Pediatric Gastroenterology

Pediatric Gastroenterology

Overview • GI system begins to develop during the third week of gestation • At birth, all structures and functions are present, but some functions are immature • Structure: mouth to anus • Function: digestion and absorption

A word on congenital defects: it is better to explain what a defect is and what the prognosis is BEFORE showing the infant to the parent. Once parents see that their baby is not their dream baby they will have a shattering grief reaction and will be unable to take in any new information • Skim London pp. 628-630

Ankyloglossia • Tight frenulum (tongue-tie) • Surgical release if frenulum is so tight infant can’t nurse adequately • May be clipped later if child experience speech problems

Cleft Lip and Palate • Two distinct midline facial defects; can occur together or alone, and can be unilateral or bilateral • Congenital anomaly involving one or more clefts in upper lip, hard palate, and/or soft palate • Use appropriate terms - NOT hare lip • Occurs very early in pregnancy, usually before mother even aware of pregnancy - mom is NOT to blame • Cleft lip occurs at about 6 weeks and cleft palate occurs at about 9 weeks gestation

Any child with one closure defect needs to be assessed for other anomalies • In 1996, folate was added to cereals and breads, and woman planning a pregnancy were advised to take folate supplements in an attempt to decrease neural tube defects. NT defects did decrease dramatically, but so did the incidence of orofacial defects

Incidence • There may be a familial tendency (therefore parents with any child with a mid-line closure defect should be referred for genetic counseling • Cleft lip occurs in 1 in 1000 live births for cleft lip and is more common in males • Cleft palate occurs in 1 in 2500 live births and the incidence in girls is double that of boys • Mid-line facial defects are more common in Japanese-Americans and are rare in African-Americans

Appearance • Mid-line defects are usually readily detectable at birth; may be apparent prenatally • Cleft lip can range from small notch in upper lip to total separation of lip and face and up into the floor of the nose; Nose may be flattened or appear mis-shaped • Cleft palate is visible with visual inspection of the palate but soft palate defects may be missed so manual palpation of hard and soft palate should be done on all newborns

Treatment • Surgical repair • Cleft lip - within first 12 weeks (typically at 4–8 wks). Early is better than later b/c lip is important to infant feeding; Also improved appearance allows for easier bonding • Cleft palate - usually between 4 to 18 months, may be up to 24 mos depending on severity of defect; May have to do repair in stages • May need dental referral, speech therapy

Nursing Care • Provide emotional support to parents as well as appropriate education; It is helpful to show parents pictures of babies after repair • Breastfeeding is to be encouraged but mom may need to express milk for several days or weeks; The expressed milk is helpful in preventing the ear infections common to cleft palate babies • Recurrent otitis media can lead to TM scarring and hearing loss • Read NCP, p. 1163-1166

Feeding (pre-op) • Cleft lip only - no big feeding problems • Cleft palate: hold baby upright and feed slowly - burp often • ESSR technique – Enlarged nipple opening, Stimulate suck, Swallow, Rest • May use specialized feeder • May need gavage feedings • Have bulb syringe handy • Assess respiratory status continuously during feedings

Post-op Care • Often only NPO for 4 hours after repair • No milk for first feedings - adheres to sutures; Offer clear water after feeding to clean sutures (clean suture line PC and PRN) • Older babies - no spoons or straws • Cleft lip repairs may have a Logan bar post-op; Elbow restraints • Avoid crying (cuddle, provided analgesics) • Both pre-op and post-op: provide appropriate infant stimulation

Pierre Robin Syndrome • Triad of micrognathia (small mandible), cleft palate, and glossoptosis (tongue malpositioned downward) • May be associated with additional defects, therefore babies with cleft palate need to be assessed • Never place supine (side is best)

TE Atresia or Fistula • Tracheoesophageal atresia or fistula (TEF) • Occurs b/w 4-5 wks gestation • Esophagus may end in blind pouch before it connects with stomach or may have fistula with trachea (unnatural connection) • Five types on next slide • Etiology unknown • Incidence is 1:3000 live births and is often associated with other anomalies

