Sie sind auf Seite 1von 43

Vol.29 No.

3 March 2008

Editor-in-Chief: Lawrence F. Nazarian,


Rochester, NY
Associate Editors: Tina L. Cheng,
Baltimore, MD
Joseph A. Zenel, Portland, OR
Editor, In Brief: Henry M. Adam, Bronx, NY
Consulting Editor: Janet Serwint, Baltimore, MD
Consulting Editor Online and Multimedia
Projects: Laura Ibsen, Portland, OR
Editor Emeritus and Founding Editor:
Robert J. Haggerty, Canandaigua, NY
Managing Editor: Luann Zanzola
Medical Copy Editor: Deborah K. Kuhlman
Editorial Assistant: Sydney Sutherland
Editorial Office: Department of Pediatrics
University of Rochester
School of Medicine & Dentistry
601 Elmwood Avenue, Box 777
Rochester, NY 14642
sydney_sutherland@urmc.rochester.edu
Editorial Board
Margie Andreae, Ann Arbor, MI
Richard Antaya, New Haven, CT
Laurence A. Boxer, Ann Arbor, MI
Latha Chandran, Stony Brook, NY
Joseph Croffie, Indianapolis, MD
Howard Eigen, Indianapolis, IN
Leonard Feld, Charlotte, NC
Jeremy N. Friedman, Toronto, ON
Vincent A. Fulginiti,Tucson, AZ
Mark Goldstein, Boston, MA
Lindsey Grossman, Baltimore, MD
Russell J. Hopp, Omaha, NE
Hal B. Jenson, Springfield, MA
Chris P. Johnson, San Antonio, TX
J. Jeffrey Malatack, Narberth, PA
Blaise Nemeth, Madison, WI
John Pascoe, Dayton, OH
DeWayne Pursley, Boston, MA
Thomas T. Sato, Milwaukee, WI
Bennett A. Shaywitz, New Haven, CT
Michael Silberbach, Portland, OR
Nancy Spector, Philadelphia, PA
Surendra K. Varma, Lubbock, TX
Maximilian Zach, Graz, Austria
Publisher: American Academy of Pediatrics
Michael J. Held, Director, Division of Scholarly
Journals and Professional Periodicals

contents

PediatricsinReview Vol.29 No.3 March 2008


Articles

75

Managing Sports Injuries


in the Pediatric Office
Jordan D. Metzl

86

Recognition of Autism Before Age 2 Years


Chris Plauche Johnson

97

Index of Suspicion
Courtney Peshkovsky, Robert J. Leggiadro, Lauren Kupersmith,
Seth Septer, Cristina Fernandez, Fernando Zapata

In Brief

103 Posttraumatic Stress Disorder


105 Enteral Feeding

Pediatrics in Review
(ISSN 0191-9601) is owned and controlled by
the American Academy of Pediatrics. It is
published monthly by the American Academy of
Pediatrics, 141 Northwest Point Blvd., Elk
Grove Village, IL 60007-1098
Statements and opinions expressed in Pediatrics
in Review are those of the authors and not
necessarily those of the American Academy of
Pediatrics or its Committees. Recommendations
included in this publication do not indicate an
exclusive course of treatment or serve as a
standard of medical care.
Subscription price for 2008 for print and online/
online only: AAP Fellow $163/$124; AAP
Candidate Fellow $153/$114; Nonmember
$204/$159; Allied Health or Resident $152/
$103. Institutions call for pricing (866-8432271). For overseas delivery, add $95. Current
single issue price is $10 domestic, $12
international. Replacement issues must be
claimed within 6 months from the date of issue
and are limited to three per calendar year.
Periodicals postage paid at ARLINGTON
HEIGHTS, ILLINOIS and at additional mailing
offices.
AMERICAN ACADEMY OF PEDIATRICS,
2008. All rights reserved. Printed in USA. No
part may be duplicated or reproduced without
permission of the American Academy of
Pediatrics.
POSTMASTER: Send address changes to
PEDIATRICS IN REVIEW, American
Academy of Pediatrics Customer Service Center,
141 Northwest Point Blvd., Elk Grove Village,
IL 60007-1098.

The printing and production of Pediatrics in


Review is made possible, in part, by an
educational grant from Abbott Nutrition.

Answer Key: 1. B; 2. D; 3. B; 4. E; 5. A; 6. D; 7. E; 8. E

Internet-Only Article
Abstract appears on page 107.

e15

American Academy of Pediatrics


Policy Statements on Bioethics:
Summaries and Commentaries: Part 2
Mark R. Mercurio, Marilyn A. Maxwell, Brenda Jean Mears,
Lainie Friedman Ross, Tomas J. Silber

Cover: The artwork on the cover of this months issue is


by one of the winners of our 2005 Cover Art Contest,
12-year-old Elizabeth Jayner of Ahoski, NC. Elizabeths
pediatrician is Charles Pierce, MD.

Article

sports medicine

Managing Sports Injuries in the


Pediatric Office
Jordan D. Metzl, MD*

Author Disclosure
Dr Metzl did not
disclose any financial
relationships relevant
to this article.

Objectives

After completing this article, readers should be able to:

1. Describe the current management of concussion.


2. Delineate the prescription for treatment of acute ankle injuries and prevention of
subsequent injuries.
3. Describe appropriate handling of neck injuries to avoid additional damage.
4. Discuss the likelihood of sustaining an ankle injury if the patient has had a previous
ankle injury.
5. Recognize which sports are more closely associated with head injury than others.

Introduction
With increasing numbers of young athletes participating in sports activities across the
United States, the issues of sports participation, sports safety, and specific return-to-play
decisions have become more common in the pediatric office. Athletic patients and their
families are turning increasingly toward the pediatrician for guidance and safe decisionmaking regarding sports participation and injury prevention.
This article examines specific issues that pediatricians and pediatric residents in training
should encounter during the practice of general pediatric medicine. These issues include
concussion, cervical spine injury, and ankle injury. Case-based teaching examples are used
to illustrate teaching points.

Current Management of Concussion


Case #1: Head Injury
A 15-year-old soccer player comes to her pediatrician for follow-up 1 day after suffering a head
injury during a soccer game. She sustained a head-to-head collision with another player and
quickly developed a headache. She tried to continue playing but was having difficulty
remembering her position and on-field responsibilities. She did not lose consciousness. She came
off the field voluntarily, was evaluated by the athletic trainer on the sideline, and was removed
from the soccer game. Her parents were worried and took her to the emergency department,
where she was evaluated. A computed tomography scan was negative for subdural or epidural
hematoma.
Closed-head injury is a common medical problem in young athletes who play in highand medium-contact sports (Table 1). Unfortunately, the majority of concussive episodes
in young athletes are not reported, but the known incidence is approximately 300,000
cases per year in the United States. The actual number is much higher.
The head injury sustained by this adolescent soccer player raises a number of issues.
How extensive are her injuries, which may be subtle in the cognitive realm, and when
should she be expected to return completely to normal? How should her progress be
monitored? When can she resume practice and competition?

Assessment
Concussion refers to a significant head injury that may cause a host of signs and symptoms,
including alterations in consciousness, confusion, amnesia, visual and hearing impairment,
irritability and mood changes, difficulties with balance, headache, lethargy, insomnia,
memory impairment, nausea, and vomiting.
*Assistant Professor, Department of Pediatrics, Hospital for Special Surgery, Cornell Medical College, New York, NY.
Pediatrics in Review Vol.29 No.3 March 2008 75

sports medicine

office management of sports injuries

Common Sports
Characterized by Level of
Contact
Table 1.

High-contact Sports

Basketball
Football
Soccer
Martial Arts
Rugby

Medium-contact Sports

Baseball
Fencing
Cheerleading
Skiing
Volleyball

Noncontact Sports

Running
Swimming
Tennis
Weight Training

The Prague Conference on Concussion in 2004 defined concussion as a complex pathophysiological


process affecting the brain, induced by traumatic biomechanical forces. (1) The Conference also pointed out
that the impairment of neurologic function typically has
a rapid onset, is short-lived, and tends to resolve spontaneously. In addition, neuropathologic changes are indicative more of a functional derangement than of a
structural injury and typically are associated with neuroimaging studies that appear grossly normal. The sequential resolution of symptoms and cognitive functioning
was emphasized.
Guidelines for the management of concussion have
been available for many years and, until recently, were
based on systems that graded the injury, with loss of
consciousness being a major criterion. These guidelines
recommended return to activity based on the grade of
concussion. More recent thinking emphasizes waiting
until after symptoms have resolved before assigning severity; stresses physical and cognitive rest; and recommends a sequential, functional progression as symptoms
clear and do not return with exertion, with careful monitoring by a physician. (1)
The patients neurologic and cognitive status can be
assessed through a variety of methods. Neurologic examination is indicated from the initial evaluation through
the follow-up period. At one end of the spectrum, office
76 Pediatrics in Review Vol.29 No.3 March 2008

assessment of mental status and cognitive functioning


by use of a series of questions is part of neurologic
testing. Intensive neuropsychological testing also is available through appropriate specialists. A middle approach
involves the use of more formal instruments that can be
administered by primary care physicians or certified athletic trainers.
Several instruments are available for an intermediate
level of evaluation. One tool that has been constructed
using a number of other instruments is the Sport Concussion Assessment Tool (SCAT), which contains both a
medical evaluation and a checklist for the athlete, as well
as information about concussion. (1) When combined
with a neurologic examination, the SCAT can be useful
in the office setting.
An instrument used commonly by certified athletic
trainers is the Standardized Assessment of Concussion
(SAC), which can be employed for sideline evaluation.
This instrument can be ordered through a medical bookstore or purchased through the Internet. Based on a
scored scale, the SAC can be used to determine orientation, immediate memory, concentration, and delayed
item recall. These different parameters are tabulated,
providing a score that grossly assesses an athletes ability
to return to competition. In the office, findings on the
SAC can be combined with evaluation of the presence of
postconcussive symptoms such as ongoing headache,
dizziness, memory loss, and irritability and a neurologic
examination to assess the athletes ability to return to
competition.
Although the SCAT and SAC are good tests of cognitive functioning, they have a ceiling effect, meaning
that a patient could do well on these tests without having
recovered fully. For this reason, a number of computerized tests are available that provide a more accurate
assessment without the need for full neuropsychological
testing. A full discussion of these instruments is beyond
the scope of this article, but pediatricians should consult
with local sports medicine physicians and athletic trainers
to learn which tools are available and in use in their area.
For milder cases of concussion in which recovery is
steady and complete over a 7- to 10-day period, mental
status screening is necessary for monitoring, but the
computerized instruments should not be necessary because physical examination and self-report of symptoms
usually are sufficient. Primary care practitioners should
be equipped to manage these injuries.
For patients who sustain a concussion and have persistent signs and symptoms or whose symptoms recur on
exertion, both neuropsychological assessment and the
involvement of specialists may be indicated. Many ath-

sports medicine

letes who sustain head injuries become totally symptomfree and do not need major intervention. Extensive neuropsychological testing is indicated for the minority of
patients in whom there is a question about the cumulative effects of concussion or in whom there may be
permanent academic impairment and questions about
further participation in contact sports.

Postconcussive Syndrome
Whenever an athlete suffers a concussion, it is important
to advise both the athlete and his or her parents about the
possibility of postconcussive syndrome. Postconcussive
syndrome involves residual symptoms from a concussion,
including headache, dizziness, irritability, and difficulty
concentrating. These symptoms can exist after any concussion, regardless of severity. The appearance and persistence of postconcussive symptoms is more likely with
more severe concussion.
A repeated episode of concussion can result in a
prolonged period of concussive and postconcussive
symptoms and, therefore, a much greater period of time
away from sports participation. A less common but much
more severe consequence of concussion is the development of second impact syndrome (SIS), rapid and progressive brain injury resulting from a second episode of
closed-head injury while the athlete still is symptomatic
from the first episode. In this entity, an athlete who still is
recovering from an initial concussive episode is struck in
the head. The result is rapid mental deterioration, mental
status change, and often fatal uncal herniation.
SIS is associated with a mortality rate of 70% to 80%
but is completely preventable through the prompt recognition of concussive and postconcussive symptoms.
The presence of this entity, although rare, demonstrates
further the need to keep athletes out of any situation
involving direct head trauma until recovery is complete.

Making the Important Decisions


Current concepts of managing concussions related to
sports dictate that an athlete manifesting any signs or
symptoms of concussion be removed from the practice or
game immediately, not be allowed to return to play that
day, be monitored carefully, and receive medical attention. These same principles should be relevant to nonathletic activity. Return to play is allowed only when the
players signs and symptoms have resolved and when he
or she has demonstrated the ability to progress stepwise
through several levels of activity without any recurring
symptoms. Activity should move through the following
steps, with advancement only if there are no symptoms. If
symptoms recur, the athlete should rest for 24 to

office management of sports injuries

48 hours and try to progress again, dropping back if


symptoms recur. (1) The recommended steps for a logical and safe progression are:
1. Complete rest, although bed rest is not indicated;
the patient may participate in activities of daily living.
2. Light (low-intensity) aerobic exercise, such as
walking, without a component of resistance. Weightlifting is prohibited.
3. Activity specific to the sport, such as running or
skating. At this step and the next, resistance training may
be added.
4. Training drills without contact, followed by mental status testing.
5. Full-contact training after clearance by medical
personnel.
6. Participation in a game.
Such a regimen must be individualized to each athlete, and progress should be monitored by clinicians
who have the appropriate level of training. The primary care physician and the experienced athletic trainer
make a good team in determining the appropriate course
of action in most cases, and the trainer can serve as a
liaison with the coach to make sure the plan is executed
properly.

Prevention
One final note on concussion involves the screening of
athletes. As in many types of injury that create a predisposition to future injury, athletes who have had one
concussive episode are nearly six times more likely to
have a second episode of concussion compared with their
unaffected peers. Therefore, effective preseason counseling about the symptoms of concussion involves asking
about the history of previous concussive events. In addition, it is important to stress the importance to athletes,
particularly those who have had previous concussive
events, of reporting postconcussive symptoms during the
season.
Prevention involves the creation of an increased
awareness in coaches and athletic trainers who might be
involved with the care of a young athlete who had a
concussive predisposition in previous seasons. Mouthguard use in high-contact sports, in addition to reducing
oral and dental injuries, is theorized by some to reduce
the risk of concussion and should be strongly encouraged
in all athletes. All of this advice and surveillance can be
accomplished during the preparticipation evaluation and
can ensure greater safety by targeting athletes who are at
greater risk and creating both an educational and health
supervisory framework on their behalf.
Unlike professional athletes who are paid to put their
Pediatrics in Review Vol.29 No.3 March 2008 77

sports medicine

office management of sports injuries

bodies at risk for money, pediatric and adolescent athletes are volunteer athletes. Therefore, special care
should be given to protect against closed-head injury
and, more importantly, to ensure that the athlete has
recovered completely before he or she returns to sports
participation.
The Centers for Disease Control and Prevention supply information for patients, parents, and clinicians that
provides guidance in the management of head injuries at
http://www.cdc.gov/ConcussionInYouthSports/ and
http://www.cdc.gov/ncipc/tbi/Facts_for_Physicians_
booklet.pdf.

Current Management of Cervical Spine Injury


Case 2: Cervical Spine Injury
You are on the sideline of a football game when a 17-yearold player suffers an injury while tackling another player.
When trying to tackle a running back, he uses his head as
the initial contact device (spear tackling), suffers an axial
load with a flexion injury, and falls to the ground, lying
motionless. He is prone. After the initial shock, the athletic
trainer rushes onto the field and immediately calls for your
assistance. As you approach the athlete, he lies motionless in
a prone position on the field.

Epidemiology of the Cervical Spine Injury


Cervical spine injury represents a rare but potentially
life-threatening event in youth sports. Approximately
60% of all acute pediatric spinal injuries occur in the
cervical spine. Cervical spine injury occurs most frequently in high- and medium-contact sports, typically
occurring through a head-first injury mechanism, as described in Case 2.

Preparation
The appropriate sideline management of the acute cervical spine injury starts with effective preparation. Rather
than waiting for an injury to occur before devising a
algorithm for treating a cervical spine injury on the field,
treatment protocols are implemented best before the
start of the sports season.
The essential elements in preseason event preparation
for a cervical spine injury include:
1) On-site emergency personnel or the ability to access emergency medical services through the use of a
charged cell phone that is fully accessible during the
sporting event.
2) Discussion with a certified athletic trainer who is
most likely to be on the sideline regarding responsibility
in the case of a cervical spine injury.
78 Pediatrics in Review Vol.29 No.3 March 2008

3) Immobilization equipment, including a back board,


for use in the event of a cervical spine injury.
4) An understanding of the steps required in the
event of an acute cervical spine injury, including airway
control, cervical spine immobilization, and plan for
transporting injured athletes to the emergency department.

On-field Management
Appropriate on-field management of a suspected cervical
spine injury follows a similar protocol to basic life support, which begins with a focus on airway management,
breathing, and circulation (ABCs). In the United States,
American football poses the greatest risk for cervical
spine injury and accounts for approximately 50% of such
injuries in youth sports. Cervical spine injuries also can
occur during water sports, particularly diving, when an
athlete hits the water at a high velocity with the neck
flexed, similar to the spear tackle in football.
When approaching the athlete who is suspected of
having a cervical spine injury, an initial desire to assess
and treat the neck directly is common. Appropriate management of the injured cervical spine, however, begins
with assessment of the ABCs while attempting to keep
the head and neck in a stable position. If the injury occurs
in football, an appropriate initial step includes listening
for breathing or putting a hand beneath the jersey and
shoulder pads to check for thoracic expansion. If the
athlete is unconscious, it is imperative to establish the
airway first, which includes assuring that the tongue is
not obstructing the airway. The football helmet never is
removed on the field. If the airway is obstructed, the face
mask should be removed, but the helmet and shoulder
pads should remain in place to ensure neutral alignment
of the cervical spine.
If the athlete is found in the prone position, establishing airway control can be difficult. Treating the prone
athlete involves initial assessment of the airway. If the
athlete is unconscious and not breathing, the airway is
established in the supine position. In this scenario, the
log roll technique (Fig. 1) is used to move the patient to
the supine position. This technique, which should be
practiced before any injury occurs, involves rolling the
injured player by two assistants controlling the body and
the team leader controlling the head. It is essential to
keep the head turning at the same speed as the body to
lessen any chance of additional injury to a potentially
unstable cervical spine.

sports medicine

office management of sports injuries

Figure 1. Log roll technique for stabilization of pediatric

cervical spine injury. It is always important for the helmet to


stay on unless airway problems are present. This technique
requires two or three people to complete, with the person in
charge at the head giving commands and keeping the cervical
spine in line with the rotating torso.

