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NCM 109

CARE OF MOTHER AND


CHILD AT RISK OR WITH
PROBLEM
SEMIRA ANN A. PANGANIBAN, RN, PHD
CHAPTER 27
Nursing Care of a Family With a
High-Risk Newborn
CHAPTER 27
NURSING CARE OF THE CHILD
BORN WITH PHYSICAL OR
DEVELOPMENTAL CHALLENGE
NURSING PROCESS
OVERVIEW
ASSESSMENT
Determining the infant’s immediate physiologic needs required to
sustain life and the parents’ immediate emotional needs to promote
bonding.

Adequate respiration An infant-parent bond


Extrauterine
Adequate stimulation
Circulation
Body Temperature Ability to take in
Stabilization adequate nutrients
Blood Sugar Ability to achieve waste
Stabilization elimination

Prevention of Infection
diagnosis
Many nursing diagnoses established for children who are physically or
developmentally challenged address the effect of the disorder on body
function, including the child’s primary needs and the family’s coping ability.

 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
Nurses play an important role in providing immediate care to high-risk
newborns at birth as well as stabilizing them until the pediatric team arrives to
assume care or transport the newborn to a high-risk nursery.
 Consider both the short- and long-term needs of the newborn and how these
needs may affect the family.
 Supportive family members can be a critical asset to parental adjustment.
 Providing nursing support as part of an interdisciplinary team, including social
workers, therapists, nutritionists, medical specialists.
implementation
Nursing interventions for a newborn who is physically challenged include
immediate life-sustaining measures such as:
 Providing oxygen
 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 lessens the
parental anxiety and enhances self-esteem.
 Provide positive role modeling when caring for the emotional and physical
needs of the newborn.
Outcome Evaluation
Outcome evaluation should focus on establishing expected outcomes for the
child’s physical and developmental health needs as well as the family’s coping
ability for current and future health of the child.

 Parent describes positive features of child by 2 weeks.


 Parents state they are comfortable with enteral feeding by 1 month.
 Child is ambulatory with walker or wheelchair by 2 years of age.
2020
NATIONAL
HEALTH
GOALS
The following 2020 National Health Goals address the
importance of prevention and therapy post-birth:
 Increase the proportion of women delivering a live birth who
took multivitamins/folic acid prior to pregnancy from a
baseline of 30.1% to 33.1%.
 Reduce the occurrence of spina bifida from 34.2 per 100,000
live births to 30.8 per 100,000 live births per year.
 Reduce the occurrence of anencephaly from 24.6 per
100,000 live births to 22.1 per 100,000 live births per year.
 Increase the proportion of children with special healthcare
needs who have access to a medical home from 47.1% to
51.8%.
 Increase the proportion of children aged 0 to 11 years with
special healthcare needs who receive their care in family-
centered, comprehensive, and coordinated systems from a
baseline of 20.4% to 22.4%; and in children aged 12 to 17
years from 13.7% to 15.1% (U.S. Department of Health and
Human Services, 2010;
Physical &
developmental
disorders (skeletal
system)
Absent or malformed
Extremities
 In most instances, however, the cause of
the anomaly is unknown.
 Children born without an extremity or
with a malformed extremity can be fitted
with prosthesis as early in life as about 6
months so the infant can learn to stand at
the normal time or handle and explore
objects readily.
THERAPEUTIC MANAGEMENT:
 Learning to use hand prosthesis takes weeks to months, and it also involves
therapy.
 Gait training for the use of lower extremity prostheses begins with the
use of parallel bars and proceeds to independent walking and mastery of
steps.
 Children who are born with an absent extremity may need help not only in
mastering the use of prosthesis but also in forming a positive body image of
them as whole.
NOTE: If possible, in the newborn period, introduce parents to the
rehabilitation team who will be following their child.
 Visiting with a child who uses prosthesis well can be a great help in
convincing parents that their child can lead a normal life.
FINGER & TOE CONDITIONS
 Polydactyly is the presence of one or more
additional fingers or toes.
 When an entire extra finger or toe forms, the
supernumerary digit is usually amputated in
infancy or early childhood.
 Syndactyly (two fingers or toes are fused), the
fusion is usually caused by a simple webbing;
separation of the digits into two sound and
cosmetically appealing ones is usually successful.
 The bones of the fingers or toes are also fused, and
cosmetic appearance and function cannot be fully
reconstructed.
