Analysis of patients with longitudinal intestinal lengthening procedure referred for intestinal transplantation

Longitudinal intestinal lengthening procedures (LILP) in patients with short gut syndrome (SGS) enhances small intestinal peristalsis and decreases bacterial overgrowth without reducing absorptive surface. Therefore, patients theoretically may be easily weaned off TPN. The aim of this study was to evaluate the impact of failed LILP in SGS patients referred for intestinal transplantation.

Twenty-seven (11%) of 230 children with SGS and total parenteral nutrition (TPN) dependency evaluated for intestinal transplantation at our institution had undergone LILP. This was performed at a mean age of 1.7 years (range, 1 day to 14.7 years); the mean age at the time of evaluation was 3.3 years (range, 0.4 to 17 years). Two patients underwent LILP immediately after birth. The principle diagnoses producing SGS were gastroschisis (n = 8), intestinal atresia (n = 11), neonatal volvulus (n = 7) and necrotizing enterocolitis (n = 1). Before LILP, the mean length of intestine was 32 cm (range, 8 to 70 cm). Fifteen (56%) patients had jaundice at the time of evaluation.

All but one child were considered candidates for intestinal transplantation. The mean intestinal length achieved after LILP was 48 cm (range, 16 to 100). The mean follow-up from the date of LILP was 876 days (range, 109 to 4,109 days). After LILP, only 9 (33%) patients increased their caloric intake through the enteral route by > or =50%, and only 1 patient could be weaned off TPN. In the patients with liver dysfunction at the time of LILP, none recovered. Most of the patients had multiple episodes of sepsis after LILP. Fourteen (52%) of 27 patients underwent intestinal transplantation, 7 combined with a liver allograft because of TPN-induced end-stage liver disease. Six of the transplanted patients are alive and TPN free. Of the remaining 13 (48%) nontransplanted patients, 9 patients died. The main cause of death was TPN-induced liver failure. Three patients are on partial TPN, and only 1 patient was weaned off TPN. The presence of an ileocecal valve did not impact on outcome. Surprisingly, patients with > or =50% of colon at the time of LILP had poorer survival than those with less. Twelve (44%) of 27 patients had surgical complications, and in both patients with LILP performed in the neonatal period it failed immediately with acute complications. There were no differences in patient survival rate for patients with SGS without LILP (n = 203) and those with LILP (n = 27).

Based on patients with unsuccessful LILP referred for intestinal transplantation, we believe this procedure should be avoided in the neonatal period, in those patients with liver dysfunction, and when intestinal length is <50 cm.