Figure 3. Normal flexion view of the cervical spine.

Five radiographic views should be obtained in all


patients in whom cervical spine injury is suspected (Figs.
2 through 6). Note that all of these radiographs show a
normal cervical spine, with the exception of the lack of
the normal lordosis on the lateral view in Figure 6, which
suggests paracervical muscular spasm. Flexion and extension views never should be obtained when cervical instability is suspected, as is the case with the patient in
Figure 7.

Prevention

Figure 2. Normal anteroposterior view of the cervical spine.

Prevention of cervical spine injury is an important consideration for the pediatrician. Effective prevention includes the use of proper-size equipment during practice
and games, proper coaching and refereeing that discourages risky tackling, and strengthening programs that
involve sport-specific exercises for the prevention of cervical spine injury.
Pediatrics in Review Vol.29 No.3 March 2008 79

sports medicine

office management of sports injuries

Figure 5. Odontoid view showing the relationship between


the first and second cervical vertebrae. The dens, so named
because of its resemblance to a tooth, extends from the
second vertebrae into the first vertebrae for stability. The
space around the dens should be equal on both sides.
Inequality of space suggests instability between the first
two vertebrae.

3) There is no report of radicular symptoms emanating from the cervical spine.


4) Results of all neurologic examinations and associated tests are normal.
Figure 4. Normal extension view of the cervical spine.

Current Management of Ankle Injury


Case #3: Ankle Injury

In Case 2, involving a player who is unconscious and


not breathing, the physician should use the athletic
trainer and a coach to assist in the log roll technique.
Once the player is supine, the face mask should be
removed quickly by the athletic trainer while the physician keeps his knees around the players helmet to minimize motion. The airway subsequently is secured quickly
with appropriate jaw positioning, and the athlete is transported to the emergency department.

Returning to Activity
Athletes who are suspected of having a cervical spine
injury but have normal radiographic findings can be
returned safely to activity when:
1) There is no pain with motion of the cervical spine.
2) There is no pain with palpation of the cervical
spine.
80 Pediatrics in Review Vol.29 No.3 March 2008

A 12-year-old soccer player limps into your office one day


after rolling her ankle. When asked, she describes a rolling episode that occurred during soccer practice yesterday
when she stepped in a hole on the field. She sits on the
examination table and asks when she can return to play.

History
As with all medical problems, obtaining a history is an
essential first step in the diagnostic approach to injury
(Table 2). In this case, the mechanism of injury, rolling
of the ankle, needs proper clarification. One helpful
method of assessment is to ask the patient to demonstrate
the mechanism of injury in the uninjured extremity. The
most common mechanism, the inversion injury, accounts
for approximately 80% of ankle injuries and is the result
of a twisting or rolling of the ankle when stepping into a
hole or running on an uneven portion of the field. In
contrast, the eversion injury, a less common and more

sports medicine

office management of sports injuries

Figure 6. Lateral view of the cervical spine showing straightening of the normal cervical lordosis but otherwise no evidence of bony abnormality.

forceful injury pattern, is associated with more severe


injuries, often involving both the medial and lateral
ankle. The examiner also should ask about the patients
activity after the injury occurred, including ability to bear
weight and return to activity. The more serious the
injury, the less likely is the ability to continue playing and
to bear weight comfortably during running or walking.
Finally, assessing the past history of ankle injury is essential. The most common reason for an ankle injury in an
athlete is the presence of a previous injury that has been
rehabilitated incompletely. Therefore, the presence of
previous injury provides important clues in terms of
present injury patterns and likely diagnoses.

Physical Examination
The physical examination begins with observation and
inspection. Observation of how a patient is walking is
essential and can assist greatly in differentiating the more
serious fracture from the less serious ligament injury.
Inspection for swelling and ecchymosis also is important;
swelling often is noted in the area of injury in many foot
and ankle injuries.

Figure 7. Lateral view of the cervical spine showing a cervical

fracture with cervical instability. This patient requires complete immobilization and immediate referral to a trauma
center for consultation with an orthopedic spine specialist or
neurosurgeon.

With the information gleaned from the history, the


examiner can use the hands-on portion of the ankle
examination to look for specific injuries. The more common inversion mechanism produces injury to the lateral
portion of the foot and ankle, including the distal fibula
and the ligaments around the distal fibula, specifically the
anterior and posterior talofibular ligaments and the calcaneofibular ligament as well as the proximal fifth metatarsal (Figs. 8 and 9). In addition, a fracture of the fibular
physis (growth plate) is the most likely injury in the
Pediatrics in Review Vol.29 No.3 March 2008 81

sports medicine

office management of sports injuries

Important Questions to
Ask of an Athlete Who Has an
Ankle Injury

Table 2.

1. How did this injury happen?


Examiner should gain appreciation of the mechanism
of injury, inversion, eversion, direct loading, often with
the aid of patient demonstrating the uninjured ankle.
2. What happened after the injury?
Examiner should gain appreciation for the severity of
injury. The ability to continue playing denotes a less
serious injury than an injury that required assistance
from the field and cessation of playing.
3. Have you injured this ankle before?
Examiner should gain insight into previous injuries and
underlying predisposing injury patterns.

athlete who has inverted the ankle, has open growth


plates, and experiences pain on palpation of the lateral
ankle.

Figure 9. Palpation of the proximal fifth metatarsal. This site

can be injured during an inversion injury. The examiner must


palpate the proximal fifth metatarsal in any inversion injury. If
pain and swelling are present, radiographs of the foot should
be obtained to rule out a fifth metatarsal fracture.

Further Studies
Radiographs of the ankle and possibly of the foot are
indicated in any suspected bony injury. Bony injury
usually is accompanied by swelling and pain. Ankle views
should include anteroposterior (AP), lateral, and mortise
views of the ankle, which provide a complete assessment
of the bony architecture (Figs. 10 and 11). In the case of
a suspected lateral foot injury to the fifth metatarsal, AP,
lateral, and oblique views of the foot are indicated.

Prevention

Figure 8. Palpation of the lateral ankle at the area of the

anterior talofibular ligament (ATFL). This is the most common


site of pain in the athlete who has had closure of the distal
fibular physis. This closure generally occurs by age 13 years in
girls and 15 years in boys, but depends on sexual and skeletal
development rather than chronologic age. Pain and swelling at
this site are suggestive of an injury to the ATFL, otherwise
known as a sprain. Such injuries are graded on a scale of 1 to
3, depending on severity. The line across the distal fibula in the
photo is that of the distal fibular physis. Pain on palpation of
this location is diagnostic for a distal fibular physeal fracture.
82 Pediatrics in Review Vol.29 No.3 March 2008

If there is no bone-specific tenderness and the athlete can


walk without much of a limp, the pediatrician can suggest
exercises as a precursor or possible alternative to physical
therapy. The premise is that the initial regimen of RICE
(Rest, Ice, Compression, Elevation) is helpful for the first
48 hours after injury. The process of ankle mobilization
can proceed after that time. The initial step is to start the
ankle moving (tracing the alphabet with the first toe is a
good start), after which progressive strengthening can
proceed. The following exercises can enhance strength
and can be learned easily in the pediatric office.
An elastic band is used to strengthen the muscles
gradually as they flex against resistance (Figs. 12 and 13).
These exercises are done in three sets of 15 repetitions
daily for a 6-week period. After 6 weeks, the exercises
usually can be discontinued. If the ankle injury persists
for more than a few weeks and if radiographs are negative

sports medicine

office management of sports injuries

Figure 11. Mortise view of the ankle in a skeletally immature


patient who suffered an eversion injury. In this case, there is
subtle widening and pain at the medial ankle in the area of the
distal tibial physis, consistent with a growth plate fracture of
the medial malleolus. The arrow points to the site of subtle
widening on the radiograph, which also correlates to the site
of the patients pain. This patient should be referred for
additional treatment.
Figure 10. Normal mortise view of skeletally immature ankle.

This view is taken with the ankle plantar flexed and the foot
rotated inwards, thus exposing the spatial relationship around
the talotibial and talofibular joints in the ankle. Alteration of
these spaces, which should be equal, as shown in this radiograph, suggests ligamentous instability in the ankle.

and there is no bone-specific tenderness, referral to a


physical therapist is indicated.
The prevention of ankle injuries is especially important in any young athlete who has suffered a previous
ankle injury. Multiple studies have demonstrated that the
most common cause of an ankle injury is the existence of
a previous ankle injury. The prevention of an ankle injury
involves the implementation of programs designed to
strengthen the muscles around the ankle, including the
peroneus muscle groups on the lateral aspect of the lower
leg and the tibialis posterior muscle on the medial aspect

of the leg. The same exercises used to strengthen the


injured ankle are useful in preventive strengthening.

Safe Return to Sports


The safe return to sports following an injury is a complicated and important decision made by thousands of
pediatricians daily. The decision can include medical issues,
such as the safe return after a concussion or acute asthma
attack, and orthopedic issues, such as the safe return after an
ankle, knee, or shoulder injury. In all cases, the question
must be asked: Does the return of this athlete to sports put
him or her at greater risk for additional complication? The
answer always should be no.
Important questions to ask in the case of nonorthopedic conditions are:
1. Have all medical considerations been addressed
(eg, is there an inhaler on the sideline for an athlete who
has asthma)?
Pediatrics in Review Vol.29 No.3 March 2008 83

sports medicine

office management of sports injuries

Figure 12. Elastic band strengthening exercises with the band


positioned along the lateral ankle and the foot flexed to 90
degrees is a good approach to starting the process of increasing lateral strength in the ankle. Over a 4-week period, this
type of lateral strengthening can increase the baseline
strength in the ankle, reducing the risk of additional injury.
Such exercises also can be helpful with acute injury. For
moderate-to-severe injuries, it is best to refer the patient to
physical therapy for an individualized program.

2. Is there a system in place to report symptoms if


they recur?
3. Are the athlete and his or her family aware of the
importance of reporting early symptoms (eg, dizziness
with an injury can mean a concussion)?
Helpful questions for orthopedic injuries and safe
return to sports include:
1. Is there any limitation of normal athletic function
with the injury? If so, the athlete should be held until this
limitation resolves (eg, the soccer player who has shin
pain that limits his or her ability to run or the gymnast
who has back pain that limits his or her ability to bend
backwards).
2. Is there any ongoing swelling or loss of motion in
an affected joint? If so, this injury may require additional
evaluation before clearance.
3. Has the proper preventive strategy been employed? Prevention can include ankle strengthening for
ankle injuries, shoulder strengthening for shoulder injuries, and back strengthening for back injuries. In each

84 Pediatrics in Review Vol.29 No.3 March 2008

Figure 13. Medial strengthening of the ankle. As with lateral

strengthening of the peroneal muscles, medial strengthening


of the tibialis posterior muscles aids greatly in the prevention
of additional injury.

case, has the athlete learned preventive strengthening,


often through the assistance of a certified athletic trainer
or physical therapist, to reduce the likelihood of reinjury?

Reference
1. McCrory P, Johnston K, Meeuwisse W, et al. Summary and
agreement statement of the 2nd International Conference on Concussion in Sport, Prague 2004. Clin J Sport Med. 2005;15:48 55

Recommended Reading
The Pediatric Clinics of North America, June and August, 2002.
Both editions are dedicated to pediatric sports medicine and are
very helpful.
Pediatric Annals, 2000;29(3) and 2002;31(1). The first number is
orthopedic-focused and the second is focused on medical sports
issues (ie, concussion).
American Academy of Pediatrics. Care of the Young Athlete. Elk
Grove, Ill: American Academy of Pediatrics; 2002, 2008 (in
press). This book is very helpful and comprehensive.
Sports Medicine: The School-aged Athlete. Philadelphia, Pa: Saunders;
1997. An excellent resource for the office that is orthopedicfocused and covers most adolescent sports injuries.
Sports Medicine in the Pediatric Office: Multimedia Text with DVD
Companion. Elk Grove Village, Ill: American Academy of Pediatrics; 2007. This comprehensive book uses both written material and DVD/video technology to teach musculoskeletal skill
examination technique.

sports medicine

office management of sports injuries

PIR Quiz
Quiz also available online at www.pedsinreview.org.
1. Which of the following statements about current understanding and management of head injury is true?
A.
B.
C.
D.
E.

A history of previous concussion is irrelevant to the management of a present concussion.


A sequential, functional progression of activity is a critical aspect of the management of concussion.
Grade of concussion is the most significant factor in planning management.
In the recovery from concussion, symptoms that recur with exercise can be ignored.
The role of the athletic trainer ceases when a concussion victim consults a physician.

2. The management of a suspected cervical spine injury in an athlete who is lying prone on the field can
include all of the following except:
A.
B.
C.
D.
E.

Assessment of breathing.
Assessment of circulation.
Removal of the face mask.
Removal of the helmet.
Use of the log roll to bring the athlete to a supine position.

3. In the United States, a cervical spine injury is most likely to occur in which of the following sports?
A.
B.
C.
D.
E.

Basketball.
Football.
Lacrosse.
Soccer.
Wrestling.

4. Of the following, the most common reason for an ankle injury in an athlete is:
A.
B.
C.
D.
E.

Flat feet.
Improper shoes.
Incorrect training methods.
Participation in a contact sport.
Presence of a previous ankle injury.

Pediatrics in Review Vol.29 No.3 March 2008 85

Article

cognition

Recognition of Autism Before


Age 2 Years
Chris Plauche Johnson,
MEd, MD*

Author Disclosure
Dr Plauche Johnson
did not disclose any

Objectives

After completing this article, readers should be able to:

1. Characterize the deficits of a child who has autism.


2. Describe the role of observing linguistic and social development in children who have
autism.
3. Implement a strategy for surveillance and screening of young children.
4. Discuss the management of autism, including the use of medication.

financial relationships
relevant to this
article.

Introduction
Primary care physicians (PCPs) caring for 1,000 children in a general practice should
expect approximately six of their patients to meet the diagnosis of an autism spectrum
disorder (ASD). (1) The impact of this prevalence is illustrated by a 2004 survey of PCPs
revealing that 44% cared for 10 or more patients who had an ASD. (2) The ASDs include:
autistic disorder (AD), Asperger disorder, and pervasive developmental disordernot
otherwise specified (PDD-NOS), a threshold term used when a child meets some but not
all criteria necessary for a diagnosis of either AD or Asperger disorder.
Research has documented the positive benefit of developmental and behavioral intervention for children who have ASDs, particularly when it is initiated prior to 3 years of age.
(3) Early recognition of ASDs also is important for timely genetic counseling because
younger siblings of children diagnosed as having ASDs have a 10 times increased risk of
also having an ASD. The purpose of this review is to help the clinician recognize signs of
ASDs that occur before age 2 years. Because the signs of Asperger disorder usually appear
later, they are not discussed. The term autism is used in this review to represent both AD
and its milder form, PDD-NOS.
Currently, autism is diagnosed infrequently in children younger than 3 years of age. The
diagnostic process is prompted most often by parental concern about absent or delayed
speech. However, signs of autism, especially deficits in social skills and in preverbal gestural
language, are present in most children by 18 months of age. Such signs are subtle and may
not be noticed by parents. New information regarding very early signs of autism is
emerging through prospective studies of high-risk infant siblings. (4) Using these early
red flags, one study reported that approximately 50% of affected children could be
diagnosed reliably by 14 months of age. (5) To facilitate earlier recognition through
surveillance and screening strategies, the American Academy of Pediatrics (AAP) has
published two guidelines: Identifying Infants and Young Children with Developmental
Disorders in the Medical Home: An Algorithm for Developmental Surveillance and
Screening (6) and Identification and Evaluation of Children with ASDs. (7) In an effort
to lower the average age of diagnosis and promote earlier access to intervention services,
these guidelines recommend ongoing surveillance for autism (and general development) at
every health supervision visit in all children and heightened surveillance in high-risk
younger siblings. An increased awareness of early signs of autism can facilitate surveillance
and enable PCPs to play a key role in early recognition that, when coupled with earlier
access to appropriate interventions, could improve outcomes markedly.

The Importance of Surveillance


Diagnosing ASD is very difficult because there are no pathognomonic clinical signs or
confirming laboratory tests. Over the past 3 decades, the DSM (American Psychiatric
*Editorial Board.
86 Pediatrics in Review Vol.29 No.3 March 2008

cognition

Association Diagnostic and Statistical Manual of Mental


Disorders) criteria have served as the gold standard for
making a clinical diagnosis; newer standardized tools that
operationalize the DSM-IV criteria (eg, Autism Diagnostic Observation Schedule [ADOS] (8)) optimize diagnosis. However, some of the DSM-IV criteria address
developmental skills that do not apply to children
younger than 2 years of age, and standardized tools have
not yet been validated in this age group. For example,
failure to form age-appropriate peer relationships is
not applicable to children younger than 2 years of age.
Additionally, it is impossible to apply criteria relating to
abnormal conversational skills and stereotypic language
in a young, preverbal child. Often, ritualistic behaviors
and a need for routines appear in children who have
autism after the third birthday. Thus, even children who
have severe AD may not meet full criteria at very young
ages, making diagnosis even more challenging.
Realizing this diagnostic dilemma, especially in the
face of widespread media promotion of early diagnosis
and intervention, Stone and associates (9) have suggested applying only the following four of the possible
DSM-IV criteria to children younger than 3 years of age:

Lack of spontaneous seeking to share enjoyment,


interests, or achievements with other people (eg, by lack
of showing, bringing, or pointing out objects of interest)
Lack of social and emotional reciprocity
Marked impairment in the use of multiple nonverbal
behaviors, such as eye-to-eye gaze, facial expression, body
postures, and gestures to regulate social interaction
Delay in or total lack of the development of spoken
language (not accompanied by an attempt to compensate through alternative modes of communication such
as gesture or mime)

Three of these criteria address social skills, illustrating


their relative importance in defining autism in very young
children. These researchers propose that all four criteria
be present to make the provisional diagnosis of autism
in the very young child; re-evaluation using the full
DSM-IV-TR (10) criteria, combined with a standardized
tool, is recommended after the third birthday. Because
referring young children for diagnosis depends on the
PCPs ability to recognize behaviors illustrating these
criteria, the following discussion targets early social and
prelanguage deficits. Currently, no standardized autismspecific screening tests for children younger than 18
months of age are available, although some are in development. Thus, surveillance, coupled with a high degree
of suspicion, is very important and should lead to earlier
diagnosis and intervention.

recognition of autism

Clinical probes are provided in most sections of this


article to assist the PCP during the surveillance process,
but they should not be used as a screening test. Rather,
they are only one part of the total package of developmental screening. The PCP may choose to use one or
several of these procedures at each visit to monitor for
autism. In addition, an autism screening tool (6)(7)(11)
should be used at the 18- and 24-month visits and at any
time there is concern about ASD.

