Long-Segment Aganglionosis: a 15 - year Experience

To access the surgical progress of patients with long segment aganglionosis and long-term evaluation. During the past 15 years, we reviewed the therapy and outcome of 15 patients with Long-Segment Agangelionosis (LSA) in Mofid children''s hospital. 2 with Agangelionosis to the midtransvers colon (MTA(, 8 to the ascendant and cecum (A/CA), 4 with total colonic and distal ileum involvement (TCA), and one child with total intestinal Agangelionosis (TIA). The patient with TIA had only laparatomy and staged biopsies. 12 were treated by Swenson technique and 2 patients by other procedures. All patients received total parenteral nutrition up to 10 days postoperative to ensure appropriate fluid and electrolyte status. The patients with TCA needed supplementary therapies (Diet, Drugs) up to 4 months to reduce the stool frequency. 2 patients died. The patient with TIA died 6 months after Biopsies. Another one died 5 months after pull-through: small bowel ileus with total intestinal necrosis. 9 Of the 13 alive patients have normal stool pattern: 1-3 times per day. 2 patients have 3-6 stools per day with 1-2 soiling per week. An adolescent patient has occasionally nightly soiling (1- 2 per month). No actual data about one patient (A/CA). One TCA patient doing well immediately postoperative becomes obstipated 3 months later (after having Entrocolitis). He had washout enemas for 9 months. Now stools regularly. Awareness of long-segment Agangelionosis should lead to earlier diagnosis. Appropriate surgery and improvements in supportive care led to a significantly increased rate of survival. Patients with LSA even involving ileum (but no more than 50 cm of ileum) should attain a normal stooling behavior. Long-term outcome is quite favorable.