Nursing Care of

the Child Born

with a Physical
or
Developmental
Challenge
Factum per
Anna Irish Belen, RN MSN
Objectives:
1. Identify and describe common physical and developmental disorders that occur in newborns, infants, and
children.
2. Assess and formulate nursing diagnoses for newborns who are born physically or developmentally challenged
and establish expected outcomes to meet their needs by implementing nursing interventions.
3. Evaluate expected outcomes to determine achievement and effectiveness of care.
4. Assist parents and other family members to manage transitions across differing healthcare settings.
ASSESSMENT
 The immediate established physiologic needs required to sustain life and the parents’ immediate emotional needs
to promote bonding.
 Adequate respiration
 • Extrauterine circulation
 • Body temperature stabilization
 • Blood sugar stabilization
 • Prevention of infection
 • An infant–parent bond
 • Adequate stimulation
 • Ability to take in adequate nutrients
 • Ability to achieve waste elimination
NURSING DIAGNOSIS
 Examples of possible diagnoses include:

 • Imbalanced nutrition, less than body requirements, related to inability to take in

 adequate nutrition secondary to a physical challenge
 • Impaired physical mobility related to congenital anomaly
 • Risk for impaired parenting related to the birth of child with a congenital anomaly
 • Anticipatory grieving (parental) related to loss of the idea of the “perfect” child
OUTCOME IDENTIFICATION AND PLANNING

 Providing immediate care to high-risk newborns at birth

 Stabilizing them until the pediatric team arrives to assume care or transport the newborn to a
high-risk nursery.
 Consideration of the family’s resources, both emotional and financial.
 Consideration of both the short- and long-term needs of the newborn and how these may affect
the family.
 Providing nursing support as part of an interdisciplinary team, including social workers,
therapists, nutritionists, medical
IMPLEMENTATION
 Providing oxygen or adequate intake of nutrients when a disorder prevents the infant from
establishing respirations or sucking.
 Encouraging skin-to-skin contact and interacting with the newborn promotes infant–parent bonding.
 Educating the parents about procedures the infant may undergo.
 Parents experience the same stages of grief: denial, anger, bargaining, depression, and adjustment as
those whose child has died at
 Provide positive role modeling when caring for the emotional and physical needs of the newborn.
OUTCOME EVALUATION
 Child’s physical and developmental health needs
 The family’s coping ability for current and future health of the child.
 Addressing the family concerns
 Providing resources to support the family during and after discharge.
NICHOLAS JAMES “NICK” VUJICIC
ZION ZACHARIAH CLARK
HOW CAN YOU HELP
 Involve in therapy
 Help parents to think of interesting activities when introducing the prosthesis so the child can
immediately see how useful it will be to use.
 Gait training for the use of lower extremity prostheses
 Suggesting activities the child needs to walk to do offers motivation for trying to use the prosthesis.
 Help them in forming a positive body image of themselves as whole.
 Visiting with a child who uses a prosthesis well can be a great help in convincing parents that their child
can lead a normal life.
PHYSICAL AND DEVELOPMENTAL DISORDER
OF THE SKELETAL SYSTEM
 ABSENT OR MALFORMED EXTREMITIES

 Maternal drug ingestion or

 Virus invasion or
 Amniotic band formation in utero
 Unknown.
FINGER AND TOE
 Polydactyly is the presence of one or more additional fingers or toes. Fingers are
often just cartilage or skin tags, and removal is simple.
 Syndactyly is when two fingers or toes are fused. Cosmetic surgery is usually successful.
In other instances, the bones of the fingers or toes are also fused, and cosmetic appearance and
function cannot be fully reconstructed.
 The Nurse can…
 Encourage them to air their feelings and concerns as they deliver the news to family and friends.
 Reassurance at health maintenance visits that the child is perfect in other ways so they can accept
 Help the child develop self-esteem
 Identifying a skill or talent that the child excels in can provide another avenue for developing self-
esteem.
 CHEST DEVIATIONS
 Pectus Excavatum, or “funnel chest,” is an indentation of the lower portion
of the
sternum. It is the most common congenital deformity of the anterior chest, occurs
in. The concern may not be present at birth but becomes more obvious as the
child grows to school age or adolescence.
As a result of the deformity, lung volume is apt to be decreased and the heart is
displaced to the left.
 With Pectus Carinatum the sternum is displaced
anteriorly, increasing the anteroposterior diameter of the
chest.
TORTICOLLIS (WRY NECK)
 Torticollis is a term derived from the terms tortus (“twisted”) and collum (“neck”).
Torticollis (wry neck) occurs as a congenital anomaly when the sternocleidomastoid
muscle is injured The infant holds the head tilted to the same side as the muscle that is
involved; the chin rotates to the opposite side. A thick mass over the muscle can usually
be palpated at that time.
How can a nurse help?

