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DEVELOPMENTAL DISORDER

GASTROINTESTINAL DEVELOPMENTAL DISORDERS

1. Ankyloglossia (Tongue-Tie)
- An abnormal restriction of the tongue caused by an abnormally tight frenulum.
Frenulum the membrane attached to the lower anterior tip of the tongue. Normally it
is short and near the tip of the tongue. As the anterior portion of the tongue grows the
frenulum becomes located farther back.
Signs and symptoms:
a) Difficulty lifting the tongue to the upper teeth or moving the tongue from side to
side
b) Trouble sticking out the tongue past the lower front teeth
c) A tongue that appears notched or heart shaped when stuck out
- Rarely causes speech difficulty or destructive pressure on gingival tissue. If it
does, surgical release may be performed

2. Thyroglossal Cyst
- Arises from an embryogenic fault that leaves a cyst formed at the base of the
tongue that drains through a fistula open to the anterior surface of the neck.
- The cyst may involve the hyoid bone or may contain aberrant thyroid gland
tissue.
Cause: dominantly inherited trait
Signs and symptoms:
- A small, soft, round lump in the center front of the neck.
- Tenderness, redness, and swelling of the lump, if infected
Dx Procedure:
- Ultrasound
- CT scan of the neck
- Fine Needle Aspiration
Mgt:
- Surgical removal of the cyst is performed to avoid future infection of the space or
if thyroid tissue is present, the possible carcinoma later in life.
- IV therapy is given after surgery until the edema at the incision recedes
somewhat and swallowing is safe once more (approx. 24 hours)
Nsg mgt:
- Observe infants closely in the immediate post-operative period of respiratory
distress, because of the operative area will develop some nearby edema.
- Side lying position so secretions drain freely from their mouth.
- If mother is breast feeding, encourage her to express milk manually to preserve
her milk supply.
- Observe infants closely the first time they take fluid orally to be certain that they
do not aspirate. Ensure that the infant Is swallowing safely prior to discharge.
Possible complications:
- As the cyst fills with fluid, swelling and obstruction can lead to respiratory
difficulty.
- If infected, the cyst appears swollen and reddened, with drainage of mucus or
pus from the anterior neck.
- if thyroid tissue is present, the possible carcinoma later in life.
Nsg Dx:
- Risk for aspiration
- Risk for infection

3. Cleft Lip
- Are openings or splits in the upper lip. Deviation may be unilateral or bilateral.
- The fusion fails in varying degrees, with the defect ranging from a small notch in the
upper lip to a total separation of the lip and facial structure up into the floor of the
nose.
- Nose is generally flattened because the incomplete fusion of the upper lip has
allowed it to expand in a horizontal dimension.
- More prevalent among boys than girls
- approx. 1 in every 700 live births
Causes: Fusion of the maxillary and median nasal processes fails to occur between 5
weeks to 8 weeks of intrauterine life.
Dx Procedure:
- Readily apparent on inspection at birth.
- May be detected by sonogram while the infant is in utero.
Mgt:
Pre-operative:
- Repaired surgically shortly after birth, sometimes at the time of the initial hospital
stay and sometimes between 2 and 10 weeks. A revision of the original repair may be
necessary when the child reaches 4 to 6 years of age.
Post-operative:
- Infant is kept on NPO status approx. 4 hours. The infant is introduced to liquids
(plain water) at the end of this time, begin the process gradually to prevent vomiting.
- Infant is usually fed using a specialized feeder because bottle or breast feeding is
contraindicated.
- Acetaminophen may be prescribed to keep children comfortable.
Nsg Dx:
- Risk for imbalanced nutrition, less than body requirements, related to feeding
problems caused by cleft lip/palate
- Risk for ineffective airway clearance r/t oral surgery.
- Impaired tissue integrity at incision line r/t cleft lip/ palate surgery
- Risk for infection r/t surgical incision
- Risk for impaired parenting r/t the birth of infant who is physically challenged.
- Risk for situational low self-esteem r/t facial surgery.

Nsg mgt:
Pre-operative Period:
- Breast feeding method for the child with cleft lip may be to support the baby in
upright position and feed the infant gently using a commercial cleft lip nipple. A
breck feeder, an apparatus similar to a bulb syringe, or a Haberman feeder may be
used,
- If surgical repair will be done immediately, the mother will be able to breastfeed as
early as 7 to 10 days after surgery. Teach her how to manually express breastmilk to
maintain milk supply
- Ensure that infant is bubbled well after feeding because of the tendency to swallow
air caused by the inability to grasp a nipple or syringe edge securely with the
mouth,
- If cleft lip extends to the nares, the infant will breathe through the mouth; the
infants mucous membranes and lips can become dry. Offer small sips of fluids
between feedings to help keep the mucous membranes moist and prevent cracks
and fissures that could lead to infection.
Post-operative:
- Make sure that no tension is placed on a lip suture line,
- Observe closely in the immediate post-operative period for respiratory distress,
- Infants may need suction to remove mucus, blood and unswallowed saliva. Be
gently and do not touch the suture line with catheter.
- Do not lay infants on their abdomen. Doing so puts pressure on the suture line.
- Most surgeons prescribe cleaning the suture line with sterile water, sterile saline or
50% Hydrogen Peroxide in sterile water used with sterile cotton-tipped applicators
after every feeding or whenever serum that forms on suture lines accumulates. Use
smooth, gentle, rolling motion to apply solution. Do not rub because this can loosen
sutures. Gently dry with sterile cotton-tipped applicator.

Cleft Palate
- An opening of the palate. Usually in the midline and may involve just the anterior
hard palate, the posterior soft palate or both. It may be a separate anomaly but as a
rule it occurs in conjunction with a cleft lip.
- As single entity, it occurs more frequently in girls than boys.
- In connection with cleft lip, the incidence is approx. 1 in every 2,000 births.
Causes: polygenic inheritance or environmental influences.
Dx procedure:
- can be determined by depressing the tongue with a tongue blade. This reveals
the total palate and extent of cleft palate.
Mgt:
- surgical repair of cleft palate is usually postponed until the child is 6 to 18 months
old to allow anatomic change in the palate contour that occurs during the first year of life
to take place.
- Because cleft palate is a component of many syndromes, a child with a cleft palate
must be assessed for other congenital anomalies that would suggest it is only one of a
combination problem.

Post-operative:
- Infant is kept on NPO status approx. 4 hours. The infant is introduced to liquids (plain
water) at
the end of this time, begin the process gradually to prevent vomiting.
- Liquids are generally continued for the first 3 or 4 days and then a soft diet is given until
healing is complete.
Nsg Dx:
- Risk for infection (ear) r/t altered slope of Eustachian tube with cleft palate surgery.
- Risk for impaired verbal communication r/t cleft palate.
Nsg mgt:
Post-operative
- Do not use spoon because the child will invariably put it against the roof of the
mouth and possibly disrupt sutures.
- Be certain that milk is not included in the first fluid s offered because milk curds
tend to adhere to the suture line.
- After feeding, offer child with clear water to rinse the suture line and keep it as
clean as possible.
- Review the signs of infection with parents of children with cleft palate. Also remind
them of the importance of reporting pharyngeal infection to their primary care
provider promptly so it can be treated before the infection spreads to the middle
ear.

4. Pierre Robin Syndrome


- A syndrome us a triad of micrognathia (small mandible), cleft palate and
glossoptosis (a tongue malpositioned downward)
- It is an example of cleft palate occurring as only one disorder of syndrome of others.
Signs and symptoms:
- Episodes in which they have difficulty of breathing. (due to the small jaw , their
tongue is too large for their mouth. This causes this to drop backward and obstruct
the airway.)
Dx Procedure:
- Children may have associated disorders of congenital glaucoma, cataracts or
cardiac disorders. They need to go through physical and genetic assessments to be
certain that none of these associated disorders is present.
Mgt:
- An infant having extensive airway obstruction, a suture is attached to the anterior
aspect of the tongue pulling it forward to provide relief. This position is maintained
by attaching a suture to the mucous membrane of the lower lip (creating an
artificial tongue-tied condition)
- Gastrostomy tube or button may be inserted to relieve feeding difficulty.
Nsg mgt:
- Observe carefully to be certain they are free of airway obstruction.
- They may need frequent nasopharyngeal suction to remove unswallowed saliva.
- Side lying or prone position is recommended. If left in supine position, they are in
grave danger of anoxia.
- Instruct parents to feed infant with the same concern given to children with cleft
palate.

