Hirschsprung’s Disease
This condition is also known as congenital aganglionic megacolon. This is characterized by an intractable constipation from birth resulting in abdominal distention and failure to grow. What is happening here is that, there is a congenital absence of ganglion cells of the colon, resulting in constriction of the involved segment with consequent dilatation of the proximal bowel segment.
Clinically, the newborn may present as a delay in the passing of meconium (the first stool of the newborn). The constipation is due to the defect in the peristalsis. However, in some cases, diarrhea may occur. Abdominal distention with gas is prominent secondary to dilatation of the colon above the diseased bowel. By palpating softly the abdomen, the physician can feel fecal masses. There will be signs of intestinal obstruction. Repeated enterocolitis with signs of dehydration may be found and in severe degree may be fatal. In doing a rectal examination, the rectal vault is empty.
The diagnosis of Hirschprung’s disease is suggested by clinical manifestations and physical findings. Plain films of the abdomen will reveal dilated bowel segments. In Barium enema, narrowed segments with dilated loops can be appreciated. Also, failure to evacuate the barium effectively in 24-36 hours is helpful in the diagnosis. But, the definitive diagnosis is through rectal biopsy that will show absence of ganglion cells.
Hirschprung’s disease may be managed medically and surgically. In the presence pf infection and dehydration, fluid and electrolyte disturbances should be corrected. Appropriate antibiotics are given if infection is shown by positive stool cultures.