 The nurse should teach the mother to perform baby neck stretching exercises.

 Help parents to understand that their actions are important therapy. In the few instances
in which simple exercises are not effective and the condition still exists at 1 year of age,
surgical correction followed by a neck immobilizer may be necessary.
CRANIOSYNOSTOSIS
 Craniosynostosis is the premature closure of the sutures of the skull. This may occur in
utero or early in infancy because of rickets or irregularities of calcium or phosphate
metabolism; it also occurs as a dominantly inherited trait and occurs more often in boys
than in girls.

What Nurses can do:

 Observe the head circumference for all infants

Craniosynostosis is diagnosed by X-ray or ultrasound, which reveals the fused

suture line. If the suture line involved is the sagittal, treatment may involve only careful
observation; if the coronal suture line is involved, it will need to be surgically opened to
prevent brain compression and an abnormally shaped head by 9 to 12 months
Sagittal suture line is the one that closes prematurely, the child’s head tends to grow
anteriorly and posteriorly. If the coronal suture line fuses early, the orbits of the eyes
become misshapen and the increased intracranial pressure may lead to eye disorders.
exophthalmos, nystagmus, papilledema, strabismus, and atrophy of the opticnerve with consequent loss of vision.
ACHONDROPLASIA
 Achondroplasia (chondrodystrophia) is a failure of bone growth
inherited as a dominant trait, which causes a disorder in cartilage
production in utero. The epiphyseal plate of long bones cannot produce
adequate cartilage for longitudinal bone growth; this results in both arms
and legs becoming stunted. Because the bones of the cranium are of
membranous origin, the head continues to grow normally,
 can be diagnosed in utero by ultrasound or at birth by X-ray by
 comparing the length of extremities to the usual length.
 Children with achondroplasia rarely reach a height of more than 4 ft 6 in.
Nursing Responsibilities:
 There is a high probability their children will inherit the disorder.
 This can make adolescence a particularly difficult time for these children.
 Both some occupational and reproductive options may be limited for them.
 Continued guidance or counseling can help them to emerge from this period with feelings of high
self-esteem as adults
TALIPES DISORDERS
 The word talipes is formed from the Latin talus (“ankle”) and pes (“foot”).
 Permanent crippling to many people
 Some newborns who appear to have a talipes disorder actually have only an unusual foot position (a
pseudotalipes) that developed because of their cramped intrauterine position.

Nursing Care:

 Be certain to demonstrate to parents that, if a pseudodisorder is present, the foot can easily be brought
into line or is not deformed.
 Stretching the foot into line every day will solve the problemin a short time.
A true talipes disorder can be one of four separate types:
 Varus deviation or valgus deviation
 Most children with talipes deformities have a combination of these conditions or have
an equinovarus or a calcaneovalgus disorder (a child walks on the heel with the foot
everted).
Assessment

 The earlier a true disorder is recognized, the better will be the correction. Make a habit, therefore, of
straightening all newborn feet to the midline as part of the initial assessment to detect this disorder

Therapeutic Management
 Correction is achieved best if it is begun in the newborn period.
 For correction, a series of casts or braces are applied to gradually mold the foot into good alignment
 The cast or brace extends above the knee to ensure a firm correction (every 1 or 2 weeks; 6 weeks)
 Changing of diapers
 Assess for good circulation
DEVELOPMENTAL DYSPLASIA OF THE HIP
 Congenital hip dysplasia is improper formation and function of the hip socket and is considered a spectrum of
abnormalities affecting the hip joint.
 Common musculoskeletal condition found in newborns, with the prevalence estimated to range from 1.6 to 28.5
per 1,000 infants, averaging about 5% of newborns
 Leading cause of orthopedic disability in childhood and adult life because it can lead to premature arthritis
requiring hip replacement
 Dislocation of the head of the femur
CAUSE:

 Unknown
 may be from a polygenic inheritance pattern
 It may also occur from a uterine position that causes less-than-usual pressure of the femur head on the
acetabulum.
 more likely to occur with breech birth, a female infant
 It is usually unilateral and found 6 times more frequently in girls than in boys,

