SICK GROWING CHILD

Alterations in
Gastrointestinal
Function
Reported by Kintanar, Frances Lorelle - Montana, Flos Carmeli - Subires, Julie - Tan, Laurence
STRUCTURAL DEFECTS
CLEFT LIP (CHEILOSCHISIS)
CLEFT PALATE (PALATOSCHISIS)
CLEFT LIP, AN EXAMPLE OF A
MALFORMATION, OCCURS AT
APPROXIMATELY 5 WEEKS OF GESTATION
WHEN THE DEVELOPING EMBRYO
NATURALLY HAS TWO CLEFTS IN THE
AREA. NORMALLY BETWEEN 5 AND 7
WEEKS, CELLS RAPIDLY DIVIDE AND
MIGRATE TO FILL IN THOSE CLEFTS. IF
THERE IS AN ABNORMALITY IN THIS
DEVELOPMENTAL PROCESS, THE EMBRYO
IS LEFT WITH EITHER A UNILATERAL OR
BILATERAL CLEFT LIP THAT MAY ALSO
INVOLVE THE PALATE.
 MULTIFACTORIAL DISORDER

ETIOLOGY Gender: Isolated CL with or without CP is more common in males, and CP

alone is more common in females.

CL appears more often in Native Americans and Asians and less frequently in

African- Americans (Moller and Glaze, 2009).

Cleft lip occurs as a familial tendency or most likely occurs from the

transmission of multiple genes. The gene(s) responsible for clefting in

individuals without syndromes is not yet known. However, some genetic

syndromes are known to be associated with clefting in approximately 15% of

individuals with clefts (such as 22q11.2 deletion).

Formation may be aided by teratogenic factor present during weeks 5 to 8 in

intrauterine life, such as viral infection or possibly a disorder to avoid any local

bruising of tissue.

Alcohol use

Cigarette smoking

Prescription drugs, including anticonvulsants, steroids, and retinoids, are

associated with higher rates of oral clefting. Conversely, folic acid

supplements may protect against clefting.

Signs and Symptoms

POOR FEEDING NATAL TEETH

SKILLS

CHRONIC EAR A SPLIT IN

INFECTIONS THE LIP AND
ROOF OF THE
MOUTH
 PATHOPHYSIOLOGY
DEVELOPMENT OF THE PRIMARY AND SECONDARY

PALATES TAKES PLACE AT DIFFERENT TIMES AND

INVOLVES DIFFERENT DEVELOPMENTAL PROCESSES,

WHICH IS WHY CLEFTS CAN VARY SIGNIFICANTLY.

FAILURE OF THE MAXILLARY PROCESSES TO FUSE

WITH THE NASAL ELEVATIONS ON THE FRONTAL

PROMINENCE TO FORM THE PRIMARY PALATE,

WHICH NORMALLY OCCURS DURING THE SIXTH

WEEK OF GESTATION.

NORMAL DEVELOPMENT IS INTERRUPTED BEFORE

7TH WEEK OF GESTATION

CLEFT LIP
 PATHOPHYSIOLOGY
DEVELOPMENT OF THE PRIMARY AND SECONDARY

PALATES TAKES PLACE AT DIFFERENT TIMES AND

INVOLVES DIFFERENT DEVELOPMENTAL PROCESSES,

WHICH IS WHY CLEFTS CAN VARY SIGNIFICANTLY.

FAILED FUSION OF THE SECONDARY PALATE (HARD

AND SOFT PALATES) BETWEEN THE SEVENTH AND

TWELFTH WEEKS OF GESTATION

CLEFT PALATE: THE DEGREE MAY VARY FROM A

BIFID UVULA (THE MILDEST FORM OF A CLEFT

PALATE WITH ESSENTIALLY NO FUNCTIONAL IMPACT

ON SPEECH OR FEEDING), TO A COMPLETE CLEFT

PALATE THAT EXTENDS FROM THE SOFT PALATE

INTO THE HARD PALATE.

