Infancy Health Problems II
•Neural Tube Defects
•Hydrocephaly
•Anencephaly
•Pyloric Stenosis
•Intusseption
•Hirschsprung’s Disease
•Tracheo Esophageal Fistula (TEF)
Neural Tube Defects
 50% or more from folic acid deficiency; others are
multifactoral etiology
 Neural tube = embryonic beginning for the brain &
spinal column
 Brain & spinal cord encased in meninges/bone
 Neural tube should close ~ 30 days after
conception; defect in closure may involve entire
length of neural tube or small area
 Most common defects: anencephaly & spina bifida
Spina Bifida or Myelodysplasia
 Failure of the bony (osseous) spine to close
 Two types:
 Spina bifida occulta – not visible externally
 Spina bifida cystica – visible, saclike protrusion
Meningocele
 Myelomeningocele – most commonly referred to by the term
Spina Bifida

Spina Bifida Occulta
 Usually lumbosacral L5-S1
 Skin indicators:
 Sacral dimple, angioma or port-wine nevus
 Sacral tufts of dark hair or lipoma
 Usually no problems unless there is an adhesion to
bony or fixed structure
Altered gait
 Bowel/bladder problems
 Foot deformities
 These usually not evident in early infancy

Myelomeningocele vs Meningocele
 Meningocele – contains meninges and spinal fluid
– no neural elements

No neurological deficits
 Myelomeningocele – also contains nerves
 Anywhere along spinal column
 Varying & serious degrees of neuro deficit
 Location & magnitude of defect determine extent of
impairment
 Below L2, flaccid paralysis of lower extremities & sensory
deficit; bowel & bladder dysfunction
 Below S3 – no motor impairment; bowel/bladder def.
Differentiating Meningocele from
Myelomeningocele
 By definition, these two neural tube
defects differ based on the presence or
absence of the spinal cord or cauda
equina within the sac at the base of the
spine.
 Meningocele—fluid only
 Myelomeningocele—fluid and spinal
cord both
What determines the severity of
Myelomeningocele??
 The degree of neurological deficit is directly related to the
level of the spinal cord defect and its extent.
 If only the bottom of the spinal cord is involved (conus),
there may be only bowel and bladder dysfunction, while
the most extensive lesions can result in total paralysis of
the legs with accompanying bowel and bladder
dysfunction.
 It is because of the varying neurological manifestations of
meningomyelocele that there has been so much
controversy regarding appropriate treatment.
Variations in Spinal Cord Anomalies
Normal
Spina bifida
occulta
Meningocele Myelomeningocele
Myelomeningocele
Lateral view
Statistics showing improvement with
Folic Acid
supplements
 Folic acid was deemed mandatory for
certain foods after 1992. Plus there was
the big educational push to have women
take supplemental Folic Acid as soon as
they thought about getting pregnant.
 CDC Stats on Folic Acid
 Folic Acid Quiz
Management
 Prevention of infection & trauma to the “sac” –
keep covered w/sterile saline dressing; monitor
closely
 Observe for early signs of infection
↑temp; no rectal for this babies!!
 Nuchal rigidity, irritability, lethargy, ↑ICP

 Prone position; no diapers
 Assess neurological deficits
 Surgical repair in 12 – 72hrs. of birth
How to manage bowel and bladder dysfunction Neurogenic bladder is one of the primary
dysfunctions that children with spina bifida
encounter.
 Reference for lay persons on management
 Reference from NIH on management
Latex Allerg;y
 All children with any neural tube defect must be
treated as if they had a LATEX ALLERGY ! See pp.
408-409 Hockenberry , 2011 9th ed.
 There are various resources available to give families
re: environmental sources of latex.

The Spina Bifida Association offers a handout with resources.
Hydrocephaly—why study it with
Neural Tube Defects
 Hydrocephaly is commonly associated with
Meningocele and Myelomeningocele.
 It is critical for nurses to assess the head
circumference of all babies with NTD daily to
note changes in the head size so that
interventions can be made to prevent
complications from  ICP.
Common signs and sx of Hydrocephaly
in neonate and infancy
 Bulging fontanel 7 separated sutures
 Increasing head circumference
 “setting sun eyes”
 Frontal bossing
 Macewen sign
 High-pitched cry
 Changes in behavior—in older child
 Nausea & vomiting—in older child
where fontanels are closed
Hydrocephalus
 Commonly seen with myelomeningocele
 80-85% will develop this
 May not be obvious at first; may appear after closure of
defect
 Critical to measure head circumference daily & palpate
fontanel for changes
 Also can be other congenital causes or acquired as
a result of infection, neoplasm or hemorrhage
 It is a syndrome that results from disturbances in
dynamics of CSF
Anatomy of the Ventricles of the Brain
 This shows a picture of the ventricles of the
brain, so that you can get a visual feel for how the
obstruction may cause increased intracranial
pressure.
Web Site Alert!!
 This web site gives lots of facts about hydrocephalus. It is
easy to read and understand.
 http://www.ninds.nih.gov/disorders/hydrocephalus
/detail_hydrocephalus.htm
What Happens??
 CSF circulates throughout the ventricular system &
is then absorbed within the subarachnoid spaces
 2 problems can occur:
Communicating hydrocephalus: impaired absorption of
CSF
 Noncommunicating hydrocephalus: obstruction to flow
of CSF occurs