THERAPEUTIC MANAGEMENT:
 Encourage them to air their feelings and concerns as they deliver the news
to family and friends.
Rationale: Because hands are so important for writing, self-care, or
computing, they may need reassurance at health maintenance visits for the
first few years of their child’s life that the child is perfect in other ways so
they can accept and help the child develop self-esteem.
Note: Identifying a skill or talent that the child excels in can provide
another avenue for developing self-esteem.
 If a child expresses a desire to learn these activities, allowing the child to
participate (if medically safe and sound) typically leads to profound
beneficial outcomes for all.
Chest deviations
 Pectus excavatum, or “funnel chest,” is an indentation of
the lower portion of the sternum.
 The concern may not be present at birth but becomes more
obvious as the child grows to school age or adolescence.
 Lung volume is apt to be decreased and the heart is
displaced to the left.
 The condition can be repaired, for either cosmetic reasons
or physiologic reasons, such as to expand lung volume.
 The sternum is displaced anteriorly, increasing the
anteroposterior diameter of the chest.
 This anomaly can be surgically corrected.
Torticollis (wry neck)
 Torticollis is a term derived from the terms
tortus (“twisted”) and collum (“neck”).
 It occurs as 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 either with a
vaginal or cesarean birth.
craniosynostosis
 It is the premature closure of the sutures
of the skull.
 This may occur in the utero or early in
infancy because of rickets or
irregularities of calcium or phosphate
metabolism.
 NOTE: This condition needs to be
detected early because early closure of
the suture line may compromise brain
growth.
Diagnosis and treatment:
 It is diagnosed with X-ray or ultrasound, which reveals the fused suture
line.
NOTE:
a) If the suture line involved in the sagittal, treatment may involve only
careful observation,
b) If the coronal suture is involved, it will need to be surgically opened
to prevent brain compression and an abnormally shaped head by 9 to
12 months.
ANCHONDROPlasia
 It is also known as chondrodystrophia where
there is a failure of bone growth inherited as a
dominant trait which causes a disorder in
cartilage production in utero.
 Because the bones of the cranium are of
membranes origin, the head continues grow
normally, causing children’s heads to appear
unusually large in contrast to their
exxtremities.
 Presence of thoracic kyphosis and lumbar
lordosis would be present and observed.
Diagnosis & THERAPEUTIC
MANAGEMENT
 DIAGNOSIS: It can be diagnosed in the utero by ultrasound or at birth X-
ray by comparing the length of extremities to usual length.
 It will also reveal characteristic abnormally flaring epiphyseal lines.
 THERAPEUTIC MANAGEMENT:
1) They may be prescribed with growth hormone or although controversial
leg lengthening may be possible.
2) Continued guidance and counseling can help them to emerge from the
period with feelings of high self esteem as adults.
Talipes disorders
 It is formed from the Latin word “talus”
(ankle) and “pes” (foot).
 It is an akle-foot disorder popularly known
as club foot.
 It implies permanent crippling to many
people and because this is no longer true
with effective surgery.
 It can be one of the four separate types:
a) Plantar flexion
b) Dorsiflexion
c) Varus Deviation
d) Valgus Deviation
assessment:
 Make a habit of strengthening all newborn feet to the middle as part of the
initial assessment of this disorder.
 Refer to the pediatric physician and orthopedist specialist to begin the
process of evaluating the infant properly.
THERAPEUTIC MANAGEMENT:
 Correction is achieved best if it is begun in the newborn period.
NOTE: A series of casts or braces are applied gradually to mold the
foot into good alignment.
 Change diapers frequently to prevent a wet diaper from touching the cast and
causing it to become soaked with urine or meconium.
 Review with parents how to check the infant’s toes for coldness or blueness and
how to blanch a toe nail bed and watch it return pink to assess for good circulation.
 Parents must evaluate crying episodes in the infant carefully.
 Infants casts must be changed or adjusted almost every 2 weeks.
 Parents may need to perform passive foot exercises such as putting the infant’s foot
and ankle to a full range motion.
Developmental dysplasia of the hip
 It is the improper formation and
function of the hip socket and is
considered a spectrum of abnormalities
affecting the hip joint.
 It is a fairly common musculoskeletal
condition found in newborns.
 The acetabalum of the pelvis is
unusually flattened or shallow.
