Patient was born by vaginal delivery at 37 weeks gestation to a 17 yo G1P1 mother. Pregnancy was complicated by oligohyramnios. Patient’s father and paternal grandfather had a history of Hirschsprung’s disease. The patient did not pass stool in the first 48 hours of life. Multiple xrays and a suction rectal biopsy were inconclusive. The patient began stooling in the first hospital day and was sent home. The patient returned to the hospital multiple times for suspected obstructions. At three months of age the patient presented and was again worked up for Hirschsprung’s disease due to signs of obstruction and family history.
Patient was found to be irritable with abdominal distention and a mass felt in left upper quadrant.
On the scout film, gaseous dilation of the colon is present extending nearly to the anal verge and the distal colon is slightly narrower than more proximal colon. Barium flowed into the rectum via gravity. The distal colon is narrower than colon proximal to the transitional zone is suggested to lie approximately 4 cm from the anal verge. The colon above this level is distended with the sigmoid colon extending into the right lower quadrant, before reaching the left flank. The sigmoid colon shows mild wall thickening compatible with colitis. Colonic filling was stopped at this time because of the condern for colon rupture in the face of colitis. The patient did not initially evacuate air or barium.
Dx: Short-segment Hirschsprung's Disease
Dx Confirmed by: Barium enema and rectal biopsy pathology report which demonstrated the absence of ganglion cells.
The Soave repair was uncomplicated. The patient returned multiple times for episodes of constipation and colitis, which is a common problem in these patients.
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