 Additional risk factors include family history of DDH, oligohydramnios, large birth weight for gestational age

 Generally plain radiography (X-ray) is not as effective in children less than 6 months of age due to
musculoskeletal immaturity and unreliability to accurately screen for DDH
Assessment
 performing the Ortolani & Barlow maneuver up to 3 months

Therapeutic Management
 Close monitoring in mild DDH cases because 60% to 80% of clinically identified abnormalities and
90% of ultrasonographic abnormalities spontaneously resolve without treatment in early infancy.
 Flexion-abduction splinting devices.
 Positioning the hip into a flexed, abducted (externally rotated) position to press the femur head
against the acetabulum and cause the acetabulum to deepen its contour from the pressure.
 Corrective and therapeutic hip surgery, which may involve having a pin inserted to stabilize the hip.
 Pavlik harness or Spica cast
PHYSICAL DEVELOPMENT DISORDERS OF
THE GASTROINTESTINAL SYSTEM
 Most common congenital anomalies of the gastrointestinal system, such as
cleft lip and cleft palate, occur because of midline closure failure extremely early in
intrauterine life.
ANKYLOGLOSSIA
 An abnormal restriction of the tongue occurring by an abnormally tight frenulum, the membrane
attached to the lower anterior tip of the tongue
 Congenital anomaly in which a tight or shortened lingual frenulum causes restricted tongue
mobility and impaired tongue function.

ASSESSMENT
 Tongue tie is often evaluated based on the mobility and how close to the tip of the tongue the
leading edge of the frenulum is attached.
 Types 1 and 2 are the most common, accounting for 75% of tongue-ties and are often called
“classic” and “anterior ties.”
 Types 3 and 4 account for remaining 25 % and are often referred as “posterior ties.”

 frenotomy release
THYROGLOSSAL CYSTS
 Arises from an embryogenic fault that leaves a cyst formed at the base of the tongue, which then
drains through a fistula (an abnormal or surgically made passage between a hollow or tubular organ
and the body surface, or between two hollow or tubular organs) to the anterior surface of the neck
 Thyroglossal cysts are congenital defects located in or around the midline of the neck, extending to the
base of the tongue.
 The cyst may involve the hyoid bone (the bone at the anterior surface of the neck at the root of the
tongue) and may contain aberrant thyroid gland tissue.
 As the cyst fills with fluid, swelling and obstruction can lead to respiratory difficulty from pressure on
the trachea.
 If infected, the cyst often appears swollen and reddened, with drainage of mucus or pus from the
anterior neck and requires antibiotic therapy.
 Management:

 Removal of csyt
 Position infants on their sides so secretions drain freely from their mouths.
 Intravenous (IV) fluid therapy is given after surgery until the edema at the incision recedes
 If the mother is breastfeeding, encourage her to express her milk via a hospital-grade pump or manual
expression
 Be certain parents have a chance to feed their infant before the infant is discharged from the surgical
unit.
 OROFACIAL CLEFTS: CLEFT LIP AND CLEFT PALATE
 The maxillary and median nasal processes normally fuse between weeks 5 and 8 of intrauterine life.
 The disorder ranges from a small notch in the upper lip to total separation of the lip and facial structures up into
the floor of the nose, with even the upper teeth and gingiva absent.
 The deviation may be unilateral or bilateral. The infant’s nose generally appears flattened because the
incomplete fusion of the upper lip has allowed it to expand in a horizontal dimension
 It is more prevalent among boys than girls
 It appears to be caused by the transmission of multiple genes aided by teratogenic factors present during weeks 5 to
8 of intrauterine life, such as a viral infection, certain seizure medicines such as phenytoin, maternal smoking or
binge drinking, hyperthermia, stress, and maternal. Folic acid deficiency may also be associated with incomplete
anterior midline closures.

 A cleft palate is an opening of the palate and occurs when the palatal process does not close as usual at
approximately weeks 9 to 12 of intrauterine life.
Assessment
 Ultrasound
 Inspection of the mouth at birth

Therapeutic Management
 Surgery can repair the condition, between 2 and 12 weeks of age. Because the deviation of the lip
 Early repair also helps infants experience the pleasure of sucking and increases speech development as
soon as possible.
 Parent may need caring support to bond with an infant whose face is deformed in this way
 Nasal rhinoplasty to straighten a deviated nasal septum may be necessary when the child reaches 4 to 6
years of age.
 Surgery may be recommended as a two-stage palate repair, with soft palate repair at 3 to 6 months of age
and hard palate repair at 6 to 18 months of age, called the Malek protocol