 It is more likely to occur at breech
births, a female infant and the mother’s
first pregnancy.
assessment:
 All infants should be screened for DDH from birth and up until 3 months of
age performing the Ortolani and Barlow Manuever.
NOTE: Detecting developmental dysplasia of the hip in the newborn is
important because the longer the condition goes undetected, the more
difficult to correct.
 Affected leg may appear slightly shorter than the other because the femur
heads rides on the socket.
 Unequal number of skin folds may also be present on posterior thighs.
 Subluxated or dislocated hips are best assessed by noting whether the hips
are abduct.
THERAPEUTIC
MANAGEMENT:
 Infants less than 6 months of age are usually treated by using flexion-
abduction splinting devices.
 Brace and splints such as von Rosen, Pavlik, Craig or Frejka may be
utilized for treatment of an unstable hip and patient family preferences
should have a substantial influence of which type is chosen.
 The method of corrective and therapeutic hip surgery, which may involve
having a pin inserted to stabilize the hip.
Physical & developmental
disorders
(gi system)
Ankyloglossia (Tongue-Tie)
 Ankyloglossia is an abnormal restriction of
the tongue occurring in a small number of
newborns caused by an abnormally tight
frenulum, the membrane attached to the
lower anterior tip of the tongue
 Tongue-tie (ankyloglossia) is a 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.”
 The Hazelbaker Assessment tool can also be used to evaluate an infant’s
tongue-tie. The grading levels range from Class I (least severe) to Class IV
(most severe) and are based on the tongue’s appearance and function
decreased tongue mobility may cause short- and long-term consequences
including feeding, speech, orthodontic problems, mandibular abnormalities,
and difficulty with oral cleanliness.
Thyroglossal Cysts
 A thyroglossal cyst 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. This
condition may occur as an autosomal dominant
trait.
THERAPEUTIC MANAGEMENT:
 Observe infants closely in the immediate postoperative period for respiratory
distress because the operative area will develop at least minimum edema from
surgical trauma.
 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 somewhat and swallowing is safe once more (approximately 24
hours).
 Observe infants closely the first time they take fluid orally to be certain they do
not aspirate.
 Be certain parents have a chance to feed their infant before the infant is
discharged from the surgical unit so they can be assured the infant is swallowing
safely, thus allowing them to feel confident at feeding the infant at home in a
relaxed and comfortable way.
Orofacial Clefts: Cleft Lip And Cleft
Palate
 In infants with cleft lip, the fusion fails to
occur in varying degrees, causing this
disorder to range 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.
 Cleft lip is the most common orofacial cleft.
Orofacial Clefts: Cleft Lip And Cleft
Palate
 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.
 The incomplete closure is usually on the midline
and may involve the anterior hard palate, the
posterior soft palate, or both.
 It may occur as a separate anomaly or in
conjunction with a cleft lip. As a single entity, in
contrast to cleft lip, it tends to occur more
frequently in girls than boys.
ASSESSMENT:
 Cleft lip may be detected by a sonogram while an infant is in utero. If not
detected then, it is readily apparent on inspection of the mouth at birth.
 When assessing newborns, be sure you have good lighting so you can
visualize the palate clearly. Because cleft palate is a component of many
syndromes, assess the child for other congenital anomalies as well
THERAPEUTIC MANAGEMENT:
 If a cleft lip is discovered while the infant is still in utero, fetal surgery can repair the
condition, although this procedure is not usually attempted.
 If the disorder is discovered at birth, a cleft lip can be repaired surgically shortly thereafter,
often at the time of the initial hospital stay or between 2 and 12 weeks 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.
This type of repair results in less need for future surgery and better facial results
 Using infant orthodontic devices and delaying hard palate closure until later has not been
shown to increase speech clarity or overall wellness
Esophageal Atresias And
Tracheoesophageal Fistulas
 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:
1. The esophagus ends in a blind pouch; there is a tracheoesophageal fistula
between the distal part of the esophagus and the trachea.
2. The esophagus ends in a blind pouch; there is no connection to the trachea.
3. A fistula is present between an otherwise normal esophagus and trachea.
4. The esophagus ends in a blind pouch. A fistula connects the blind pouch of
the proximal esophagus to the trachea.
5. There is a blind end portion of the esophagus. Fistulas are present between
both widely spaced segments of the esophagus and the trachea.