Nursing Diagnosis

IMBALANCED

IMPAIRED NUTRITION,
RISK FOR
SWALLOWING LESS THAN
IMPAIRED
RELATED TO BODY
PARENT/INFA
IMPAIRED REQUIREMENT
NT/CHILD
SUCKING AND S RELATED TO
ATTACHMENT
PALATAL PALATAL

CLEFT DEFECT
Nursing Diagnosis:
Post Operative Care
RISK FOR

TRAUMA TO

SURGICAL IMBALANCED
SITE RELATED NUTRITION,
ACUTE PAIN
TO SURGICAL LESS THAN
RELATED TO
PROCEDURE, BODY
SURGICAL
DEVELOPMENT
REQUIREMENT
REPAIR OF
AL AGE OF
S RELATED TO
CHILD, SKIN
CL/ CP
PALATAL
AND MUCOUS
DEFECT
MEMBRANE

FRAGILITY
 ALTHOUGH THE INITIAL CLEFT LIP

AND CLEFT PALATE ARE REPAIRED

WITHIN THE FIRST YEAR OF LIFE,

TREATMENT TO REPAIR THE

DEFORMITY MAY CONTINUE INTO

EARLY ADULTHOOD. MANAGEMENT

OF BOTH DEFECTS IS DIRECTED

TOWARD SURGICAL CLOSURE OF THE

CLEFT, PREVENTION OF

COMPLICATIONS, AND FACILITATION

OF NORMAL GROWTH AND

DEVELOPMENT OF THE CHILD.

IT IS TREATED BY A CLEFT/

CRANIOFACIAL MULTIDISCIPLINARY

TEAM, INCLUDING PLASTIC SURGERY,

ORTHODONTICS, SPEECH-LANGUAGE

MANAGEMENT PATHOLOGY,

AUDIOLOGY,
PEDIATRICS,

SOCIAL WORK,
NURSING,

AND

PSYCHOLOGY.
MANAGEMENT
When caring for a child with a cleft lip, remind the

parent that the defect is not her fault and that,

“Cleft lip is an example of a disruption and occurs

early in the pregnancy, often before you even know

you are pregnant.”

CLEFT LIP
SURGICAL
Surgical correction occurs when the baby is between 2 and

3 months old.

NASAL ALVEOLAR MOLDING (NAM)

taping of the upper lip in order to bring the segments of the lip and

alveolus into better alignment, resulting in less tension on the lip repair

following surgery and an improved aesthetic outcome. (Performed by an

orthodontist)
CHEILOPLASTY
SURGICAL LIP RESTORATION
Medical and
Pharmacologic
Administer analgesics for pain after surgery.

Apply a thin layer of antibiotic ointment to

the suture line for three days, followed by

application of petroleum jelly for several

weeks.
NURSING:
Avoid the prone position to prevent suture damage.

Elbow restraints may be applied immediately after

POST-
surgery to prevent the infant from rubbing the suture

line, although many surgeons no longer support this

OPERATIVE
practice.

Teach parents how to properly clean the sutures by

CARE using water or diluted hydrogen peroxide.

Avoid the use of suction or objects in the mouth such

as tongue depressors, thermometers, spoons, or

straws.

Gentle aspiration of mouth and nasopharyngeal

secretions may be necessary to prevent aspiration and

respiratory complications.

Watch out for excessive swallowing as it may be a sign

of bleeding and swallowing blood.

NURSING CARE
FEEDING

Begin by positioning the infant upright with the head supported

by the caregiver’s hand or cradled in the arm. This position

helps gravity to direct the flow of liquid so that it is swallowed

rather than entering into the nasal cavity.

NUK (orthodontic) nipple or a Playtex nurser are bottle-fed

systems with a wider base which allows infants with CL to feed

more successfully

Cheek support (i.e., squeezing the cheeks together to

decrease the width of the cleft) may also help the infant

achieve an adequate anterior lip seal during feeding.

CLEFT PALATE
SURGICAL
PALATOPLASTY IS A SURGICAL
PROCEDURE USED TO CORRECT OR
RECONSTRUCT THE PALATE IN A PERSON
WITH A CLEFT PALATE.
Cleft Palate repair is typically completed between 6 and 12

months. Delaying palate repair after first words emerge may

result in significant speech disorders.