5. Tracheoesophageal Atresia and Fistula


- Esophageal atresia is an obstruction of the esophagus, often a fistula occurs
between the closed esophagus and trachea.
- Serious disorder because during feeding, milk can fill the blind esophagus and
overflow into the trachea, resulting in aspiration.
- Incidence is approx. 1 in 3,000 live births.
- Tracheoesophageal atresia must be ruled out in any infant born to a woman with
hydramnios.
- A newborn who has so much mucus in the mouth that he or she appears to be
blowing bubbles is suspected.

5 usual types of esophageal atresia:


a. The esophagus ends in a blind pouch; there is a tracheoesophageal fistula
between the distal part of the esophagus and the trachea.
b. The esophagus ends in a blind pouch; there is no connection to the trachea.
c. A fistula is present between an otherwise normal esophagus and trachea.
d. The esophagus ends in a blind pouch of the proximal esophagus to the trachea.
e. There is a blind end portion of the esophagus. Fistulas are present between both
widely spaced segments of the esophagus and the trachea.
Causes: Teratogen that does not allow the esophagus and trachea to separate normally.
(Between 4 weeks and 8 weeks of intrauterine life, the laryngo-tracheal groove develops
into the larynx, trachea and beginning lung tissue. The esophageal lumen is formed
parallel to this.

Dx Procedure:
- A firm catheter that cannot be passed through the infants esophagus to the
stomach and stomach contents cannot be aspirated. If radiopaque catheter is used,
it can be demonstrated coiled in the blind end of esophagus on x-ray.
- Flat plated x-ray of the abdomen may reveal a distended stomach with air that is
passing from the trachea into the esophagus and stomach.
- Barium swallow or bronchial endoscopy examination will reveal the blind-end
esophagus and fistula.
Mgt:
- Gastrostomy may be performed and the tube allowed to drain by gravity to keep the
stomach empty of secretions and prevent reflux into the lungs,
- Antibiotics
- Surgery consists of closing the fistula and anastomosing the esophageal segments.
- Esophageal dilatation at periodic intervals to keep the repaired esophagus fully
patent in some infants where stenosis or stricture at the anastomosis site.
Pre-operative:
- IV therapy or TPN can supply fluid and calories to the infant.
Post-operative:
- Gastrostomy feeding.
Possible Complications:
- Upper right lobe pneumonia.
- Dehydration or an electrolyte imbalance from lack of oral intake.
- Pneumothorax (Post-operative; from leaks occurring at anastomosis sites)
- Gastroesophageal reflux may occur after a repair if the esophagus is left shorter
than usual.
Nsg Dx:
- Risk for imbalanced nutrition less than body requirements r/t inability for oral intake
- Risk for infection r/t aspiration or seepage of stomach secretions into the lungs
- Risk for impaired skin integrity r/t gastrostomy tube insertion site.
Nsg Mgt:
- Position the infant upright or on the right side to prevent gastric juice from entering
the lungs form the fistula..
- Oropharyngeal suctioning of mucus as necessary.
Post-operative:
- Observe closely for respiratory distress.
- Continue to suction as ordered. (shallow)
- Turn child frequently to discourage fluid form accumulating in the lungs.
- Keep an infant laryngoscope and ET readily available at bedside in case extreme
edema develops, increasing the infants risk for airway obstruction.
- Gastrostomy: protect the skin by using a cream or commercial skin protector
system.

6. Omphalocele
- Protrusion of abdominal contents through the abdominal wall at the point of junction
of the umbilical cord and abdomen.
- The herniated organs are usually the intestines but they may include the stomach
and liver.
- They are usually covered and contained by a thin transparent layer of peritoneum.
- 1 in 5,000 live births.
- 30%- 60% of infants have accompanying disorders.
Causes:
- At approx. 6 -8 weeks of intrauterine life, the fetal abdominal contents are extruded
from the abdomen into the base of the umbilical cord. At 7-10 week, the intestine
returns to the abdomen. Omphalocele occurs when the abdominal contents fail to
return to the abdomen.
- Teratogenic insult that prevented normal intestinal growth.
Dx:
- Prenatal sonogram. If not, presence of omphalocele is obvious on inspection at
birth.
Mgt:
- When identified in utero, cesarean birth may be performed, however, if this is the
only disorder, vaginal birth is considered safe. Document the omphaloceles general
appearance and its size in cm at birth to demonstrate its extent and appearance.
- Immediate surgery to replace the bowel before the thin peritoneal membrane
ruptures or becomes infected.
- If omphalocele is large, topical application of solution such a silver sulfadiazine to
prevent infection of the sac, followed by delayed surgical closure.
- NGT is inserted to prevent intestinal distention, which would enlarge the bowel
lumen, making it more difficult to replace.
- Infant must not be fed orally or suck on pacifier until the bowel repair is complete.
- Hospitalized or home care for a long time (1-2months) waiting for 2 nd stage or even
3rd stage operation, depending on the extent of bowel involved.
- Post-operative: maintain TPN. Once the final stage of bowel repair is completed, a
normal infant diet can be introduced gradually.
Possible Complication:
- Volvulus: twisting of bowel causing obstruction.
- If the total bowel were replaced into this small abdomen, respiratory distress might
result from the pressure of the visceral bulk on the diaphragm and lungs. Bowel
may be contained by a silastic pouch suspended over the infants bed. Over the
next 5 to 10 days, this is decrease in size as more bowel is gradually returned to the
abdomen. TPN is done to supply nutrients and keep bowel from filling.

Nsg Dx:
- Risk for infection r/t exposed abdominal contents
- Risk for imbalanced nutrition, less than body requirements, r/t exposed abdominal
contents.
Nsg mgt:
- Do not leave infants under a radiant heat source because this will quickly dry the
exposed bowel.
- Cover with sterile saline soaked gauze or a sterile plastic bowel bag until surgery.
- Observe infants carefully for signs of obstruction (abdominal distention, constipation
or diarrhea, or vomiting) when they begin oral feedings.
- Offer support to parents to help them accept that this treatment method is the best
way to manage this type of intestinal disorder.
7. Gastoschisis
- Condition similar to omphalocele , except that the abdominal wall defect is a
distance from the umbilicus and abdominal organs are not contained by peritoneal
membrane but rather spill freely from the abdomen
- Surgical procedure is same with omphalocele.
- Children may often have decreased bowel mobility and even after surgical
correction they may have difficulty with absorption of nutrients and passage of
stool.
- Long term follow up maybe necessary to ensure that nutrition and elimination are
adequate
8. Intestinal obstruction
Causes:
- If canalization of the intestine does not occur in the utero at some point in the
bowel, an atresia (complete closure) or stenosis (narrowing) of the fetal bowel
can occur.
- Mesentery of the bowel twists as the bowel re-enters the abdomen or from the
looseness of the intestine in the abdomen of the neonate (this continues to be a
problem for the first 6 months of life.)
- Thicker than usual meconium formation, blocking the lumen.
Signs and Symptoms:
- Passes no meconium
- Pass one stool and then halt
- Distended abdomen
- Tender abdomen- evidence pain by hard forceful indignant crying and by pulling the
legs up against the abdomen
- Vomiting
- Greenish (bile stained) vomitus if obstruction is in the ampulla of Vater (junction of
bile duct and duodenum.
- Increased RR (diaphragm is pushed up against the lungs)
Dx:
- May be anticipated if mother had hydramnios during pregnancy
- if more than 30ml of stomach contents can be aspirated form the newborns
stomach by catheter and syringe at birth.
- Abdominal flat plate x-ray or sonogram will reveal no air below the level of
obstruction in the intestines.
- Barium swallow or barium enema x-ray film may be used to reveal the position of
the obstruction.
Mgt:
- OGT or NGT is inserted and then attached to low suction or left open to the air to
prevent further gastrointestinal distention from swallowed air. (low intermittent
suction)
- NPO
- IV therapy to restore fluid
- Immediate surgery. Area of atresia or stenosis is removed.
- If the repair is anatomically difficult or the infant has other anomalies that interfere
with overall health, a temporary colostomy may be constructed and surgery will be
rescheduled at age of 3 to 6months.
Nsg Dx:
- Risk for fluid volume r/t vomiting

