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Long-segment Aganglionosis-Treatment and Results (Abstract)

M-A. Ardelean, J. Bauer, C. Schimke, J. Schnöll, C. Heil, B. Ludwikowski
Salzburg, Austria



Purpose: To assess the surgical progresses of patients (pts) with long segment aganglionosis.

Material and methods: During the past 13 years, 56 cases of Hirschsprung's disease have been treated at our clinic. We reviewed the therapy and outcome of 15 pts with long-segment aganglionosis (LSA): 3 with aganglionosis to the midtransverse colon (MTA), 7 to the ascendent and coecum (A/CA), 4 with total colonic and distal ileum involvement (TCA), and one child with total intestinal aganglionosis (TIA). The pt with TIA had only laparotomy and staged biopsies. Twelve were treated by Swenson technique and 2 pts by other procedures. All pts received total parenteral nutrition up to 10 days postoperative to ensure appropriate fluid and electrolyte status. The pts with TCA needed supplementary therapies (diet, drugs) up to 4 months to reduce the stool frequency. Results: Two patients died. The pt with TIA died 6 months after biopsies. Another patient died 5 months after pullthrough: small bowel ileus with total intestinal necrosis. Nine of the 13 alive pts have normal stool pattern: 1 – 3 time per day. Two pts have 3 – 6 stools per day with 1 – 2 soiling per week. An adolescent pt has occasionally nightly soiling (1 – 2 per month). No actual data about one patient (A/CA pt). One TCA pt doing well immediately postoperative becomes obstipated 3 month later (after having enterocolitis). He had washout enemas for 9 months. Now he stools regularly. Conclusions: Awareness of long-segment aganglionosis should lead to earlier diagnosis. Appropriate surgery and improvements in supportive care led to a significantly increased rate of survival. Pts with LSA even involving ileum (but no more than 50 cm of ileum) should attain a normal stooling behavior.

Key words: long-segment aganglionosis, total colonic aganglionosis, stooling behavior


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