CHAPTER 27 PHYSICAL OR DEVELOPMENTAL CHALLENGE

IMMEDIATE CARE AT BIRTH OF AN INFANT BORN PHYSICALLY OR DEVELOPMENTALLY CHALLENGED - when a parent gives birth to a child with defects there is usually a delay from medical term in informing the parents - this leads to parents thinking either they have given to a perfect child and the team lacks enthusiasm OR - they have given birth to a child so deformed the team are horrified to talk to them - for this reason, nurses need to be familiar with the most frequently encountered physical or developmental anomalies so that they can explain the problems to the parents - nurses must also be ready to serve as back-up informants - explain to parents what the disorder consist of and what the usual prognosis is before showing the baby to them - plan for adequate pain management for neonate

PHYSICAL & DEVELOPMENTAL DISORDERS OF THE GASTROINTESTINAL SYSTEM - many congenital anomalies involve the GI tract coz GI tract is formed first as a solid tube ===> undergoes canalization - if canalization does not occur ===> partial or complete blockage or obstruction occur - other disorders such as CLEFT LIP and CLEFT PALATE are result of midline closure failure extremely early in intrauterine life - all of these disorders interfere with infant’s ability to take in nourishment at birth

ANKYLOGLOSSIA (TONGUE-TIE) - abnormal restriction of the tongue - rarely causes speech diff or destructive pressure on gingival tissue - short frenulum is normal CAUSE - abnormally tight frenulum (membrane attached to lower anterior tip of tongue) - normally, in NB, frenulum appears short and is positioned near tip of tongue - as anterior portion of infant’s tongue grows, the frenulum becomes located farther back - in most instances, infant is suspected of being tongue-tied has a normal tongue at birth // it just seems short to parents who are unaware of a NB’s appearance

THYROGLOSSAL CYST CAUSE - arises from an embryogenic fault that leaves a cyst formed at the base of tongue, which then drains thru a fistula (opening) to the anterior surface of the neck - occur as dominant inherited trait - cyst may involve hyoid bone (bone at anterior surface of neck at root of tongue) or may contain aberrant thyroid gland tissue - as cyst fills with fluid ===> swelling and obstruction ===> RR diff from pressure on trachea - if infected cysts appears swollen and reddened, with drainage of mucus or pus from anterior neck THERAPEUTIC MANAGEMENT - cyst is surgically removed - to avoid future infection of the space OR

- if thyroid is present, the possibility of developing thyroid carcinoma later in life ASSESSMENT - assess infant after surgery for RR distress - operative area will develop some edema from surgical trauma - position infant on sides so secretion drain freely from their mouths - IV fluid is given after surgery until edema at incision recedes somewhat and swallowing is safe once more (approx. 24 hrs) - if mother is BF and infant is NPO, ask her to express her milk manually to preserve her milk supply - observe infant the first time the take fluid orally to be certain they do not aspirate - be certain parents feed infant before infant is discharged from unit so they can see that infant is swallowing safely - important in helping confidence

CLEFT LIP & PALATE CLEFT LIP - normally the maxillary and median nasal processes fuse between 5-8 weeks of intrauterine life - infant with CLEFT LIP, fusion fails to occur in varying degrees ===> disorder range from small notch in upper lip to total separation of lip and facial structure up into the floor of nose, with even the upper teeth and gingiva absent - deviation maybe unilateral or bilateral - nose is generally flattened coz the incomplete fusion of upper lip allowed it to expand in horizontal dimension - more prevalent among boys than girls - higher in Asian population - lower in Black population CAUSES - familial tendency - transmission of multiple genes - teratogenic factors present during week 5-8 of intrauterine life - viral infection - deficiency of folic acid CLEFT PALATE - normally palatal process closes at approx. weeks 9-12 of intrauterine life - CLEFT PALATE (an opening of the palate) is usu on the midline and may involve the anterior hard palate, the posterior soft palate, or both - maybe a separate anomaly but as a rule it occurs in conjunction with cleft lip - occurs more in girls than boys CAUSES - polygenic inheritance - environmental influences ASSESSMENT CLEFT LIP - detected by sonogram in utero - if not detected, apparent at birth CLEFT PALATE - determined by depressing NB’s tongue with tongue blade - reveals total palate and extent of any cleft present - if child has this, assess for other congenital anomalies - cleft palate is a component of many syndromes

THERAPEUTIC MANAGEMENT CLEFT LIP .may need frequent nasopharyngeal suctioning to remove un-swallowed saliva . micrognathia (small mandible) 2.with soft palate repair at 3-6 months of age .hard palate repair at 15-18 months of age . cataracts 3. cardiac disorders ASSESSMENT .allows parents to sleep as well from worrying .at birth.if cleft lip is discovered while infant is still in utero === fetal surgery .if severe.as child grows.palate repair narrows upper dental arch ===> less space in upper jaw for eruption of teeth . children may have diff breathing === as a result of small jaws === tongues are too large for their mouths ===> tongue drops backward and obstruct airway .growth with repair of cleft palate ===> decrease RR problems .tell parents to use RR monitor at night to monitor infant’s breathing .some infants will have nasal mold apparatus applied before surgery to shape a better nostril .usu occur when child in supine position .an example of cleft palate occurring as only one part of syndrome .aka Pierre Robin sequence TRIAD OF 1. congenital glaucoma 2.RECOMMENDED IS two-stage palate repair .parents have hard time bonding with infant with deformity .follow up treatment by pedodontist PIERRE ROBIN SYNDROME . glossoptosis (tongue malpositioned downward) .when child reaches 4-6 years old === revision of original repair because facial contours change as child grows CLEFT PALATE .early repair increases speech development but may result in a necessary second-stage repair as child’s palate arch grows . attached a suture to anterior aspect of tongue === pulls tongue forward .be certain infant with this disorder is not developing airway obstruction .use a side-lying position when sleeping . mandible grows as well .gastrostomy tube or button is inserted to relieve feeding diff . cleft palate 3.rare disorder (1 in 8500 births) MAY HAVE ASSOCIATED DISORDERS OF 1.if disorder not discovered until birth === surgical repair often at time of initial hospital stay between 2-10 weeks of age .early repair helps infant experience pleasure of sucking as soon as possible .