9. Meconium Plug Syndrome


- Extremely hard portion of meconium that has completely blocked the intestinal
lumen, causing bowel obstruction.
- Usually form in the lower end of the bowel.
Cause: Unknown, probably reflect normal variation of meconium consistency.
Signs and symptoms:
- gentle rectal examination may reveal the presence of hardened stool
Dx:
- x-ray or sonogram may reveal distended air-filled loops of bowel up to the point of
obstruction.
- Barium Enema to reveal the level of obstruction but also may be therapeutic in
losing the plug.
- Assess family history of a newborn who has meconium plug for cystic fibrosis or
aganglionic megacolon. Both these disorders ma present with absence of
meconium.
Mgt:
- Administration of saline enemas may cause enough peristalsis to expel the plug.
- Acetylcysteine with Diatrizoate rectally may be prescribed to dissolve the plug.
- Gastrografin, a highly osmotic radiographic substance can be administered as
enema.
- Infant must be observed for further passage of meconium over the next 3 days to
be certain that additional plugs do not exist farther up in the bowel.
10. Meconium Ileus
- Obstruction of the intestinal lumen by hardened meconium,
- A specific phenomenon tgat occurs almost exclusively in infants with Cystic Fibrosis.
Signs and symptoms:
- No meconium passage
- Abdominal distention
- Vomiting of bile-stained fluid
Mgt:
- Enemas
- If obstruction is too high for enemas to reduce it, the bowel must be incised and the
hardened meconium surgically removed.
- Further assess infant for Cystic Fibrosis

11. Diaphragmatic Hernia


- Protrusion of an abdominal organ (usually the stomach or intestine) through a
defect in the diaphragm into the chest cavity.
- Usually occurs on the left side.
- 1 in 3,00 live births
Cause:
- The chest and abdominal cavity are one, at approx. 8 weeks of growth, the
diaphragm forms to divide them. If it does not form completely, intestines can
herniate through the diaphragm opening into the chest cavity.
Signs and Symptoms:
- Respiratory difficulty from time of birth (because at least one of the lobes of their
lungs is unable to expand satisfactorily.
- Cyanosis
- Intercostal or subcostal retractions
- Sunken abdomen
- Absent breath sounds on affected side of the chest.
Dx:
- Occasionally detected in utero by sonogram however it is diagnosed at birth.
Mgt:
- If seen extreme in sonogram, surgery to remove the bowel from the chest can be
attempted by fetoscopy while fetus is still in the utero.
- Emergency surgical repair of the diaphragm and replacement of the herniated
intestine.
- If the defect of the diaphragm is large, an insoluble polymer (Teflon) patch may be
used in reconstruction
- NGT or Gastrostomy tube is inserted immediately to prevent distention of the
herniated intestine. Low intermittent suction is used to avoid injuries in the lining of
the stomach.
- They may be treated with nitric oxide or maintained on ECMO (Extracorporal
membrane oxygenation; A heart lung machine) after surgery until lung tissue is able
to function.
- TPN
Nsg Dx:
- Risk for infective airway clearance r/t displaced bowel
- Risk for imbalance nutrition less than body requirements r/t NPO status
Nsg Mgt:
- Elevate head of bed. This allows the herniated intestine to fall back as far as
possible into the abdomen, providing maximum amount of respiratory space in
chest.
- Post-operative: semi-fowlers position, to keep pressure of the replaced intestine off
the repaired diaphragm.
- Suction as necessary
- Keep infant in a warmed humidified environment to encourage lung fluid drainage.
- Chest physiotherapy maybe ordered to ensure that lung secretions do not pool and
prevent pneumonia
- When starting oral feedings, be certain to bubble the infant well after feeding to
reduce the amount of swallowed air and limit bowel pressure against the
diaphragm.
Possible Complication:
- Cardiac displacement to the right side of the chest and collapse the left lung (if
occurred in the left)
- Pulmonary hypertension because the blood is unable to perfuse readily through the
unexpanded lung. This leads to right-to-left shunting through the foramen ovale of
the heart and also causes Patent Ductus Arteriosus.
- Mortality rate ranges from 25% to 50% with death due to anomalies of the heart,
lung and intestine.
12. Umbilical hernia
- Protrusion of a portion of the intestine through the umbilical ring, muscle and fascia
surrounding the umbilical cord.
- -occurs frequently in African-American children and more often in girls than boys.

Signs/symptoms:
- Bulging protrusion under the skin at the umbilicus. It is rarely noticed at birth and
becomes increasingly noticeable at health care visit during the first year. The
structure is generally 1-2cm in diameter but may be as big as orange when children
cry or strain.
Mgt:
- If the facial ring is less than 2cm, closure will usually occur spontaneously and no
repair will be indicated.
- If more than 2cm, surgery for repair will generally be indicated to prevent intestinal
obstruction or bowel strangulation. Usually done when the child os 1 to 2 years of
age.
- Surgery is generally accomplished on an ambulatory out-patient basis.
- Educate parents to avoid placing belly bands or taping silver dollar over the area .
it may lead to bowel strangulation and should be avoided.
- Post-operative: Pressure dressing remains in place until the sutures are well healed.
- Educate parents to sponge-bath the child until they return for post-operative visit
and the dressing is removed.
13. Imperforated Anus
- Stricture of the anus.
- 1 in 5,000 live births.
Cause:
- failure of the upper bowel to elongate pouch and combine with a pouch invaginating
from the perineum by absorbing the membranes during the 7 th week of intrauterine
life.
- May occur as an additional complication of the spinal cord defects, because both
the external anal canal and spinal cord arise from the same region.
Sign and symptoms:
- No stool will be passed
- Abdominal distention
- Inspection of the perineum that reveals no anus is present.
- Inability to insert a rubber catheter into the rectum.

Dx:
- X-ray or sonogram will reveal the defect if the infant is held in a head-down position
to allow swallowed air to rise to the end of the blind pouch of the bowel. This is also
helpful to estimate the distance the intestine is separated from the perineum.
- Urinalysis to determine whether the child has a rectal-bladder fistula (presence of
meconium). Meconium-stained discharge or a rectovaginal fistula in girls
Mgt: (degree of difficulty in repairing an imperforated anus depends on the extent of the
problem)
- NPO
- NGT attached to low intermittent suction (relieve vomiting and prevent pressure on
other abdominal organs or diaphragm from the distended abdomen.
- IV therapy
- Simple anastomosis of the separated bowel segments if rectum ends close to the
perineum and the anal sphincter is formed.
- The repair becomes complicated if the end of the rectum is at distance from the
perineum or the anal sphincter exists only in an underdeveloped form.
- Repairs are complicated if a fistula to the bladder or urethra is present.
- Colostomy may be created temporarily if repair will be extensive and anticipating
final repair when the infant is 6-12 months old.
Post-operative:
- Still with NGT and removed once bowel sounds are present and start small oral
feeding with milk or glucose water.
- Low residue diet for those with colostomy and are scheduled for second stage
repair.
- Stool softener daily to keep stool from becoming hard and tear the healing suture
Nsg Dx:
- Imbalanced nutrition, less tha body requirement r/t bowel obstruction and inability
for oral intake
- Impaired tissue integrity at rectum r/t surgical incision.
- Risk for impaired parenting r/t difficulty in bonding with infant ill from birth,
Nsg mgt:
Post-operative:
- Take axillary or tympanic temperature rather than rectal.
- Clean the suture line well after bowel movement by irrigating normal saline.
- Side-lying position. Do not place infant in prone position. Newborns tend to place
their knees under causing tension in the perineal area.
Possible complications:
- Fistula to the bladder in boys and to the vagina in girls further complicating a
surgical repair.

