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