often a fistula (opening) occurs between the closed esophagus and the trachea 5 USUAL TYPES OF ESOPHAGEAL ATRESIA 1.leaks occurring at anastomosis sites can occur at this time .if NB have so much mucus in mouth that they appear to be blowing bubbles should be suspected of tracheoesophageal fistula DIAGNOSIS .these are serious disorders coz during feeding. there is a tracheoesophageal fistula between the distal part of the esophagus and the trachea 2.esophageal atresia is obstruction of the esophagus . esophagus ends in a blind pouch.esophageal lumen forms parallel to this .many fetus with this condition are born preterm coz of accompanying hydramnios (compounding their original problem with immaturity) . and beginning lung tissue .cardiac .tracheoesophageal atresia must be ruled out in any infant born to woman with hydramnios (excessive amniotic fluid) .renal .emergency surgery is essential to prevent development of . trachea.vertebral . cyanosis.assess infant for VACTERL syndrome that could occur during same week in gestation as condition above .barium swallow or bronchial endoscopy examination can also reveal blind-end esophagus and fistula THERAPEUTIC MANAGEMENT .if catheter cannot be passed thru infant’s esophagus to stomach OR stomach contents cannot be aspirated .a number of anomalies formed when the two organs are not allowed to separate but remained connected .dehydration .TRACHEOESOPHAGEAL ATRESIA AND FISTULA .a flat-plate radiograph of abdomen or UTZ can reveal a stomach distended with air that is passing from trachea into esophagus and stomach . diff breathing . there is a blind end of the esophagus // fistulas are present between both widely spaced segments of the esophagus and the trachea . fistula is present between an otherwise normal esophagus and trachea 4. milk can fill the blind esophagus and overflow into trachea OR a fistula can allow milk to enter trachea ===> aspiration ASSESSMENT .anal .tracheoesophageal .if infant not diagnosed and is fed ===> coughing.observe infants at postoperative days 7-10 when sutures dissolve .pneumonia from leakage of stomach secretions to lungs . there is no connection to the trachea 3.between 4-8 weeks of intrauterine life.maybe necessary to complete surgery in different stages and to use portion of colon to complete the anastomosis if esophageal segments are far apart from each other . esophagus ends in a blind pouch // a fistula connects the blind pouch of the proximal esophagus to the trachea 5.electrolyte imbalance from lack of oral intake . laryngotracheal groove develops into larynx. esophagus ends in a blind pouch.limb anomalies .a fetus with this condition cannot swallow ===> amount of amniotic fluid grow abnormally large .surgery consists of closing fistula and anastomosing esophageal segments .

when identified in utero === CS birth is performed to protect exposed intestine . growing faster than fetal abdomen. infant is fed by total parenteral nutrition and keep bowel from filing with air or stool .if omphalocele is small === one-stage repair .when > 10 cm === true omphalocele OCCURS .antibiotics are given to help prevent infection .if large === use of prosthetic patch repair that bridges the unformed gap on abdomen with synthetic material with skin drawn tight and closed over patch .gastroesophageal reflux may occur after a repair if esophagus is left shorter than usual ===> recurrent fistula formation from presence of stomach acid in the esophagus .during this time.this condition occurs coz at approx.occurrence is associated with chromosomal aberrations .. it is obvious during birth .also bowel might not have room for effective peristalsis .if defect is simple fistula.usu covered and contained by a thin layer of transparent layer of amnion and chorion with umbilical cord protruding from exposed sac .if total bowel were replaced into this small abdomen ===> RR distress could result from pressure of visceral bulk on diaphragm and lungs .upper R lobe pneumonia from aspiration is one of major complications of this disorder OMPHALOCELE .at 7-10 weeks when abdomen has enlarged sufficiently.if it occurs === esophageal dilatation at periodic intervals to keep the repaired esophagus fully patent is necessary .if not.herniated organs usu intestines.if it does occur ===> fluid and air leak out into chest cavity ===> pneumothorax (collapse of lungs) results .diagnosed by prenatal sonogram . but may include stomach and liver . intestine returns to abdomen .if this is the only disorder identified === vaginal birth can be allowed to proceed THERAPEUTIC MANAGEMENT .second approach is to replace only a portion of bowel .a protrusion of abdominal contents thru abdominal wall at point of junction of umbilical cord and abdomen . fetal abdominal contents.maybe revealed by elevated maternal serum alpha-fetoprotein (MSAFP) examination during pregnancy . weeks 6-8 intrauterine life. bowel is gradually returned to abdomen .gastrostomy is done (under local anesthesia) and tube allowed to drain by gravity to keep stomach empty of secretions and prevent reflux into lungs . can be repaired with endoscopic technique and application of fibrin glue to seal fistula .over the next 5-7 days. are extruded from abdomen into base of umbilical cord .omphalocele occurs when abdominal contents fail to return in usual way . some stenosis or stricture at anastomosis site remains .previous association between serotonin-reuptake ingestion during pregnancy has proven to be false ASSESSMENT .some infants.when defect is < 4 cm ==== hernia of umbilical cord .surgery within 24 hrs to replace the bowel before thin membrane surrounding it ruptures or becomes infected .remainder is contained by a Silastic pouch termed a “silo” suspender over infant’s bed .