NERVOUS SYSTEM DEVELOPMENTAL DISORDERS

1. Hydrocephalus
- An excess of CSF in the ventricles and subarachnoid spaces of the brain.
- Can be congenital or acquired.
a. Communicating or extra-ventricular if fluid passes between the ventricles
and the spinal cord.
b. Obstructive or intra-ventricular- block to such passage of fluid.
- 3-4 per 1,000 live births.
Cause: unknown, although maternal infection such as toxoplasmosis may be a factor.
Excess CSF can result from:
a. Over production of fluid by a choroid plexus(tumor) in the first or second
ventricle (rare)
b. Obstruction of passage of fluid somewhere between point od origin and point of
absorption (most frequent cause)
c. Interference with the absorption of the fluid from the subarachnoid space.
Signs/symptoms:
- Enlargement of the head- infant cranial structures are not firmly knitted, this excess
fluid causes enlargement of the head. Becomes evident in the first few weeks or
months of life.
- Fontanelles widen and appear tense
- Suture lines of the skull separate
- Shiny scalp and scalp veins become prominent
- Sunset eyes (sclera shows above the iris because of upper lid retraction)
- Increased ICP (bradycardia, bradypnea, increased temperature and BP, hyperactive
reflexes, strabismus, and optic atrophy)
- Irritable
- Lethargic
- Failure to thrive
- Shrill, high-pitched cry
Dx:
- Can sometimes be detected on prenatal sonogram.
- Sonogram
- CT
- MRI
- Skull x-ray (will reveal separating sutures and thinning of skull bones)
- If non-communicating, dye inserted into a ventricle through the anterior fontanelle
will not appear in CSF obtained from LP.
Mgt: (depends on its cause and extent)
- Accetazolamide (diamox) may be ordered if caused by over production of fluid.
- Destruction of the portion of the choroid plexus may be attempted by ventricular
endoscopy or if a tumor in that area is responsible, removal of the tumor should
provide a solution.
- Shunting procedure (ventriculo-peritoneal shunt- threading a polyethylene catheter
under the skin from the ventricles to the peritoneum.
- Most have NGT placed during surgery.
- On NPO until bowel sounds return and tube can be removed. Introduce fluid
gradually after removal of tube.
-
Nsg Dx:
- Risk for ineffective cerebral tissue perfusion r/t increased intracranial pressure
- Risk of imbalanced nutrition, less than body requirement, r/t increased ICP.
- Risk for impaired skin integrity r.t weight and immobility od head
- Deficient knowledge r/t home care needs with hydrocephalus
- Risk for delayed growth and development r/t potential neurologic challenge.
Nsg mgt-
- Post shunt insertion, infants head is usually raised only 30 degrees so the head
remains level with the body. If raised above, may cause rapid CSF flow leading to
possible tearing od cerebral arteries,
- Assess for signs of increased ICP after surgery (tensed fontanelles, increasing head
circumference, irritability, lethargy, decreased LOC, poor sucking, vomiting,
increased BP, increased temp, decreased pulse and RR)
- Assess for signs of infection and meningitis (stiff neck and marked irritability)
- Be certain that child receives adequate pain management to minimize any upset,
crying elevates CSF pressure.
- Change the position of the head approx. every 2 hours so that no portion of the
head rest against the mattress for a long period.
- If a Kling or stockinette bandage is used to hold a surgical head dressing in place,
place a piece of gauze or cotton behind the childs ear before the bandage is
applied to prevent skin surfaces from touching and becoming excoriated.

2. Neural Tube Disorders


- Lack of fusion of the posterior surface of the embryo in early uterine life.
- 0.6 in 1,000 births
- Prognosis depends on e extent of the disorder.
Cause:
- polygenic inheritance pattern
- Poor nutrition (esp deficient folic acid) appears to be a major contributing
factor
Dx:
- Intrauterine life sonography
- Fetoscopy
- Amniocenthesis (increased AFP in amniotic fluid)
- Analysis of AFP in maternal serum.
Types:
a. Anencephaly
- Absence of cerebral hemispheres.
- Occur when the upper end of neural tube fails to close in early uterine life.
- Under developed head does not engage the cervix well thus difficult labor
- Children cannot survive with this disorder because they have no cerebral
function. However, they may survive for number of days after birth.
- Must be differentiated from craniosynthosis (normal brain growth but
premature fusion of cranial sutures) Abnormally closed fontanelles and often
show bulging. Surgery can be done and brain growth will be normal.
Dx:
- elevated AFP in maternal serum or amniocenthesis
- Confirmed by sonogram
B. microcephaly
- a disorder in which brain growth is so slow that it falls more than 3 standard
deviations below normal growth charts.
Cause:
- Intrauterine infection such as rubella, cytomegalovirus or toxoplasmosis.
- Severe malnutrition or anoxia in early infancy.
Signs and symptoms:
- Cognitively challenged because of the lack of functioning brain tissue.
- Decreased head circumference.
C. Spina Bifida Occulta
- occurs when the posterior lamina of the vertebrae fails to fuse
- Common at the fifth lumbar or first sacral level but may occur at any point
along the spinal canal
- Benign defect
- 1 of every 4 children
- may be noticeable as dimpling at the point of poor fusion.
Mgt: need no immediate surgical correction.

D. Meningocele
- meninges covering the spinal cord herniate through unformed vertebrae.
- appears as a protruding mass (approx. orange size) at the center of the back.
Generally occurs at the lumbar region, although it may be present anywhere in
the spinal canal.
- Protrusion may be covered by a layer of skin or only the clear dura mater.
E. Myelomeningocele
- The meninges covering the spinal cord herniate through the vertebrae defect
the same as with meningocele.
- Results in lower motor neuron damage
Signs and symptoms:
- Flaccidity
- Lack of sensation in the lower extremities
- Loss of bowel and bladder control
- Children often have accompanying talipes (clubfoot) disorders and
developmental hip dysplasia.
- 80% of cases are accompanied with hydrocephalus.
Mgt:
- Cesarean birth for meningocele and myelomeningocele. (to avoid pressure
and injury to the spinal cord)
F. Encephalocele
- a cranial meningocele or myelomenigocele.
- Occurs more often in the occipital area of the skull but may occur as a nasal
or nasopharyngeal defect.
Dx: X-ray or sonography will reveal the size of the skull defect.
Mgt: (Meningocele, myelomeningocele, encephalocele)
- Surgery to replace the contents that are replaceable and to close the skin
defect to prevent infection.
- Artificial bladder sphincters may be placed to help establish continence.
Nsg Dx:
- Risk for infection r/t rupture or bacterial invasion of neural tube sac
- Risk for imbalanced nutrition, less than body requirements, r/t difficulty
assuming normal feeding positions
- Risk for ineffective cerebral tissue perfusion r/t increased intracranial
pressure
- Risk for impaired skin integrity r/t required prone position
- Impaired physical mobility r/t neural tube disorder
- Risk for impaired elimination r/t neural tube disorder
Nsg Mgt:
- Position patient on prone or side-lying( supported)
- Help parents hold infant in as normal feeding position. Make certain that a
supporting arm does not press against the lesion.
- Encourage parents to take the infant to the places a child would normally
accompany parents.
- Teach parents to perform passive exercises to prevent muscle atrophy and
formation of contractures if the child has impaired lower extremity motor
control.
- Post-operative: place child on prone until the skin incision is healed (about 7
days)
- Observe frequently for signs of increased ICP, increase in head circumference
as well as behavioral changes such as irritability or lethargy.