TEF • Esophagus ends in blind pouch and there is a fistula from pouch to esophagus (rare form) • Esophagus ends in blind pouch with no fistula (esophageal atresia = 5-8%) • Esophagus ends in blind pouch plus fistula from trachea to lower esophagus (most common) • Esophagus ends in blind pouch plus fistula from pouch and from lower esophagus (rare) • Normal trachea and esophagus with TEF (“H” type = 2-3%)

TEF • Dx: history of maternal polyhydramnios; Inability to pass a catheter to aspirate stomach, flat plate x-ray of abd shows stomach distended with air; radiopaque NG tube will x-ray as curled in esophagus; Excessive oral mucous (blowing bubbles); “Double bubble” on prenatal sono; Bronchial endoscopy • Presence of other associated anomalies (VATER Syndrome - vertebral, anorectal, tracheoesophageal, rectal)

TEF • S&S: 3 C’s - coughing, choking, and cyanosis; excessive oral mucous, bubbles, drooling • **Must be dx before feeding** • Rx: may have to do in stages - what’s first? • Airway, IV flds, NG tube, surgery • Nsg care: NPO, minimize risk of aspiration, monitor FLN, technical care, support & teach parents, routine pre and post op care, don’t forget “normal” newborn care • See Figure 46-3, p. 1169 and teaching box, p. 1168

Pyloric Stenosis • Pyloric sphincter is b/w lower part of stomach and duodenum (beginning of intestine) • Stenosis causes partial obstruction so milk leaves stomach too slowly • Initially baby eats well or may have some mild spitting, but as obstruction increases and feeding volume increases, vomiting worsens and becomes more forceful • Average incidence is 3 per 100 live births • Less frequent in breastfed infants

Manifestations • Emesis immediately after feeding beginning about 2 to 4 wks of age and grows increasingly forceful until projectile, usually smells sour b/c digestion has begun • Diagnosis • History of projectile vomiting, visible peristaltic waves on abdomen, palpable olive-sized mass in LUQ • Confirmed by sonogram

Pyloric Stenosis • Treatment - Small feedings • If too severe (causing malnutrition): correct fluid and electrolyte imbalance, then surgical repair of pyloric sphincter via laparoscopy (pyloromyotomy) • Routine post op care with PO volume restricted feedings to allow suture line to heal • See Critical Pathway, pp. 1171-1172

Omphalocele • Protrusion of abdominal contents thru abd wall at junction of abd and umbilicus • Failure of abdominal contents to return to abdomen when abdominal walls begins to close about 10 weeks gestation • Umbilical cord inserts into the omphalocele sac • Usually intestines, may include liver and even stomach; covered with peritoneum - keep clean and moist • Incidence 1:5000; Dx on prenatal sono or in DR • Rx - Surgical repair

Rupture of sac results in evisceration of abdominal contents • Sac must be protected until surgery is performed • Cover sac with sterile gauze soaked with warmed saline • Sterile technique is essential • Cover gauze with plastic to keep moisture in and to decrease evaporative heat loss

Gastroschisis • Similar to omphalocele but abdominal wall defect is not at umbilicus & is not covered by peritoneal sac, so contents spill freely • Harder to repair • Incidence about 1 to 3 in 10,000 births • Sterile technique, keep warm, keep hydrated, NG to keep GI system decompressed • Repair is surgical, often done in stages

Diaphragmatic Hernia • Protrusion of abdominal contents (one or more organs) into the chest cavity through a defect in the diaphragm • Most common on left side • Incidence about 1 in 5000 live births • Occurs at about intrauterine week 8, so is usually detected by prenatal sonogram • Intrauterine surgery may be attempted

Newborns with large defect will have severe respiratory distress (life-threatening condition) • Lung on affected side is not expanded, and may not have developed properly (hypoplastic lung) • Overall death rate is 25 to 50% • Death rate increases with presence of other anomalies • Death rate decreases with no other anomalies and delivery at a tertiary center

Treatment • Requires NICU care • Surgical correction – may require staged surgery, must have ventilator support or ECMO • Pre-op: assess VS frequently, elevate HOB and position on affected side (collapsed lung down), maintain NG tube, monitor IV fluids, maintain mechanical ventilation or ECMO, provide minimal stimulation, support family

Nursing Management • Post-op • Elevate HOB, maintain NG and chest tubes, suction prn, maintain IVs and TPN (may need ECMO) Post-op: monitor for infection, continue respiratory support, organize care to decrease stimulation, assess/maintain adequate nutrition, support and educate family