 (NPO) for approximately 4 hours

 HOW CAN NURSES HELP:
 Offering small sips of fluid between feedings can help keep the mucous membranes moist and prevent cracks
 Following a feeding, be certain an infant with a cleft lip is burped well
 POST SURGERY – no spoon, no milk, infants may need their mouth suctioned to remove mucus, blood, and
unswallowed saliva.
 Nursing Diagnosis: Risk for ineffective airway clearance related to oral surgery
 Outcome Evaluation: Child’s respiratory rate remains at baseline level for age without retractions or obvious
distress.
 Nursing Diagnosis: Impaired tissue integrity at incision line related to cleft lip or cleft palate surgery
 Outcome Evaluation: Incision line appears clean and intact and free of erythema or drainage during postoperative
period.
 Nursing Diagnosis: Risk for infection related to surgical incision
 Outcome Evaluation: Infant’s temperature is below 98.6°F (37°C) by tympanic membrane; incision site is clean,
dry, and intact without erythema or foul drainage.
 Nursing Diagnosis: Risk for impaired parenting related to birth of an infant who is physically challenged
 Outcome Evaluation: Parents state they believe there will be a positive outcome for their child and demonstrate
positive coping behaviors evidenced by holding and helping with infant care.
THE PIERRE ROBIN SEQUENCE
 Pierre Robin syndrome is a rare triad of micrognathia (small mandible), cleft palate, and glossoptosis
(a tongue malpositioned downward). This triad of conditions can result in severe upper airway
obstruction,

 Unknown cause
 children born with Pierre Robin syndrome have associated disorders of congenital glaucoma; cataracts;
cardiac disorders; or other genetic, chromosomal, or medical concerns.
What can you do:
 Monitor infants for possible airway obstruction (as a result of their small jaw, their tongue tends to
drop backward and obstruct their airway).
 Suctioning to remove secretions may be necessary.
 A side-lying rather than a supine sleeping position
 The airways of older children can be well maintained using nasal continuous positive airway pressure
(CPAP); obstructive sleep apnea decrease.

 The jaw will grow somewhat, although the mandible will always be small. Parents need instructions
on how to feed these infants until the cleft palate can be repaired. The child may have a gastrostomy
tube or button inserted to relieve feeding difficulty
ESOPHAGEAL ATRESIAS AND TRACHEOSOPHAGEAL FISTULA
 Between weeks 4 and 8 of intrauterine life, the laryngotracheal groove in the chest develops into the
larynx, trachea, and beginning lung tissue; the esophageal lumen forms parallel to this. A number of
anomalies can occur if the trachea and esophagus are affected by some teratogen that does not allow the
two organs to fully form separately and possibly become connected.
 Esophageal atresia is the incomplete formation of the esophageal lumen, resulting in the proximal
(upper) esophagus forming a “blind pouch,” which then does not connect to any other structure.
 Tracheoesophageal fistula occurs when an opening develops between the closed distal (lower)
esophagus and the trachea.
Five usual types of esophageal atresia that result are:
Assessment
 Esophageal atresia must be ruled out in any infant born to a woman with hydramnios (excessive
amniotic fluid).
 diagnosing a child who has an esophageal atresia/tracheoesophageal fistula before the infant is first
fed is important. The infant will cough, become cyanotic, and have obvious difficulty breathing as
fluid is aspirated.
 Newborns who have so much mucus in their mouths that they appear to be blowing bubbles should
be suspected of having either disorder.
 Esophageal atresia can be diagnosed with certainty if a catheter cannot be passed through the
infant’s esophagus
 An X-ray will reveal a radiopaque catheter coiled in the blind end of the esophagus.
 Ultrasound also may reveal a stomach distended with the air that has passed from the trachea into
the esophagus and stomach, demonstrating that a fistula is present.
 Either a barium swallow or a bronchial endoscopy examination can also reveal the blind-end
esophagus and fistula.
Therapeutic Management
 Emergency actions are required for the infant with this group of esophageal disorders in order to
prevent the development of pneumonia, one of the major complications that is apt to occur from
leakage of oral or stomach secretions into the lungs.
 The best position for the infant is an upright, 60-degree angle so acid stomach fluids stay in the
stomach
 Nasogastric tube can be inserted into the distal end of the esophagus and connected to low intermittent
suction, oral secretions will no longer collect in the blind pouch, decreasing aspiration risks.
 IV fluids and parenteral nutrition are begun to prevent dehydration and an electrolyte imbalance from
lack of oral intake.
 IV medications that restrict the stomach’s ability to produce acid may be prescribed, and an antibiotic
may be prescribed to help prevent an infection.
 Surgery consists of closing the fistula and anastomosing the esophageal segments. If the defect was
only a simple fistula, surgery may be done by thoracoscopy to minimize the time required for surgery
 If the repair will be more complex, it may be necessary for the surgery to be completed in different
stages and to use a portion of the intestine to complete the anastomosis if the esophageal segments are
too far apart to join readily.
 After an anastomosis repair, observe infants closely at postoperative days 7 to 10 because this is the
point when their internal sutures will dissolve, possibly allowing a leak from the anastomosis site to
occur at this time. If such a leak occurs, fluid and air can leak out into the chest cavity, and
pneumothorax (collapse of a lung) can occur.
 Nursing Diagnosis: Risk for imbalanced nutrition, less than body requirements, related to inability to take in
oral feedings
 Outcome Evaluation: Child maintains weight within 10% of birth weight and maintains weight in same
percentile on growth curve.
 Nursing Diagnosis: Risk for infection related to aspiration or seepage of stomach secretions into lungs
 Outcome Evaluation: Child’s temperature remains below 98.6°F (37°C) axillary; here is absence of rales on
auscultation.
 Postoperative Care. The baby will return from final surgery with multiple tubes (e.g., chest tubes, endotracheal
tube, Foley catheter, multiple IV lines, probably an arterial line, possibly a gastrostomy tube if not previously
inserted), oxygen saturation, and electrocardiography monitoring equipment. The posterior chest tube drains
collecting fluid, and the anterior tube allows air to leave the chest space so the lungs can reexpand
ABDOMINAL WALL DEFECTS
 Umbilical Hernias is a protrusion of a portion of the intestine through the umbilical ring, muscle, and
fascia surrounding the umbilical cord. This creates a bulging protrusion under the skin at the umbilicus. It is
rarely noticeable at birth while the cord is still present but becomes increasingly noticeable at healthcare visits
during the first year.
 low–birth-weight infants, and more often in girls than in boys

