Hirschsprungs Disease

What causes Hirschsprung's disease?

Absence of ganglion cells in parts of the colon → decreased motility → mechanical obstruction.

What part of the GI tract is affected?

The distal colon (most commonly).

Another name for Hirschsprung's disease?

Congenital aganglionic megacolon.

Main risk factor?

Family history of Hirschsprung's disease.

Newborn findings?

No meconium within 24-48 hr, Bilious vomiting, Refusal to feed, Abdominal distention.

Infant findings?

Failure to thrive, Constipation, Vomiting, Episodes of diarrhea and vomiting.

Child findings?

Undernourished, anemic appearance, Abdominal distention, visible peristalsis, Palpable fecal mass, Constipation, foul-smelling ribbonlike stools.

Lab tests?

CBC, electrolytes.

Diagnostic confirmation?

Rectal biopsy showing absence of ganglion cells.

Diet before surgery?

High-protein, high-calorie, low-fiber.

Possible nutrition support?

TPN if severe.

Definitive treatment?

Surgical removal of aganglionic bowel; possible temporary colostomy.

Pre-op priorities?

Fluid & electrolyte replacement, Monitor for enterocolitis, Bowel prep with saline enemas and oral antibiotics, Prepare family for surgery developmentally.

Post-op priorities?

Maintain airway, monitor vitals, Manage pain, Assess surgical site for bleeding/infection, Foley & ostomy care, Assess bowel sounds and function.

Family teaching?

Ostomy and incision care, Monitor for infection or dehydration, Know signs of complications.

Most serious complication?

Enterocolitis (bowel inflammation).

Nursing care for enterocolitis?

Monitor VS and abdominal girth, Watch for sepsis, peritonitis, shock, Maintain hydration, replace electrolytes/blood, Administer antibiotics.

Other complications?

Anal stricture or incontinence → may need bowel retraining or dilation.