Biliary Atresia • Progressive inflammatory process that causes bile duct fibrosis • Cause is unknown, but is prenatal: viruses, toxins, or chemicals are suspected • Obstruction of bile ducts doesn’t allow flow of bile out of liver and into small intestine and gall bladder; bile plugs form and cause bile accumulation in liver; inflammation, edema and irreversible hepatic injury occur; liver becomes fibrotic, leading to cirrhosis and portal hypertension

Without bile in the intestines, fat and fat-soluble vitamins cannot be absorbed, leading to malnutrition and growth failure • Biliary atresia is fatal without treatment • Temporary treatment – supportive care and surgery to temporarily correct obstruction • Only permanent treatment is liver transplant • Assessment: appears healthy at birth, jaundice occurs within 2 wks to 2 mos, signs of hepatic failure (clay stools, brown urine, itching, abdominal distention)

Hirschsprung’s disease • Congenital anomaly resulting from an absence of ganglion cells in the colon • Also known as megacolon or congenital aganglionosis • Usually familial; incidence is about 1 in 500 (more common in Down syndrome patients) • Rectosigmoid colon is most commonly affected • Absence of innervation means that peristalsis can’t occur leading to accumulation of bowel contents and distention of bowel

Newborns fail to pass meconium stools • Also develop abdominal distention and have bile-stained emesis • Infants • FTT, constipation, abd distention, vomiting, episodic diarrhea • Toddlers and up • Chronic constipation, foul-smelling stools, abd distention, visible peristalsis, palpable fecal mass, malnourishment, anemia • Treatment: remove aganglionic bowel and create temporary colostomy (bowel is reanastamosed after about 2 years)

Other Pediatric GI Problems • Intestinal obstruction - can be due to atresia, stenosis, volvulus, intussusception, ileus • Anticipate if mother had polyhydramnios or >30cc gastric aspirate at birth, other sx same (no BM, abd distention, vomiting) • Rx same as adults: NG, NPO, surgery prn • May need temp or permanent colostomy • “Currant jelly” stool may be a sign of bowel obstruction and intussusception (may imitate VGE initially)

Meconium plug • Rx: gastrografin enema prn • Meconium ileus • Specific to cystic fibrosis d/t missing enzyme so all body fluids thick and tenacious • Usual ileus therapy plus CF test

Umbilical hernia - protrusion of a portion if intestine thru umbilical ring; more common in girls and more common in African-Americans; surgical repair in 1 to 2 yrs if >2cm; Belly bands or taping do not help • Imperforate anus - can be minor (minor surgical incision) or major with no rectum; may be accompanied by fistulas. 1:5000, more common in males. Assess in DR and if no BM w/i 24 hours

Infant GI Disorders • Gastroesophageal reflux (GER) (chalasia) • Neuromuscular problem where lower esophagus and GE sphincter (cardiac sphincter) are lax and allow for easy regurgitation of gastric contents • Spitting up immediately after meals or with laying down, effortless and non-projectile, often not sour smelling because digestion hasn’t started yet • Dx suggested by history of vomiting, usually w/i one week of birth (younger than with pyloric stenosis), irritability. Definitive dx - Ba swallow

GER Treatment • Depends on severity • Mild/uncomplicated - none, or elevate head during fdg and for 1 hour pc; May use thickened formula • Moderate - above plus antacid and/or H2 antagonist (cimetidine, ranitidine) tid to qid to reduce acidity of reflux; cisapride (propulsid) may be used to increase gastric emptying time • Severe – above, plus may need surgery • See Table 46-1, p. 1173 on reflux meds

Reflux is usually self-limiting • Fades at about 6 mos with sphincter maturity and as solids introduced • Usually fully resolved by 1 year of age • Occurs in about 3% of all newborns; higher incidence in preemies, neurologic disorders (Down), about 3 times more common in boys • Almost always symptomatic by 6 weeks of age

Peptic Ulcer Disease • Shallow ulceration in mucosal wall of stomach, pylorus, or duodenum; In infancy ulcers usually are gastric • Usually d/t H. Pylori, but may be secondary to severe stress following burns, chronic illness, medications (oral steroids) • PUD occurs in only 1% to 2% of children • Treatment is similar to adults