ASSESSMENT:
 Esophageal atresia must be ruled out in any infant born to a woman with
hydramnios (excessive amniotic fluid). This is because hydramnios occurs
because, normally, a fetus swallows amniotic fluid during intrauterine life.
 If not diagnosed in utero, diagnosing a child who has an esophageal
atresia/tracheoesophageal fistula before the infant is first fed is important.
Otherwise, the infant will cough, become cyanotic, and have obvious
difficulty breathing as fluid is aspirated.
 Newborns that have so much mucus in their mouths that they appear to be
blowing bubbles should be suspected of having either disorder.
THERAPEUTIC
MANAGEMENT:
 The best position for the infant is an upright, 60-degree angle so acid stomach
fluids stay in the stomach through the use of gravity (sitting the baby in an
infant chair does this).
 When the infant has been stabilized by this sequence of measure, 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.
 The ultimate prognosis for children with this disorder depends on the extent of
the repair necessary, the condition of the child at the time of surgery, and the
presence or absence of other congenital anomalies. Even with larger disorders,
if surgery can be performed before pneumonia develops the repair of the
esophagus and trachea will be effective.
Umbilical Hernias
 A protrusion of a portion of the intestine
through the umbilical ring, muscle, and
fascia surrounding the umbilical cord.
 It occur most frequently in Black children
(seen in 30% term infants), low–birth-
weight infants, and more often in girls than
in boys.
 Some parents believe holding an umbilical
hernia in place by using “belly bands” or
taping a silver dollar over the area will help
reduce the hernia.
Omphalocele
 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.
 This condition occurs because, at
approximately weeks 6 to 8 of
intrauterine life,
Gastroschisis
 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.
 The condition occurs because of failure of the abdominal wall to close,
usually during the fourth week of development.
 The disorder is similar to a neural tube defect (covered later in this chapter)
because both are a failure to close at about the same gestational time.
ASSESSMENT:
 The wall defect with the herniated organs may be identified by sonogram
during intrauterine life. A prenatal sonogram is also helpful to determine the
presence of both abdominal wall and spinal disorders.
 These may also be revealed by an elevated maternal serum αfetoprotein
(MAFP) examination during pregnancy, which is done at the 15th week of
pregnancy.
 When an omphalocele or gastroschisis is identified in utero, a cesarean birth
may be performed to protect the exposed intestine. If this is the only
disorder identified, however, a vaginal birth may be preferred.
 Be certain to document the general appearance of the defect and its size in
centimeters at birth to serve as a baseline assessment.
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.
 If a gastroschisis is small, a one-stage repair may be possible. If
large, one surgical approach is the use of a 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.
 For an omphalocele, if the sac is ruptured, the defect is treated
like gastroschisis because of the potential for infection
Intestinal obstruction
 Obstruction may occur because of mesentery
of the bowel twisted as the bowel re-entered
the abdomen after being contained in the based
of the umbilical cord early in intrauterine life
or from the looseness of the intestine in the
abdomen after it was returned.
 Obstruction also occur due to thicker that usual
meconium formation, blocking the lumen
(meconium plug or meconium ileus).
ASSESSMENT:
 Anticipated if mother had hydramnios during pregnancy( swallowed fluid is not
absorbed effectively by fetus) or if more than 40 ml of stomach content can be aspirated
from the newborn stomach by catheter and syringe at birth (fluid is not passing freely to
the tract).
 No meconium had pass or may pass one time and then not pass anymore; abdomen
become distended and tender.
 Spit up in neonates is normal but vomiting with sour smell , includes bile, and
spontaneous without coughing or patting, vomitus may also be black from the color of
meconium is a sign of obstruction.
 Bowel sound increase due to effort in pushing the stool pass in obstruction. Wave of
peristalsis is observable in abdomen.
 Abdominal pain is present shoes by crying hard forceful indignant crying while pulling
the legs up the abdomen
THERAPEUTIC
MANAGEMENT:
 Insertion of orogastric and nasogastric tube and attached to low suction or
left open to the air to prevent further gastrointestinal distention.
 IV therapy to begun restore the fluid and electrolyte imbalance
 Undergo surgery to repair obstruction through laparoscopy although full
abdominal surgery may be necessary.
 Area of stenosis or atresia is removed and the bowel is anastomosed.
Meconium Plug
 Extremely hard portion of meconium that has
completely blocked the intestinal lumen,
causing bowel obstruction
 Cause is unknown , probably reflects normal
variation of meconium consistency.