 Table 11-3; p. 443 has specifics
 Either way it leads to ↑ICP and this is serious!
Ventriculo-peritoneal Shunts
 Here are some examples
of VP shunts that may
be used to help
manage hydrocephaly.
 Website on Endoscopic Third
Ventriculostomy
Hydrocephalus:
Initial Management
 Treatment of excessive CSF (shunt) –
ventriculoperitoneal (VP) most common
 Treatment of complications – mainly related to the
shunt
Infection – greatest risk first 2 mos
 Malfunction – will see ↑ICP

 Manage problems related to development

2/3 are intellectually normal
Post Op Care
 Must be alert for signs of Increased ICP
 Post op: daily measurements of FOC and palpation of
fontanels for size, signs of bulging, tenseness, separation
 Initially keep flat w/o pressure on incision, gradually HOB
elevated
 Neuro assessment, VS, I&O
 Abdominal distention (peritonitis or ileus)
 Signs of infection: temp↑, poor feeding, vomiting, seizures,
↑LOC
 Inspect incision line for redness, drainage (test any
drainage for glucose in case it is CSF)
Discharge Information
 Must teach how to recognize signs of infection and
shunt malformation
 Refer to p. 1617, Box 37-1 for summary of clinical
manifestations of ↑ICP in infants and children
 Make appropriate referrals, ie early childhood
programs, early intervention services, support
groups
What is Arnold Chiari
Malformation?
 Arnold Chiari Malformations are a
form of hydrocephaly that was
discovered by
Dr. Chiari in the 1890’s.
 Chiari I – IV malformations may be an
anomaly that people live with with
minimal sx. MRI has significantly
helped in the dx.
 Chiari Type II is often found in
children with Myelomenigocele.
 NIH website with information
Anencephaly–
What is it and how do we help those families
who have babies born with it?
 This website gives resources as well as
some explanation of what it is.
 Here are family stories of their
Children
Anencephaly
 Incompatible with life: stillborn or live hours to

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weeks
Absence of both cerebral hemispheres
Brainstem function may be intact
Cause of death usually respiratory failure
Management: comfort measures, family support,
discussion of end-of-life issues like organ donation,
possible referral to hospice
Hypertrophic Pyloric Stenosis
Def: results when the circular areas of muscle surrounding
the pylorus hypertrophy & block gastric emptying.
Etiology:
Cause unknown, heredity, possibly.
 Incident: 1 in 500 live births
 ♂( x 5) > ♀ (1)

Pathophysiology:
 Pylorus narrows because of progressive hypertrophy and
hyperplasia of the circular pyloric muscle. This leads to
obstruction of the pyloric sphincter, with subsequent
gastric distention, dilatation, and hypertrophy.
Hypertrophic Pyloric Stenosis
Pyloromyotomy, creation of an
incision along the anterior pylorus to
split the muscle, is performed to relieve
the obstruction.
Link to diagram of pyloric
stenosis and surgical incision
Hypertrophic Pyloric Stenosis
Assessment preoperatively:
 Progressive, projectile, non-bilious vomiting
occurs.
 Movable, palpable, firm, olive- shaped mass in the
right upper quadrant
 Irritability, hunger, and crying.
 Sunken fontanels, dry mucus membranes, and
decreased urine output, constipation, jaundice, &
metabolic alkalosis. (Dehydration)
 U/S and upper GI series shows delayed gastric
emptying and hypertrophied pylorus.
Hypertrophic Pyloric Stenosis
Labs:
 1.↑pH & ↑bicarbonate level = metabolic alkalosis
 2. Serum chloride, sodium, potassium ≈ decreased
 3. HCT, Hgb ↑ = hemoconcentration
Management:
 Preoperative management:
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Monitor for dehydration—strict I &O, daily weight
Restore hydration through IV fluids and electrolytes (usually glucose and
electrolytes including K+
e.g. D5 1/2 NS with 20 KCl.
NPO, but may suck on pacifier
Maintain NG tube patency if part of pre-op management
Careful monitoring of VS
Accurate documentation of any vomiting and diarrhea
Teach parents about pathology and treatment after primary MD.
 Post-operative care:
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Maintain IV fluids until taking adequate po.
Small frequent feedings of clear liquids 4-6 hours after surgery. If
retained, strict diet regimen of gradual advancement of feedings until
normal formula has been resumed. Breastfed babies may be given
breastmilk in a bottle initially, and then allowed to go to breast.
NG may be maintained for a short time post op, not always.
Monitor I&O carefully
Manage pain with analgesics ‘round the clock’—using appropriate
pain scale for age of infant
Monitor surgical site for signs of infection.
Not uncommon to have vomiting in first 24-48 hrs.
Intussusception
Def: Bowel telescopes onto itself. Invagination or
telescoping of 1 portion of the intestine into
another, resulting in obstruction behind the
defect.
Epidemiology:
Most common cause of intestinal obstruction 3 yrs & under
 Rare before 3 months of age
 Peak age: 5 to 9 months
 Less common after 36 months
 Link was made to the Rotavirus vaccine initially, but new
vaccines have been found safe, but should only be given at
designated ages