from looseness of the intestine in the abdomen after it has returned .obstruction is rare above ampulla of Vater (junction of bile duct with duodenum) so vomitus will be bile stained (greenish) .obstruction may occur coz the mesentery of bowel twisted as bowel re-entered abdomen (after being contained in the base of umbilical cord early in intrauterine life) OR .long term follow up is necessary to ensure that nutrition and elimination are adequate INTESTINAL OBSTRUCTION .because meconium is black.may pass one stool (meconium that formed below obstruction) and then not pass anymore .condition similar to omphalocele .passes no meconium OR . and abdominal organs are not contained by a membrane but rather spill freely from abdomen .vomit smells barely sour // true vomit is sour smelling (stomach acid has acted on it) and occurs spontaneously without coughing or back-patting .infant will vomit as it progresses . S/S of neonate: .abdomen becomes distended and tender .greater amount of intestinal content tends to herniate ===> increase potential for volvulus and obstruction OCCURS .stenosis (narrowing) of fetal bowel develops .if obstruction not revealed.except that abdominal wall disorder is a distance from the umbilicus.thicker-than-usual meconium formation.if canalization of intestine does not occur in utero .even after surgical correction may have difficulty with absorption of nutrients and passage of stool . usually to the right.children often have decreased bowel motility .care and surgical procedure is same with omphalocele . blocking the lumen (meconium plug or meconium ileus) .GASTROSCHISIS .atresia (complete closure) .coz of ischemia to blood vessels that supply the abdominal wall during 1st trimester of pregnancy THERAPEUTIC MANAGEMENT .or if more than 30 mL of stomach contents can be aspirated from NB stomach by catheter and syringe at birth (fluid is not passing freely thru the tract) .maybe anticipated if mother has hydramnios during pregnancy (swallowed amniotic fluid could not be absorbed effectively by fetus) .this twisting pattern is termed volvulus and continues to be a problem for the first 6 months of life until infant develops firmer intestinal supports ASSESSMENT .most common site is duodenum OCCUR . vomitus is also dark .

orogastric or nasogastric tube is inserted and then attached to low suction or left open to air to prevent further GI distention from swallowed air .final surgical procedure will restore child to full health MECONIUM PLUG SYNDROME .radiograph or sonogram reveal distended air-filled loops of bowel up to point of obstruction .RR rate increase as intestine fills and diaphragm is pushed up against lungs and lung capacity decreases DIAGNOSIS .abdominal distention .waves of peristalsis is evident across abdomen .IV therapy is necessary to restore fluid and immediate surgery is scheduled coz bowel obstruction is an emergency that must be treated before dehydration.maybe associated with Hirschsprung’s disease and is strongly associated with cystic fibrosis .and pulling legs up against abdomen .meconium plug usu form in lower end of bowel coz this meconium formed early in intrauterine life and has the best chance to dry and obstruct the bowel lumen ASSESSMENT .abdominal flat-plate radiograph or sonogram will reveal no air below level of obstruction in intestines .infant is identified as infant with no meconium passage in the past 24 hrs of age . or aspiration of vomitus occurs ..vomiting . and surgery is rescheduled at about 3-6 months of age .gentle rectal exam may reveal presence of hardened stool . infant is d/c to home care. forceful.S/S of obstruction may not occur for at least 24 hrs . electrolyte imbalance.probably reflects normal variations of meconium consistency .extremely hard portion of meconium has completely blocked the intestinal lumen ===> bowel obstruction CAUSE .unknown .pain is evident by---hard.always use low intermittent suction with decompression tubes in neonates .slightly hypertonic water-soluble contrast agent enema not only reveal the level of obstruction but also may be therapeutic in loosening the plug .if repair is anatomically diff === temporary colostomy.bowel sounds increase with obstruction coz of increase in peristaltic action that occurs as intestine attempts to push stool pass point of obstruction .area of stenosis or atresia is removed . indignant crying .repair of obstruction (with exception of meconium plug syndrome) is done by laparoscopy or thru an abdominal incision .bowel is anastomosed .pressure greater than this can irritate and ulcerate their stomach lining .barium swallow or barium enema x-ray film maybe used to reveal position of obstruction THERAPEUTIC MANAGEMENT .when bowel obstruction is confirmed.

abdominal distention .protrusion of an abdominal organ (usually stomach or intestine) thru a defect in diaphragm into chest cavity .observe infant for further passage of meconium (should occur at least once daily) over the next 3 days .THERAPEUTIC MANAGEMENT .gastrografin (a highly osmotic radiographic substance) can be given as an enema . a neonate passes a small plug of hardened meconium -.specific phenomenon that occurs in infants with cystic fibrosis .thought of as lung disorder because of severe s/s of tenacious secretions in lungs (tenacious lung fluid leads ===> stasis and infection and alveolar obstruction ===> to reduce air exchange) .these disorders maybe the cuase of hardened meconium .subnormal body temp .substance pulls fluid into bowel coz of its low osmotic pressure ===> allowing stool to soften and plug to pass .in the first 1-2 days of life .cystic fibrosis .no meconium passage . infant should have no further diff and over the next several hrs may pass a great amount of stool .is a recessive disease .S/S of hypothyroidism .enzyme that moistens and makes all body fluids free-flowing is absent ===> all body fluids are thick and tenacious .hard enough it would have caused an obstruction except that it is small -.be certain this blood test is obtained in any NB with meconium plug MECONIUM ILEUS .occasionally.lethargy .hypothyroid and cystic fibrosis screening is done along with phenylketonuria screening .S/S OF BOWEL OBSTRUCTION .large protruding tongue .intestinal and pancreatic secretions are also affected (signaled at birth by hardened obstructive meconium at ileus level from lack of pancreatic trypsin section (meconium ileus) ===> bowel obstruction .administration of saline enemas (never use tap water in NB coz it can lead to water intoxication) may cause enough peristalsis to expel the plug .obstruction of the intestinal lumen by hardened meconium .assess family history of NB who has meconium plug for cystic fibrosis === recessively inherited disorder or aganglionic megacolon (Hirschsprung’s diseease).reflects extreme meconium plugging .infants need to be well hydrated before and after the procedure or they can become hypovolemic from effect of contrast medium .hypothyroidism is yet another disorder that may present with constipation or hardened stool in NB .vomiting of bile-stained fluid .if obstruction is too high for enema to reduce === bowel is incised and hardened meconium removed by laparotomy DIAPHRAGMATIC HERNIA .be certain to record and report such finding coz infant will need close observation for continued defecation .once thickened meconium passed.a polygenic inherited disorder .instillation of acetylcysteine (mucomyst) with diatrizoate (hypaque) rectally may be used to dissolve the plug .