Encourage cup feeding before surgery (alternative methods

may be syringe feeding)

 VEAU-WARDILL- KILNER
V-Y PUSHBACK

FURLOW DOUBLE
OPPOSING Z-PLASTY
 Observe child closely for signs of airway obstruction, hemorrhage

and laryngeal spasm

NURSING: A tongue stitch may be used to prevent the tongue from

obstructing the airway

POST- It is taped to the cheek and may be removed per surgeon’s

preference

OPERATIVE The child with a CP repair may be placed on clear liquids for 24

hours followed by a liquid diet for 2 weeks.

CARE A soft diet may be encouraged for a full 6 weeks following palate

repair.

Acceptable feeding devices include open cup for liquids, but rigid

utensils such as spoons, straws, or hard-tipped sippy cups should

be avoided to avoid accidental injury to the repair

Elbow restraints can keep the hands away from the mouth, and the

parents should maintain this precaution at home until the palate is

healed.

Teach parent to careful position the child in the car seat and in

sleeping on a supine position

 NURSING CARE
FEEDING

Modified bottles or specialized feeding

Recommend the following to the mother:

-- She may try pumping her breast milk and providing the milk with an adapted

bottle made for children with clefts.

-- Skin-to-skin contact should be encouraged when possible.

-- After bottle-feeding, the infant may be put to the mother’s breast for nonnutritive

sucking.

The soft palate must elevate and separate the oral and nasal cavities in order to

achieve negative intraoral pressure and achieve suction.

 NURSING CARE
FEEDING
Several specialized bottles are available that do not require suction,

including the:

-- Special Needs Feeder (formerly Haberman bottle), Pigeon, and the

Mead- Johnson Cleft Palate Nurser.

Both uses a one-way flow valve that allows the infant to feed by

compressing the nipple chamber between the tongue and the intact

palatal segments. The liquid flows into the baby’s mouth rather than

back into the bottle chamber when the nipple is compressed.

These infants should be burped following every ounce of liquid, or two to

three times during a feeding.

Watch for indications that the infant needs to stop feeding momentarily (

facial signal, which involves elevated eyebrows, a wrinkled forehead, or

watery eyes)
ESOPHAGEAL ATRESIA
AND TRACHEO-
ESOPHAGEAL FISTULA
ESOPHAGEAL ATRESIA
AND TRACHEO-ESOPHAGEAL
FISTULA
CONGENITAL ESOPHAGEAL ATRESIA (EA), OBSTRUCTION OF
THE ESOPHAGUS, AND TRACHEOESOPHAGEAL FISTULA (TEF),
AN ABNORMAL CONNECTION IN ONE OR MORE PLACES
BETWEEN THE ESOPHAGUS AND THE TRACHEA, ARE RARE
MALFORMATIONS THAT REPRESENT A FAILURE OF THE
ESOPHAGUS TO DEVELOP AS A CONTINUOUS PASSAGE AND A
FAILURE OF THE TRACHEA AND ESOPHAGUS TO SEPARATE INTO
DISTINCT STRUCTURES. THESE DEFECTS MAY OCCUR AS
SEPARATE ENTITIES OR IN COMBINATION.
ETIOLOGY

Tracheoesophageal atresia and fistula occur

from failure of the trachea and esophagus to
divide appropriately in intrauterine life.
Surgical intervention often needs to be
performed in several stages.
Preterm
Maternal alcohol and smoking
Exposure to methimazole
Maternal polyhydamnios
 The esophagus develops from the first segment of the

embryonic gut.

PATHOPHYSIOLOGY   ↓
During the fourth and fifth weeks of gestation, the foregut

normally lengthens and separates longitudinally.

↓
Each longitudinal portion fuses to form two parallel

channels (the esophagus and the trachea) that are joined

only at the larynx.

↓
                     Defective separation

↓
 Incomplete fusion of the tracheal folds after this

separation, or altered cellular growth during embryonic

development.