Early Social Skill Deficits


Social development usually parallels overall general functioning (motoradaptivelanguagecognitive domains) in children developing typically. Social development also parallels general functioning in children who
have global developmental delays (GDD). For children
who have autism (with or without coexisting GDD), the
development of social skills and language is more delayed
and characteristically is out of sync with motor, adaptive, and cognitive functioning. The discrepancy between the development of social skills and general development is one of the most important defining criteria.
Discrepancies may be recognized late in the first year
after birth, but they become more obvious by 18 to 24
months of age in children later diagnosed with autism.
The delay in social development often is manifested
by a lack of or decreased need for social and emotional
reciprocity or social relatedness. Such children have
little or no inherent drive to connect with others and
share feelings, thoughts, and actions. Infants who have
other disabilities may have difficulties connecting with
others due to their respective motor, hearing, and visual
deficits, but the inherent drive to connect promotes
compensatory strategies. For example, infants who have
cerebral palsy establish social relatedness through eye
contact, facial expressions, sounds, and conversations.
Infants who have hearing loss often are visually hypervigilant and use eye contact and gestures to connect with
others. Children who have autism make few, if any,
attempts to compensate and relate socially to others; they
often are content being alone and usually ignore others
bids for attention, affection, or connectedness. Children
who have autism may seek comfort and affection when
injured or anxious, but such self-initiated bids solely
when in need should not be interpreted as sufficient
indicators for normal social relatedness. Children who
have autism can and frequently do form attachments
with family members; sometimes these may become unusually strong and problematic. Deficits in social relatedness are manifested by several behaviors that can be observed spontaneously or prompted during an encounter.
Pediatrics in Review Vol.29 No.3 March 2008 87

cognition

recognition of autism

Joint Attention
The single most distinguishing characteristic of very young
children who have autism is a deficit in joint attention
(JA) that is discrepant from overall functioning. JA is a
normal, spontaneously developing behavior that demonstrates the childs joy in sharing an object or event with
another person. It is triadic; the child alternates attention
between an object/event and another person by looking
back and forth between the two and connecting. It is a
core feature of the DSM-IV-TR criteria and a critical component of current autism screening and evaluation tools.
JA may be classified as: 1) spontaneous when the
child looks back and forth between an interesting
object/event and the caregiver to share interest, 2) responding when the child looks in the direction indicated by the pointing of a caregiver and connects, and
3) initiating when the child points to an object/event
to establish a connection with another person. As with all
developmental skills, JA appears to develop in graduated
stages. At 8 to 10 months of age, the typically developing
infant, while socially engaged with his or her mother, will
follow the mothers gaze if she shifts her eyes away from
the infant to look at an object or event across the room.
This action leads to sharing in a mutual experience.
Children who have autism often take no notice of the
shift in gaze. At about 10 to 12 months of age, a child can
follow a point. For example, if a parent sees something
of interest, points in its direction, and says, Oh look!,
the typically developing child looks in the direction that
the parent is pointing. On seeing the object of interest,
the child looks back at the parent and may smile (or
frown if the object is frightening). If the object is unfamiliar or the infant does not see the object, he or she may
look back quizzically at the parent. True JA requires the
child not only to follow the point, but to look back at the
caregiver and connect socially. A child who has autism
often is oblivious to the parents bid or may look briefly at
the object with little interest but does not look back at
the parent to connect.

Clinical Probe for the 12- and 15-month Health


Supervision Visits: The clinician might demonstrate
the childs ability to follow a point by saying, Look! See
the. . . . and point to an interesting object or picture on
the wall or ceiling. If there is no response, call louder and
initiate the bid with the childs name or a tap on his or
her shoulder. Often, no degree of intensity is successful in
getting the child who has autism to look.

88 Pediatrics in Review Vol.29 No.3 March 2008

Later emerging JA skills are characterized by the child


rather than the adult initiating the connection. Pointing
can serve both communicative and social functions for
children. As a communicative function, children point to
request an item that is out of reach. As a social function, children point to comment or to draw anothers
attention to an event or object (eg, the moon) out of
mere interest and simply to share. The requesting
point normally appears first, at approximately 12 to 14
months. Words later may accompany the pointing as
language develops. While pointing to the desired object
(eg, a cookie), a child looks back and forth between the
object and the caregiver to be reassured that the caregiver
understands his or her need. The gesture is triadic (child,
object, caregiver), but it is not purely social because the
cookie is the goal and the caregiver is simply a means by
which the child can obtain it. Most children who have
autism do not master this skill at age-expected times;
some, especially younger children, may make no attempt
to elicit help and just cry. Others may lead their parents
by the hand to the desired object and simply wait or
cry. Some may engage in primitive pointing involving
an outstretched arm in the direction of the desired object and opening and closing the hand in a repetitive
grasping motion. However, there is no eye contact or
connection with the parent, so this gesture does not
represent true JA.
At 14 to 16 months of age, typically developing
children point to comment or call attention to and
share an interesting object/event. The same triad exists
(child, object, caregiver), but the goal is reversed. The
object now is the means by which the child obtains his or
her goal, the adults attention. The child looks alternatively between the object and the caregiver for reassurance that the object has been observed and appreciated.
Again, depending on the childs language level, he or she
may use words when pointing. Such pointing serves a
purely social purpose and represents fully developed JA.
Absence of this type of pointing in the 18- to 24-monthold child is a strong indicator of autism. Such JA pointing
should be distinguished from pointing simply to label
objects with little eye contact or sharing of affect. Although children who have autism can be adept, even
advanced, at self-initiated, ritualistic labeling, they often
are unable to point to the same objects on request, and
the labels rarely are used in social conversation. Around
14 to 16 months of age, typically developing children
also begin to show or bring an object to the parent,
as if to say, Look at this! This JA act is qualitatively

cognition

different from bringing an object to the parent to request


help, such as to open a container, but even this latter type
of request is infrequent in autism.
Lack of or significantly delayed JA skills appear to be
specific to autism. Children who have GDD without
coexisting autism develop JA skills at rates similar to
those of the development of other skills and begin pointing to comment when they attain a mental age of 14 to
16 months. Thus, it is necessary to know the childs
overall level of functioning as well as his or her progress in
JA development to help distinguish autism from GDD. If
there is a discrepancy between the two domains in which
JA skills are significantly more delayed than general skills,
autism is likely. If the delays in all domains are similar,
GDD is more likely. JA appears to be a pivotal skill; that
is, functional language is dependent on its mastery. Studies have shown that once JA is mastered, functional
language begins to emerge within 1 year. (3) This insight
has stimulated the development of curricula that have
resulted in successful promotion of both JA and language
development. This accomplishment underscores the urgency of an early diagnosis and prompt access to intervention.

Social Orienting
Social orienting is the ability to orient to verbal stimuli, in particular, turning to respond to ones own name
being called. At about 8 to 10 months of age, most
typically developing children consistently turn their
heads toward the speaker when their names are called.
Children who have autism usually do not, prompting
parents to call louder or tap their childs shoulder in an
effort to get a response. Parents may begin to have
concerns that their child cannot hear. With greater vigilance, they realize that the child seems to hear well in
other situations. This dichotomy occurs because children
who have autism often attend to environmental sounds
extremely well but ignore human voices. Retrospective
evaluations of birthday videos of 1-year-old children later
diagnosed as having autism have demonstrated that
blinded viewers could recognize autism with accuracy
at this age. (12)(13) The best distinguishing factor was
failure to orient to name because JA pointing skills may
not be universally present in typically developing children at 1 year of age. Newer prospective studies of
high-risk infant siblings later diagnosed with autism have
confirmed this finding. (14)

recognition of autism

Clinical Probe for the 12-month Health Supervision Visit: The PCP can evaluate the childs ability to
orient socially by calling out his or her name and noting
whether the child turns and connects. If he or she does
not, the PCP should try again. Typically developing
children usually respond by the second trial.

Pretend (Symbolic) Play


Lack of or delayed symbolic play (using pretend actions
with objects) appears to be a relatively reliable distinguishing feature of autism. Play usually evolves in a
highly predictable manner in typically developing children. Once children can grasp and retain objects (approximately 4 months of age), sensory-motor play begins. They mouth and manipulate objects (eg, blocks).
Children who are 8 to 10 months old may throw the
blocks, bang them on the table, or bang them together.
Oral-motor play evolves into a more functional type of
play as children become aware of the intended use of
objects. At 12 to 14 months of age, using this new
understanding as well as evolving imitation skills, children begin to build towers with the blocks. Pretend play
subsequently emerges and, with time, increases in complexity and imagination. Simple pretend play usually
begins at approximately 16 to 18 months of age, when
children begin to use miniature representative items,
such as a tiny plastic bottle or spoon to feed a doll or a toy
telephone to talk with parents. At 18 to 20 months of
age, children begin to engage in complex pretend play.
They use generic items (eg, blocks) rather than miniatures to represent food and pretend to feed the doll or
pretend that a banana or stick is a phone. Pretend play
also becomes more complex as children engage in several
successive pretend scenarios (feeding, changing clothes,
and putting the doll or teddy bear to sleep).
Children who have severe autism and coexisting GDD
often remain in the sensory-motor stage throughout
their lifespans; they continue to mouth, throw, twirl, and
bang objects stereotypically. Some progress to functional
play, but true pretend play is rare before the age of 2
years. Furthermore, they often have little interest in toys,
frequently preferring everyday items such as string,
sticks, rocks, dirt, chains, strings of beads, and books or
videos (simply for carrying, not for reading or viewing).
When they are interested in typical toys, they may play
with them in unusual ways. For example, rather than
typical play with a miniature truck, a child who has autism
might turn it upside down and spin the wheels repeatedly. Some children, particularly those who have normal
Pediatrics in Review Vol.29 No.3 March 2008 89

cognition

recognition of autism

nonverbal intelligence, may be proficient in constructive


play (eg, using objects in combination to create a product, such as stacking blocks, nesting cups, putting puzzles together) and in computer games because mastery
of such play depends on independent trial and error
problem-solving rather than on observation and imitation of others. Children who have autism excel in tasks
learned through trial and error. Occasionally, parents
may state that their child is unusually good, that is, he or
she is content to play alone for hours, requiring little
attention or intervention from the parent. Such solitary
play usually is sensory-motor and ritualistic or constructive. Many children who have autism appear to enjoy
roughhouse play (being tickled or tossed in the air)
and show fleeting moments of social relatedness.
Although additional social deficits are associated with
ASDs, they often are not recognized until later, especially
in children who have Asperger disorder. Other deficits
are more difficult to assess in a clinical setting and impractical for use in surveillance.
Clinical Probes for the 12- and 18-month Health
Supervision Visits: The PCP should ask the parents
about the childs favorite toys and manner of play. The
PCP also might ask the parents to describe a typical
day to ascertain the amount of time spent in solitary
play. If parents report that their child enjoys playing
with other children, probe further. If play consists solely
of chasing and roughhousing, the enjoyment simply
may reflect sensory-motor pleasure rather than social
engagement.

Early Language Skills Deficits


Historically, delays and deviancies in language development have been the most common presenting signs in
children later diagnosed with autism. However, parents
usually did not raise such concerns until after their childs
second birthday, making such concerns less helpful in
reaching an early diagnosis. Because autism has been
spotlighted frequently in the lay media, more parents are
aware of the condition and may ask about it regardless of
the nature of their childs symptoms. To reassure the
parents or to suggest appropriate referrals, the PCP
should be familiar with the more subtle and earlier appearing prelinguistic language deficits associated with
autism. Parents may not be able to verbalize such subtle
deficits, requiring the PCP to probe with specific questions. Parents may be unable to answer some questions
decisively at the time of questioning, but once the subtle
90 Pediatrics in Review Vol.29 No.3 March 2008

characteristics are brought to their attention, they often


become very vigilant. In such cases, the PCP should
encourage the parents to report back once they have had
the opportunity to observe their child systematically.

Prelinguistic Language Abnormalities


Prelinguistic abnormalities characterizing autism during
the first postnatal year became apparent when investigators began retrospective evaluation of infant videos of
children later diagnosed with autism. (12) Recent prospective studies of high-risk infant siblings have confirmed video findings and revealed new red flags. (4)
Some infants later diagnosed with autism are unusually
quiet and make few vocalizations. Other infants make
atypical vocalizations; instead of cooing and babbling,
they may hum or grunt for extended periods, squeal
stereotypically, or laugh inappropriately. Others may be
very irritable, cry for long periods of time, and be difficult
to console. Infants later diagnosed with autism demonstrate fewer gestures. They may not wave bye, extend
their arms to be picked up, or engage in interactive hand
movements associated with childrens songs and nursery
rhymes.
Normal babbling usually begins by 6 months of age,
with repetition of the same phoneme (ba ba) in a
monotone voice. Later, the babbling advances in complexity, with utterances becoming longer and including
several phonemes (ba da pa pa da da). Typically developing infants develop a to-and-fro pattern of babbling
and silence (or apparent listening) that is coordinated
with the caregivers speech and is similar to true turntaking conversation seen in older children. For example,
many infants babble to themselves playfully on awakening. When the mother approaches the bedside to say
good morning, the infant, on seeing the mother,
smiles and stops babbling at the sound of her voice.
When the mother turns away to get diaper supplies and
pauses in her speech, the infant may resume babbling but
stop again when mother speaks. High-risk siblings who
are later diagnosed with autism often continue to vocalize as if they are not aware of their mothers speech; thus,
vocalizations overlap and do not resemble the protoconversations described previously. Often, there is less
eye contact, and the mother may feel that her child does
not recognize her voice or notice when she enters or
leaves the room. At 10 to 12 months of age, the typical
infant adds inflection to utterances in an attempt to tell a
story (jargoning), causing his or her speech to sound like
animated gibberish. Children who have autism often do
not add inflection at this age.

cognition

Other prelinguistic language deficits overlap with social skills. For example, not responding to ones name
being called (previously discussed in the section on social
orienting) traditionally has been considered a receptive
language deficit, but recent studies of infants later diagnosed with autism have revealed that the deficit is linked
more closely to the infants lack of social relatedness than
to a deficit in receptive language or auditory comprehension. (14) The possibility of a hearing loss also must be
considered, but as noted previously, children who have
hearing impairments fail to orient to both social and
environmental sounds.
Clinical Probes for the 9- and 12-month Health
Supervision Visits: Does your baby . . .
Look at you when you are speaking?
Babble? If yes, also ask . . . Does he or she take turns
vocalizing back and forth with you? Are vocalizations becoming more varied in terms of types of
sounds and length of sequences? Are these associated
with a growing repertoire of gestures?
Wave bye bye? Raise his or her arms to be lifted?
Hear your voice as well as he or she hears environmental sounds?
Make any unusual vocalizations or laugh inappropriately?

recognition of autism

If hearing is normal but receptive language is delayed, the PCP must consider the possibility of autism
or GDD; a wait and see approach is not appropriate. In such a case, the PCP should refer the child
immediately to a developmental or autism specialist
(or a team of specialists) and to an early intervention
program. (7) The clinician should not delay; a definitive diagnosis is not necessary to begin intervention. Staff can begin an intervention program that is
tailored to the childs deficits, and if necessary, the
strategies can be revised after a diagnosis becomes
evident. Unfortunately, parents of children who have
autism sometimes complain that they received inappropriate reassurance regarding their concerns, the
clinician counseled a wait and see approach, and
referrals were delayed.
Clinical Probes for the 12- and 15-month
Health Supervision Visits: The AAP has recommended formal screening for developmental skills at
9, 18, and 24 or 30 months of age using standardized
tools that include social and language milestones.
These tools also can provide useful probes for the 12and 15-month visits. Developmental tools are described in both the AAP Developmental Surveillance
and Screening (6) and ASD (7) guidelines. Selected
tools are included in the AAP ASD Toolkit (11).

Absent or Delayed Speech


Absent or delayed speech has been the most common
presenting sign in children who have autism. Although
most parents sense something is wrong by the time their
child is 18 months of age, they often do not share such
concerns with the clinician until much later, when they
realize that the child has little or no speech. Sometimes
they may rationalize that the delays are due to the childs
temperament (shy) or to a lack of opportunity to interact
with peers if the child is an only child. Although these
and other environmental factors may cause delayed
speech, receptive language is normal. Because parents
sometimes overestimate their childs receptive language
abilities and think that their child understands everything, referrals to audiology to evaluate hearing status
and to a speech and language pathologist to evaluate
both expressive and receptive language are indicated. If
hearing and receptive language are normal, a wait and
see approach may be appropriate. However, the PCP
should recommend strategies (by means of developmental handouts, attendance at a preschool with verbal peers,
etc) to stimulate speech.

Language Regression
Approximately 25% to 30% of children later diagnosed
with autism seem to develop normally and then regress.
Parents may report that children stopped talking and
gesturing (eg, pointing, waving bye) and made less eye
contact. Such autism regression usually occurs between 15 and 24 months of age, with most occurring
between 18 and 21 months. Although children appear to
develop normally until the regression, recent prospective
studies have revealed that some children have subtle
social and language deficits prior to regression. (13) (15)
Loss of language skills is not pathognomonic for autism;
it occurs in Rett syndrome and other neurodegenerative
disorders. Loss of speech associated with seizures is characteristic of Landau-Kleffner syndrome, but regression is
later (usually older than 3 years) and not associated with
loss of eye contact or social skills. Sleep electroencephalographic tracings in affected children often are characteristic.
Pediatrics in Review Vol.29 No.3 March 2008 91

cognition

recognition of autism

Clinical Probe for the 15-, 18,- and 24-month


Health Supervision Visits: Although most parents
are keenly aware when regression occurs and are
likely to raise a concern, others may rationalize that
some event in the childs life (birth of a sibling, move
to a new house, death of a family member) caused the
loss of skills. Because they may not raise a concern
spontaneously, a probe might be needed to highlight
the importance of skill loss.