Intussusception
Telescoping of the intestine
leading to possible ischemia
Common Symptoms:
•Acute pain, vomiting
•Current-jelly-like stools
•Apathy
•Abdomen tender, distended
Treatment: Barium enema and/or surgery
Intussusception
Etiology:
 Idiopathic (90%)
 Pathologic lesion
Risk factors:
 Cystic Fibrosis
 Indwelling gastrointestinal tubes
 Recent infections
 Acute Gastroenteritis
Intussusception - Symptoms
Acute:
 Child appears well between
episodes of pain
 Vomiting
 Abdominal pain:
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Acute pain with sudden onset
Colicky with paroxysms and of
pain @ 20 minute intervals
 Stool changes
 Watery stools @ first (12-24
hours)
 Red Current Jelly Stools later
(bloody mucus)
 Lethargy
Chronic:
 Diarrhea
 Anorexia
 Weight loss
 Occasional
vomiting
 Periodic pain
Hirschsprung Disease
Congenital Aganglionic Megacolon
 Definition: congenital anomaly results from absence of
ganglion cells in colon which then results in a mechanical
obstruction d/t inadequate motility from one section of
the colon.
 Etiology:
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Congenital defect ;1:5000 live births
4:1 ratio [affects 4x’s >♂’s than ♀]
Down's syndrome is frequently associated with it.
Genitourinary abnormalities accompany it.
Hirschsprung’s Disease
Hirschsprung Disease cont.
 Pathophysiology:
 Lack of the intramural ganglionic cells
 Lack of innervations:
 Hypertonic bowel results in functional stenosis
 Internal sphincter does not relax
 Partial or complete colonic obstruction
 Proximal intestine markedly dilated with feces, gas.
Hirschsprung Disease cont.
Symptoms:
Newborns:
• Failure to pass meconium in first
24-48 hours
• Bile-stained emesis *
• Abdominal distention
• Reluctance to eat
Infants:
 Failure to thrive, inadequate wt
gain
 Constipation
 Abdominal distention
 Vomiting
 Episodic diarrhea
 Ominous Signs: bloody diarrhea,
fever, severe lethargy
Toddlers and older children:
 Chronic constipation
 Foul-smelling stools, ribbon
like stools
 Abdominal distention
 Visible peristalsis
 Palpable fecal mass
 Malnourishment
 Signs of anemia and
Hypoproteinemia
Hirschsprung Disease cont.
Assessment:
1. Rectal exam: absence of stool and rectum
2. X-ray: enlarged portion of the colon
3. Rectal biopsy: absence of ganglion cells
Treatment:
1. Remove ganglionic bowel
2. Temporary colostomy
3. Reanastomosis around age two
Nursing considerations:
1. Prepare for surgical procedure
2. Teach ostomy care
3. Monitor fluid and electrolytes
4. Monitor nutrition
5. Help with body image
Tracheoesophageal Fistula (TEF)
Esophageal Atresia (EA)
 Atresia: absence of normal opening or normally
patent lumen
 Failure of esophagus to develop as a continuous
passage and/or trachea & esophagus do not separate
 May occur separately or in combination
 High association w/cardiac anomalies
Tracheoesophageal Fistula
Signs / Symptoms /Management
 3 C’s:
 Coughing
 Choking episodes w/eating
 Cyanosis may occur d/t aspiration of formula or saliva →
laryngospasm and/or apnea
 Frothy saliva in mouth & nose; drooling
 Can literally drown if esophagus is connected to
trachea
 Attempt made to pass NG/OG catheter is
unsuccessful
 Polyhydraminois & SGA are big warning signs
 Pure Esophageal Atresia
 Fistula from trachea to upper esophageal segment
 Blind pouch proximal esophagus; distal is connected to trachea or
bronchus by short fistula near bifurcation
 Fistula from trachea to upper and lower segments of esophagus
 “H type” – normal trachea and esophagus but fistula connects them
Management
 Goal: prevent aspiration pneumonia, ensure
hydration & nutrition
 Immediately NPO; IV fluids started
 Antibiotics started d/t ↑ risk of aspiration
 Frequent suctioning & HOB ↑30°
 Gastrostomy frequently done
 Surgery – usually completed in 1-2 tries
 Near 100% survival rate if no other problems
 May have long term feeding problems so need
close monitoring
Post Op and Discharge
 Gastrostomy feedings at first, then oral
 Always begin w/water & close supervision
 Assess for skin breakdown & provide care
 Provide infant stimulation, pacifier, til able to suck
from breast/bottle
 Observe for respiratory problems and teach S/S to
parents; teach CPR
 Extreme care when introducing foods
 Increased risk for GERD
 Refer to Care Plan in book p.438
The End!
Surgical Repair of TEF