3. Arnold-Chiari II Malformation (Charii II Malformation)


- Over growth of neural tube inn weeks 16-20 of fetal life.
- Projection of cerebellum, medulla oblongata and fourth ventricle into the
cervical canal.
- Causes the upper cervical spinal cord t jackknife backward, obstructing CSF
flow causing hydrocephalus.
- 50% of these children has lumbrosacral myelomeningocele
- Prognosis depends on the extent of the defect and the surgical procedure
possible.
Signs and symptoms:
- Gagging and swallowing reflexes may be absent, increasing risk of tracheal
aspiration.

SKELETAL DEVELOPMENTAL DISORDERS

1. Absent or malformed Extremities


- Congenital skeletal disorders may result from unknown reasons such as
maternal drug ingestion, virus invasion during pregnancy or amniotic band
formation in the utero.
- These children can be fitted with prosthesis early in life
- Children who are born with an absent extremity may need help not only in
mastering the use of prosthesis but also in mastering a positive body image
of themselves as whole.

2. Finger Conditions
a. Polydactyly presence of one or more additional fingers. These extra
fingers are often just cartilage or skin tags. Removal is simple and
cosmetically sound.
b. Syndactyly two fingers are fused. The fusion is usually caused by simple
webbing; separation of the fingers into two sound and cosmetically appealing
ones is usually successful.
- In other instances, the bones of the fingers are also fused and
the cosmetic appearance and function of fingers cannot be fully
reconstructed.
3. Pectus Excavatum
- Is an indentation of the lower portion of the sternum
- Children are usually born with this condition but they may also develop it
after chronic obstructive lung disease or rickets.
- Lung volume decreases and the heart is displaced to the left.
- It can be repaired for cosmetic reasons or to expand lung volume.
4. Pectus Carinatum
- Sternum is displaced anteriorly, increasing the AP diameter of the chest.
- This condition can be repaired for physiologic or cosmetic reasons.
5. Torticollis
- A congenital anomaly when the sternocleidomastoid muscle is injured and
bleeds during birth.
- This tends to occur in newborns with wide shoulders when pressure is exerted
on the head to deliver the shoulder,
- It may not be noticeable in newborn and may become evident only as the
original hemorrhage recedes and fibrous contraction occurs at age 1 to 2
months.
- Thick mass over the muscle can be palpated
Mgt:
- Passive stretching exercises and encouraging the infant to look in the
direction of the affected muscle.
- If manual stretching is begun early and consistently by the parents, further
treatment usually is not necessary.
- In few instances which simple exercises are not effective and condition still
exists at 1 year of age, surgical correction followed by a neck immobilizer will
be necessary
Pos. complication:
- Continued elevation of one shoulder (if with extreme injury to the muscle)
- Scoliosis later in life
6. Craniosynostosis
- Premature closure of skull sutures.
- May occur early in utero or early infancy
- More often on boys than girls
- Should be detected early because premature closure of the suture line will
seal the skull closed and compromise brain growth.
- Cardiac anomalies, choanal atresia or defects of elbows and knee joints are
also associated.
Cause:
- rickets or irregular calcium or phosphate metabolism
- unknown
S&S:
- sagittal suture line closes prematurely - head tends to grow anteriorly and
posteriorly
- coronal suture line fuses early orbits of the eye becomes misshapen
- Increased ICP may lead to: exophthalmos, nystagmus, papilledema,
strabismus and atrophy of the optic nerve with consequent loss of vision.
Mgt:
- Measure head circumference on all children age 2 years or younger ar health
maintenance visits
- Sagittal careful observation only
- Coronal surgically opened to prevent brain compression
Dx:
- X-ray or sonogram which reveals fused suture line.
Possible Complication:
- Loss of vision.

7. Achondroplasia (Chondrodystrophia)
- Failure of bone growth
- Defect in cartilage production in utero
- Epiphyseal plate of long bones cannot produce adequate cartilage for
longitudinal bone growth, which results in both arms and legs becomes
stunted.
Cause: inherited dominant trait (high probability that any child will inherit the
disorder)
S&S:
- Heads appear unusually large. Forehead is prominent and bridge of the nose
is flattened. Intelligence is generally normal
- Thoracic kyphosis and lumbar lordosis of the spine may develop.
- Height less than 4ft 6 inches (140cm)
- Small pelvis ( difficult child bearing)
Dx:
- Can be diagnosed in the utero or at birth by comparing the length of
extremities to the normal length
- X-ray, which will reveal characteristic abnormally flaring epiphyseal lines
Mgt:
- may be prescribed with growth hormones
- continued guidance or counseling can help them emerge from this period
with feelings of high self-esteem in themselves as adults

8. Talipes Deformities
- Ankle-foot disorder club foot
- 1 on every 1,000 children
- More often in boys than girls
- Foot cannot be properly aligned without further intervention.
Types:
a. Plantar flexion- an equinus or horsefoot position with the foot lower than
the heel.
b. Dorsiflexion- the heel is held lower than the foot or the anterior foot is flexed
towards the anterior leg.
c. Varus deviation- foot turns in
d. Valgus deviation- foot turns out
- most children have a combination of these conditions or have an equinovarus
or a calcaneovalgus deformity (child walks on heel with foot everted)
Causes: inherited as a polygenic pattern
S&S:
- Shoe size may vary as much as two shoe sizes
- Asymmetry of leg length
Mgt:
- Cast is applied while the foot is placed in an over-corrected position. The cast
extends above the knee to ensure firm correction.
- Change diapers frequently to prevent a diaper from touching the cast.
- Cast is changed every one to 2 weeks (infants grow rapidly)
- Infant may have to sleep in Denis Browne splints (shoes attached to a metal
bar to maintain position) or high top shoes at night for a few more months
- If unsuccessful, surgery is an option to achieve final correction

Nsg mgt:
- Review with parents on how to check the infants toes for coldness or
cyanosis and how to blanch a toe nail bed and watch it turn pink to assess for
good circulation
- Teach parents to perform passive foot exercises such as putting the infants
foot and ankle through a full range of motion several times a day for several
months (after cast is removes approx. after 6weeks)
9. Developmental Hip Dysplasia
- Improper formation and function of the hip socket, it may be evident as
subluxation or dislocation of the head of the femur
- Occurs most in children of Mediterranean ancestry.
- 6 times more frequent in girls than boys. (Possibly because the hips are
normally more flaring in females and possible because the maternal hormone
relaxin causes the pelvic ligaments to be more relaxed.)
- Usually unilateral
Cause:
- Unknown. May be from a polygenic inheritance pattern
- may also occur from a uterine position that causes less-than-usual pressure
of the femur head on the acetabulum