Atypical Language
In addition to delays, children who have autism may
demonstrate atypical language during their second
year. Such atypicalities sometimes can give the false
impression of advanced speech, but the
speech is echolalic, ritualistic, and not functional.
Most typically developing children pass through a
stage where they imitate or echo anothers speech
(echolalia). They repeat the last one or two words of
the sentence they have just heard. Echolalia usually is
a temporary phenomenon and occurs when toddlers
are rapidly gaining new words. Echolalia is classified as
immediate (parroting that occurs immediately after
the partners vocalization) or delayed (parroting that
occurs at a later time). Whereas typically developing
children demonstrate the immediate variety, children
who have autism demonstrate both types. Autistic
echolalia also is much more pervasive and enduring
and includes larger chunks of verbal material. Children who have autism may demonstrate exceptional
verbal memory linked to echolalia and recite nursery
rhymes, advertisement jingles, or the ABCs at much
younger ages. The complexity of such speech utterances can mask true deficits in both functional speech
and receptive language skills. For example, a child may
be able to recite the ABCs or sing a television advertisement jingle but be unable to ask the parents for a
drink or follow a command. Unless this discrepancy
between echolalic and functional language is recognized, a speech deficit may be overlooked.
Some children who have autism, especially those who
are of normal intelligence, may become obsessed with
labeling colors, shapes, and numbers, yet they are unable
to use the terms in conversation or point to them on
request. Unlike typically developing toddlers, they demonstrate less interest in common everyday objects or
pictures in books and rarely point to them to request new
words. They may use overlearned gestalt pseudophrases
92 Pediatrics in Review Vol.29 No.3 March 2008

Downloaded from

or sentences spoken as a single giant word (eg,


Whatisit? Idontknow.), yet they lack the ability to combine words in novel phrases that convey meaning. Some
children who have autism say pop-up words that seem
to be out of context and have no communicative intent
to the listener. As suddenly as these words pop up for no
apparent reason, they also disappear. Occasionally, the
parent might discover the stimulus for the verbalizations.
For example, on seeing his mother dressed in a pink
striped blouse, a toddler who has little if any functional
speech exclaims elephant. No elephants (real or
stuffed) or pictures of elephants are within sight. He
repeats this several times that day but not the next. The
next time mother wears the same pink blouse, he again
begins saying elephant repeatedly. The mother may
recall that she wore the blouse the day they went to the
zoo and he saw his first elephant.

Clinical Probes for the 15,- 18,- and 24-month


Health Supervision Visits: The clinician may ask the
parent about echolalia, pop-up words, and exceptional
labeling skills. Formal screening is recommended at the
18- and 24-month visits.

Restrictive Interests, Stereotypies, and


Repetitive Behaviors
Although many typically developing children form
attachments to a stuffed animal, a special pillow, or a
blankee, children who have autism often prefer hard
items (ballpoint pens, flashlights, a piece of rope, or
particular action character). Moreover, the attachment is
much more robust; they may insist on holding the object
most of the day, even during meals. Many children
develop stereotypies (hand flapping, twirling, finger
movements, rocking, head nodding, toe walking, licking,
sniffing). Although stereotypies are distinctive and obvious, they usually appear later than 2 years of age. They
are not specific for autism; children who have severe
GDD or visual impairment also may demonstrate stereotypies. Even typically developing children can flap their
hands briefly when excited. Some children engage in
repetitive behaviors (eg, lining objects up) and may
protest violently when they are directed to a new activity.
The anger and protest may escalate quickly to a prolonged temper tantrum and aggression or self-injurious
behaviors.

cognition

Clinical Probes for 9,- 12,- and 15-month Health


Supervision Visits: Note gait and whether the child
carries a comfort item into the office. Probe for unusual
attachments, stereotypic movements, self-injurious behaviors, and unusually severe temper tantrums with
transitions or for no apparent reason.

Additional Early Autism Characteristics Not


Included in the DSM-IV-TR Criteria
Children who have autism may demonstrate simultaneous hyposensitivities and hypersensitivities for different stimuli within the same sensory modality. For example, the slightest sound of water dripping may irritate the
child, yet the child may seem oblivious to his mother
calling his name loudly. Affected children may explore
toys visually in unusual ways, holding objects very close
to their eyes, looking at objects out of the corners of the
eyes, or demonstrating an unusual head tilt. Others may
have oral aversions and intolerance to certain food textures that contribute to self-imposed restricted diets.
Additionally, children may demonstrate tactile defensiveness and not tolerate soft touch, garment labels, or
certain textures. On the other hand, they may be indifferent to noxious stimuli and injuries that typically are
painful to others. The dichotomy is puzzling, but it is
believed to be due to an abnormal arousal level or sensory
gating system.
The motor skills of children who have autism may seem
advanced, especially running, climbing, and jumping skills.
However, some children may have subtle deficits in fine
motor skills, coordination, and motor planning and sequencing of movements. Most affected children exhibit
fewer imitative motor patterns, although this may be linked
more closely to social rather than pure motor deficits. In
addition to abnormal quality of motor actions, children
who have autism may demonstrate abnormal amounts of
activity. Some may appear to be hyperactive and motor
driven, with an exterior focus of attention. Others may be
hypoactive or withdrawn, move little, and seem to have an
interior focus of attention.
Clinical Probes at the 12,- 15,- 18,- and 24-month
Health Supervision Visits: The clinician should ask the
parents about diet, unusual visual behaviors, hypo- or
hypersensitivities, and activity level. A neurologic examination or gross and fine motor screening tools can be
helpful in evaluating coordination and motor skills.

recognition of autism

Autism is not associated with a classic physical phenotype. More than 90% of children who have ASDs have
idiopathic ASD, meaning that the cause currently is
unknown and that there is no known associated syndrome. Such children look normal and have few, if any,
dysmorphic features (eg, posteriorly rotated ears). Some
of these children demonstrate accelerated head growth
beginning at about 6 months of age and continuing for
the next few months, sometimes to the point of macrocephaly. However, the head size often normalizes in late
childhood. The remaining 5% to 10% have an associated
syndrome (eg, fragile X syndrome, tuberous sclerosis,
phenylketonuria) and manifest the physical signs that are
characteristic of that syndrome. (16)
Clinical Strategy for All Health Supervision Visits:
Generally, most dysmorphic features are recognized at
birth; some may be subtle and require ongoing surveillance
as they become more prominent. The PCP should measure
the head circumference and plot head growth to monitor
for growth acceleration. Although the PCP should consider
the possibility of hydrocephalus, computed tomography scan
or magnetic resonance imaging often is not indicated
unless there are associated neurologic signs or other indicators for neuroimaging studies.

Screening and Evaluation


In addition to conducting surveillance at every health
supervision visit, the AAP has recommended formal
screening of all children with a standardized ASDtargeted tool at the 18- and 24-month visits and whenever a concern is raised. (7) To operationalize this recommendation, the AAP has developed an ASD toolkit.
(11) Although the toolkit contains several screening
tools, no screening tool is ideal, and most target children
18 months and older. Screening tools for younger children are in development and should be available soon.
The AAP tool kit also can serve as a resource guide for
both clinicians and parents because it contains related
policy statements, clinical reports, surveillance and
screening algorithms and tools, and two collections of
fact sheets (one set for physicians and one for parents)
that address major health, developmental, educational,
transition, and family support issues associated with
ASDs.
The ASDs surveillance and screening algorithm published in the AAP ASD clinical report (7) states that if a
child has two or more risk factors (eg, older sibling
having an ASD, parent concern, other caregiver concern,
Pediatrics in Review Vol.29 No.3 March 2008 93

cognition

recognition of autism

or physician concern), the PCP should make three simultaneous referrals: to an early intervention or school program (depending on the childs age), to an ASD specialist
or team of specialists for a comprehensive evaluation, and
to audiology (if this has not already been done). If the
child has only one risk factor, the PCP should use an
appropriate screening tool based on the age of the child.
If the result is positive, the PCP should make the same
three referrals. If the results of the screening are negative,
an additional appointment should be made within 1
month to monitor progress and address any residual
concerns the parent or PCP might have in spite of a
negative screen. If there are no risk factors, the child
should be screened with an ASD-specific tool at 18 and
24 months. This process is described in much greater
detail in the AAP ASD clinical report. (7)
Whereas surveillance and screening are the responsibility of the PCP, the comprehensive evaluation may be
accomplished best by a specialist or team of specialists
who have expertise in autism. Such evaluations usually
include the following components: 1) a thorough history, including a three-generation pedigree; 2) a detailed
physical examination focusing on neurologic signs and
dysmorphic features; 3) thorough developmental or psychometric evaluations, depending on the childs mental
age and level of cooperation; 4) assessment with a standardized ASD tool (eg, ADOS) that operationalizes the
DSM-IV-TR criteria, or if not available, assessment of
the criteria themselves using clinical judgment; 5) an
assessment of the familys strengths and needs; and 6) an
etiologic search based on results of the first five components. The PCP may be involved in this process to
varying degrees, depending on the available local resources and his or her level of comfort.

Management
The PCPs most important role is to provide a medical
home, as with all children (www.medicalhomeinfo.org).
This can be more challenging for several reasons:
1) Many children who have ASD are unable to describe
their symptoms or localize their pain; 2) Care coordination can be complex due to a variety of practitioners
(developmental and behavioral specialists, pediatric subspecialists, therapists, teachers, social workers, vocational
staff); 3) Parents may be stressed and require more family
supports; 4) Children may have ongoing pica and continue to need lead screening; 5) Children may have
coexisting medical (seizures, nutritional), psychiatric
(anxiety, obsessive compulsive disorder), and behavioral
(aggression, sleep disorders) problems; and 6) Infant
siblings need heightened surveillance. The PCP also may
94 Pediatrics in Review Vol.29 No.3 March 2008

need to counsel parents regarding the 10 times increased


risk of ASDs in subsequent siblings if this information
was not conveyed by the specialist making the diagnosis.
Management of autism generally is the province of
early intervention specialists, educators, therapists, and
behavior management specialists. Early intervention programs and schools should provide services that are individualized, appropriate, and intense and should address
development, social skills, behavior, and academic issues.
Although all children should receive appropriate developmental, therapeutic, and educational services, not
all require medication. There is no medical cure for
autism, and only one medication (risperidone) has been
approved by the United States Food and Drug Administration for use in children who have ASDs. Medication
is an adjunctive treatment that often is used to address
challenging behaviors after behavioral and environmental interventions have failed. However, medications
never should be started without first performing a physical examination to rule out a medical cause (eg, tooth
abscess, ear infection, abdominal discomfort) for a newonset behavior. When the child puts him- or herself or
others in danger, medication may become a front-line
intervention.
Medications should be considered when behaviors
(eg, self-injurious behaviors, aggression, anxieties, obsessions, stereotypies, sleep disorders) negatively affect or
prevent the progress of educational, therapeutic, and
behavioral interventions. When choosing a medication,
the clinician should target the most challenging behavior. When prescribing the medication, the clinician
should start low and go slow. Children should be
monitored for adverse effects, especially when they are
unable to verbalize symptoms. Parents of children who
have ASDs often seek complementary and alternative
medicine (CAM). (17) Although it is difficult to be
familiar with the myriad CAM possibilities, the PCP
should be familiar with some of the most popular local
options and provide the family with evidence-based information, if available. The AAP clinical report provides a
detailed review of the management of children who have
ASD. (18)

Conclusion
A growing body of evidence is revealing that many
children who have autism can be recognized before age 2
years. PCPs now have the opportunity to play a pivotal
role in early recognition. PCPs who recognize autism in
children younger than 2 years of age, refer them for
comprehensive evaluations, and help them enroll in appropriate early intervention programs benefit the chil-

cognition

dren, their families, their future schools, and society.


Early diagnosis and intervention improve outcomes, empower families, decrease the need for special education
services in later years, and increase the childs chance for
independence and gainful employment as an adult, especially if there are no coexisting cognitive deficits. A high
degree of suspicion and a solid understanding of early
deficits in social and preverbal skills are critical to early
recognition. The AAP ASD clinical reports and toolkit
should help guide and support PCPs in this endeavor.

References
1. Centers for Disease Control and Prevention. Prevalence of autism spectrum disordersAutism and Developmental Disabilities
Monitoring Network, 14 Sites, United States, 2002. MMWR Surveill Summ. 2007;56(SS-1):1228
2. Dosreis S, Weiner CL, Johnson L, Newschaffer CJ. Autism
spectrum disorder screening and management practices among
general pediatric providers. J Dev Behav Pediatr. 2006;27:
S88 S94
3. National Research Council, Committee on Interventions for
Children With Autism. Educating Children With Autism. Washington, DC: National Academies Press; 2001
4. Zwaigenbaum L, Bryson S, Rogers T, Roberts W, Brian J,
Szatmari P. Behavioral manifestations of autism in the first year of
life. Int J Dev Neurosci. 2005;23:143152
5. Landa R. Early communication development and intervention
for children with autism. Ment Retard Devel Dis Res Rev. 2007;13:
16 25
6. American Academy of Pediatrics, Council on Children With
Disabilities, Section on Developmental and Behavioral Pediatrics,
Bright Futures Steering Committee, Medical Home Initiatives for
Children with Special Needs Project Advisory Committee. Identifying infants and young children with developmental disorders in
the medical home: an algorithm for developmental surveillance and
screening. Pediatrics. 2006;118:405 420
7. Johnson CP, Myers SM. Council on Children with Disabilities.
Identification and evaluation of children with autism spectrum
disorders. Pediatrics. 2007;120:11831215
8. Lord C, Risi S, Lembrecht L, et al. The Autism Diagnostic
Observation Schedule generic: a standard measure of social and
communication deficits associated with the spectrum of autism. J
Autism Dev Disorder. 2000;30:205223
9. Stone WL, Lee EB, Ashford L, et al. Can autism be diagnosed
accurately in children under 3 years? J Child Psychol Psychiatry.
1999;40:219 226
10. American Psychiatric Association. Diagnostic and Statistical
Manual of Mental Disorders, Fourth Edition, Text Revision (DSMIV-TR). Washington, DC: American Psychiatric Publishing, Inc;
2000
11. AAP Autism Expert Panel. Autism: Caring for Children With
Autism Spectrum Disorders: A Resource Toolkit for Clinicians. Elk
Grove Village, Ill: American Academy of Pediatrics; 2007
12. Osterling JA, Dawson G, Munson J. Early recognition of

recognition of autism

1-year-old infants with autism spectrum disorder versus mental


retardation. Dev Psychopathol. 2002;14:239 251
13. Maestro S, Muratori F, Cesari A, Pecini C, Apicella F, Stern D.
A view to regressive autism through home movies. Is early development really normal? Acta Psychiatr Scand. 2006;113:68 72
14. Nadig AS, Ozonoff S, Young GS, Rozga A, Sigman M, Rogers
S. A prospective study of response to name in infants at risk for
autism. Arch Pediatr Adolesc Med. 2007;161:378 383
15. Werner E, Dawson G. Validation of the phenomenon of
autistic regression using home videotapes. Arch Gen Psychiatry.
2005;62:889 895
16. Muhle R, Trentacoste SV, Rapin I. The genetics of autism.
Pediatrics. 2004;113:472 486
17. American Academy of Pediatrics, Committee on Children with
Disabilities. Counseling families who choose complementary and
alternative medicine for their child with chronic illness or disability.
Pediatrics. 2001;107:598 601
18. Myers SM, Johnson CP. Council on Children with Disabilities. Management of children with autism spectrum disorders.
Pediatrics. 2007;120:11621182

Suggested Reading
Chawarska K, Volkmar FR. Autism in infancy and early childhood.
In: Volkmar FR, Klin A, Paul R, eds. Handbook of Autism and
Pervasive Developmental Disorders. 3rd ed. Hoboken, NJ: John
Wiley & Sons, Inc; 2005:223246
Johnson CP. Early clinical characteristics of children with autism.
In: Gupta VB, ed. Autism Spectrum Disorders in Children.
New York, NY: Marcell Decker; 2004:85123
Landa RJ, Holman KC, Garrett-Mayer E. Social and communication development in toddlers with early and later diagnosis of
autism spectrum disorders. Arch Gen Psychiatry. 2007;64:
853 864
Mitchell S, Brian J, Zwaigenbaum L, et al. Early language and
communication development of infants later diagnosed with
autism spectrum disorder. J Dev Behav Pediatr. 2006;27(2
suppl):S69 S78
Sigman M, Dijamco A, Gratier M, Rozga A. Early detection of core
deficits in autism. Ment Retard Dev Disabil Res Rev. 2004;10:
221233
Wetherby AM, Prizant BM, Schuler AL. Understanding the nature
of communication and language impairments. In: Wetherby
AM, Prizant BM, eds. Autism Spectrum Disorders. Baltimore,
Md: Paul H. Brookes Publishing Co; 2000:109 141
Autism Speaks, a nonprofit organization dedicated to increasing
awareness of autism and raising money to fund autism research,
together with First Signs, the leader in early identification and
intervention of children with developmental delays and disorders, and Florida State University, have developed a first-of-itskind web-based video glossary to help professionals learn more
about the early warning signs of ASDs. The glossary, available
free of charge at www.autismspeaks.org, www.firstsigns.org,
and http:// firstwords.fsu.edu, contains more than 100 video
clips that illustrate both typical and atypical development.

Pediatrics in Review Vol.29 No.3 March 2008 95

cognition

recognition of autism

PIR Quiz
Quiz also available online at www.pedsinreview.org.
5. Differences in pointing behaviors, as an example of joint attention, may be used in descriptions of
children believed to have autism. Mature joint attention is demonstrated best with a point whose purpose
is to:
A.
B.
C.
D.
E.

Comment.
Direct.
Distract.
Label.
Request.

6. Of the following, the best DSM-IV-TR-based criteria to identify children younger than 2 years of age who
have autism are:
A.
B.
C.
D.
E.

Abnormal conversational skills and stereotypic language.


Delay in achieving speech and language milestones.
Failure to form age-appropriate peer relationships.
Impairment in use of nonverbal behaviors.
Ritualistic behaviors and need for routine.

7. The failure of an 18-month-old child who has autism to respond to his or her name when called is believed
to be the result of deficits in:
A.
B.
C.
D.
E.

Auditory processing.
Cognition.
Hearing.
Receptive language.
Social relatedness.

8. Careful observation of typical children at play has been important in better understanding children who
have autism. Children who have autism rarely demonstrate evidence of:
A.
B.
C.
D.
E.

Constructive play.
Oral motor play.
Roughhouse play.
Sensory motor play.
Symbolic play.

96 Pediatrics in Review Vol.29 No.3 March 2008

index of suspicion

The reader is encouraged to write


possible diagnoses for each case before
turning to the discussion. We invite
readers to contribute case
presentations and discussions.
Please inquire first by contacting Dr.
Nazarian at LFredN@aol.com.

Author Disclosure
Drs Peshkovsky, Leggiadro,
Kupersmith, Septer, Fernandez, and
Zapata did not disclose any financial
relationships relevant to these cases.

