Hirschsprung's Disease | Congenital Megacolon | Symptoms | Pathophysiology | Nursing Management

Hirschsprung's Disease is a congenital disease characterized by absence of sympathetic ganglionic cells in nerve plexuses of large intestine. It is also called congenital aganglionic megacolon or congenital aganglionosis.
The cause of Hirschsprung's disease includes mutations in RET and EDNRB genes. Hirschsprung's Disease is the most common cause of intestinal obstruction in infants. The incidence is 1 in 5000 live births. It is 4 times more common in males than in females.
The Clinical manifestations include the failure to pass meconium, abdominal distention, bile stained vomiting, dehydration, shock, enterocolitis and so on.
Diagnosis is based on failure to pass meconium, squirt or blast sign, X ray and suction biopsy.
The mainstay of treatment is surgery including Duhamel's, Swenson's and Soave's operation.
The nursing diagnoses include,
1) Constipation related to reduced bowel motility as evidenced by infrequent bowel movements,
2) Imbalanced nutrition less than body requirements as evidenced by weight loss.
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