S&S:
- affected leg appear slightly shorter than the normal one (because the femur
rides so high in socket. This is noticeable when child is lying in supine and the
thighs are flexed to a 90 degree angle toward the abdomen.
- In some, hips abduct normally at a newborn assessment but at approx. 4 to 6
weeks, a secondary shortening of the abductor muscles will have occurred.
Dx: (will reveal the shallow acetabulum and a more lateral placement of the
femur head than is ordinarily seen)
- X-ray
- Sonogram
- MRI
Mgt:
- Splints, halter or cast may be used (to press the femur head against the
acetabulum and cause it to deepen its contour by the pressure.)
- Traction is used first for older children (to bring the femur head into good
position with the acetabulum)
- Surgery (pin inserted to stabilize the hip. For those who did not achieve
correction)
- Some children wil be 2 years old before the final cast or harness is removed.
Splints:
a. Frejka Splints- made of plastic and buckles onto the child like a huge
confining diaper. Keep splint in place at all times except when changing
diapers or bathing the infant.
b. Pavlick Harness- an adjustable chest halter that abducts the legs, method of
choice for long term therapy because it reduces the time interval for therapy
to 3 to 4 weeks and simplifies care. Soft plastic stirrups with quick fastening
closures attach to leg extension straps and hold the hips flexed, abducted
and externally rotated. Instruct parents to lie the infant supines, grasp the
infants thigh and abduct them to place the femoral head into the
acetabulum and then aply the harness.
c. Spica Cast- if hip is fully dislocated or the subluxation is severe, the infant
may be placed immediately in frog-leg cast or a spica cast to maintain an
externally rotated hip position. The child may be placed in bryants traction
for a week to better position the hip. The hip is then placed in an abducted
position and a large hip spica cast or an A-line case is applied.
Nsg Dx:
- Deficient parental knowledge r/t splint, halter or cast correction for hip
dysplasia
Nsg mgt:
- Remind parents of good diaper area care. Padding the edges of the brace
with an additional diaper can increase comfort and decrease irritation.
- Advice parents to assess the skin under the straps daily for irritation or
redness.
- Assess hourly for circulation to the extremities for the first 24 hours after the
cast is placed and daily thereafter.

COMMON CHROMOSOMAL DISORDERS THAT RESULT IN PHYSICAL OR COGNITIVE


DEVELOPMENTAL DISORDERS

1. Trisomy 13 syndrome (Pataus syndrome; 47XX13+ or 47Xy13+)


- A condition which children have an extra chromosome 13.
- Severely cognitively challenged children
- 0.45 per 1,000 live births
- Most of these children do not survive past early childhood.
S&S:
- Microcephaly with abnormalities in the forebrain and forehead
- Eyes are smaller than normal (microphthalmia) or absent
- Cleft lip and palate
- Low set ears
- Hearts defects (particularly ventricular septal defects.
- Abnormal genitalia
2. Trisomy 18 syndrome
- Have 3 number 18 chromosomes
- Severely cognitive challenged
- 0.25 per 1,000 live births
- These children do not survive beyond early infancy
S&S:
- Small for gestational age
- Markedly low-set ears
- Small jaw
- Congenital heart defects
- Misshapen fingers and toes
- Soles of feet are often rounded instead of flat

3. Cri du Chat Syndrome


- short arm on chromosome 5.
- Severely cognitively challenged
S&S:
- Abnormal cry, sounds of a cat
- Small head
- Wide set ears
- Downward slant to the palpebral fissure of the eye

4. Turners Syndrome
- Has one functional X chromosome
- 1 per 1,000 live births

S&S:
- Child is short
- Hairline at the nape of the neck is low-set
- Neck may appear to be webbed and short
- Edema of the hands and feet
- Congenital anomalies (coarctation of the aorta and kidney disorders)
- May be cognitively challenged, more commonly intelligence is normal.(some
may have learning disabilities.
- Small and nonfunctional gonands (secondary sex characteristics do not
developed puberty)
- Females cannot reproduce because of limited ovarian function
Mgt:
- growth hormone can be helpful to achieve additional height
- estrogen at 13 years old (secondary sex characteristics will appear. However
does not correct the problem of sterility)

5. Klinefelters Syndrome
- Boys with an XXY chromosome pattern
- 1 in 1,000 live births
S&S: (Characteristics of the syndrome may not be noticeable at birth
- At puberty child does not develop secondary sex characteristics
- Testis are small and produce ineffective sperm
- Gynecomastia
Possible complication:
- Increased risk of breast cancer

6. Fragile X syndrome
- X-linked pattern of inheritance in which one long arm of a X chromosome is
weakened
- 1 in 1,000 live births
- Most common cause of cognitive challenge in boys
- Carrier females may show some evidence of the physical and cognitive
characteristics
S&S:
- Before puberty, maladaptive behaviors such as hyperactivity and autism,
- Reduced intellectual functioning
- Deficits in speech and arithmetic
- Large head
- Long face with high forehead
- Prominent lower jaw
- Cardiac disorders may be present
- After puberty, enlarged testicles may become evident (fertile and can
reproduce)
Mgt:
- Although intellectual function cannot be improved, folic acid and
phenothiazine administration mat improve symptoms of poor concentration
and impulsivity

7. Down Syndrome (Trisomy 21)


- most frequently occurring chromosomal abnormality
- 1 in 800 live births
- Occurs more frequently in women who are over 35 years of age (1 in 100 )
Dx:
- fetal diagnosis Is possible in sonogram in utero
S&S:
- broad and flat nose
- eyelids have an extra fold of tissue in the inner canthus
- palpebral fissure (opening between the eyelids) tend to slant laterally upward
- Brushfields spot (white specks in the iris).
- Tongue may protrude from the mouth (oral cavity is smaller)
- Back of the head is flat
- Shorter neck
- Low-set ears
- Poor muscle tone
- Fingers are shorter and thick
- Wide space between the first and second toes
- Simian line (palm of the hand shows a peculiar crease or a horizontal palm
crease rather than the normal 3 creases in the palm
- Degree of cognitive challenge (not evident at birth)
- Altered immune function (making them prone to upper respiratory infection)
Mgt:
- Expose to early educational and play opportunities
- Good hand washing hygiene technique should always be taken when caring
for them (prone to infection)
- Feed child slowly (large tongue may interfere swallowing and may cause
choking
- Counselling and support for parents can begin.

IMMUNODEFICIENCY DISORDERS

A. Primary (congenital) Immunodeficiency


- Children born without an essential immune substance or function or with
inadequate amounts of immune substance.
1. B-Lymphocyte Deficiencies

a. Abnormally low Ig (IgA) or totally, which is referred to as hypoammaglobulinemia


or agammaglobulinemia
- Inhireted X-linked recessive defect in the maturation of B lymphocytes that
results in abnormally low levels of all immunoglobulins.
- Rheumathoid arthritis or SLE may occur later in life.
- Cellular or T-lymphocyte response remains adequate allowing the child to
resist viral, fungal and parasitic infections
S&S:
- Frequent respiratory, digestive and throat infections ( at 6months of age after
maternal antibodies fade)
Mgt:
- Monthy IVIg injections to supply immunoglobulin (set up a schedule)
- Bone marrow transplant (to restore immune competency
- Teach parents to recognize early infection

b. Common variable immunoglobulins Deficiencies


- Most common if deficiency in IgA in surface secretions.
- Overall B-lymphocytes is normal but IgA production is reduced or absent
- Without IgA, infection on surfaces exposed to the external environment and
normally protected by mucus becomes common (sinusitis, atopic dermatitis)
- Can occur as a secondary type due to treatment og phenytoin and
penicillamine.
Possible complication:
- Malignancy of the respiratory, gastrointestinal and lymphoid systems (form
chronic irritations due to these large numbers of antigens predispose tissue
to malignant transformation.)
Mgt: - prevention by preventing infections and perhaps administering prophylactic
antibiotics to prevent respiratory infections

2. T- Lymphocyte Deficiencies
- Inadequate numbers or inadequate functioning of one or more types of T-
lymphocytes. This affects cell mediated immunity and also because of helper
T-lymphocyte function, possibly humoral immunity.