Celiac Disease • Chronic malabsorption syndrome d/t a sensitivity to protein, particularly the gluten protein found in grains (wheat, barley, rye, oats) *corrected list as of 1/25/05 • Patients usually can eat corn, and sometimes rice • When children with celiac disease ingest such protein, changes occur in the intestinal mucosa or villi that prevent absorption of foods across the intestinal villi into the bloodstream; Most noticeable malabsorption developed is inability to absorb fat

Manifestations • Steatorrhea, vitamin deficiencies of ADEK, malnutrition • Distended abdomen with skinny extremities • Anorexia • Irritability • Growth failure • May develop rickets d/t vit D deficiency and clotting disorders d/t vit K deficiency

Relatively rare but early dx is essential for continued health • Dominant inheritance in children of northern European descent; children have varying degrees of involvement (disease can be mild to severe) • Rx: gluten-free diet for life (priority is pt. education) • Hint: so you need to know what to teach them what they can eat and not eat

Gastroenteritis in Infancy • Usually viral (VGE) • Nsg dx: R/O FVD r/t vomiting or diarrhea; Alt nutrition <body req.; Pot alt in skin integrity • Need to do full nsg assessment, esp hydration status assessment and ht & wt • S&S dehydration: thirst, dec skin turgor, dry or clammy skin - may feel cold, dec urine output, lethargy, sunken fontanel/eyes, wt loss

Vomiting • Usually viral; treatment for vomiting is to be NPO until vomiting stops then SLOWLY introduce clear liquids • Know difference b/w regurgitation and vomiting and review with UAPs • Treated with oral rehydration unless volume loss is large

Diarrhea • Major cause of infant mortality in developing countries • Primarily viral or bacterial • Most commonly viral, usually d/t rotaviruses or adenoviruses • Bacterial pathogens usually are C. difficile, salmonella, Giardia, and Camplyobacter • Always serious in infancy d/t small ECF reserve, therefore can dehydrate quickly

Infant Diarrhea • See table 46.3 on p. 1185 on causes of diarrhea in children • Know how to differentiate normal stool from diarrhea • Mild (<10 BM/day), fever 101-102, may have sl. dry mouth, anorectic & irritable, *urine output is normal (5-6/day) • Parent ed - stay ahead of fluid loss; control fever • Continue breastfeeding; if on formula may switch to clear liq (Pedialyte NOT Gatorade) and advance slowly • Going out of vogue: BRAT diet

Severe Diarrhea • Infant is obviously ill, fever >102, high pulse, weak and listless, sunken fontanel, sunken eyes, poor turgor, no saliva, decreased or absent urine output • May have liquid stool every few minutes; stool may be green and contain mucous or blood • Estimation of dehydration done by weight loss • Mild >5% wt loss, mod 5 to 10% wt loss, severe >10% loss - requires immediate intervention

Diarrhea Treatment • Correct fld and lyte disturbance; Bowel rest; Comfort care • Stool culture with initiation of appropriate antibx • Initial IV in hospital is without K until child voids, then KCl is added. Dehydrated infants usually are K depleted (d/t GI loss), but have to be certain kidneys function before giving any potassium • See box on p. 1186 and read gastroenteritis NCP, p. 1187

Rotavirus Diarrhea • Most common cause of severe, dehydrating diarrhea • Accounts for 500,000 office visits and 50,000 hospitalizations (3 day avg stay) in children less than 5 yrs old • Strikes almost all children at least once in the first 5 years of life • Primary prevention: breastfeed for at least 6 mos to get antibody protection from mother; Work is continuing on vaccine

Constipation • Characterized by a decrease in stool frequency or the formation of hard, dry stools • May be due to underlying disease, diet, or psychological factors • Rare during infancy • Almost unheard of in breastfed infants • Usually due to feeding mismanagement or medications • Most common in toddler and preschool children

Corrected by dietary changes • Remove constipating foods such as bananas, rice, and cheese • Increase fluids and fiber-rich foods such as whole grains, fruits, vegetables • Dietary management is treatment of choice; if that doesn’t work a simple glycerin suppository usually does. • Caution parents to avoid use of laxatives, stool softeners, enemas • Herbal remedies OK for kids >12 years

Pediatric Gastroenterology