Frequently Used Abbreviations


ALT:
AST:
BUN:
CBC:
CNS:
CSF:
CT:
ECG:
ED:
EEG:
ESR:
GI:
GU:
Hct:
Hgb:
MRI:
WBC:

alanine aminotransferase
aspartate aminotransferase
blood urea nitrogen
complete blood count
central nervous system
cerebrospinal fluid
computed tomography
electrocardiography
emergency department
electroencephalography
erythrocyte sedimentation rate
gastrointestinal
genitourinary
hematocrit
hemoglobin
magnetic resonance imaging
white blood cell

Case 1 Presentation

Case 2 Presentation

A 16-month-old girl is evaluated in


the ED for increased work of breathing. She had been seen 4 days ago for
rhinorrhea, cough, vomiting, diarrhea, and fever, all of which appeared
to have resolved. At that time, she
had a normal CBC and serum electrolyte measurement and was treated
with intravenous fluids and discharged. Two days later, she again
developed cough with posttussive
vomiting and rhinorrhea, but no fever or diarrhea.
On physical examination, the girl
is awake and alert but in mild-tomoderate respiratory distress. Her
respiratory rate is 50 breaths/min,
pulse oximetry saturation is 94%, and
heart rate is 161 beats/min. She is
afebrile. She has moderate pharyngeal erythema, clear rhinorrhea, and
a hyperemic right tympanic membrane. Intercostal retractions, mild
wheezing, and occasional scattered
crackles are present. The rest of her
physical findings are normal. She is
given three treatments of albuterol
and prednisolone and responds with
a lower respiratory rate of 40
breaths/min but a pulse oximetry
saturation of 92% in room air.
Initial laboratory studies include
negative results for respiratory syncytial virus (RSV) and influenza A and
B antigens. A chest radiograph shows
no acute disease and mild hyperinflation. She is admitted to the hospital.
Over the next 3 days, she develops
a temperature to 101F (38.4C) and
has no improvement, despite frequent
albuterol treatments. On day 4, her
WBC is 9103/mcL (9109/L),
with 41% neutrophils, 2% bands, 43%
lymphocytes, and 7% monocytes, and
her platelet count is 775103/mcL
(775109/L). A urine culture is negative, but a blood culture is reported
positive for gram-negative diplococci.

A 10-year-old boy is admitted to


the hospital after 4 days of progressive vision loss in his right eye. His
symptoms began 4 days ago with eye
pain on lateral movement, followed
by worsening blurriness and vision
loss that have progressed upward
through his right visual fields over
the past 3 days. Now he has lost
complete vision in his right eye. He
denies fever, headache, or trauma to
the eye. He recently was exposed to
mosquitoes on a camping trip, but he
denies getting tick bites or having
any recent illnesses.
Physical examination reveals a
pleasant boy in good spirits. He still
complains of pain in his right eye on
lateral movement, but all extraocular
motions appear intact. An external
examination of the eye gives normal
results, with no periorbital swelling
or conjunctivitis. His visual acuity is
20/20 in the left eye, and he has only
light perception in the right. A more
extensive eye examination reveals the
diagnosis.

Case 3 Presentation
A 14-year-old boy is evaluated for
excessive nighttime eating. Since
early childhood, he awakens every
night around midnight and consumes large quantities of food, even
those he dislikes. Alarms, locks, and
punishment have not stopped this
habit. He remembers his eating in
the morning but has no remorse or
regret. In addition, he eats a small
breakfast and a lunch and dinner normal for his age. He has gained 50 lb
in the last year.
He has been evaluated by a pediatrician, psychologist, psychiatrist,
neurologist, and educational specialist; has been diagnosed as having
mild autism, obsessive-compulsive
disorder (OCD), and learning problems; and has been treated with
Pediatrics in Review Vol.29 No.3 March 2008 97

index of suspicion

methylphenidate, divalproex, and


clonidine. He was adopted at 6 days
of age, and no family medical history
is available.
Physical examination reveals an
interactive, conversational boy who
answers questions appropriately. His
vital signs are normal, weight is
171 lb (above the 95th percentile),
height is 69 in (90th percentile), and
body mass index is 25.2 (95th percentile). He has mild hypotonia and
mild weakness of the legs. Other than
obesity, all other physical findings are
normal.

Case 1

Discussion

The patients age of 16 months in


conjunction with cough, rhinorrhea,
wheezing, and chest wall retractions
were consistent with a diagnosis of
bronchiolitis. Bronchiolitis is predominantly a viral illness (RSV, parainfluenza 3 virus, adenovirus, influenza virus) but also can be caused by
Mycoplasma. Notwithstanding negative antigen tests for RSV and influenza A and B, she was treated for
presumptive viral lower respiratory
tract disease.
The blood culture grew Moraxella catarrhalis, and she was treated
for 7 days with parenteral ceftriaxone
followed by 7 days of oral cefuroxime
axetil. She was discharged without
complications.

The Clinical Clue


Typically, bronchiolitis reaches its
most critical stage during the first
48 to 72 hours after the onset of
cough. This patients course was
atypical because she developed her
initial fever after 3 days of cough
and wheezing, which proved to be
indicative of a bacterial infection.
Although uncommon, bronchiolitis
may be complicated by bacterial infection (about 2% to 10% of cases).
Bacterial otitis media and pulmonary
98 Pediatrics in Review Vol.29 No.3 March 2008

bacterial coinfection, as well as associated urinary tract infection, are examples from recent literature.
In general, a clinician should suspect sepsis whenever a patient has fever
associated with behavioral changes
such as irritability, fussiness, lethargy,
poor feeding, and altered mental status. Tachycardia and tachypnea also
may reflect sepsis. Petechiae and purpura are well-known cutaneous indicators of possible sepsis, especially
meningococcemia. A weak cry and
jaundice may indicate sepsis in neonates. The presentation of sepsis depends on the competency of the
patients immune system. Subtle presentations can occur in young infants
and immunocompromised children,
with the clinical picture influenced by
the patients level of immunity.

The Agent
M catarrhalis is an aerobic, gramnegative diplococcus in the family
Neisseriaceae that commonly inhabits the upper respiratory tract (nasopharynx), with increased seasonal
colonization in fall and winter. M
catarrhalis can cause acute, localized
infection such as otitis media, sinusitis, conjunctivitis, and pneumonia in
children. Although it causes a large
proportion of cases of lower respiratory tract infection in elderly patients
who have chronic obstructive pulmonary disease and chronic bronchitis, this association has not been seen
in pediatric patients.
M catarrhalis generally is not
thought of as causing invasive, systemic disease (such as meningitis
and endocarditis) except in immunocompromised conditions, but documentation of bacteremia occurs in
children in rare instances. Risk factors for bacteremia include viral infection, sickle cell disease, malignancy, acquired immunodeficiency
syndrome, and other immunodeficient states. This child did not have

any other evidence of immunodeficiency; thus, additional evaluation


for that state was not undertaken. It
is possible that her hyperemic tympanic membrane was caused by an M
catarrhalis infection, but the finding
also could result from underlying viral infection.
In the clinical laboratory, isolates
of M catarrhalis must be differentiated from Neisseria sp. The management and infection control differences between Neisseria sp and M
catarrhalis are important. As in this
case, identification of gram-negative
diplococci in a patients blood culture warrants droplet precautions
for suspected meningococcemia for
24 hours while the patient receives
appropriate antimicrobial therapy.
Confirmation of N meningitidis also
warrants antimicrobial prophylaxis
for appropriate contacts.
Also, major management and social implications are associated with
the differentiation between N gonorrhoeae and M catarrhalis when gramnegative diplococci are identified in
the smear of an eye discharge from a
baby who has neonatal conjunctivitis. Gonococcal neonatal conjunctivitis requires systemic antimicrobial
therapy for the baby as well as evaluation and management of the mother
and her partner. A final diagnosis of
M catarrhalis does not raise any of
these issues.

Therapy
More than 85% of M catarrhalis
isolates are ampicillin-resistant because of beta-lactamase production.
First-line antibiotics for focal infections (otitis media, sinusitis, pneumonia) are oral amoxicillin-clavulanate
or oral second- and third-generation
cephalosporins. Other antibiotics active against this organism include
macrolides, trimethoprim-sulfamethoxazole, and fluoroquinolones.
Sepsis generally is treated paren-

index of suspicion

Case 2 Discussion

circumstances, when the light is


shone in one eye, the pupils of both
eyes constrict equally. However,
when the light is shone in the affected eye of a patient who has an
APD, the pupil of the affected eye
paradoxically dilates rather than constricts. This abnormal response signifies that the afferent pathway to the
brain is not functioning normally and
the brain is not receiving the message
properly. However, when the light is
shone in the unaffected eye, the afferent pathway in the unaffected eye as
well as the efferent pathway in the
affected eye function normally, with
both pupils constricting. If the light
is moved back to the affected eye, the
pupil appears, paradoxically, to dilate
again.
On funduscopic examination,
edema of the right optic disc with
blurring of the disc margin was noted
in this patient; the left optic nerve
appeared normal. Generally, an APD
in combination with papilledema
confirms the diagnosis of acute-onset
optic neuritis. This diagnosis was
confirmed by an ophthalmologist,
and the boy was admitted for additional evaluation as well as to receive
a course of high-dose corticosteroids. One week later, the acuity in
his right eye had improved to 20/
100, and the pain had resolved completely. At the time of discharge and
at follow-up with the ophthalmologist, he continued to have the diagnosis of optic neuritis of unknown
cause.

The boy also had an afferent pupillary


defect (APD) in his right eye. An
APD, also referred to as a MarcusGunn pupil, occurs when there is a
problem transmitting an afferent signal through the optic nerve to the
brain. An APD is identified by examining the eyes with a bright light,
known as the swinging flashlight
test. A flashlight is moved back and
forth between the eyes. In normal

Optic neuritis refers to inflammation


of the optic nerve. This condition
can occur at any age, although it is
more common in adults than in children and is more common in females,
who comprise 60% to 75% of affected
patients.
It previously was believed that
most children who acquired optic

terally until the patient becomes


asymptomatic and has a negative repeat blood culture, when treatment
may be changed to oral therapy to
complete a 7- to 14-day course, depending on the organism. When receiving an initial report of gramnegative diplococci growing in a
blood culture, it is prudent to administer parenteral third-generation
cephalosporins (to cover Neisseria sp
and M catarrhalis) until the isolate is
identified. Then, therapy can be individualized, depending on the clinical
course and antimicrobial susceptibility results.

Lessons for the Clinician


When the course of a patients illness
does not follow the usual expectations, it is appropriate to consider the
possibility of multiple diseases occurring simultaneously. Although rare,
M catarrhalis has the potential to
cause a serious bacterial infection.
The identification of gram-negative
diplococcus in the blood of a patient
who has fever and respiratory disease
or otitis media should alert the physician to the possibility of the uncommon M catarrhalis as well as
the more dangerous N meningitidis.
(Courtney Peshkovsky, MD, Robert J.
Leggiadro, MD, Lincoln Medical and
Mental Health Center, Bronx, NY,
and Weill Medical College of Cornell
University, New York, NY)

The Condition

neuritis experienced bilateral visual


loss, but new data have shown that
unilateral visual loss is the most common presentation in children and
adults. Other common presenting
symptoms in children include pain
with ocular movements, headache,
and a viral prodrome. On physical
examination, it is most common to
see visual acuity in the affected eye of
20/400 or worse. Some children can
perceive light only, and some do not
even have that ability. Visual fields
and color vision are abnormal in
more than 80% of patients, and an
APD is seen in more than 50% of
patients.
Generally, disc edema is visible in
the affected eye on funduscopic examination. This edema can resemble
the swelling seen with papilledema,
but the presence of an APD points
toward the diagnosis of optic neuritis. The pediatric generalist should
perform frequent funduscopic examinations in the course of practice and
be able to discern abnormalities of
the optic disc that require referral for
additional study.

Differential Diagnosis
Many underlying conditions have
been associated with optic neuritis,
but it is not always possible to determine the specific cause for each
patient. Because as many as 85% of
cases are associated with a viral infection or recent immunization
(most commonly, measles-mumpsrubella), it is important to inquire
about a recent viral prodrome. Optic
neuritis also has been associated with
other infectious entities, such as
Lyme disease, varicella, syphilis, catscratch disease (Bartonella henselae
infection), pertussis, infectious mononucleosis, toxoplasmosis, brucella,
and tuberculosis as well as with toxocariasis and helminthic infestations.
Some of these infectious causes are
associated with distinct historic or
Pediatrics in Review Vol.29 No.3 March 2008 99

index of suspicion

physical findings. For example, patients who have Lyme disease may
have a positive travel history or a
history of a rash consistent with erythema migrans. Any preceding history of varicella or herpes zoster, especially in an immunocompromised
patient, is especially important because varicella-zoster virus infection
can progress from direct infiltration
of the optic nerve to retinal necrosis if
not treated appropriately.
The pathogenesis of the other
viral causes is a cell-mediated attack
on optic nerve myelin. However, the
pathogenesis of disease following
bacterial infection is less clear. In the
case of cat-scratch disease, gramnegative organisms tend to invade
endothelial cells, leading to an inflammatory response. A similar response within the retina can lead to
both generalized inflammation and
focal areas of endothelial cell damage
and proliferation. Such areas of retinal damage are consistent with funduscopic signs of neuroretinitis, including stellate macular exudates and
splinter hemorrhages. These findings
also can be seen in toxoplasmosis.
In the case of Borrelia burgdorferi
infection, the spirochete invades the
eye, causing direct inflammation of
the optic nerve. With syphilis, a vitreous cellular reaction typically is
present, most likely due to a direct
infection of the optic nerve. Syphilis
associated with optic neuritis also is
seen more commonly in immunocompromised patients.
Optic neuritis in adults classically
is associated with the development of
multiple sclerosis (MS); in children,
this association is not as substantial.
MS is a more likely cause if the child
presents with bilateral involvement
or has recurring episodes. Another
possible cause of optic neuritis is neuromyelitis optica or Devic syndrome.
In these patients, demyelinating lesions are found in the optic nerve or
100 Pediatrics in Review Vol.29 No.3 March 2008

the spinal cord, but not in the brain,


and their clinical pictures are suggestive of a demyelinating disease. Optic
neuritis also may be due to systemic
conditions such as sarcoidosis, in
which there have been reports of
choroidal and retinal masses representing granulomas, and of systemic
lupus erythematosus (SLE).

Making the Diagnosis


MRI of the brain and orbits with
contrast showing enhancement of
the optic nerve is essential to confirm the diagnosis. The MRI also
can show changes in the white matter
that may represent demyelination
and signify the possibility of developing MS. Sarcoidosis should be
considered if a mass is seen at the
head of the optic nerve on MRI. This
lesion should be biopsied; histologic
changes demonstrating noncaseating
granulomas are diagnostic of sarcoidosis. An MRI of the spinal cord also
should be performed in any patient in
whom Devic syndrome is a consideration.
Performing a lumbar puncture for
the collection of CSF is another
important part of the evaluation of
optic neuritis. CSF studies may indicate an acute infectious process.
Analysis of CSF for oligoclonal banding can help determine if MS is likely.
The evaluation also can include testing the CSF for markers of syphilis,
Lyme titers, acid-fast bacteria, fungal
infection, and varicella-zoster infection by culture or polymerase chain
reaction. It also is possible to test for
other infections, such as B henselae,
Epstein-Barr virus, and B burgdorferi, with blood cultures and antibody tests. CSF analysis can include
antinuclear antibodies to screen for
SLE as well as lymphocyte analysis
for sarcoidosis.

Treatment
The current treatment of optic neuritis is administration of corticosteroids, but there are various protocols.
The Optic Neuritis Treatment Trial
recommends a 3-day course of highdose steroids followed by an 11-day
course of oral prednisone. (1) It also
is important to treat any infection
that may be present.

Prognosis
No extensive data address the prognosis of childhood optic neuritis. In
general, the prognosis for visual recovery is excellent regardless of therapy. In fact, it has been shown that
steroid treatment has no effect on
long-term outcome, only that patients recover visual acuity more
quickly when steroid therapy is initiated. The prognosis for optic neuritis
is worse for children who have bilateral presentation, MRI changes, and
recurring episodes because these features are associated with a higher risk
for developing MS. In one prospective study, bilateral optic neuritis in
combination with MRI white matter
lesions was associated most strongly
with development of MS. Conversely, of the patients they reviewed,
none who had normal MRI findings
developed MS after 2-year followup. (2)
Long-term follow-up of patients
diagnosed as having Devic syndrome
shows a poorer outcome and relapses. Of those patients experiencing multiple relapses, Devic syndrome may be the first presentation
of SLE, suggesting an autoimmune
etiology for optic neuritis in those
patients.

Lessons for the Clinician


Optic neuritis offers a wide differential diagnosis, but history and physical examination (including a comprehensive ophthalmologic assessment)
may help to focus and guide diagnos-

index of suspicion

tic testing. An MRI and lumbar


puncture for CSF analysis are essential, although they may not lead to
discovery of a cause. It is important
to seek all underlying causes that can
be treated (Lyme disease, syphilis,
varicella) in the initial diagnostic testing. Affected patients should be followed to monitor the recovery of
visual function, the occurrence of
neurologic complaints, and the recurrence of optic neuritis, which may
indicate a predisposition for the development of MS. (Lauren Kupersmith, MD, University of California
at Davis Medical Center, Sacramento, Calif.)

References
1. Beck RW, Trobe JD, for the Optic Neuritis Study Group. What we have learned
from the Optic Neuritis Treatment Trial.
Ophthalmology. 1995;102:1504 1508
2. Wilejto M, Shroff M, Buncic J, et al. The
clinical features, MRI findings, and outcome of optic neuritis in children. Neurology. 2006;67:258 262

Case 3 Discussion
This boy had an eating disorder characterized by overeating at night in
conjunction with associated psychological problems, including OCD
and learning disability. His nighttime
eating was causing numerous problems, including a loss of sleep that
might be affecting his school performance. The long-term effects on his
health from the gain of 50 lb in 1 year
are significant and could include
metabolic syndrome, diabetes, hypercholesterolemia, and coronary artery disease. His eating pattern is
troubling socially and has been disruptive to the family.

Differential Diagnosis
Nighttime eating can have many
causes, including sleep-related eating
disorder (SRED), binge eating disor-

der with binges at night, night eating


syndrome (NES), and behavioral disorders that have nonconventional
eating patterns. Another consideration in this patient who has an associated learning disability is PraderWilli syndrome, which was ruled out
via genetic testing. It should be
noted that some nighttime snacking
is normal, especially in teenagers, but
eating greater than 50% of total calories at night and awakening from
sleep to eat are not normal eating
behaviors. Discerning the cause of
nighttime eating requires clinical
judgment aided by a thorough history, including dietary logs and
eating questionnaires.
SRED and NES are similar conditions, but the history can elicit differences. SRED is associated with other
sleep-related disorders, such as sleep
apnea and restless legs syndrome,
that are not seen in NES. Polysomnography can aid in differentiating
the conditions because the only abnormality usually reported in NES is
low sleep efficiency. Patients who
have SRED often consume unusual
foods and nonfood items, in contrast
to the consistent food choices in
NES. Another variance between the
two disorders is that patients who
have NES are aware of their eating
(and show no remorse), whereas
SRED sufferers often are not aware
of their eating and do not remember
the event.
Bulimia nervosa also can present
with nighttime eating binges, often
with purging, but purging is not reported typically in NES. The abnormal feature seen in NES is more the
timing of food intake than the actual
amounts. Intake of massive quantities of food is more characteristic of
bulimia, although patients who have
NES can eat enough to gain excessive
weight.