 Form in the lower end of the bowel because
meconium formed early in intrauterine life
and has the best chance to become dry and
obstructive.
 Associated with hirschsprung disease, cystic
fibrosis, hypothyroidism, and magnesium
sulfate administration.
ASSESSMENT:
 Distention and vomiting, but this does not occur for atleast 24 hour.
 No meconium passage in 24 hours after post birth.
 Rectal examination may reveal presence of hardened stool although plug may
be high up in the bowel to be palpated.
 X-ray and sonogram may reveal distended air filled loops in bowel
 Barium enema reveals level of obstruction and also therapeutic in loosening
the plug.
THERAPEUTIC
MANAGEMENT:
 There is a spontaneous resolutions of the meconium plug without treatment
 Used of saline barium enema has 97%of success as a treatment
 Instillation of acetylcysteine rectally to soften stool and gastrografin as
enema.
 Child should pass stool in upcoming days if not observe the client and
assess for obstruction higher than the previous one.
Meconium ileus
 Obstruction in the intestinal lumen by
hardened meconium.
 Occurs almost exclusively to infant with
cystic fibrosis resulting from abnormal
pancreatic enzyme function seen with cystic
fibrosis.
 Symptoms are no meconium passage,
abdominal distension, and vomiting of bile
strained fluid.
 Treatment is incision and hardened
meconium is removed by laparotomy.
Meconium ileus
 Obstruction in the intestinal lumen by
hardened meconium.
 Occurs almost exclusively to infant with
cystic fibrosis resulting from abnormal
pancreatic enzyme function seen with cystic
fibrosis.
 Symptoms are no meconium passage,
abdominal distension, and vomiting of bile
strained fluid.
 Treatment is incision and hardened
meconium is removed by laparotomy.
Diaphragmatic Hernia
 Weakness of musculature that permits a
portion of the abdominal organ such as
stomach or intestine, to protrude through the
chest wall.
 Cause by early intrauterine life, the chest and
abdominal cavity is in one.
 As diaphragm is not completely develop the
intestine can herniate through the diaphragm
opening into the chest cavity as a
diaphragmatic hernia.
ASSESSMENT:
 Detected in utero by sonogram
 At birth newborn has extremely difficulty in breathing
 Scaphoid (sunken) abdominal caused by displacement of abdominal content in
chest
 Absent of breath sound in affected side due to inability to expand properly
 Cyanotic with intercostal and subcostal retraction
 Abdominal generally appear sunken
 Has potential to develop persistent pulmonary hypertension.
THERAPEUTIC
MANAGEMENT:
 Surgical repair may be done as an imminent surgical procedure. Includes
repair to diaphragm and replacement of herniated intestine and organs back
in the abdomen possibly both requiring abdominal and chest incision. If the
disorder of the diaphragm is large, an insoluble polymer patch may be used
to reconstruct a better diaphragm shape.
 Gastrochismis surgery later in life
Imperforate Anus
 Stricture or the absence of anus.
 At 7 weeks in the womb the upper bowel
elongates to pouch and combine with a
poutch invaginating from the perineum when
this two meets, membrane between them are
absorbed and the bowel is then patent outside
if this development does not happen this
causes imperforate anus.
ASSESSMENT:
 Detected by prenatal sonogram
 Discovered during birth inspection, the area is filled with black meconium
protrude from the anus .
 Wink reflex cannot elicited if sensory nerve ending in rectum are not intact
 By 24 hours no meconium pass and distention will become evident
 X-ray and sonogram detect the disorder
THERAPEUTIC
MANAGEMENT:
 It the rectum ends is close to the perineum and the anal sphincter is formed,
repair involves simple laparoscopy with anastomosis of the separated
bowel segment.
 If repair is extensive child may be given temporary colostomy and final
repair perform when the child is older (6 to 12 months).
Physical & developmental
disorders
(NERVOUS system)
HYDROCEPHALUS
 The accumulation of an excess amount of CSF
in the ventricles or the subarachnoid space.
 Thought to result from aqueductal stenosis and
blockage of the CSF outflow from the fourth
ventricle.
 Because cranial sutures are not firmly knitted in
infants, the pressure of this excess fluid causes
enlargement of their skull.
 If there is a block to CSF so it cannot circulate
into the subarachnoid space, the disorder is
termed obstructive or intraventricular
hydrocephalus.
