INTENSIVE NUTRITIONAL SUPPORT AND REMEDIAL SURGICAL INTERVENTION FOR EXTREME SHORT-BOWEL SYNDROME

Management of extreme short bowel syndrome (SBS) has changed dramatica lly over the last 20 years with notable improvements in survival and q uality of life in patients with this syndrome. A review of our institu tion's medical records over a 12-year period (1980-1992) revealed 32 p atients with <100 cm (range, 14-94; median, 40) of functional small bo wel after intestinal resection. The causes of intestinal loss included necrotizing enterocolitis (11 cases), atresias (8 cases), long-segmen t Hirschsprung's disease (5 cases), midgut volvulus (5 cases) and gast roschisis (3 cases). The mean follow-up period was 4.2 years, and four deaths were recorded (12.5%). Survival of eight of nine (88.9%) patie nts without an ileocecal valve (ICV) and with <40 cm of small bowel wa s noted. The absence of an ICV, however, was associated with significa ntly prolonged total parenteral nutrition. Follow-up surgical procedur es, including intestinal lengthening, tapering enteroplasty, Martin's procedure, longitudinal myectomy-myotomy, and ostomy take-down, were p erformed in 20 of the patients (64%). Prolonged survival and normal de velopment can be expected for the patient with severe SBS who is given meticulous nutritional support and treated with carefully planned sec ondary surgical intervention. These results are also seen in patients with extreme SBS (<40 cm residual small bowel length) and no ICV.