may develop pulmonary HTN coz blood cannot perfuse thru unexpanded lungs ===> R-to-L shunting thru foramen ovale and also PDA remains open THERAPEUTIC MANAGEMENT .more often in girls than boys . diaphragm forms to divide them .this creates a bulging protrusion under the skin at umbilicus .sunken abdomen because it lacks intestine .it is covered by silicone elastomer (Silastic) and left to be closed at a later date after abdomen has grown .over the next week. 8 week.protrusion of a portion of the intestine thru the umbilical ring.RR diff at birth (one of the lungs cannot expand well or not fully formed) .structure is generally 1-2 cm (0.size of protruding mass is not as important as size of fascial ring thru which the intestine protrudes . or extracorporeal membrane oxygenation (ECMO) UMBILICAL HERNIA .if disorder of diaphragm is large === insoluble polymer (Teflon) patch is used in reconstruction . compressed lung will gradually expand and begin to function .5 .if fascial ring > 2 cm === surgery to prevent herniation and intestinal obstruction or bowel strangulation . mm.no difference between male & female incidence CAUSE .if fascial ring < 2 cm === closure occur spontaneously and no repair is necessary .occur most frequently in African American . the chest and abdominal cavity are one .abdominal incision may not be closed but left open to allow intestine to protrude abdominally .1 in) in diameter .if extreme === surgery is done to remove bowel from chest by fetoscopy while fetus is still in utero S/S .usually occurs on L-side ===> cardiac displacement to R-side of chest & collapse of L-lung .rarely noticed @ birth while cord is still present . and fascia surrounding the umbilical cord .difficult repair requires thoracic incision and placement of chest tubes .intercostal and subcostal retractions .repair is complicated if there is not enough room in abdomen for intestine to be returned .if diaphragm does not form completely ===> intestines herniate thru diaphragm opening into chest cavity ASSESSMENT .surgery repair is done by laparoscopy .becomes increasingly noticeable during first year .this is done when child is 1-2 years of age .cyanosis .requires therapy with high-frequency oscillatory ventilation. inhaled nitric oxide..with severe pulmonary HTN .surgery to repair diaphragm and replacement of herniated intestines back into abdomen .occurs coz early in intrauterine life.at approx.detected by sonogram .but maybe as large as orange when children cry or strain .if lungs are hypoplastic from pressure of intestine to uteru .

child returns from surgery with a pressure dressing. they need to keep diapers folded down below the dressing to prevent contaminating the suture line with stool IMPERFORATE ANUS .in week 7 of intrauterine life === upper bowel elongates to pouch and combine with a pouch invaginating from perineum .condition can be minor (involving just surgical incision) or much severe (involving sections of bowel that are many inches apart with no anus) .this method is helpful to estimate the distance the intestine is separated from perineum or extent of correction that will be necessary .some parents believe that holding an umbilical hernia in place by using “belly bands” or taping a silver dollar over the area will help to reduce the hernia .if child not yet toilet trained.remind parents to sponge-bathe the child until they return for postoperative visit and the dressing is removed . discovered @ birth when no anus is present (this is not helpful coz some condition anus is normal but imperforation exist far inside) 3.it can be discovered by inserting a rubber catheter into rectum .placing a urine collector bag over the vagina in girls may reveal a meconium-stained discharge or a rectovaginal fistula .if membrane between 2 surfaces does not dissolve ===> imperforate anus .may occur as additional complication of spinal cord disorders coz both external anal canal and spinal cord arise from same germ tissue layer .radiograph or sonogram will reveal disorder if infant is held in head-down position to allow swallowed air to rise to end of the blind pouch of bowel .surgery done on ambulatory outpatient basis .. prenatal sonogram 2.problem is more common in boys than girls ASSESSMENT METHODS FOR DETECTING IMPERFORATE ANUS 1.if this motion toward each other does not occur OR . condition maybe revealed coz membrane filled with black meconium can be seen protruding from anus 4. WINK REFLEX (touching skin near rectum should make it contract) will be absent if sensory nerve endings in rectum are not intact .when infants fail to pass stool after first 24 hours === investigate reason why .collect a urine specimen on infants with imperforate anus === detects presence of meconium to help determine whether the child has a rectal-bladder fistula . which remains in place until the sutures are well healed .if these methods fail to detect condition .these actions lead to bowel strangulation and should be avoided THERAPEUTIC MANAGEMENT .is stricture of anus .there maybe an accompanying fistula to bladder in boys and to vagina in girls === further complicating surgical repair .the 2 sections of bowel meet ===> membranes between them are absorbed ===> bowel is then patent to the outside OCCURS IN .no stool is passed and abdominal distention evident .