3. Comnined T and B lymphocyte Deficiency


- Most frequently seen disorder characterized by an absence or reduction of
both humoral and cell-mediated immunity.
Cause:
- X-linked or autosomal recessive disorder
- Developmental abnormality which prevents the formation of T lymphocytes,
Mgt:
- Stem cell transplantation

B. Secondary (Acquired) Immunodeficiency


- can occur from factors such as severe systemic infection, cancer, renal
disease, radiation therapy, severe stress, malnutrition (because rapidly
growing cells need protein synthesis), immunosuppressive therapy
(prednisone) and aging.
- Can be complete or partial loss of both B and T lymphocytes
a. Acquired Immunodeficiency Syndrome (AIDS/HIV)
- Caused by the RNA human immunodeficiency retrovirus HIV.
- The virus acts by attacking the lympho-reticular system, in particular
CD40bearing helper T lymphocytes. The virus replicates in these lymphocytes
and in the process destroys them.
- No defense against the virus
- When monocytes and macrophages become affected as well, the person with
HIV infection cannot resist normal infection and is susceptible to opportunistic
ones such as fungal infections.
- Incubation period of 10 years in adult
Transmission:
- It is contracted through blood and body secretions by sexual contact, sharing
of contaminated needles for injection, transfusion of contaminated blood and
blood products, breast feeding.
- prenatally from mother to fetus /newborn (most common reason for childhood
HIV. Declined to 80% since HIV positive women have been prescribed with
Zidovudine during pregnancy)
Dx:
- PCR (polymerase chain reaction-determine of antigen)
- ELISA (Enzyme linked immunosorbent assay- test for antibody)
- Western blot
- CD4 counts-to document the disease status and detect disease progression

3 CLASSIFICATIONS OF HIV/AIDS IN CHILDREN


i. Category A mildly symptomatic. Two or more symptoms such as
enlarged lymph nodes, liver, spleen or recurrent persistent upper
respiratory infections, sinusitis or otitis media.
ii. Category B- moderately symptomatic. More serious illness such as
oropharengeal candidiasis, pneumonia, sepsis, herpes zoster, etc.
iii. Category C - prenatally from mother to fetus /newborn (most common
reason for childhood HIV) and through Severely symptomatic. Serious
bacterial infections such as septicemia, meningitis, bone or joint
infection, candidiasis, herpes simplex lasting for 1 month,
mycobacterium or Pneumocystitis Carinii pneumonia. Karposi sarcoma
rarely develops in children.
Mgt:
- Zidovudine administration during pregnancy to HIV positive women
- Complex regimen of nutritional supplements to prevent weight loss
- vaccines to prevent infections (except OPV and Varicella vaccines)
- anti-viral and antibacterial agents to combat HIV and opportunistic infections
- specific antiretroviral medication to prevent progressive deterioration of the
immune system and to provide prophylactic measures against opportunistic
infections.
3 Classes of drugs are the mainstay of therapy
i. NRTI (nucleoside reverse transcriptase inhibitor)
- Designed to block production of viral DNA, limiting the ability of
the virus to infect cells. (Zidovudine)
ii. NNRTI (non-nucleoside reverse transcriptase inhibitor)
- Inhibit the DNA synthesis of viruses but act at different sites on
the viral enzyme. (Nevirapine and Efavirenz)
iii. Protease inhibitor- stop the ability of the virus produce
protease, limiting metastasis. (Amprenavir, Nelfinavir and
Ritonavir)

Nsg Dx:
- risk for infection related to decreased immune function
- risk for compromised family coping r/t diagnosis of HIV. AIDS in child
Nsg Mgt:
- instruct children and parents to maintain strict personal hygiene and to avoid
lose contact between the child and to anyone with respiratory infection and
try to prevent the child from contracting dangerous opportunistic infections
ALLERGY
- Result of an abnormal antigen-antibody response.
- 3 in every 15 children
- Can be chronic or minor
- Cause is difficult to pinpoint
- Symptoms vary from minor to acute without warning
Hypersensitivity
- The underlying cause of all allergic disorders appears to be an excessive
antigen-antibody response when the invading organism is an allergen rather
than a simple immunogen.
Type 1: anaphylaxis
- Acute reaction characterized by extreme vasodilation that ;leads to
circulatory shock and extreme bronchoconstriction that decreases the air
lumens
- IgE receptor antibodies attached to the surface of mast cells bind to IgE
antibodies responding to presence of antigen.
S&S:
- Vascular congestion and edema (IgE triggers the release of intracellular
granules which contains histamine, leukoctrienes and chemotactic substance.
Histamine and leukotrienes cause this)
Type 2: Cytotoxic response
- Cells are detected as foreign and immunoglobulin directly attack and destroy
them without harming surrounding tissue.
- Example is a foreign RBCs that are introduced to an Rh-negative w by Rh
positive fetus that are destroyed by this process.
- Tumor cells may be destroyed by this process (unknown why)
Type 3: immune complex
- An IgG or IgE mediated antigen antibody complex reaction that involves
complement ad initiates the inflammatory response.
- Glomerulonephritis and SLE
Type 4: Cell mediated hypersensitivity
- T lymphocytes react with antigens and release lymphokines to call
macrophages into the area.
- Mantoux or PPD test is an example. Redness and induration of the site do not
begin initially but only after 12 hours from the injection. Reaction peacks at
24-72 hours (delayed reaction)
- Contact dermatitis is another example. Redness and vesicles may occur and
pruritus may be intense because lymphocytes and macrophages infiltrate the
area and attempt to destroy the offending proteins.
Dx:
- Family history
- Exact symptoms of the allergy are important in helping to identify allergy
(rhinitis, airborne antigen)
- Radioalergosorbent test (RAST), an indirect radioimmunoassay in which
childs serum IgE s allowed to react with specific allergens impregnated in
laboratory disk. Determination of IgE serum antibodies (increased eosinophil
count 250 or more per cubic mm is significant. Can also be ova or parasite)
Skin testing- is done to detect the presence of IgE in the skin or to isolate an
antigen. Because this appears quickly, the test should be read in 20mins.
- Intracutaneous injections are done by injecting a small amount of a solution
of allergen bellow the epidermis of the skin.
- After testing, the child will have have a wheal and erythema will occur at test
site.
- Have a syringe filled with 1ml Epinephrine 1:1000 on hand to counter act
unexpected anaphylactic reaction from skin testing.
- Epinephrine is given subcutaneously in doses of 0.01mg/kg up to 0.5mg.
Mgt:
- 3 goals of therapy
Reduce childs exposure
- Environmental control means removal of as many common allergens as
possible.
Hyposensitize the child to produce a state of increased clinical tolerance
- Done when the childs allergy symptoms cannot be controlled by avoidance
of the allergen or conventional drug therapy.
- Works by increasing the plasma concentration of IgG antibodies.
- After specific allergens have been recognized with skin testing, small
amounts of the allergy extract are inject subcutaneously at 3-5 days interval.
- After hyposensitization has been achieved, the child needs periodic injections
every 3 to 4 weeks to maintain hyposensitization.
- A newer technique is the sublingual administration of chosen allergen
solution.
Pharmacologic therapy
- Antihistamine
o Diphenhydramine (benadyl) is a prototype of this drug but
causes severe drowsiness.
o 2nd and 3rd generation such as cetirizine and Loratadine cause
less drowsiness and provide longer effect.
COMMON IMMUNE REACTION
1. Anaphylactic shock
- immediate, life threatening, type I hypersensitivity reaction after exposure to an allergen in a
previously sensitized child.
S&S:
- Nauseated, Vomiting, Diarrhea (sudden increase in gastrointestinal secretions by
stimulation of histamine.
- urticaria ad angioedema
- dyspnea (from bronchospasm)
- blood pressure and pulse rate may fail (as blood vessels dilate)
Mgt:
- Epinephrine is the drug of choice
- if from an insect sting, give the epinephrine in the opposite arm. Place a tourniquet in the
extremity of allergenic