The Condition
As part of his evaluation, the patient
completed a diet log that revealed an
average consumption of 1,417 kcal
from 10:00 PM to 6:00 AM and consumption of 1,388 kcal during the
other 16 hours of the day (more than
50% of calories after the evening
meal). Nighttime eating was reported during 7 of 7 nights, and he
was fully aware of his eating. Anorexia was present each morning.
The boy also met criteria for depression (by use of a standard depression scale). This patient, therefore,
seemed to fit closely a diagnosis of
NES. The diagnosis of NES is difficult in pediatrics because criteria are
established only for adults.
Although first described in 1955,
NES was not studied extensively until the recent societal increase in obesity, and the disorder still is in the
early stages of investigation. Most research has been performed in adults,
and pediatric diagnostic criteria still
are being developed. The major
characteristics are evening or night
hyperphagia, insomnia with waking
to eat at night, and morning anorexia. Birkvedt and associates (1)
include additional criteria: consuming greater than 50% of total daily
calories at night, awakening at least
once per night, consuming a snack
during awakenings, having symptoms for at least 3 months, and not
meeting the criteria for bulimia or
binge-eating syndrome.
Although the criteria for inclusion
have changed over the years, the
central feature is a delay in the circadian pattern of eating. Circadian oscillators may signal food intake at a
different phase than is signaled normally, and the circadian timing of
food intake is delayed. Other problems associated with NES include
psychological disorders such as depression, anxiety, and low selfesteem. This boys learning problems
Pediatrics in Review Vol.29 No.3 March 2008 101

index of suspicion

and OCD are consistent with such


observations about NES. In one
study, 5.6% of patients who had NES
had OCD as well.
The prevalence of NES is estimated to be 0.5% to 1.6% of the
general population and 6% to 14% of
patients who attend obesity clinics.
Outpatient psychiatric clinics report
an incidence of NES as high as 16%.
Patients who have NES can be of
normal weight as well as obese. The
mean body mass index in a study of
106 patients was 30.8. (2)

rotonin. The use of selective serotonin reuptake inhibitors (SSRIs),


therefore, has been studied in treating NES. A study by OReardon and
colleagues (3) showed a decreased
number of awakenings, episodes of
nocturnal ingestion, and percent of
calories eaten after supper after
12 weeks of treatment with sertraline. Approximately 25% of the patients in this study experienced remission and had an average weight
loss of 4.8 kg (10.8 lb).

Lessons for the Clinician


Treatment
Optimal treatment of NES still is
subject to research. The nighttime
food preferences primarily seeming
to be carbohydrates may signify an
attempt to promote sleep or improve
mood. Tryptophan uptake is promoted by insulin release after carbohydrate ingestion, and tryptophan is
a precursor to serotonin synthesis.
Circadian rhythms of eating and
sleep are controlled by a part of the
hypothalamus that is affected by se-

102 Pediatrics in Review Vol.29 No.3 March 2008

NES is an unusual form of eating


disorder whose pathophysiology,
treatment, and prognosis still are being explored and require more study,
especially in children. As additional
research is completed in children, a
more concrete set of criteria may be
developed for diagnosis in pediatric
patients. Treatment with SSRIs offers some promise based on a recent
study. This diagnosis should be considered in patients who demonstrate
excessive nighttime eating or in those

who are obese without clear cause,


especially if they have sleep disturbances or other psychological disorders. A history that involves detailed
documentation of eating and sleeping habits is the most important diagnostic tool. (Seth Septer, DO, Cristina Fernandez, MD, Fernando
Zapata, MD, University of Nebraska/
Creighton University School of Medicine, Omaha, Neb.)

References
1. Birkvedt GS, Florholmen J, Sundsfjord
J, et al. Behavioral and neuroendocrine
characteristics of the night-eating syndrome. JAMA. 1999;282:657
2. de Zwaan M, Roerig DB, Crosby RD,
Kraz S, Mitchell JE. Nighttime eating: a
descriptive study. Int J Eating Dis. 2006;39:
224 232
3. OReardon JP, Sunnkard AJ, Allison
KC. Clinical trial of sertraline in the treatment of night eating syndrome. Int J Eating
Dis. 2004;35:16 26

To view Suggested Reading lists for


these cases, visit www.pedsinreview.
org and click on Index of Suspicion.

The following Suggested Reading


lists are included online only for the
Index of Suspicion.

Case 1 Suggested Reading


Abbasi S, Pendergrass LB, Leggiadro
RJ. Influenza complicated by Moraxella
catarrhalis bacteremia. Pediatr Infect
Dis J. 1994;13:937938
Abuhammour WM, Abdel-Haq NM, Asmar
BI, Dajani AS. Moraxella catarrhalis
bacteremia: a 10-year experience. South
Med J. 1999;92:10711074
Coffin S. Bronchiolitis: in-patient focus.
Pediatr Clin North Am. 2005;52:
10471057

Case 3 Suggested Reading


OReardon JP, Peshek A, Allison KC. Night
eating syndrome: diagnosis, epidemiology and management. CNS Drugs.
2005;19:9971008

in brief

In Brief
Posttraumatic Stress Disorder
Nikeea Copeland-Linder, PhD, MPH
Johns Hopkins School of Medicine
and the Bloomberg School of
Public Health
Baltimore, Md.

Author Disclosure
Drs Copeland-Linder and Serwint
did not disclose any financial
relationships relevant to this In Brief.

Children and Trauma in America:


A Progress Report of the National
Child Traumatic Stress Network.
National Child Traumatic Stress
Network 2004. Available at www.
nctsnet.org./nctsn_assets/pdfs/
reports/NCTSNProgressReport
2004.pdf. Accessed November
2006
Diagnostic and Statistical Manual of
Mental Disorders. 4th ed, Text
Revision. American Psychiatric
Association. Washington, DC:
American Psychiatric Press; 2000:
463 468
Posttraumatic Stress Disorder in Children and Adolescents. Yule W. Int
Rev Psychiatry. 2001;13:194 200
Posttraumatic Stress Disorder: Clinical
Guidelines and Research Findings.
Scheeringa MS. In: Luby JL, ed.
Handbook of Preschool Mental
Health: Development, Disorders,
and Treatment. New York, NY:
Guildford Press; 2006:165185
Substance Abuse and Mental Health
Services Administration Model
Programs: Trauma-focused Cognitive Behavioral Therapy. Available
at http://www.modelprograms.
samhsa.gov/pdfs/model/TFCNT.pdf.
Accessed December 2006.

Violence and Risk of PTSD, Major Depression, Substance Abuse/


Dependence, and Comorbidity: Results from the National Survey of
Adolescents. Kilpatrick DG, Ruggiero
KJ, Acierno R, Saunders BE, Resnick
HS, Best CL. J Consult Clin Psychology. 2003;71:692700
Rape Trauma Syndrome. Burgess AW,
Holmstrom LL. Am J Psychiatry.
1974;131:981986
Treating the Trauma of Rape:
Cognitive-Behavioral Therapy for
PTSD. Foa EB, Rothbaum BO. New
York, NY: The Guilford Press; 1998

According to the National Child Traumatic Stress Network, 25% of youth


experience a traumatic event by the
time they are 16 years old, including
natural disasters (eg, hurricanes),
violence (eg, rape, physical assault,
witnessing violence), combat or warrelated events, and traumatic events
related to illness or injury. Youths reactions to catastrophic events or major
stressors can vary from temporary distress to severe forms of psychopathology. Factors that influence the development of severe stress reactions
include the proximity, intensity, and
duration of exposure to the stressor as
well as the childs age, prior mental
health, coping strategies, and quality of
family support.
A subset of youth exposed to a
traumatic event develop posttraumatic
stress disorder (PTSD), which is characterized by intense fear and the occurrence of three categories of symptoms
following exposure. The first category
involves re-experiencing the event. The
trauma may be re-experienced in a
number of ways, including intrusive
thoughts, memories, flashbacks, recurring dreams, or nightmares. In addition,

PTSD sufferers may experience intense


emotional distress and physiologic reactions when exposed to cues or situations that remind them of the experience. In young children, this category
of symptoms may be manifested by
repetitive play and drawings that involve themes related to the event as
well as by reenactments of aspects of
the experience. In addition, childrens
sense of fear and helplessness may be
manifested by agitated or disorganized
behavior.
The second category of symptoms
relates to emotional numbing and avoidance of stimuli associated with the
event. Sufferers of PTSD may feel disconnected or estranged from others,
lose interest in participating in activities, and have difficulty expressing feelings. This category of symptoms also
includes avoidance of thoughts, feelings, places, people, or activities that
are reminders of the trauma. In addition, it is common to have difficulty
remembering aspects of the event. Individuals may feel hopeless about the
future.
The third category of symptoms involves increased arousal, which may
encompass sleep disturbances, irritability, poor concentration, hyperalertness,
and easy startling. Criteria for diagnosing PTSD are the presence of at least
one symptom related to re-experiencing, three or more emotional numbing/
avoidance symptoms, and two symptoms of hyperarousal, with symptoms
being present for more than 1 month.
Additional symptoms associated
with childrens stress reactions include
an increase in tantrums and aggressive
behavior as well as new fears that are
thematically unrelated to the traumatic
event. Regressive behaviors, such as
Pediatrics in Review Vol.29 No.3 March 2008 103

in brief

bedwetting and clinginess, often increase among young children, although


an increase in physical complaints (headaches, stomachaches) also may occur. Children and adolescents may experience separation difficulties, needing
to remain in close proximity to their
parents. PTSD sufferers may feel guilty
about surviving when others died or
they may feel guilty about what they
had to do to survive.
PTSD can interfere with childrens
emotional, social, and cognitive development. Traumatized youth often have
difficulties managing their behavior,
tolerating and expressing feelings, and
trusting others. Such difficulties can
have a negative impact on their ability
to develop and sustain healthy relationships. Youth who have been raped or
otherwise physically and mentally assaulted may feel permanently damaged,
which may compromise their identity
development. Among adolescents,
symptoms of PTSD are associated with
high rates of depression, suicidal ideation, and substance abuse.
The lifetime prevalence of PTSD
among adults has been estimated to be
8%. Among adolescents, the estimated
prevalence is 3% to 6%, with higher
rates reported among girls. Rates of
PTSD vary considerably among individuals who have been exposed to trauma,
based on the type of event, with some
studies reporting rates as high as 90%
among witnesses of domestic violence
and rape survivors. Trauma symptoms
are so common among rape survivors
that prior to the introduction of PTSD in
the Diagnostic and Statistical Manual
of Mental Disorders, researchers described a constellation of trauma symptoms that they labeled rape trauma
syndrome. Although this term sometimes is used, most researchers and
practitioners agree that rape trauma
syndrome is characterized best as PTSD.
The appropriateness of applying
some PTSD diagnostic criteria to young
children continues to be debated. The
104 Pediatrics in Review Vol.29 No.3 March 2008

numbing/avoidance symptoms are very


difficult to detect among preverbal
children because endorsement of such
symptoms depends greatly on verbal
abilities. Thus, this category of symptoms may be underreported in young
children. Regardless of whether diagnostic criteria for PTSD are met, individuals who have been exposed to
trauma warrant concern because they
often are highly symptomatic and functionally impaired.
Youth who have been exposed to
trauma and are symptomatic should be
referred to a mental health professional. A multi-informant approach is
preferred and involves gathering information from the youth and the parents
about the nature and frequency of the
event and the severity, intensity, and
duration of the symptoms. Validated
symptom checklists and structured
clinical interviews can aid in assessment. Direct observations of play may
be incorporated into assessments of
young children. Information from all
modes of assessment should be examined, with special attention to the
youths age, developmental stage, prior
history of trauma exposure, family history of psychopathology, support network, and protective factors.
Cognitive behavioral therapy (CBT)
has received the most empiric support
as an effective treatment for PTSD. CBT
aims to modify maladaptive thoughts
and behaviors that have developed in
response to the traumatic event. Most
forms of CBT involve education about
the nature of the trauma and symptoms, gradual exposure to feared stimuli (ie, recount memories, confront situations), and cognitive restructuring to
address maladaptive thoughts. Traumafocused CBT (TF-CBT) is an evidencebased intervention designed to help
youth ages 3 to 18 years and their
parents who have been affected by
trauma. Youth components focus on
factors such as identification of feelings, cognitive reframing, gradual expo-

sure, and stress management. The intervention addresses issues such as low
self-esteem, difficulty trusting others,
and risky behavior. Combined parentchild sessions as well as separate parent
sessions are used. Parent sessions help
parents process their feelings and distress related to their childs traumatic
exposure and assist them with effective
parenting skills. TF-CBT consists of
12 to 16 sessions. It has been recognized as a model program by the Substance Abuse & Mental Health Services
Administration and the United States
Department of Health and Human Services and has been classified as Selective and Indicated by the Institute of
Medicine.
Comment: PTSD has been acknowledged more in the media since September 11, with the war in Iraq, and as acts
of terrorism have become more common. However, events that occur in
everyday life, such as motor vehicle
crashes, homicides, intimate partner
violence, or even invasive medical procedures also must be recognized and
considered. Although the diagnosis can
be more challenging in younger children because symptoms of distress may
not be as obvious, PTSD may have more
pronounced effects on younger children because of the vulnerability of a
rapidly developing central nervous system. Societal barriers to mental health
counseling exist, and some urban families may find the inciting events to be
commonplace in their environments,
but health-care practitioners must be
vigilant for a suggestive history and
symptoms of PTSD and should present
therapy in a socially acceptable manner to help break the cycle and facilitate healing. Recognition and treatment of PTSD is imperative for the
ultimate optimal function of children
and families.
Janet R. Serwint, MD
Consulting Editor

in brief

In Brief
Enteral Feeding
Taryn E. Weissman, MD
Barry K. Wershil, MD
The Childrens Hospital at Montefiore
Bronx, NY

Author Disclosure
Drs Weissman, Wershil, and Adam
did not disclose any financial
relationships relevant to this In Brief.

Enteral Nutrition. Abad-Sinden A, Sutphen I. In: Walker WA, Goulet O,


Kleinman RE, Sherman PM, Schneider
BL, Sanderson IR, eds. Pediatric Gastrointestinal Disease. 4th ed. Hamilton, Ontario, Canada: BC Decker;
2004:19811991
Short-Bowel Syndrome and Intestinal
Adaptation. Vanderhoof J. In: Walker
WA, Goulet O, Kleinman RE, Sherman
PM, Schneider BL, Sanderson IR, eds.
Pediatric Gastrointestinal Disease.
4th ed. Hamilton, Ontario, Canada:
BC Decker; 2004:750 753
Nutrition Support. Tiberio E, Surkhang
D, Brusco T. In: Piccini JP, Nilsson
KR, eds. The Osler Medical Handbook.
2nd ed. Baltimore, Md: Johns Hopkins University; 2006
Nutrition in Gastrointestinal Diseases.
DeLegge M. In: Feldman M, Friedman
LS, Sleisenger MH, eds. Feldman,
Sleisenger & Fordtrans Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders; 2006:365378

Nutrition support is essential for patients unable to meet daily caloric or


fluid requirements orally and can be
provided either by an enteral or parenteral route. Enteral support is preferred
because it is more physiologic, less
expensive, safer, and associated with
fewer adverse effects than parenteral
nutrition. Enteral feeding stimulates

gastrointestinal (GI) motility, minimizes


atrophy of the GI mucosa, decreases the
risk of bacterial overgrowth, and prevents translocation of bacteria or bacterial products into the circulation. Enteral nutrition avoids the need for
central venous access, thus diminishing
infections and eliminating the thrombotic and hepatic complications of parenteral nutrition.
Malnourished children unable to
maintain adequate nutrition can benefit
from enteral feeding. Conditions warranting enteral nutrition include feeding of
preterm infants, prolonged anorexia, severe protein-energy malnutrition, neurologic disease or impairment, liver failure,
cardiopulmonary disease, inflammatory
bowel disease, short gut syndrome, critical illness, and conditions of hypermetabolism (burn injury, cancer, human immunodeficiency virus infection/acquired
immune deficiency syndrome, trauma/
head trauma), and renal disease. Enteral
feeding also may be indicated for children
at high risk for aspiration.
Enteral feeding provides calories and
fluid to the GI tract by means other than
the oral route and requires some type of
tube delivery system, either temporary
for acute conditions or permanent for
chronic disabilities. Parenteral nutrition
ranges from supportive therapy, in which
the tube supplies a portion of the nutrients, to primary therapy, in which the
tube delivers all nutrients.
A temporary nasoenteric tube (nasogastric, nasoduodenal, or nasojejunal) is
recommended when the anticipated duration of nutrition support is limited (1
to 3 mo). For longer-term needs or for
patients who have chronic esophageal
abnormalities, tube enterostomy is the
preferred access route and includes percutaneous tubes (eg, endoscopic gastros-

tomy and jejunostomy) and surgically


placed feeding tubes (eg, open gastrostomy).
Gastric administration of feedings is
preferred because of the ease of management, ability to use a bolus feeding regimen, and potential benefits of gastric
acid as a bactericidal agent. However,
jejunal placement should be considered
for patients who have significant upper
tract disease or when there is a high risk
of aspiration, as in critically ill patients.
Enteral feedings can be delivered by
bolus or continuously. Bolus feedings
deliver formula as rapidly as an oral
feeding. This technique is simple, requires
minimal supplies, and may facilitate the
transition to home care. Most patients
tolerate bolus feeding, but vomiting,
painful abdominal distention, large gastric residuals, or diarrhea may be evidence
of intolerance. When intolerance of bolus
feedings is suspected, continuous infusion pump feedings are indicated. Continuous administration also is preferred
for patients fed through the small intestine or at high risk of aspirating and
appears to be particularly beneficial for
patients suffering impaired absorption
(chronic diarrhea, short bowel syndrome).
When selecting an enteral preparation, formula variables to consider include
digestibility and availability of the nutrients, nutritional adequacy, and osmolality. Patient variables include nutritional
status and requirements, digestive and
absorptive capacity, and disease state.
Special attention should be paid to osmolality and carbohydrate content when
patients exhibit signs of intolerance. The
osmolality of a formula is affected by the
concentration of amino acids, carbohydrates, and electrolytes. A formula that
has an osmolality greater than that of
normal body fluids can produce an osPediatrics in Review Vol.29 No.3 March 2008 105