is an excess of CSF in ventricles or the subarachnoid space .if there is a block to passage of fluid OTHER CLASSIFICATIONS OF HYDROCEPHALUS . overproduction of fluid by choroid plexus in 1st or 2nd ventricle.congenital (occurs @ birth) .abnormal accumulation of cerebrospinal fluid (CSF) or hydrocephalus .if rectum ends close to perineum (below or at level of levator ani mm) and anal sphincter is formed === repair involves simple anastomosis of separated bowel segments .degree of difficulty in repairing imperforate anus depends on extent of problem .abnormalities associated with neural tube closure (meningocele or spina dysraphism) .repair becomes complicated if end of rectum is at a distance from perineum (above levator ani mm) or the anal sphincter exist only in an underdeveloped form .all of these are results of multifactoral inheritance and nutritional deficits HYDROCEPHALUS .all repairs are complicated if fistula to bladder or vagina is present .anticipating final repair when infant is somewhat older (6-12 months) . as could occur from a growing tumor (rare) .toxoplasmosis or infant meningitis EXCESS OF CSF IN NB OCCURS IN 1 OR 3 MAIN REASONS: 1.if repair is extensive === doc may do temporary colostomy .most common developmental disorders of the NS (nervous system) at birth include .in infant whose cranial sutures are not firmly knitted ===> excess fluid cause enlargement of skull CLASSIFICATIONS OF HYDROCEPHALUS .formed in 1st and 2nd ventricles of brain ===> passes thru aqueduct of Sylvius and 4th ventricle ===> empty into subarachnoid space of spinal cord (where it is absorbed) .CSF .Communicating Hydrocephalus OR Extraventricular Hydrocephalus .if fluid reaches spinal cord .for successful repair === it is unnecessary for internal rectal sphincter to be present as long as the subrectal mm is judged to be intact PHYSICAL AND DEVELOPMENTAL DISORDERS OF THE NERVOUS SYSTEM .Obstructive Hydrocephalus OR Intraventricular Hydrocephalus .unknown .acquired (incident later in life) CAUSE .THERAPEUTIC MANAGEMENT .but associated with maternal infection .

2nd.infant shows S/S of increased ICP (intracranial pressure) .increased VP . separated suture line 6. Hemorrhage from trauma or a growing tumor also may obstruct the passage of CSF.head diameter enlarges . obstruction of passage of fluid in narrow aqueduct of Sylvius (most common cause).if atresia is in aqueduct of Sylvia ===> 1st. 3rd ventricles will dilate .S/S . as occurs with surgery for meningocele.S/S may develop rapidly or slowly depending on extent of atresia .have a typical shrill. Obstruction occurs coz infections such as meningitis or encephalitis may leave adhesions behind that block fluid flow. “bossing” of forehead 3.decreased RR . An Arnold-Chiari disorder (elongation of the lower brain stem and displacement of the 4th ventricle into the upper cervical canal) is yet another cause 3. prominent scalp veins 7.S/S OF ICP . Other common sites of obstruction include: foramina of Magendie & Luschka.increased temp .eyes become “sunset eyes” (sclera shows above the iris because of upper lid retraction) .optic atrophy .infant is irritable or lethargic and fail to thrive .becomes evident in first few weeks or months of life .decreased P . “sunset” eyes 4. an interference with absorption of CSF from subarachnoid space if a portion of the subarachnoid membrane is removed. or after extensive subarachnoid hemorrhage. shrill cry 10.suture lines on skull separate .strabismus .if hydrocephalus is present prenatally === can be detected by prenatal sonogram .increased temp .can be shunted in utero .@ birth it is not evident coz of intrauterine pressure .as fluid accumulation continues === scalp becomes shiny and scalp veins become prominent . the openings that allow fluid to leave the 4th ventricle.3 ASSESSING AN INFANT WITH HYDROCEPHALUS 1.increased BP . lethargy or irritability 9. increased head circumference 8. enlarged fontanelles 2. high-pitched cry BOX 27.decrease P .hyperactive reflexes .infant’s fontanelles widen and appear tense .brow bulges forward (bossing) . when portions of membrane absorption surface become obscured ASSESSMENT .decreased RR .when obstruction is present === excessive fluid accumulates and dilates the system above the point of obstruction .2. hyperactive reflexes 5.if it is at exit from 4th ventricle ===> all ventricles will dilate .

laser surgery to reopen the route of flow OR .may suggest point of obstruction .transillumination (holding a bright light such as a flashlight or a specialized light [a Chun gun] against the skull with child in darkened room) will reveal skull is filled with fluid rather than solid brain .however. it could become enclosed in a fold of peritoneum and become obstructed or it could become infected .computed tomography (CT .acetazolamide (Diamox) === a diuretic is given to promote excretion of excess fluid .skull that is enlarging anteriorly with a shallow posterior fossa === obstruction is in aqueduct or 3rd ventricle .ultrasound .if caused by overproduction of fluid .once ICP becomes acute ===> brain tissue is damaged and motor or mental deterioration results .bypassing the point of obstruction by shunting the fluid to another point of absorption .all children < 2 years should have their head circumference recorded and plotted on an appropriate growth chart at health care visits .infant’s motor function becomes impaired as head enlarges .destruction of portion of choroid plexus is attempted by ventricular endoscopy OR if a tumor in that area is responsible for overproduction of fluid.older children who suffered severe head trauma should have their head circumference noted at time of accident .measure NB head circumference within an hour of birth . whether parents can recognize when it needs to be replaced to prevent ICP NEURAL TUBE DISORDERS .aside from general enlargement of head.even the best shunting procedure cannot repair this damage to brain cells .magnetic resonance imaging (MRI) .. as long as child has more than 1 cm of cerebral tissue present === motor function often not impaired .because both neurologic impairment and atrophy caused by inability to move such a heavy head .if hydrocephalus is noncommunicating type .skull x-ray will reveal separating sutures and thinning of the skull .treatment is most effective when disorder is recognized early .because neural tube forms in utero first as a flat plate and then molds to form brain and spinal cord ===> susceptible to malformation .fluid drains via this route into the peritoneum and is absorbed across peritoneal membrane into body circulation .as another complication.dye inserted into a ventricle thru the anterior fontanelle will not appear in CSF obtained from a lumbar puncture THERAPEUTIC MANAGEMENT . note any asymmetry that is occurring .treatment depends on its cause and extent .the ultimate prognosis for a child with hydrocephalus is whether brain damage occurred before shunting and if shunt is in place. removal of tumor should provide solution .again before discharge from health care facility to establish baseline .this type of shunt usu has to be replaced as child grows or it will become too short .shunting procedure involves threading a thin polyethylene catheter under skin from ventricles to peritoneum .even with extremely enlarged head === children’s intelligence may remain normal although fine motor development maybe affected DIAGNOSIS .