↓
Formation of fistula between trachea and esophagus
TYPES

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SIGNS AND
SYMPTOMS
FROTHY SALIVA IN THE MOUTH AND NOSE
DROOLING, CHOKING, AND COUGHING
VOMITING
DIFFICULTY BREATHING.
VERY ROUND, FULL ABDOMEN
INABILITY FOR AN NG-OROGASTRIC (OG) CATHETER TO PASS
INTO THE ESOPHAGUS
OBSTRUCTION ENCOUNTERED UPON INSERTION OF A
RADIOPAQUE CATHETER INTO THE HYPOPHARYNX
CHEST FILMS TO DETERMINE THE ESOPHAGEAL PATENCY
RESPIRATORY DISTRESS MAY BE MILD OR SIGNIFICANT,
DEPENDING ON THE TYPE OF DEFECT AND THE INFANT’S
GESTATIONAL AGE.
IF FED, THE INFANT MAY SWALLOW NORMALLY BUT
SUDDENLY COUGH AND GAG, WITH RETURN OF FLUID
THROUGH THE NOSE AND MOUTH.
THE INFANT MAY BECOME CYANOTIC AND APNEIC BECAUSE
OF ASPIRATION OF FORMULA OR SALIVA
VISUALIZED FISTULA UPON BRONCHOSCOPIC EXAMINATION
POLYHYDRAMNIOS
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Nursing Diagnosis

RISK FOR
RISK FOR IMPAIRED RISK FOR
SUFFOCATION PARENTING DISPROPORTION
RELATED TO RELATED TO ATE GROWTH
ABNORMAL INFANT’S RELATED TO
OPENING PHYSICAL INADEQUATE
BETWEEN DEFECT AND NUTRITIONAL
ESOPHAGUS AND ENVIRONMENTAL INTAKE
TRACHEA FACTORS SECONDARY TO

CAUSING SURGICAL

PARENT-INFANT REPAIR OF TEF

SEPARATION
Medical and
Pharmacologic

IV fluids are initiated as soon as EA is suspected and

deprived from oral intake

Broad- spectrum antibiotic therapy is often instituted

if there is a concern about aspiration of gastric

contents. To decrease the chance of infection.

Pain medications  may be given after the surgery

SURGICAL
A double-lumen catheter should be placed into the upper esophageal

pouch and attached to intermittent or continuous low suction.

With measures instituted to prevent aspiration pneumonia and to ensure

adequate hydration and nutrition, surgery may be postponed to allow for

more effective treatment of pneumonia and physiologic stabilization so

that the infant can better withstand the complex surgery.

Thoracotomy with division and ligation of the TEF and an end-to-end or

end-to-side anastomosis of the esophagus.

Gastrostomy, ligation of the TEF, and constant drainage of the

esophageal pouch.

Esophageal replacement procedure

Esophageal growth induction procedure

Percutaneous gastrostomy tube is inserted and left open so that any air

entering the stomach through the fistula can escape, thus minimizing the

danger of gastric contents being regurgitated into the trachea.

NURSING
The infant is positioned in supine (sometimes prone) with the head elevated on an inclined plane of at least 30

degrees to facilitate drainage of secretions and decrease the likelihood of aspiration

A double-lumen catheter should be placed into the upper esophageal pouch and attached to intermittent or

continuous low suction.

Adequate thermoregulation is provided

The gastrostomy tube (if applicable) is returned to gravity drainage until feedings are tolerated

The nurse must carefully observe the initial attempt at oral feeding to make certain the infant is able to swallow

without choking. Oral feedings are begun with sterile water, followed by frequent small feedings of breast milk

or formula. Until the infant is able to take a sufficient amount by mouth, oral intake may need to be

supplemented by bolus or continuous gastrostomy feedings. Ordinarily infants are not discharged until they can

take oral fluids well. The gastrostomy tube may be removed before discharge or maintained for supplemental

feedings at home.

Weigh the baby daily.

Nonnutritive sucking should be provided with a pacifier.