in brief

motic effect in the stomach and small


intestine, drawing water into the gut and
diluting the formula. This excess water
can cause diarrhea, nausea, cramping,
and distention. Isotonic formulas are designed to alleviate these problems. The
osmolality of full-strength isotonic formulas is similar to that of normal body
fluids, approximately 300 mOsm/L.
Specialized formulas have been tailored to a wide variety of physiologic
circumstances while attempting to minimize specific types of intolerance. A
complete discussion of the many formulations is beyond the scope of this review, but examples are worth mentioning. Some formulas address the special
nutrient requirements of preterm infants
while diminishing osmolality and improving digestibility. Patients who have
severe GI disease or protein allergies
may require semi-elemental or elemental
formulas, which also are free of lactose
and fructose, to optimize tolerance. Other
specialized formulas include calorie- and
protein-dense preparations for fluidrestricted patients and fiber-enriched formulas for patients who have severe constipation.
Complications of enteral feeding include GI, mechanical, and metabolic
problems. GI complications include nausea, vomiting, constipation, and diarrhea.
In addition to formula intolerance, nausea and vomiting can be caused by too
rapid an infusion rate, delayed gastric
emptying, or GI tract obstruction. Constipation may result from dehydration,
obstruction, decreased bowel motility,
or a decrease in dietary fiber. Diarrhea
can be caused by diminished absorptive
capacity, formula hyperosmolality, bacterial overgrowth, or enterally administered medications, including antibiotics,
formulations containing sorbitol, and
magnesium-containing antacids.
Aspiration, tube obstruction (ie, dislodged or occluded feeding tube), and
mucosal damage (ie, skin breakdown) are
examples of mechanical complications.
Aspiration, potentially the most serious
106 Pediatrics in Review Vol.29 No.3 March 2008

complication of enteral feeding, is most


likely to occur in patients who have
neurologic injury, an incompetent lower
esophageal sphincter, large gastric residuals, significant gastroesophageal reflux,
impaired gastric emptying, improper tube
placement, or migration of the tube tip
into the upper stomach or esophagus.
Percutaneous feeding tubes also are associated with a risk of infection or erosion
at the insertion site. Excessive traction
may cause gastric wall necrosis and perforation of the stomach. More commonly,
patients may experience chronic drainage, erythema, or excessive build-up of
granulation tissue with intermittent
bleeding.
Metabolic complications are less
common with enteral than with parenteral feeding. Dehydration and fluid shifts
may occur with formulas of high osmolarity. Overly aggressive caloric administration to severely malnourished patients
can lead to refeeding syndrome, characterized by the development of hypokalemia, hypophosphatemia, and hypomagnesemia. This disorder can result in
cardiac arrhythmias, heart failure, respiratory failure, liver dysfunction, coma,
and death.
Often when enteral feeding is initiated, medications are administered by
tube, but this approach has limitations.
For example, phenytoin concentrations
can be affected because phenytoinformula complexes adhere to the wall
of the tube. Ciprofloxacin has been
shown to bind with tube feedings, reducing its absorption.
Special considerations should be
given to the patient who is transitioning from parenteral to enteral feedings
after bowel resection. The timing of this
transition depends on the activity of
the underlying disease and whether GI
function is sufficient for enteral nutrition. Immediately following resection,
parenteral nutrition is necessary, primarily because of bowel ileus. Initially,
large volumes of fluid and electrolytes
may be secreted and gastric fluid lost

from nasogastric suctioning. An ostomy


may produce additional significant
losses. When fluid and electrolyte losses
have resolved, continuous enteral infusion may be started, taking into account the extent and specific portions
of resected bowel. The initial rate
should be slow, and dilute formulas
commonly are used. However, if tolerated, the concentration can be increased rapidly to full-strength. Once
full-strength feedings are tolerated, the
volume of enteral feedings can be increased gradually until caloric goals are
reached, and the volume of parenteral
nutrition can be decreased simultaneously.
When the oral route is restricted,
enteral feeding often is the safest, most
cost effective, and most physiologic
approach to providing adequate nutrition to both acute and chronically ill
patients. Several approaches and multiple formulas are available, and each
choice should be individualized for the
specific needs and circumstances of the
patient.
Comment: Within the memory of
some of us, enteral tube feeding meant
placement and repeated replacement of
nasogastric tubes, with fear of misplacement into the airway instead of
the GI tract. Surgically placed gastrostomy tubes and inconspicuous buttons made G-tubes more cosmetically
acceptable. Now we have percutaneous
endoscopic gastrostomy, which avoids
laparotomy and usually can be accomplished with sedation rather than anesthesia. This procedure involves insufflation of the stomach to bring it into
apposition with the abdominal wall,
followed by the percutaneous passage
of a cannula and subsequently a wire
into the stomach, over which the gastrostomy tube is guided into place.
Ingenious!
Henry M. Adam, MD
Editor, In Brief

Article

policy statements

American Academy of Pediatrics


Policy Statements on Bioethics:
Summaries and Commentaries: Part 2
Mark R. Mercurio, MD,

Introduction

MA,* Marilyn A. Maxwell,

The American Academy of Pediatrics (AAP) has a strong and longstanding interest in the
field of bioethics and periodically publishes policy statements pertaining to specific ethical
questions relevant to pediatrics. The subjects addressed cover a wide range of topics, from
parental refusal of immunization to the care of critically ill children. These policies are
authored initially by the AAPs Committee on Bioethics and undergo extensive internal
review by other committees prior to publication.
This article is the second in a series of three intended to familiarize readers with many of
the AAP policies currently in place that address issues in bioethics. In this series, 16 policies
published by the AAP are summarized, each followed by a brief commentary. The
commentaries are intended to address, at least on a cursory level, some of the ethical
principles underlying the policies. Some briefly point out possible alternative viewpoints.
The policies referenced in this article represent the efforts of various committees and
committee members over the years. Each of the summaries presented here, as well as the
commentaries that follow, represents the work of an individual serving on the Executive
Committee of the Section on Bioethics, as indicated at the beginning of each summary.
Understandably, some of the wording of these summaries is taken directly from the
published policies. When quotations are used within a summary and not referenced, it can
be assumed that the quote is taken directly from the policy being summarized. For ease of
use, the references for each policy are provided with each individual summary and
commentary.

MD,* Brenda Jean Mears,


MD, MS,* Lainie Friedman
Ross, MD, PhD,* Tomas J.
Silber, MD, MAAS*

Author Disclosure
Drs Mercurio, Maxwell,
Mears, Friedman Ross,
and Silber did not
disclose any financial
relationships relevant
to this article.

Policies Reviewed
Part 1 of this series reviews:
1. Informed Consent, Parental Permission, and Assent in Pediatric Practice
2. Religious Objections to Medical Care
3. Responding to Parental Refusals of Immunization of Children
4. Sterilization of Minors With Developmental Disabilities
5. Human Embryo Research
Part 2 of this series reviews:
6. Guidelines on Forgoing Life-sustaining Medical Treatment
7. Forgoing Life-sustaining Medical Treatment in Abused Children
8. Do-Not-Resuscitate Orders for Pediatric Patients Who Require Anesthesia and
Surgery
9. Do-Not-Resuscitate Orders in Schools
10. Ethical Issues With Genetic Testing in Pediatrics
11. Ethics and Care of Critically Ill Infants and Children
Part 3 of this series reviews:
12. Female Genital Mutilation
13. Appropriate Boundaries in the Pediatrician-Family-Patient Relationship
14. Infants With Anencephaly as Organ Sources: Ethical Considerations
15. Palliative Care for Children
16. Institutional Ethics Committees

Guidelines on Forgoing Life-sustaining Medical Treatment


Committee on Bioethics. Guidelines on forgoing life-sustaining medical treatment.
Pediatrics. 1994;93:532536. Available at: http://aappolicy.aappublications.org/cgi/
*On behalf of the American Academy of Pediatrics Section on Bioethics.
Pediatrics in Review Vol.29 No.3 March 2008 e15

policy statements

bioethics

content/abstract/pediatrics;93/3/532.pdf. Reaffirmed
January 2004. Summary and comment by Mark R. Mercurio, MD, MA.

Summary of Policy Statement


Life-sustaining medical treatment (LSMT) refers to
any intervention that could prolong the life of the patient, including but not limited to mechanical ventilation, dialysis, and artificial nutrition and hydration.
Forgo in the context of these guidelines is meant to
encompass withholding (failing to initiate) or withdrawing a therapy. The guidelines acknowledge that there is
no ethical or legal distinction between not instituting a
treatment and withdrawing it. That is, it is equally acceptable to try a treatment for a while and then withdraw
it and to refrain from initiating that same treatment.
Either decision might be acceptable in certain circumstances and should be based primarily on the relative
benefits and burdens to the patient. Those benefits and
burdens usually are assessed in light of the values of the
patient and the patients family. It is acknowledged that
individuals may value benefits differently and that families should be given wide latitude:
Our social system generally grants patients and families wide discretion in making their own decisions about
health care and continuing, limiting, declining, or discontinuing treatment, whether life-sustaining or otherwise. Medical professionals should seek to override family wishes only when those views clearly conflict with the
interests of the child.
The guidelines emphasize the following points:
1. Physicians have an obligation to provide decisionmakers (generally the parents) with relevant information regarding risks and benefits of available
options and to provide a specific recommendation,
rather than simply offer a menu of choices.
2. Families should be given adequate time to consider the options because they may reconsider as
events gradually unfold or become clearer to them.
Decision-making should be treated as a process,
rather than an event.
3. A physician cannot be compelled by patients or
families to provide any treatment he or she feels is
unlikely to benefit the patient. Hospitals should
have policies addressing disagreements that persist
after all efforts at communication and collaborative
decision-making have failed.
4. Many pediatric patients, such as emancipated minors and some mature minors (often judged to
be 14 years old) generally should be viewed as
having decision-making capacity for their care. All
e16 Pediatrics in Review Vol.29 No.3 March 2008

children should have the opportunity to participate in decisions about LSMT to whatever extent
their abilities allow.
5. Decisions for infants and young children should be
based on the best interest standard, which dictates
a choice based on the relative benefits and burdens
of the proposed treatment to the child. The Committee describes potential benefits as follows:
The benefits may include prolongation of life
(understanding that the continuation of biologic
existence without consciousness may not be a benefit); improved quality of life after the LSMT has
been applied (including reduction of pain or disability); and increased physical pleasure, emotional enjoyment, and intellectual satisfaction. (1)

Comment
It is widely agreed that, as the policy states, there is no
significant ethical difference between withholding and
withdrawing an LSMT. Nevertheless, many clinicians
have observed a psychological difference in that it often is
far more difficult for parents to agree to remove LSMT,
such as mechanical ventilation or intravenous nutrition/
hydration, than it is to agree not to initiate it.
The foundation of this guideline is the patients best
interest standard, which states that decisions should be
based on an assessment of the benefits and burdens of the
proposed treatment. Of course, that assessment often is
subjective, with the outcome of the analysis ultimately
being a value judgment. Whether the benefits of ongoing LSMT to the patient outweigh the burdens frequently is a matter of opinion. The guidelines state that
the values (and, hence, the opinion) of the family generally should prevail, rather than the values of the physician, with one notable exception: Medical professionals
should seek to override family wishes only when those
views clearly conflict with the interests of the child. The
word clearly, perhaps, is the most consequential in the
guideline.
An ethical gray zone may have various definitions, but
in this case, it is useful to think of it in terms of the childs
best interest. The ethical gray zone might be thought of
as the area wherein the best interest of the child cannot
be discerned easily. In some cases, the parents wishes
regarding LSMT clearly (in the opinion of the clinicians)
are consistent with the childs best interests, and no
conflict exists. In other cases, parental wishes clearly
(again, in the opinion of the clinicians) are opposed to
the childs best interests, and the guidelines rightly advise
that steps be taken to override parental wishes. Within
the gray zone, the physician might be uncomfortable

policy statements

with the parental choice and, perhaps, even recommend


against it, but does not feel that his or her choice clearly
opposes the best interests of the child. In such a case, the
guidelines do not advocate overriding parental decisions.
Only when it seems to the clinicians that the parental
wishes are clearly opposed to the childs interests should
they be overridden. The location of the borders of the
gray zone, for any given physician, essentially is determined by the degree of certainty (or clarity) he or she
feels about the childs best interest. Within the ethical
gray zone, where the best interests of the child seem
unclear, the values and judgment of the parents generally
should hold sway.
In the case of LSMT, adherence to these guidelines
might result in a child being kept alive because of parental request, despite the misgivings or even the frank
opposite recommendation of the physician. The physician might be concerned about the pain of the ongoing
course or the potential burden of severe disability should
the child survive, but be uncertain whether the burdens
outweigh the benefit of possible survival. Ongoing patience, communication, and compassion provide the best
approach in such a case.
The patients best interest standard is strictly interpreted to require exclusive consideration of the benefits
and burdens to the patient. Some have suggested that this
is too narrow a view and that it may be legitimate to
consider the interests of family members as well. (2) How
much weight should be accorded to the interests of the
family is a central question in decisions regarding LSMT.
Although the AAP guideline generally seems to endorse
the patients best interest standard, it also opens the door
to other considerations, at one point stating that physicians and families should include the interests of others,
such as family members and loved ones, in decisions
about LSMT.

References
1. Meisel A. The Right to Die. New York, NY: Wiley Law Publishers; 1989:295

2. Hardwig A. Is There a Duty to Die? New York, NY: Routledge


Publishers; 2000

Forgoing Life-sustaining Medical Treatment


in Abused Children
Committee of Child Abuse and Neglect and Committee
on Bioethics. Forgoing life-sustaining medical treatment
in abused children. Pediatrics. 2000;106:11511153.
Available at: http://aappolicy.aappublications.org/cgi/
content/full/pediatrics%3b106/5/1151. Reaffirmed Oc-

bioethics

tober 2006. Summary and Comment by Tomas J. Silber,


MD, MAAS.

Summary of Policy Statement


In this joint statement, it is affirmed that the decision to
forgo LSMT for a critically ill child injured as the result of
abuse should be made using the same criteria as those
used for any critically ill child. In addition, the parent or
guardian of an abused child may be considered to have a
conflict of interest when a decision to forgo LSMT risks
changing the legal charge faced by a parent, guardian,
relative, or acquaintance from assault to manslaughter or
homicide. Therefore, the statement recommends that if a
physician suspects that a parent or guardian is not acting
in a childs best interest, additional review and consultation should be sought in hopes of resolving the conflict.
Such consultation includes requesting the appointment
of a guardian ad litem, who represents the childs interests regarding LSMT, in all cases in which a parent or
guardian may have a conflict of interest. The statement
also clearly addresses the issue of the brain-dead child,
suggesting that although brain death is a clinical determination based on established criteria, in cases of abuse,
given the likelihood of criminal prosecution, it may be
prudent to supplement the clinical determination of
brain death with an ancillary test, such as a cerebral flow
study. The document ends with five recommendations:
1. Pediatricians, pediatric subspecialists, and pediatric
surgeons should be aware of the legal and ethical
issues in caring for children who have been seriously
injured as a result of abuse.
2. Regardless of the cause, nature, and extent of a childs
injuries, the parent(s) or guardian(s) should be involved, as appropriate, in all aspects of the childs care
and treated with respect and due consideration for
their privacy.
3. Decisions to forgo LSMT for a critically ill child
whose injuries are the result of abuse should be made
using the same guidelines as those used for any critically ill child.
4. A guardian ad litem for medical decision making
should be appointed in all cases of child abuse requiring LSMT in which a parent, guardian, or prosecutor
of the alleged abuser may have a conflict of interest.
5. The medical examiners office should be involved
early and before forgoing LSMT. Local procedures
for collecting evidence and performing postmortem
examinations should be developed to allow for organ
and tissue donation.
Pediatrics in Review Vol.29 No.3 March 2008 e17

policy statements

bioethics

Comment
This is among the most tragic circumstances that a pediatrician may encounter in his or her practice. Because it is
only human to be enraged at the suffering of the most
vulnerable at the hands of those who abuse their power,
betray, and hurt them, it initially is counterintuitive to
accept the advice that decisions to forgo LSMT be based
on complete and compassionate communication with
the family, even if one or both parents are suspected of
causing the injury. However, additional reflection on
who we are as pediatricians and what our role and obligations are clarifies that we have been granted by society
the special mission of providing support for the parents
of critically ill children, which includes identifying for
them and offering them the possibility of meeting with
bereavement counselors, chaplains, or any other professional or group they may need in the midst of this
emotional and spiritual crisis.
The document also is on solid footing when it identifies the best interest of child as the primary focus when
considering whether to forgo LSMT and appropriately
advises that when the pediatrician suspects that the parent is not acting in the best interest of the child, an ethics
consult should follow in hopes of resolving the conflict. Indeed, an ethics consult can be very useful in
assuring the hospital administration that reasonable steps
were taken before proceeding to a court hearing. The
responsibility of the physician does not end when a court
appoints a guardian ad litem. Pediatricians need to function as child advocates, continuing to provide input so
the guardian ad litem makes an informed decision. Pediatricians need to be aware that prosecutors may not
support a decision to forgo LSMT out of concern that
the case against the alleged abuser may be weakened.
At the end, the thoughtful and systematic approach of
the statement comes around to helping us understand
that decision-making in the treatment of abused children
is no different from the painful dealings with other
injured or moribund children only more so.

Do-Not-Resuscitate Orders for Pediatric


Patients Who Require Anesthesia and
Surgery
Fallet ME, Deshpande JK, and the Section on Surgery,
Section on Anesthesia and Pain Medicine, Committee
on Bioethics. Do-not-resuscitate orders for pediatric
patients who require anesthesia and surgery. Pediatrics.
2004;114:1686 1692. Available at: http://aappolicy.
aappublications.org/cgi/content/full/pediatrics;114/6/
1686. Summary and Comment by Brenda Jean Mears,
MD, MS.
e18 Pediatrics in Review Vol.29 No.3 March 2008

Summary of Policy Statement


This clinical report from 2004 discusses preexisting donot-resuscitate (DNR) orders for the pediatric patient
who undergoes anesthesia and surgery.
DNR orders may be written for a child at the request
of the parent when the burdens of resuscitation exceed
any expected benefit. Such orders should not preclude
the provision of appropriate surgical interventions. Surgical procedures and anesthesia may increase the risk of
hemodynamic instability, but arrests in this setting have a
higher percentage of successful resuscitation. Honoring
the DNR order may allow a potentially reversible death
to occur, but a requirement that the order be suspended
in the operating room may not be in the best interest of
the patient.
Required reconsideration specifies that DNR orders be reviewed during the informed consent process.
The various components of the surgery are reviewed and
the implications of the DNR considered. An older child
is included in the discussions when possible and assent
obtained. A family may choose to suspend or modify the
DNR order intraoperatively. Procedure-directed planning documents decisions on the use of various interventions but lacks flexibility. Goal-directed approaches
focus on patient goals, values, and preferences rather
than on individual resuscitation procedures. The timing
of resuming previous DNR orders should be planned.
Documentation of all discussions and communication to
all involved in providing care are needed. If the physician
cannot comply with the decisions made by the family,
transfer of the patient or involvement of other consultants may be necessary. This consideration of options
allows care to be individualized to the needs of the child.

