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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 closeTwo 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-S1Skin 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 onlyMyelomeningocele—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 AcidFolic 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 diapersAssess neurological deficitsSurgical 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 managementReference 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 bossingMacewen signHigh-pitched cryChanges in behavior—in older childNausea & 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 changesAlso 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 occursTable 11-3; p. 443 has specificsEither way it leads to ↑ICP and this is serious!
Ventriculo-peritoneal Shunts
Here are some examples of VP shunts that maybe 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 familieswho 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 weeks
Absence of both cerebral hemispheresBrainstem function may be intactCause of death usually respiratory failureManagement: 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
Link to diagram of pyloric stenosis and surgical incision
Pyloromyotomy, creation of an incision along the anterior pylorus to split the muscle, is performed to relieve the obstruction.
Hypertrophic Pyloric Stenosis
Assessment preoperatively:Progressive, projectile, non-bilious vomiting
occurs.Movable, palpable, firm, olive- shaped mass
in the right upper quadrantIrritability, 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
alkalosis2. Serum chloride, sodium, potassium ≈
decreased3. HCT, Hgb ↑ = hemoconcentration
Management:
Preoperative management: 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: 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 lesionRisk factors:Cystic FibrosisIndwelling gastrointestinal tubesRecent infectionsAcute Gastroenteritis
Intussusception - Symptoms
Acute:Child appears well
between episodes of painVomitingAbdominal pain:
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:DiarrheaAnorexiaWeight lossOccasional
vomitingPeriodic pain
Hirschsprung Disease
Congenital Aganglionic MegacolonDefinition: 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: 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 cellsLack 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 rectum2. X-ray: enlarged portion of the colon3. Rectal biopsy: absence of ganglion cellsTreatment:1. Remove ganglionic bowel2. Temporary colostomy3. Reanastomosis around age twoNursing considerations:1. Prepare for surgical procedure2. Teach ostomy care3. Monitor fluid and electrolytes4. Monitor nutrition5. 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 combinationHigh 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 apneaFrothy saliva in mouth & nose; droolingCan literally drown if esophagus is
connected to tracheaAttempt made to pass NG/OG catheter is
unsuccessfulPolyhydraminois & 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 startedAntibiotics started d/t ↑ risk of aspirationFrequent suctioning & HOB ↑30°Gastrostomy frequently doneSurgery – usually completed in 1-2 triesNear 100% survival rate if no other
problemsMay have long term feeding problems so
need close monitoring
Post Op and Discharge
Gastrostomy feedings at first, then oralAlways begin w/water & close supervisionAssess for skin breakdown & provide careProvide infant stimulation, pacifier, til able
to suck from breast/bottleObserve for respiratory problems and teach
S/S to parents; teach CPRExtreme care when introducing foodsIncreased risk for GERDRefer to Care Plan in book p.438
The End!
Surgical Repair of TEF