Educate the parent on feeding techniques and signs of respiratory difficulty and esophageal stricture upon

discharge
PYLORIC STENOSIS
Pyloric stenosis occurs when the circumferential muscle of the pyloric sphincter becomes thickened,

resulting in elongation and narrowing of the pyloric canal. This produces an outlet obstruction and

compensatory dilation, hypertrophy, and hyperperistalsis of the stomach. This condition usually

develops in the first few weeks of life, causing nonbilious vomiting, which occurs after a feeding;

projectile vomiting may develop and the infant is fussy and hungry after vomiting. If the condition is

not diagnosed early, dehydration, metabolic alkalosis, and failure to thrive may occur.
ETIOLOGY
The exact cause is unknown, but multifactorial inheritance is the likely cause.

Inheritance is polygenic, with an increased risk in the siblings and offspring of affected

persons.

It occurs less frequently in breastfed infants than in formula-fed infants. Formula-fed infants

typically begin having symptoms at approximately 4 weeks of age. Breastfed infants begin

having symptoms at 6 weeks because the curd of breast milk is smaller than that of cow’s

milk, and it passes through a hypertrophied muscle more easily.

For unknown reasons, an increased incidence is seen in infants who receive a macrolide

antibiotic such as erythromycin.

It is more common in firstborn children, and boys are affected four to six times more

frequently than girls.

Race (PS is seen less frequently in African-American infants than in Caucasian infants, and it

is rare in Asians)

It is more likely to affect full-term infants than premature infants.

SIGNS AND
Projectile vomiting

Metabolic acidosis: decrease in serum chloride levels (below 96 mEq/L)

SYMPTOMS
Persistent hunger

Stomach contractions

Dehydration: increase BUN (above 20 mg/dL)

Changes in bowel movements

Weight problems

Distended upper abdomen

Readily palpable olive-shaped tumor in the epigastrium just to the right of

the umbilicus

Visible gastric peristaltic waves that move from left to right across the

epigastrium visible via examination

Pathophysiology

The circular muscle of the pylorus thickens as a result of hypertrophy.

  ↓
This produces severe narrowing of the pyloric canal between the

stomach and the duodenum. Consequently, the lumen at this point is

partially obstructed.

  ↓
Over time the size of the opening is reduced, and the partial

obstruction may progress to complete obstruction.

  ↓
                                       Pyloric Stenosis

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Nursing Diagnosis

DEFICIENT RISK FOR RISK FOR

FLUID DEFICIENT IMPAIRED

VOLUME: FLUID PARENTING

VOMITING R/T VOLUME R/T R/T INFANT’S

NARROWED INABILITY TO FEEDING

PYLORIC RETAIN FOOD DIFFICULTY

CANAL
 MEDICAL AND
PHARMACOLOGIC

THE INFANT IS KEPT NPO AND GIVEN IV

FLUIDS OF DEXTROSE AND

ELECTROLYTES BASED ON SERUM

ELECTROLYTE VALUES— USUALLY

SODIUM CHLORIDE SOLUTION WITH

ADDED POTASSIUM (WHEN THERE IS

ADEQUATE URINARY OUTPUT). CAREFUL

MONITORING OF THE IV FLUIDS AND

STRICT MONITORING OF INTAKE AND

OUTPUT ARE IMPORTANT. RECORD

ACCURATE DESCRIPTION OF ANY

VOMITING AND THE NUMBER AND

CHARACTER OF STOOLS.
PYOLOROMYOTOMY WO
OR FREDET-RAMSTEDT
NURSING
Encourage feedings after 4 to 6 hours postoperatively, beginning with small,

frequent feedings of water or electrolyte solution.

Must be alert to signs of HPS in infants and refer them for medical evaluation

Monitor vital signs. Infants undergoing pyloromyotomy between 2 and 8

weeks of age are at higher risk for postoperative apnea as a result of CNS

immaturity and anesthesia; such infants should be closely monitored for 24

hours prior to discharge

Ensure that the NG tube is patent and functioning properly and that the type

and amount of NG drainage is recorded.

Assess the skin and mucous membranes for alterations in hydration status.

Observe the operative site for any drainage or signs of inflammation.