The size of the protruding mass is not as important as the size of the fascial ring through which the intestine
protrudes. If this fascial ring is less than 2cm, closure will usually occur spontaneously after the child begins to walk
to around 2 years of age, so surgical repair is often not necessary for mild (small) cases.
 Omphaloceles is a protrusion (herniation) of abdominal contents through the abdominal wall at the
point of the junction of the umbilical cord and abdomen.
 The herniated organs involved are usually the intestines, but they may include the stomach and liver.
 The organs are usually covered and contained by a thin transparent layer of amnion and chorion with the
umbilical cord protruding from the exposed sac.
 This condition occurs because, at approximately weeks 6 to 8 of intrauterine life, the fetal abdominal contents,
which grow faster than the fetal abdomen, are pushed out from the abdomen into the base of the umbilical cord.
 At 7 to 10 weeks, when the fetal abdomen has enlarged sufficiently, the intestine normally returns to the
abdomen.
 An omphalocele occurs when abdominal contents fail to return in the usual way.
 Gastroschisis a term derived from the Greek word for “stomach cleft” or “fissure,”
is a condition similar to an omphalocele, except the abdominal wall disorder is a
distance from the umbilicus, usually to the right, and abdominal organs are not
contained by a membrane but rather spill freely from the abdominal wall

 Also, a greater amount of intestinal contents tends to herniate, increasing the

potential for volvulus and obstruction.

 The condition occurs because of failure of the abdominal wall to close, usually
during the fourth week of development,
Therapeutic Management

 With both omphalocele and gastroschisis, until surgery and the bowel is effectively returned to the abdomen, the
infant will be fed by TPN to supply nutrients and keep the bowel from filling with air or stool.
 Most infants with gastroschisis will have surgery within 24 hours to replace the bowel before the blood supply
becomes hampered, the intestinal membranes dry, bowel volvulus occurs, or the bowel becomes infected.
 prosthetic patch repair that bridges the unformed gap on the abdomen with a synthetic material; the skin is then
drawn tight and closed over the patch.
 A second approach is to replace only a portion of the bowel at one time. The remainder is contained by a Silastic
pouch termed a “silo” that is suspended over the infant’s bed.
ICE BREAKER
 Maia tells the nurse her baby was born on an unlucky day because the baby
born before hers had his bowel outside his body. What is the most important
nursing consideration at birth in the care of a baby with gastroschisis?