infant will have difficulty in labor coz the underdeveloped head does not engage in cervix well .SPINA BIFIDA === Latin for “divided spine” .prenatal sonogram is also helpful to determine presence of disorder TYPES OF DISORDERS 1. they may survive for several days after birth . spina bifida occulta 4.prenatal sonogram . . encephalocele ANENCEPHALY . however.these are also midline closure disorders FACTORS .all of the disorders occur coz of lack of fusion of the posterior surface of embryo in early intrauterine life .poor nutrition (diet deficient in folic acid) === major contributing factor .serum MSAFP is done at week 15 of pregnancy when AFP reaches its peak conc..many such infants present in breech position . parents have option of abortion .amniocentesis .pregnant women are advised to take 600 micrograms of folic acid daily to help prevent this disorder . microcephaly 3.occurs when upper end of neural tube fails to close in early intrauterine life DIAGNOSIS .can be compared with cleft palate or cleft lip --.if result is elevated === amniocentesis is then done to assess level of AFP in amniotic fluid .but since RR and cardiac centers are located in intact medulla.used as a collective term for all spinal cord disorders .a woman who have one child with this disorder are advised to have a maternal serum assay or amniocentesis for AFP levels to determine if such a disorder is present in 2nd pregnancy (level is abnormally increased if there is open spinal lesion) .when condition is discovered prenatally. anencephaly 2. meningocele 5.ethical problem arise when parents decide to continue pregnancy to use infant for organ transplant .absence of cerebral hemispheres OCCURS .take note that not all neural tube disorders involve spinal cord .polygenic inheritance pattern .revealed by elevated level of AFP in maternal serum . myelomeningocele 6.children cannot survive with this disorder coz they have no cerebral function .

when meninges covering spinal cord herniate thru unformed vertebrae .abnormal tufts of hair .term “spina bifida” denote all spinal cord anomalies MENINGOCELE OCCURS . so motor and sensory function is absent beyond this point ===> lower motor neuron damage . although it might be present anywhere along spinal canal .rubella .protruding mass .spinal cord and meninges protrude thru the vertebrae the same as with meningocele .S/S .usu approx.difference is that spinal cord ends at the point.toxoplasmosis .severe malnutrition .child will have S/S .cytomegalovirus .occurs most commonly at 5th lumbar OR 1st sacral level but may occur at any point along spinal canal .disorder in which brain growth is so slow that it falls more than 3 standard deviations below normal on growth charts CAUSE . size of an orange at center of back .when posterior laminae of vertebrae fail to fuse .with surgery.generally occurs in lumbar region.lack of sensation of lower extremities .simple spina bifida is a benign disorder .discolored skin is present .protrusion may be covered by a layer of skin or only the clear dura mater MYELOMENINGOCELE .S/S .flaccidity .true microcephaly must be differentiated from craniosynostosis (normal brain growth but premature fusion of the cranial sutures) which also causes decreased head circumference .noticeable as dimpling at point of poor fusion .infant with craniosynostosis have abnormally closed fontanelles and often show bossing of forehead and S/S of increased ICP similar to infants with hydrocephalus .loss of bowel and bladder control .MICROCEPHALY .anoxia in early infancy .disorder in brain development associated with intrauterine infection such as: . craniosysnostosis can be relieved and brain growth will be normal SPINA BIFIDA OCCULTA OCCURS .generally infant is cognitively challenged coz of lack of functioning brain tissue .

CT scan .most often in occipital area of skull but may occur as a nasal or nasopharyngeal disorder .development of hip dysplasia .hydrocephalus accompanies myelomeningocele coz of lack of adequate subarachnoid membrane for CSF absorption .MRI ENCEPHALOCELE .CT.but they maybe open or covered only by dura .differentiation will be further established by UTZ or MRI THERAPEUTIC MANAGEMENT .infant’s legs are lax and they do not move them .SPINA BIFIDA OCCULTA .transillumination of sac will reveal solid substance or fluid in sac .ultrasound .fetoscopy .diff to tell size of encephalocele if only CSF is trapped in protruding meninges or whether brain tissue could be involved .. MRI.assess nature and pattern of voiding and defecation .generally covered fully by skin .needs no immediate surgical correction . or UTZ will reveal size of skull disorder ASSESSMENT .normal infant appears to be “always wet” from voiding but actually voids in amounts of approx.prenatal UTZ .observing these feature aids in differentiating between meningocele and myelomeningocele .S/S .cranial meningocele or myelomeningocele OCCURS .if condition is discovered in utero === maybe possible to close lesion by fetoscopic surgery .children will have talipes (clubfoot) disorders .pattern is same for defecation .discovered in intrauterline life by: .urine and stools continually dribble coz of lack of sphincter control .amniocentesis (discovery of increased AFP in amniotic fluid) or analysis of AFP in maternal serum .assess infant born with neural tube disorders whether he has spontaneous movement of lower extremities === assess if child has lower motor function .the higher the myelomeningocele occurs in cord === the more likely it is that hydrocephalus will occur DIAGNOSIS .infant with motor or sphincter control voids continuously .infants maybe born CS birth to avoid pressure and injury to spinal cord .some kids may need surgery to prevent vertebral deterioration coz of unbalanced spinal column . 30 mL and then is dry for 2-3 hrs before voiding again .