Three Main Reasons explain why CSF accumulates:
1) Overproduction of fluid by the choroid plexus in the first or second
ventricle as could occur from a growing tumor (rare).
2) Obstruction of the passage of fluid in the narrow aqueduct of Sylvius (the
most common cause) or the foramina of Magendie and Luschka, the
openings that allow fluid to leave the fourth ventricle.
3) Interference with the absorption of CSF from the subarachnoid space if a
portion of the subarachnoid membrane has been removed.
ASSESSMENT:
 With an obstruction present, excessive fluid accumulates and dilates the system
forward of the point of obstruction.
 Symptoms may develop rapidly or slowly depending on the extent of the
atresia.
 The brow bulges forward (bossing), 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
 Neurologic, neurosurgical, urologic, and orthopedic consultations should be
considered to rule out any comorbid conditions.
 Measure the head circumference of all newborns within an hour of birth and
again before discharge from the healthcare facility to establish a baseline.
 Note any asymmetry that is occurring because this may suggest the point of
obstruction.
THERAPEUTIC
MANAGEMENT:
 The treatment for hydrocephalus depends on its cause and extent.
 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.
 As more and more obstructions in the third or fourth ventricles are relieved by
endoscopy, the next generation of children with isolated hydrocephalus may
not need artificial shunting.
 A shunting procedure involves threading a thin polyethylene catheter under
the skin from the ventricles to the peritoneum (a ventriculoperitoneal shunt)
Neural Tube Disorders
 The term “neural tube defect” (NTD) is used to
describe many distinctly different malformations of
the neural tube .
 Because the neural tube forms in utero first as a flat
plate and then molds to form the brain and spinal
cord, it is susceptible to malformation
 The term spina bifida (Latin for “divided spine”) is
most often used as a collective term for all spinal
cord disorders, but there are well-defined degrees of
spina bifida involvement, and not all neural tube
disorders even involve the spinal cord
Types of NEURAL
TUBE DEFECTS
Anencephaly
 It is the absence of the cerebral hemispheres. It
occurs when the upper end of the neural tube fails to
close in early intrauterine life. It is revealed by an
elevated level of MAFP, amniocentesis, or a
prenatal sonogram.
 Accommodate the needs of the family as best as
possible, especially if the mother chooses to birth
her baby. Allowing the parents to hold their
newborn and to take photos can help with the
grieving process. Many families opt to name their
child and hold a funeral after the infant passes. This
again can be helpful in their grieving process.
Microcephaly
 It is a disorder in which the fetal brain grows
so slowly that it falls more than three standard
deviations below normal on a growth chart at
birth.
 The cause might be a disorder in brain
development associated with an intrauterine
infection such as rubella, cytomegalovirus, or
toxoplasmosis.
 A pregnant woman infected with Zika can
pass the virus to her fetus, causing a range of
certain birth defects, including microcephaly
Spina Bifida Occulta
 It 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, the mildest form of all
NTDs, occurs when the posterior laminae of
the vertebrae fail to fuse
Meningocele
 The spinal cord is protected by three layers of
meninges or membranes: the pia mater, the
arachnoid, and the dura mater.
 The protrusion generally occurs in the lower
lumbar and lumbosacral region, although it
might be present anywhere along the spinal
canal.
 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
 This is the defect that most people think of
when they say “spina bifida” because it is the
most common birth defect affecting the
central nervous system and is frequently
viewed as the most complicated birth defect
compatible with survival
 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.
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.
 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
ASSESSMENT:
 Neural tube disorders may be discovered during intrauterine life by prenatal
ultrasound, fetoscopy, amniocentesis (discovery of increased AFP in amniotic
fluid), or analysis of MAFP.
 If the condition is discovered in utero, it may be possible to close the lesion by
fetoscopic surgery.
 An infant may be born by cesarean birth to avoid pressure and injury to the
spinal cord. It is generally difficult to tell from visual appearance whether the
disorder is a meningomyelocele or the simpler meningocele.
THERAPEUTIC MANAGEMENT:
 They should also receive adequate patient education so that they can
recognize more serious symptoms as the child grows such as numbness,
weakness, or pain, which might indicate a need for re-evaluation.
 Treatment for a meningocele or encephalocele involves immediate surgery to
replace the meninges and to close the gap in the skin to prevent infection
 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.
 Serious levels of sleep apnea may also occur (Fuller, Sinha, Caruso, et al.,
2016), which require surgical intervention

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