9.3 Hirschsprung's Disease and GER(D)

Question 1

Hirschsprung’s Disease

Answer 1

• Congenital Aganglionic Megacolon
• Mechanical obstruction of intestine from inadequate motility (most common cause of neonatal obstruction)
• Can be associated with down syndrome

Pathophysiology
- Absence of ganglion cells (most often in the recto-sigmoid region) causes that portion of the colon to not have motility.
- Accumulation of intestinal contents causes obstruction in this area
- Obstruction can cause ischemia/inflammation of bowel.

S/S
- Massive bowel distention proximal to defect
- Failure to pass meconium in the first 24-48 hours
- Bile stained vomitus
- Ribbon like stools (flat and stringy)
- Poor feeding, chronic constipation
- May have explosive stools

Diagnosis
- Bowel x-ray
- Biopsy of intestines (it will show absence of ganglion cells) - ULTIMATE DIAGNOSIS
- Anal rectal manometry (will show failure of internal sphincter to relax but normal constriction of external sphincter)

Treatment
- Resection of aganglionic section of bowel (called a pull-through) - most common
- Can also be done in stages where first you do a colostomy then do a pull-through and close the colostomy

Question 2

Gastroesophageal Reflux (GER)

Answer 2

• Transfer of gastric contents into esophagus.
• GERD is the chronic form (must be tissue damage or complication from the reflux)
• Most common around 4 months and usually resolves around 12 months.

Manifestations (Infant)
- Spitting up (vomiting)
- Irritable
- Weight loss
- Cough, wheeze, choking

Manifestations (Children)
- Heart burn
- Non-cardiac chest pain
- Dysphagia

Risk Factors
- Prematurity
- Asthma
- Cystic Fibrosis
- Cerebral Palsy
- Tracheoesophageal Repairs

• Occurs most common after meals and is due to lower esophageal sphincter not relaxing fully
• Can also be caused by anything that causes increased intraabdominal pressure such as scoliosis or overeating

Diagnosis
- History/Physical
- 24 Hour pH Probe (GOLD STANDARD) - NG tube that is meant to track pH within GI system
- Scintigraphy - Feed baby radioactive compound and it looks at gastric emptying.

Treatment
- Encourage small frequent feedings
- Thicken formula with rice cereal (so it’s harder to spit up)
- SEVERE GER can be treated with NG tube feeding
- BABY SHOULD BE HELD AT GREATER THAN 45 DEGREES FOR FEEDING AND SITTING UP BABY 30 MINUTES AFTER EATING

Medications
- H2 blockers or Proton Pump Inhibitors

Surgery
- Nissen Fundoplication (only for severe patients) where fundus of stomach is wrapped around esophagus to re-enforce lower esophageal sphincter.

Question 3

Nonpharmacological Treatment

Answer 3

• Avoid foods that exacerbate acid reflux (caffeine, citrus, tomatoes, alcohol, peppermint, spicy/fried food)
• Lifestyle modification (weight control, small frequent meals, thickening food, feeding baby up right and leaving baby upright for 30 minutes after feeding.

Food thickening
- 1 teaspoon to tablespoon of rice cereal per ounce of formula

Question 4

Pharmacological Treatment

Answer 4

H2-Blockers
- Ranitidine (Zantac)
- Famotidine (Pepcid)

Proton Pump Inhibitors (PPI) - reduce gastric hydrochloric acid secretions
- Pantoprazole
- Rabeprazole