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= Abstract =

Total Colonic Aganglionosis With Skip Area

Suk Koo Lee, M.D., Woo-Yong Lee, M.D., Hyun-Hahk Kim, M.D.

Devision of Pediatric Surgery Department of Surgery, Samsung Medical Center Sungkyunkwan University College of Medicine

Seoul, Korea

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. -If there are interven- ing ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the dis- ease. There were aganglionic segments from the anal margin to the terminal ile- um 3.7cm proximal to the ileocecal valve. The entire transverse colon and ap- pendix were normally ganglionated.

Index Words: Total colonic aganglionosis, Skip area

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Fig. 1. Infantogram at admission shows multiple dilatation of bowel loops in the abdomen.

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Fig. 2. Delayed film of the small bowel series, shows delayed passage of barium and dilatation of the small bowel.

(a) (b) (c)

Fig. 3. Photomicrograms: (a)frozen biopsy of the distal ileum; no ganglion cell, (b)frozen biopsy of the dilatated ileum; ganglion cells(black arrow) noticed, (c)permanent section of the ascending colon; no gan- glion cell.

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;lion cells present in frozen biopsy, 3; perforation ite and colostomy site.

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