Comment
Parents, acting as surrogates for their children, may
refuse treatment without implying a choice to hasten the
childs death. (1) Several points bear emphasis. When a
DNR order is in place, there still should be the freedom
to request beneficial interventions without fear of prolonged resuscitation or unwanted intensive care. (2)
Despite the possible risks of an iatrogenic arrest during
anesthesia and surgery, it is not always in the best interest
of a child to be resuscitated, and a family may refuse
resuscitation attempts. Policies requiring the suspension
of a DNR order force families to balance the benefits of
surgery against the risks of unwanted resuscitation. (3)
Procedures commonly used in the operating room may
be considered resuscitation in other situations (3). Reconsideration of the DNR order allows a review of therapeutic interventions that might be used and possible

policy statements

responses to expected and unexpected events as well as


the risks and benefits of the surgery and anesthesia.
Suspension or modification of the DNR order can be
planned and documented for use during the procedure.
(2)(3) If a conflict cannot be settled to the satisfaction of
the participants, the preference of the family should
prevail.

References
1. American Academy of Pediatrics. Committee on Bioethics.
Guidelines on forgoing life-sustaining medical treatment. Pediatrics. 1994;93:532536. Available at: http://pediatrics.
aappublications.org/cgi/reprint/93/3/532
2. Truog RD, Waisel DB, Burns JP. Do-not-resuscitate orders in
the surgical setting. Lancet. 2005;365:733735
3. American Society of Anesthesiologists, Committee on Ethics.
Ethical Guidelines for the Anesthesia Care of Patients with Do Not
Resuscitate Orders or Other Directives that Limit Treatment. Available at: www.asahq.org/publicationsAndServices/standards/09.html

Do-Not-Resuscitate Orders in Schools


Committee on School Health and Committee on Bioethics. Do-not-resuscitate orders in schools. Pediatrics.
2000;105:878 879. Available at: http://aappolicy.
aappublications.org/cgi/content/full/pediatrics;105/4/
878. Reaffirmed June 2003. Summary and Comment by
Marilyn A. Maxwell, MD.

Summary of Policy Statement


This statement addresses the challenging situation of
DNR orders in schools. With the advances of medical
knowledge and technology, many children who previously would have died are surviving serious conditions.
Challenges involved in the care of these children who
have continuing life-threatening health problems can be
encountered as they reach school age. Public Law 94142, the Education for All Handicapped Children Act,
provides that children who have disabilities or handicaps
have access to education in the least restrictive environment appropriate for their needs. The risk of dying
while attending school is a real possibility for some
children who have chronic and terminal conditions. Parents who have chosen no cardiopulmonary resuscitation
for their child also may want this decision carried out
while the child is at school. In this statement, the committees stress, a DNR order is not synonymous with
abandonment of all medical treatment and does not, of
itself, rescind the obligations of the health care team to
provide quality care, such as suction, oxygen, and pain
medication.
School officials may have concerns about DNR orders
in schools. Such orders possibly could be misinterpreted

bioethics

by medically untrained staff, which could result in harm


to a child. There also is concern that school personnel
may not respond to easily reversible situations, such as a
mucous plug in a child who has a tracheostomy. Finally,
there are concerns that situations not anticipated by a
DNR order, such as a child choking on food or being
injured, could cause confusion among personnel. All of
these issues raise liability concerns. In addition, there is a
consideration of the effect on other students exposed to
a death at school.
The AAP believes that pediatricians should be involved with these decisions and work with families and
school personnel to provide guidance and advice in this
area. The goal of working together would be to ensure
that a childs participation in the education process continue for as long as reasonably possible.

Comment
Children who have life-threatening problems are at risk
of dying while in school. Parents, after appropriate consultation, may decide on DNR orders and may wish for
these orders to be followed at school. From an ethical
standpoint, parents could argue that they, along with
their child, have a right to have the DNR order followed
at school. On the other hand, school officials may be
worried about a variety of other issues, some of which
may involve liability for the school. In addition, there is a
concern about the effect of a death in school on other
students, as well as parents of other children not wanting
their children exposed to death at school. This last point
also could be presented as a rights-based argument.
Avoiding adversarial struggles between school personnel and parents is important. The AAP recommendations in this area are both helpful and practical in attaining common ground for the school and the family. The
AAP recommends that pediatricians work with parents of
at-risk children as well as with school and nursing personnel, teachers, administrators, and emergency medical
service personnel. Concerted efforts to accommodate
all points of view will help avoid confrontation and
possible litigation. More importantly, finding common
ground is in the best interest of children at increased risk
of dying in school, should the parents desire a DNR
order be followed in the school.

Ethical Issues With Genetic Testing in


Pediatrics
Committee on Bioethics. Ethical issues with genetic
testing in pediatrics. Pediatrics. 2001;107:14511455.
Available at: http://aappolicy.aappublications.org/cgi/
content/full/pediatrics;107/6/1451. Reaffirmed JanuPediatrics in Review Vol.29 No.3 March 2008 e19

policy statements

bioethics

ary 2005. Summary and Comment by Lainie Friedman


Ross, MD, PhD.

unclear, and the decision to test takes away the childs


right not to know and to make such decisions for him- or
herself.

Summary of Policy Statement


In this statement, the Committee on Bioethics seeks to
address three areas of genetic testing and screening in
children that raise serious ethical issues: 1) newborn
screening, 2) carrier screening, and 3) predictive testing
for late-onset disorders.
The two primary ethical issues in newborn screening
that the statement addresses are: 1) consideration of how
to introduce new tests into newborn screening ethically
and 2) examination of the current policy of mandatory
screening without parental permission. The statement is
aligned with the traditional position that as a public
health program, newborn screening must provide a clear
benefit to the child, a position that currently is being
challenged by some patient advocates. (1) The Committee on Bioethics is against mandatory screening because
it fails to acknowledge the role of parents in making
decisions for their children. The Committee recommends replacing current mandatory screening practices
with a program that requires mandatory offering,
which is consistent with the position of the AAP Committee on Genetics (although some of those committee
members wanted to leave the program as mandatory),
(2) the AAP Newborn Screening Task Force, (3) and
many other ethical analyses of genetic screening programs. (4)(5)
Carrier detection may occur as an incidental finding
(eg, during newborn screening for sickle cell disease or
cystic fibrosis) or it may be part of a program for providing reproductive information to adolescents and young
adults. The latter rarely has been attempted in the United
States but has been undertaken internationally. (6)(7)
The Committee on Bioethics takes the position that newborns should not be screened for the purpose of determining carrier status because the primary use of such information is reproductive and is irrelevant to the child. However,
when carrier information is discovered incidentally, the
Committee states that the information should be conveyed
to parents. They also argue against routine carrier screening
in adolescents on the grounds that adolescents should decide whether they want this reproductive information as
adults. Again, these positions are similar to other United
States statements. (4)(5)
Finally, the statement addresses the issue of predictive
testing for late-onset disorders, supporting the delay of
testing until the child has the capacity to make the
choice. This position is based on the premise that
whether the psychological benefits outweigh the risks is
e20 Pediatrics in Review Vol.29 No.3 March 2008

Comment
The position outlined in this statement is mainstream
and consistent with many earlier ethics documents about
genetic testing and genetic screening. (4)(5) The great
expansion of genetic technologies in the past 5 years has
not created much of a change in ethical analysis, although it has influenced genetic practice. First, newborn
screening has expanded rapidly without appropriate systems in place to ensure that the policy decisions can be
re-evaluated. (8)(9) Second, although many argue
against predictive testing and screening of children and
adolescents for carrier detection and late-onset conditions, empiric data show that such testing is occurring.
(10)(11)

References
1. Bailey DB Jr, Skinner D, Warren SF. Newborn screening for
developmental disabilities: reframing presumptive benefit. Am J
Public Health. 2005;95:1889 1893
2. American Academy of Pediatrics, Committee on Genetics. Newborn screening fact sheets. Pediatrics. 1996;98:473501
3. American Academy of Pediatrics, Newborn Screening Task
Force. Serving the family from birth to medical home: a report from
the Newborn Screening Task Force convened in Washington DC,
May 10 11, 1999. Pediatrics. 2000;106(suppl):386 427
4. National Research Council. Genetic Screening: Programs, Principles, and Research. Washington DC: National Academy of Sciences; 1975
5. Institute of Medicine. Assessing Genetic Risks. Implications for
Health and Social Policy. Washington DC: National Academy Press;
1994
6. Scriver CR, Clow CL. Compliance factors in Tay-Sachs screening. Progr Clin Biol Res. 1977;18:379 380
7. Barlow-Stewart K, Burnett L, Proos A, et al. A genetic screening
programme for Tay-Sachs disease and cystic fibrosis for Australian
Jewish high school students. J Med Genet. 2003;40:e45
8. Botkin JR, Clayton EW, Fost NC, et al. Newborn screening technology: proceed with caution. Pediatrics. 2006;117:17931799
9. Botkin JR. Research for newborn screening: developing a national framework. Pediatrics. 2005;116:862 871
10. Duncan RE, Savulescu J, Gillam L, Williamson R, Delatycki
MB. An international survey of predictive genetic testing in children
for adult onset conditions. Genet Med. 2005;7:390 396
11. Cadet E, Capron D, Gallet M, et al. Reverse cascade screening
of newborns for hereditary haemochromatosis: a model for other
late onset diseases? J Med Genet. 2005;42:390 395

Ethics and Care of Critically Ill Infants and


Children
Committee on Bioethics. Ethics and care of critically
ill infants and children. Pediatrics. 1996;98:149 152.

policy statements

Available at: http://aappolicy.aappublications.org/cgi/


content/abstract/pediatrics;98/1/149. Reaffirmed June
2003. Summary and Comment by Brenda Jean Mears,
MD, MS.

Summary of Policy Statement


Current LSMT can prolong the lives of patients who, in
the past, might not have been treated vigorously. The
value of LSMT may be uncertain, which elicits the moral
question: When is it appropriate to apply LSMT to a
critically ill infant or child? Although a critically ill infant
or child ultimately may have multiple physical or mental
limitations, it is impossible to predict the outcome accurately for any individual.
This policy references the 1984 Child Abuse Amendments. These Baby Doe rules resulted from a perception in the early 1980s that beneficial treatment was
being denied to infants. Misconceptions about these
requirements may have hampered attention to concerns
about possible overuse of LSMT. The actual language
may permit more discretion than frequently realized.
Exceptions to mandated treatment include permanent
unconsciousness, futile treatment, and virtually futile therapy that imposes excessive burdens on the infant. A trial of therapy may be necessary to clarify a
clinical situation and the pertinent ethical values in the
particular situation.
The AAP supports parental involvement in decisions
from the earliest possible moment for all children. Parents are presumed to accept responsibility for providing
care for their children and should be informed of available options. Informed decisions should be made together by the family and medical personnel, considering
both burdens and benefits. Parents may perceive and
value these benefits and burdens differently than medical
personnel. Only rarely should the involvement of child
protective services be necessary.

bioethics

LSMT should be provided in conformity with current


medical, ethical, and legal norms. (1) Treatment should
be individualized and re-evaluated frequently. (2)(3)(4)
Withdrawal of LSMT in some settings may be appropriate; one AMA policy supports withdrawal when the risks
of pain and suffering are greater than any potential for
joy. (3)
This policy has created controversy because it may
relate to the 1984 Child Abuse Amendments (the Baby
Doe rules) and, more recently, to the 2002 Born Alive
Act. (6)(7)(8)(9)(10)(11)(12)(13) The Born Alive
Act defines human being to include any infant born
alive at any stage of development. (13) Although the
AAP Neonatal Resuscitation Program Steering Committee said that no changes in approach are necessary, there
has been concern that this definition may jeopardize the
normative ethical practice of offering discretionary palliative care. (8)(11)(12)(14)
This policy has been said to give support to the Baby
Doe rules, be inconsistent with other guidelines, and be
responsible for altered standards of care by limiting the
ability to select individualized treatment plans and act in
the best interests of infants (9) and by disregarding
parental concerns about pain and suffering. (15) Predictions of outcome are imperfect, but survival alone is not
necessarily in a childs best interest. (6)(16) The definition of child abuse does include failure to provide medically necessary treatment, (15)(17) and court decisions
concerning required treatment have varied. A Texas case
upheld the right of parents to make decisions to withhold
treatment except in an emergency, (6)(9)(18)(19) but a
Wisconsin case seemed to require maximal treatment
unless there is irreversible coma or dying. (6)(8)
In brief, each case requires careful consideration by
the family members and medical and support personnel
that possibly involves multiple group meetings prior to
the making of acceptable, appropriate decisions.

Comment
This policy has much in common with other AAP and
American Medical Association (AMA) policies and statements. The primary consideration should be the best
interests of the child. (1)(2)(3) Physicians should justify
treatments to families, patients, and themselves based on
possible benefits and burdens. (1)(2)(3)(4)(5) Parents
need to be fully informed as early as possible (1)(2)(3)(4)
because different individuals judge the value of benefits
and risks differently. (1) Parents are granted wide discretion in decision making, and physicians should use judicial intervention to override parents only when their
decisions clearly conflict with the childs interests. (1)

References
1. American Academy of Pediatrics. Committee on Bioethics.
Guidelines on forgoing life-sustaining medical treatment. Pediatrics. 1994;93:532536
2. American Academy of Pediatrics. Committee on the Fetus and
Newborn. The initiation or withdrawal of treatment for high-risk
newborns. Pediatrics. 1995;96:362363
3. American Medical Association. E 2.215 Treatment Decisions for
Seriously Ill Newborns. Available at: http://www.ama-assn.org/
apps/pf_new/pf_online?f_nresultLink&docpolicyfiles/HnE/
E-2.215.HTM&s_te2.215&catgAMA/HnE&catgAMA/
BnGnC&catgAMA/DIR&&nth1&&st_p0&nth2&.
7/
10/06
Pediatrics in Review Vol.29 No.3 March 2008 e21

policy statements

bioethics

4. American Academy of Pediatrics. Clinical report. Perinatal care


at the threshold of viability. Pediatrics. 2002;110:1024 1027
5. American Medical Association. H-480.992 Moral and Ethical
Issues in the Use of Health Care Technologies. Available at: http://
www.ama-assn.org/apps/pf_new/pf_online?f_nresultLink&doc
policyfiles/HnE/H-480.992.HTM&s_twithdrawal&catgAMA/
HnE&catgAMA/BnGnC&catgAMA/DIR&&nth1&&st_p
0&nth8&.7/20/06
6. Clark FI. Treatment decisions for critically ill infants: the abrogation of the best interests standard. Juv Family Court J. 2004;
55(3):1122
7. Frader JE. Baby Doe rules: in reply. Pediatrics. 2005;116:
16011602
8. Hurst I. Baby Doe rules. Pediatrics. 2005;116:1600 1601
9. Kopelman LM. Are the 21-year-old Baby Doe rules misunderstood or mistaken? Pediatrics. 2005;115:797 802
10. Kopelman LM, Irons TG, Kopelman AE. Neonatologists
judge the Baby Doe regulations. N Engl J Med. 1988;318:
677 683
11. McCullough LB. Neonatal ethics at the limits of viability.
Pediatrics. 2005;116:1019 1021
12. Sayeed SA. Baby Doe redux? The Department of Health and
Human Services and the Born-Alive Infants Protection Act of
2002: a cautionary note on normative neonatal practice. Pediatrics.
2005;116:e576 e585
13. Born-Alive Infants Protection Act of 2001, HR. 2175, 107th
Cong, 2nd Sess (2001)
14. Boyle D, Carlo WA, Goldsmith J, et al. Born-Alive Infants
Protection Act of 2001, Public Law No. 107207. Pediatrics.
2003;111:680 681
15. Clark F. Baby Doe rules: in reply. Pediatrics. 2005;116:1601
16. Boyle RJ, Salter R, Arnander MW. Ethics of refusing parental
requests to withhold or withdraw treatment from their premature
baby. J Med Ethics. 2004;30:402 405
17. Fost N. Decisions regarding treatment of seriously ill newborns. JAMA. 1999;281:20412043
18. Hurst I. The legal landscape at the threshold of viability for
extremely premature infants: a nursing perspective, part I. J Perinat
Neonatal Nurs. 2005;19:155166
19. Robertson JA. Extreme prematurity and parental rights after
Baby Doe. Hastings Center Report. 2004;34(4):3239

Conclusion
The AAP periodically publishes policy statements and
guidelines addressing difficult ethical issues that physi-

e22 Pediatrics in Review Vol.29 No.3 March 2008

cians caring for children continue to face. This review


is intended to provide readers with an overview of some
of those guidelines and possibly stimulate additional
thought and dialogue within the profession. It is presented by the AAP Section on Bioethics as part of its
mission to foster education in this area among pediatricians. As the commentaries suggest, there may not be
unanimity about the positions taken, and that is important to recognize. The full text of each policy, as well as
other relevant references and information, can be found
on the web site for the Section on Bioethics at http://
www.aap.org/sections/bioethics.
The Section on Bioethics serves primarily an educational role within the AAP and beyond. They organize
educational forums in bioethics at the annual AAP National Conference and Exhibition, publish a newsletter
that includes original articles in the area of bioethics, and
carry out other educational efforts intended primarily for
pediatricians. In addition, they provide input to the
Board of Directors and other committees regarding proposed policy statements and guidelines. Membership in
the Section is open to all AAP Fellows who have an
interest in bioethics. Affiliate membership also is available
to physicians and other health professionals not eligible
for AAP membership.
This is the second in a series of three articles. Part 1
appeared in the January 2008 online-only issue of
Pediatrics in Review, and Part 3 will appear in the May
issue.
ACKNOWLEDGMENTS. The authors would like to
thank Mary B. Adam, MD, MA, and Edwin N.
Forman, MD, their colleagues on the AAP Section on
Bioethics Executive Committee, for guidance and
helpful comments in the preparation of this manuscript.

Das könnte Ihnen auch gefallen