 a. Position the infant on his or her stomach so the intestine is well contained.
 b. Wrap the intestines with chilled gauze to prevent intestinal swelling.
 c. Keep the infant seated upright under a radiant warmer for warmth.
 d. Contain the intestine in a warmed, sterile saline–lined bowel bag.
INTESTINAL OBSTRUCTIONS
 If canalization of the intestine does not occur in utero at any point, an atresia (complete closure) or stenosis
(narrowing) of the fetal bowel can develop, although the most common site is the duodenum.
 This twisting pattern is termed a volvulus and continues to be a potential problem for the first 6 months of life until
the infant develops firmer intestinal supports.
 Yet another reason obstruction can occur is because of thicker-than-usual meconium formation, blocking the lumen
(meconium plug or meconium ileus).
 Mesentery
Assessment
 If more than 30 ml of stomach contents can be aspirated from the newborn stomach by catheter and
syringe at birth (fluid is not passing freely through the tract).
 The infant passes no meconium or may pass one stool
 The abdomen becomes distended and tender.
 the infant will vomit.
 True vomiting from intestinal obstruction is usually sour smelling (stomach acid has acted on it),
 Vomitus may also be black from the color of meconium.
 The infant may reveal that abdominal pain is developing by crying
 The child’s respiratory rate will increase as the intestine fills, the diaphragm is pushed up against the
lungs, and lung capacity decreases.
Management
 As soon as a bowel obstruction is confirmed, an orogastric or nasogastric tube is inserted and then
attached to low suction or left open to the air to prevent further gastrointestinal distention.
 IV therapy is begun to restore fluid and electrolyte balance; immediate surgery is scheduled to relieve the
obstruction
 The area of stenosis or atresia is removed, and the bowel is anastomosed.
MECONIUM PLUG SYNDROME
 A meconium plug is an extremely hard portion of meconium that has completely blocked the
intestinal lumen. Meconium plugs usually form in the lower end of the bowel .
Assessment
 Typically, the infant will be identified first as an infant who has had no meconium passage in the first
24 hours postbirth. A gentle rectal examination may reveal the presence of hardened stool, although
the plug may be too high up in the bowel to be palpated.
 An X-ray or sonogram may reveal distended air-filled loops of bowel up to the point of obstruction.
 A barium enema not only reveals the level of obstruction but also may be therapeutic in loosening the
plug.
MECONIUM ILEUS
 Obstruction of the intestinal lumen by hardened meconium

 The usual symptoms of bowel obstruction occur: no meconium passage, abdominal distention, and
vomiting of bile-stained fluid.

 Unlike simple meconium plugging, the obstruction point may be too high in the intestine for enemas to
reduce it; instead, the bowel must be incised and the hardened meconium removed by laparotomy.
DIAPHRAGMATIC HERNIAS
 A diaphragmatic hernia is a weakness in the musculature that permits a portion of the abdominal organs, such as
the stomach or intestine, to protrude through the chest wall.
 This can cause a collapse of the left lung due to cardiac displacement on the right side of the chest
 Increased frequency in boys and a decreased frequency in Blacks
 CAUSE: early in intrauterine life, the chest and abdominal cavity are one; at approximately week 8 of
intrauterine growth, the diaphragm forms and divides them. If the diaphragm does not form completely, the
intestines can herniate through the diaphragm opening into the chest cavity
Assessment
 At birth, breath sounds are usually absent on the affected side of the chest cavity
 The infant may be cyanotic with intercostal or subcostal retractions.
 The abdomen generally appears sunken because it is not as filled with intestine as usual.

Therapeutic Management
 Surgery includes repair of the diaphragm and replacement of the herniated intestine and organs back
into the abdomen, possibly requiring both thoracic and abdominal incisions.
 The repair can be complicated if there is not enough room in the abdomen for the intestine to be
returned. In these infants, the abdominal incision may not be closed.
 If the lung that was compressed in utero and cannot function, it will be removed at the time of
surgery. If it is developed but just deflated, over the next week after surgery, the lung will gradually
expand and begin to function.
IMPERFORATED ANUS

 is a stricture or the absence of the anus.