obstructing CSF flow and causing hydrocephalus .serious levels of sleep apnea may occur that also require surgical intervention PHYSICAL AND DEVELOPMENTAL DISORDERS OF THE SKELETAL SYSTEM . knee extension and weak knee flexion. medulla oblongata.MENINGOCELE.CONGENITAL SKELETAL DISORDERS RESULT FROM .child with myelomeningocele will continue to have paralysis of lower extremities and loss of bowel and bladder function after surgery coz the absent lower cord cannot be replaced RISK FOR SURGERY . ENCEPHALOCELE .because of upper motor neuron involvement ===> gagging and swallowing reflexes are absent ===> increase risk for tracheal aspiration . ambulation with maximal support Hip flexion.done as soon after birth as possible (usu within 24-48 hrs) so infection thru exposed meninges does not occur . kyphosis and scoliosis is common.specific anomaly is a projection of cerebellum.loss of meninges by surgery === limit rate of absorption in CSF ===> buildup of CSF ===> resulting in hydrocephalus TABLE 27. with preservation of some foot and ankle movement. ambulation without support L1-2 L3-4 L5 S1-2 S3 ARNOLD-CHIARI DISORDER (CHIARI II MALFORMATION) .FACTORS . ambulation with moderate support As above.prognosis depends on extent of disorder and surgical procedure possible .environmental ABSENT OR MALFORMED EXTREMITIES .caused by overgrowth of neural tube in weeks 16-20 of fetal life . and knee extension present. paraplegia.1 MOTOR FUNCTION ABILITY IN CHILDREN WITH MYELOMENINGOCELE SPINAL CORD LESION T6-12 RESULTANT EFFECTS complete flaccid paralysis of the lower extremities.immediate surgery to replace contents that are replaceable and to close skin disorder to prevent infection . adduction. ambulation with maximal support Hip flexion present. hip dislocation common.this causes upper cervical spinal cord to jackknife backward. and 4th ventricle into cervical canal . ambulation with minimal support Mild loss of intrinsic foot muscular function possible. and varying degrees of abduction. paralysis of lower legs and feet. weakened abdominal and trunk musculature in higher lesions.. some control of hip and knee movement possible. adduction.genetic . 50% in children with this anomaly .lumbosacral myelomeningocele is also present in approx. ambulation with moderate support Hip flexion. MYELOMENINGOCELE.

maternal drug ingestion .removal is simple and cosmetically sound .pectus excavatum .children born without an extremity or malformed extremity can be fitted for prosthesis early in life .introducing prostheses early also prevents child from adjusting to a missing extremity.gait training for use of lower extremity prostheses begins with use of parallel bars and proceeds to independent walking and mastery of steps .amniotic band formation in utero .two fingers or toes are fused .children who are born with absent extremity may need help in mastering not only the use of a prosthesis but also a positive body image of themselves as a whole . such as writing with feet or sliding across floor rather than walking .these extra fingers are often just cartilage or skin tags .separation of digits into 2 is usu successful .lower extremity prostheses are fitted as early as age 6 months (so infant will learn to stand at normal time) .usu amputated in infancy or early childhood .upper extremity prostheses are fitted this early also. so infant can handle and explore objects daily .fusion usu caused by simple webbing ..virus invasion during pregnancy .polydactyly .syndactyly .pectus carinatum .but maybe developed after chronic obstructive lung disease or rickets .children will have better function if malformed portion of extremity is amputated before prosthesis is fitted .result is lung volume decreases and heart is displaced to left this condition can be repaired either for cosmetic or to expand lung volume .children with a congenital extremity do not grieve over loss of extremity as adults or older children do === meaning they move on quickly to rehabilitation FINGER AND TOE CONDITIONS .a well fitted prosthesis that a child learns to use at early age will provide more function and allow a more normal childhood and adult life than if original disorder is left unchanged .learning to use a hand prosthesis takes weeks to months .indentation of lower portion of sternum .presence of one ore more additional fingers or toes .this condition can be repaired for physiologic or cosmetic reasons TORTICOLLIS (WRY NECK) .children usu are born with this condition .if bones of fingers or toes are fused === cosmetic appearance and function cannot be reconstructed CHEST DEVIATIONS .these self-adjustments will greatly limit a child’s potential .sternum is displaced anteriorly ===> increasing anterior-posterior diameter of chest .

premature closure of coronal suture line is associated with syndactyly .papilledema .also occurs as a dominantly inherited trait .speaking to and handling child objects from affected side to make child look that way are also helpful exercises .occur in utero or early in infancy coz of rickets or irregularities of calcium or phosphate metabolism .syndactyly .parents should encourage infant to look in direction of affected mm .premature closure of sutures of the skull OCCUR .lying infant on flat surface and rotating head thru a full range of motion .tends to occur in NB with wide shoulders when pressure is exerted on head to deliver the shoulder .cardiac anomalies .strabismus .nystagmus .exophthalmos .occurs more often in boys than girls .injury may not be noticeable in NB and may become evident only as original hemorrhage recedes and fibrous contraction occurs at 1-2 months of age .place a mobile on child’s crib to encourage child to look toward affected side is also helpful .botulism (botox) injection maybe given as treatment .hold the child to feed in such a position that child must look in desired direction .S/S .choanal atresias .when sagittal suture line closes prematurely ===> child’s head tends to grow anteriorly and posteriorly .torticollis (wry neck) occurs as a congnital anomaly when sternocleidomastoid mm is injured and bleeds during birth OCCUR ..infant holds head titled to side of mm involved .chin rotates to opposite side .if condition still exist at 1 years of age ==== surgical correction followed by a neck immobilizer is necessary .if extreme injury to mm occured ===> torticollis can lead to elevation of one shoulder ===> risk for scoliosis later in life .however not necessary or recommended for most infants CRANIOSYNOSTOSIS .needs to be detected early coz premature closure of suture line ===> seals the skull closed ===> compromise brain growth .atrophy of optic nerve with consequent loss of vision .parents need to begin a program of passive stretching exercises .term derived from tortus (twisted) and collum (neck) .assess for the foll associated with craniosysnostosis: .observe infants with syndactyly for head circumference .a thick mass over mm can usu be palpated at this time THERAPEUTIC MANAGEMENT .disorders of elbows and knee joints .if coronal suture line fuses early ===> orbits of eyes become misshapen and increased ICP may lead to .