2. Urticaria (hives) and Angioedema


Urticaria - refers to flat wheals surrounded by erythema arising from the chorion layer if the sin.
They are intensely pruritic.
- Occurs from type 1 Allergic reaction
- In chronic urticaria, no causative allergen may be found.
- there is dilatation of capillaries and venules with increased permeability
Angioedema- edema of the skin and subcutaneous tissue.
- frequent on eyelids, hands, feet, genitalia and lips.
- Not dependent, generally asymmetrically distributed and usually occurs with
urticaria.
- Cause: includes drugs, foods and insect stings.
- Mgt: subcutaneous epinephrine or oral antihistamine
- Complication: laryngeal edema that may lead to airway obstruction and
subsequent asphyxiation and death.
3.Serum Sickness
- type 3 hypersensitivity response.
- examples of foreign sera given to children include tetanus antitoxin, diphtheria antitoxin, and
rabies antiserum.
- from reaction to drug (penicillin) but is rare
S&S: (begin 7 to 12 days after serum injection. Some may occur as early as 1 to 5 days,)
- Itching, Edema, Erythema at injection site
- Generalized urticarial
- Angioedema
- Erythrmatous maculopapular rashes
- Erythema multiforme (a generalized maculo eruption with dark red
papules)
- Purpura (hemorrhage into the skin)
- Fever
- Arthralgia
- Lymphadenopathy
- Weight gain
- Nausea and vomiting
- Abdominal pain
- In extreme instances, the childs nervous system may be involved and
optic neuritis, stupor and coma may occur.
Mgt:
- Treatment is symptomatic
- Antihistamine or epinephrine in relieving symptoms
- ASA or NSAID such as Ibuprofen for fever and pain.
4. Atopic Disorders
- examples are hay fever (allergic rhinitis), eczema (atopic dermatitis) and asthma.
Though with familial tendency, different family members may have different symptoms.
The gene responsible for an immune response is located chromosomally near the human
leukocyte antigen that is responsible for graft rejection
these children have a higher than normal production of IgE antibody that makes them more
responsive to allergies than other people.
Children whose parents smoke have twice the incidence of atopic disorders compared with
children with parents who does not smoke.
Allergic Dermatitis
- Caused by a type 1 or immediate hypersensitivity immune response.
- Occurs in 10% to 15% of children
- Causes are generally pollens or molds rather than foods or drugs.
S&S:
- Sneezing
- Nasal engorgement
- Profuse watery nasal discharge
- Eyes may water
- Pruritic conjunctiva, often with a distinctive pebbly appearance
- Frontal headache (children after 6 years old)
- Sore throat and cervical adenopathy may be present, but these are rare with
allergic rhinitis.
Dx:
-nasal smear discharge will reveal an increased eosinophil count.
- RAST analysis may reveal the offending allergens
Mgt:
- Avoidance of allergens
- Pharmacologic agents (antihistamine, leukotriene inhibitors or corticosteroid)
- Immunotherapy
- Intranasal corticosteroids
Perennial Allergic Rhinitis
- When allergen is one that is capable of affecting the child year-round such as
dust mites or pet hair.
- Environmental control plays a big role in the control of the disorder.
Possible Complication:
- Otitis media
Atopic Dermatitis (infantile eczema)
- Primarily a disease of infants, beginning as early as 2 nd month of life and
possibly lasting until2 or 3 years old.
- Primarily related to a food allergy (tends to occur in formula-fed infants than
in breastfed infants and more frequently if infants are fed solid foods before
6months of age.
- Sweating, heat, tight clothing and contact irritants such as soap increases
pruritus.
- Common site includes the scalp and forehead, the cheeks, neck, behind the
ears and the extensor surfaces of the extremities.
S&S:
- Loss of serous fluid out into the tissues (increased capillary membrane)
- Popular and vesicular skin eruptions with surrounding erythema. The vesicles
rupture and exude yellow, sticky secretions that form crusts on the skin as
they dry.
- Depigmented and lichenified (shiny) dry, flaky scales form as infected lesions
heal.
- Fussy and irritable (from uncomfortable lesions)
Mgt:
- Reduce exposure to allergen
- Reduce pruritus
- Hydrate the skin by bathing or applying wet dressings for 15-20mins followed
by application of a hydrating emollient such as petroleum jelly
- Antihistamine
- Corticosteroid ointment
- Hydrocortisone mixed with antibiotic if lesions are secondary infections
- Skin emollient and moisturizer such as Eucerin or baths with a substance to
lubricate the skin such as Alpha-keri are prescribed to prevent excessive skin
dryness.
Nsg Dx:
- Risk for impaired parenting r/t feelings of inadequacy secondary to infants
atopic dermatitis
- Impaired skin integrity r/t infantile atopic dermatitis
Nsg Mgt:
- Instruct parents to trim the infants finger nails short or cover his or her hands
with cotton socks to prevent scratching.
In older children, it frequently occurs at puberty or late adolescence.
- Often occurs in the eyebrows
- Depigmentation or hyperpigmentation is usually present
- Lichenification is marked
Mgt:
- Use only prescription soap
- Avoid swimming in chlorinated pools
- Shower to remove perspiration which is irritating to the skin
(other treatments are same with infants)

DRUG AND FOOD ALLERGIES


Drug allergies- the drug itself may not be an allergen, but when the drug combines with
body protein, it becomes an allergen. This is why allergic responses occur not with the initial
administration of drug but only after the protein interaction has occurred.
Reaction to drugs differ but skin manifestations seen frequently include
-urticaria
- angioedema
- allergic contact dermatitis
- pruritus
- purpura
- wheezing and rhinitis
- there may be thrombocytopenia and hemolytic anemia
- Anaphylactic shock and serum sickness may occur
- drugs used in children that are frequently involved in allergic reactions include
parenteral penicillin and vaccines.

Food allergies- manifest themselves differently from one child to another but
common
symptoms are:
- Urticaria
- Angioedema
- Pruritus
- stomach pain
- colic
- cramps, diarrhea
- respiratory symptoms and atopic dermatitis

- symptoms such as urticaria, begins to manifest itself only minutes after an


offending food is eaten.
- most common cause are egg white, fish and other seafood, berries and nuts.
Dx:
- encourage the child or parents to keep a food diary or a record of everything
the child eats each day is the best way to spot offending foods.
- An elimination diet can be used to detect food allergens. (parents feed the
child with foods that rarely cause allergy such as rice, carrots, lamb and
sweet potatoes for about 7 days. Then they add one by one at 2-3 day
intervals, foods that are suspected of causing allergy.
Mgt:
- Eliminate offending foods from clients diet.
Milk allergy
- True incidence is probably not as high as the number of diagnosis made.
- Those with lactase deficiency are commonly diagnosed incorrectly.
S&S:
- Failure to gain weight
- Diarrhea
- Vomiting
- Abdominal pain
Mgt:
- Case in hydrolysate formula (after symptoms are relieved, milk should be
introduced the later date. If the problem will recur, it is a true milk allergy)

Stinging Insect Allergy


- Severe hypersensitivity reactions from bee stings, wasps, hornets or yellow
jackets.
- Serum sickness reaction may occur. But the usual reaction is anaphylaxis.
S&S:
- Local edema at site during the first stings
- During the 2nd time, urticarial, pruritus and edema may develop.
- The 3rd time it could be so severe that shock and death results (time interval
between sting and death is very short, 10mins)
Mgt:
- Hyposensitization
- Epinephrine
- Teach children who are allergic to stinging insects not to wear scented
preparations such as hair spray, deodorants, lotions or perfumes because
they attract bees and wasps.
Contact Dermatitis
- An example of delayed or type 4 hypersensitivity response.
- It is a reaction to skin contact with an allergen.
S&S:
- Erythema
- Pruritic papules and then vesicles
Dx:
- Patch testing. Child should not take corticosteroid at the time of patch
testing because these drugs delay hypersensitivity reactions. After 48 hours,
patches are removed and reactions are graded 1+ to 4+, the same as in
regular skin testing
Mgt:
- Removing identified allergen
- Dressings wet with water, saline or Burows solution relieve itching
- Calamine or caladryl lotion is standby
- Hydrocortisone lotions or creams reduce itching and also promote healing,
- Baths with baking soda and oatmeal
- Sedative in some children to relieve their discomfort during the period of
intense pruritus.