 The disorder can be relatively minor, requiring just surgical incision of the persistent membrane,
 There may bean accompanying fistula to the bladder in boys and to the vagina in girls (retrovaginal
fistula)
Assessment
 The condition may be detected by a prenatal sonogram.
 It is discovered at birth when inspection of a newborn’s anal region reveals no anus, a membrane filled
with black meconium protrudes from the anus,
 A “wink” reflex (touching the skin near the rectum should make the anus contract) cannot be elicited if
sensory nerve endings in the rectum are not intact.
 By 24 hours, no stool will be passed, and abdominal distention will become evident.
 If not, they may be asked to collect a urine specimen so it can be examined for the presence of
meconium to help determine whether the infant has a rectal–bladder fistula.
Therapeutic Management
 The degree of difficulty in repairing an imperforate anus depends on the extent of the problem.
 If the rectum ends close to the perineum and the anal sphincter is formed, repair involves simple laparoscopy with
anastomosis of the separated bowel segments
 A repair becomes complicated if the end of the rectum is at a distance from the perineum, the anal sphincter exists
only in an underdeveloped form, or a fistula to the bladder or vagina is present.
 If the repair is estimated to be extensive, the child may be given a temporary colostomy, and the final repair
performed when the infant is somewhat older (6 to 12 months).
PHYSICAL AND DEVELOPMENTAL DISORDER
OF THE NERVOUS SYSTEM
 The most common developmental disorders of the nervous system at birth include abnormal accumulation
of cerebrospinal fluid (CSF) which has several causes, and abnormalities associated with neural tube
closure (meningocele or meningomyelocele).
HYDROCEPHALUS
 CSF is formed in the first and second ventricles of the brain and then passes through the aqueduct of Sylvius and the fourth
ventricle to empty into the subarachnoid space of the spinal cord, where it is absorbed.
 Hydrocephalus is the accumulation of an excess amount of CSF in the ventricles or the subarachnoid space
 the pressure of this excess fluid causes enlargement of their skull.
 If fluid is able to reach the spinal cord, the disorder is called a communicating or extraventricular hydrocephalus.
 If there is a block to CSF so it cannot circulate into the subarachnoid space, the disorder is termed obstructive or
intraventricular hydrocephalus.
 Hydrocephalus can occur at birth (congenital) or) from an incident later in life (acquired).
 90% of babies with congenital hydrocephalus have a diagnosis of meningomyelocele
Reasons for CSF accumulation:

 Overproduction of fluid by the choroid plexus in the first or second ventricle

 Obstruction of the passage of fluid in the narrow aqueduct of Sylvius (the most common cause) or the
openings that allow fluid to leave the fourth ventricle.
 Hemorrhage from trauma, a growing tumor, or infections such as, meningitis, or encephalitis may
leave adhesions behind that block fluid flow at these points.
 Interference with the absorption of CSF from the subarachnoid space
ASSESSMENT
 As the fluid accumulation continues, the scalp becomes shiny and scalp veins become prominent.
 The brow bulges forward, and the eyes become “sunset eyes” (the sclera shows above the iris because of upper lid
retraction).
 Infants begin to show symptoms of increased intracranial pressure, such as decreased pulse and respirations,
increased temperature and blood pressure, hyperactive reflexes, strabismus, and optic atrophy.
 They may become either irritable or lethargic with a typical shrill, high-pitched cry
Therapeutic Management
 If it is caused by overproduction of fluid, acetazolamide (Diamox), a diuretic, may be prescribed to
promote the excretion of this excess fluid.
 Laser surgery to reopen the route of flow or bypassing the point of obstruction by shunting the fluid to
another point of absorption.
 A shunting procedure involves threading a thin polyethylene catheter under the skin from the ventricles
to the peritoneum (a ventriculoperitoneal shunt). Fluid drains by this route into the peritoneum, where it
is absorbed across the peritoneal membrane into the body circulation.
 A ventriculoperitoneal shunt removes excessive cerebrospinal fluid from the ventricles and shunts it to the peritoneum.
 A one-way valve is present in the tubing behind the ear.
NEURAL TUBE DISORDERS