causes a disorder in cartilage production in utero .occurs more in boys than girls .anterior fontanelle at 12-18 months DIAGNOSIS .failure of bone growth inherited as dominant trait .women with this condition will have diff with child bearing coz of small pelvis ===> CS birth THERAPEUTIC MANAGEMENT .and child may have asymmetry of leg length .leg lengthening maybe possible TALIPES DISORDERS .ankle-foot disorder .bridge of nose is flattened .popularly called CLUBFOOT .Latin word // talus means “ankle” // pes means “foot” === ankle foot .if suture line is sagittal one === treatment may involve only careful observation .epiphyseal plate of long bones cannot produce adequate cartilage for longitudinal bone growth ===> both arms and legs stunted .children’s head will appear unusually large in contrast to their extremities . arms can be extended to distance of midthigh) .aka chondrodystrophia .forehead is prominent .radiography ..diagnosed in utero or @ birth by comparing length of extremities to normal length (in average child.however shoe size may vary as much as two shoe sizes .radiography (reveals characteristic abnormally flaring epiphyseal lines) .cognitive development is normal (cartilage problem not brain problem) .UTZ (reveals fused suture line) .correction should leave the child with normal foot position .measure head circumference on all children age 2 years of younger .thoracic kyphosis (outward curve) and lumbar lordosis (inward curve) of spine may develop DIAGNOSIS .maybe given growth hormone to increase their ultimate height OR .usu occurs in unilateral problem .people with this disorder rarely reaches height of more than 4 feet 6 inches (140 cm) .children’s trunk are near normal size .if coronal suture line === will need surgically opened to prevent brain compression and abnormally shaped head ACHONDROPLASIA .bones of cranium are of membranous origin === continues to grow normally .posterior fontanelle normally closes at 2 months of age .inherited as polygenic pattern .

in subluxated hip === femur “rides up” coz of flat acetabulum .after approx.the cast extends above knee to ensure firm correction .often referred to as congenital hip dysplasia .maybe evident as subluxation or dislocation of the head of femur ..tingling feeling or circulatory compression (when foot is “asleep”) from too tight a cast .in a true disorder.assess crying in NB with cast // may be due to: .infant who did not have successful correction by casting ===> surgery is another option DEVELOPMENTAL HIP DYSPLASIA . valgus deviation (foot turns out) .occurs more often in children of Mediterranean ancestry .hunger .wet diapers .change diapers frequently to prevent a wet diaper from touching cast and causing it to become soaked with urine or meconium .polygenic inheritance pattern .uterine position that causes less-than-usual pressure of femur head on acetabulum . dorsiflexion (heel is held lower than forefoot or anterior foot is flexed toward anterior leg) 3.after this. plantarflexion (an equinus or “horsefoot” position with forefoot lower than heel) 2.in these infants. 6 weeks. the foot cannot be properly aligned without further intervention 4 TYPES OF TALIPES DISORDER 1.CAUSE .infant may have to sleep in Denis Browne splints (shoes attached to a metal bar to maintain position) OR .in dislocated hip === femur rides so far up that it actually leaves the acetabulum .this prevents head of femur from remaining in acetabulum and rotating adequately . varus deviation (foot turns in) 4.the earlier true talipes is recognized.the acetabulum of pelvis is flattened or shallow .make a habit of straightening all NB feet to midline as part of initial assessment to detect this disorder THERAPEUTIC MANAGEMENT . final cast is removed .most children with talipes disorder have combination of these or have an equinovarus or calcaneovalgus disorder (a child walks on heel with foot everted) ASSESSMENT . foot looks to be turned in but can be brought into a straight position by manipulation .a cast is applied while foot is placed in an overcorrected position . the better will be the correction .some newborns have pseudo-talipes disorder that has developed coz their intrauterine position .is improper formation and function of the hip socket .high top shoes at night for a few more months .colic . parents need to perform passive foot exercises such as putting infant’s foot and ankle thru a full range of motion several times a day for several months .cast must be changed almost every 1-2 weeks as infant grows .

one knee will appear to be lower than the other (a Galeazzi sign) .MRI *** reveals the shallow acetabulum and a more lateral placement of femur head than ordinarily seen *** THERAPEUTIC MANAGEMENT . hip abducts properly at NB assessment.however.sometimes the affected leg may appear slightly shorter than other one === coz femur head rides so high in socket . such as the way infants are carried.an unequal number of skin folds may be present on posterior thighs .the longer the condition goes undetected === the more difficult it is to correct . secondary shortening of adductor mm will have occurred.possibly coz hips are normally more flaring in females AND .so this disorder must be ruled out DIAGNOSIS .UTZ ..radiography . halters.coz maternal hormone relaxin causes pelvic ligaments to be more relaxed . may promote or decrease the extent of involvement ASSESSMENT . and disorder will be evident .this is most noticeable when child is lying supine and thighs are flexed to 90 degree angle toward abdomen .correction involves positioning hip into a flexed. but at time of health maintenance visit at 4-6 weeks.small number of children who do not achieve correction by these methods === surgery and a pin inserted to stabilize hip .hip dysplasia is difficult to detect at birth in an infant who was born from a footling of frank breech presentation coz knees are stiff and do not flex readily .subluxated or dislocated hips are best assessed by noting whether the hips abduct .found 6x more frequently in girls than boys . this finding is unreliable because some infants with normal hips have uneven number of posterior thigh skin folds .tight adductor mm occur in children with cerebral palsy . abducted (externally rotated) position to press the femur head against the acetabulum and cause it to deepen its contour by pressure . or casts maybe used .in some infants.splints.sociocultural methods of childbearing.involvement is usu unilateral .

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