 malformations of the neural tube

 the neural tube forms in utero first as a flat plate and then molds to form the brain
and spinal cord
 The term spina bifida (Latin for “divided spine”) is most often used as a collective
term for all spinal cord disorders,
 lack Of fusion of the posterior surface of the embryo in early intrauterine life.
Types of Neural Disorders
Anencephaly is the absence of the cerebral hemispheres. It occurs when the upper end of the neural tube fails to close in
early intrauterine life.
 Labor with an infant with anencephaly may be prolonged because the infant may present in a breech position or the
underdeveloped head may not engage the cervix well.
 Because the respiratory and cardiac centers are located in the intact medulla, infants may survive for several days
after birth but cannot survive further because they have little or no cerebral function.
Microcephaly
 Microcephaly is a disorder in which the fetal brain grows so slowly
 The cause might be a disorder in brain development associated with an intrauterine
infection.
 A pregnant woman infected can pass the virus to her fetus, causing a range of certain
birth defects, including microcephaly
 Microcephaly may also result from severe malnutrition
 The prognosis for a normal life is guarded in children with microcephaly and depends on
the extent of restriction of brain growth and on the cause. Generally, the infant is
cognitively challenged because of the lack of functioning brain tissue.
 True microcephaly must be differentiated from craniosynostosis (normal brain growth but
 premature fusion of the cranial sutures), which also causes decreased head circumference
but is curable.
Spina Bifida Occulta
 the mildest form of all NTDs, occurs when the posterior laminae of the vertebrae fail to fuse.
 Spina bifida occulta is a malformation caused by nonclosure or incomplete closure of the posterior
portion of the vertebrae.
 This occurs most commonly at the fifth lumbar or first sacral level but may occur at any point along
the spinal canal.
 Spina bifida occulta may be first noticed as a dimpling at the point of poor fusion; abnormal tufts of
hair or discolored skin may also be present
defect for them: It simply means a surface of bone is missing, and the spinal cord is intact.
Meningocele

 The spinal cord is protected by three layers of meninges or membranes: the pia mater, the arachnoid, and the dura
mater.
 If these membranes herniate through an unformed vertebrae, they protrude as a circular mass, about the size of an
orange, at the center of the back
 The protrusion generallyoccurs in the lower lumbar and lumbosacral region, although it might be present
anywhere along the spinal canal. The protrusion is either covered by a layer of skin or, more frequently, only the
clear dura mater.
 No sensory or motor deficits accompany the disorder unless the membrane sac should rupture, but damage to the
cord could occur or infection could enter the now unprotected CSF.
Meningomyelocele

 The most common birth defect affecting the central nervous system and is frequently viewed as the
most complicated birth defect compatible with survival
 not just the meninges protrude through the vertebrae, but the spinal cord usually ends at the point of
protrusion.
 Motor and sensory function will be decreased or absent beyond this point.
 Generally, the higher the defect is along the spine, the greater the degree of paralysis.
 The child will have partial or complete paralysis, partial or complete lack of sensation of the lower
extremities, as well as loss of bowel and bladder control.
 The infant’s legs may appear lax because the infant cannot move them; urine and stools continually
dribble because of a lack of sphincter control.
Encephalocele
 An encephalocele is a cranial meningocele. These occur most often in the occipital area of the skull but may
occur as a nasal or nasopharyngeal disorder.
 Encephaloceles generally are covered fully by skin, although they may be open or covered only by the dura. It is
difficult to tell from the size of the encephalocele if only CSF is trapped in the protruding meninges or whether
brain tissue could also be involved.
 Transillumination of the sac will reveal whether brain tissue is in the sac. A CT, MRI, or ultrasound will reveal
the size of the skull disorder and help predict the extent of surgery, which will be needed.
ARNOLD–CHIARI (CHIARI II) MALFORMATION
 Categorized as Chiari I, Chiari II, or Chiari III. Chiari
 Type I malformation involves the downward herniation of the caudal end of the cerebellar vermis
through the foramen magnum.
 Children with Chiari I typically show symptoms in the second or third decade of life, so it is referred to
as an “adult type,” although current sonogram scan capabilities have allowed for earlier incidental
diagnosis.
 Chiari II and the very rare Chiari III types are present at birth and so are considered primary neural tube
abnormalities.
ASSESSMENT

 A diagnosis is made by MRI and is sometimes accidently identified via an initial MRI because the older child is
being evaluated for chronic headaches.
 The prognosis for the child with an Arnold–Chiari malformation depends on the extent of the disorder and the
surgical repair procedure possible.
 Because of the upper motor neuron involvement, gagging and swallowing reflexes may be absent, increasing the risk
for tracheal aspiration.
 Serious levels of sleep apnea may also occur.

Overall prognosis is encouraging.

 A lumbar puncture (LP) should never be performed on a child with Arnold–Chiari malformation for risk of a
brainstem herniation
THANK YOU!
Sources

 Maternal and Child Health Nursing

JoAnna Silbert-Flagg
Adele Pillitteri