Delayed primary serial transverse enteroplasty as a novel management strategy for infants with congenital ultra-short bowel syndrome.

BACKGROUND: Congenital ultra-short bowel syndrome (USBS) is a challenging problem with a poor outcome. We report a new management approach for USBS infants that attempts to optimize gut growth potential.

METHODS: We report five neonates with USBS in whom no correction was performed at primary surgery except placement of a gastrostomy (G) tube. Sham feeds were started with intermittent G-tube clamping to induce bowel dilatation/growth. Serial fluoroscopy was done until bowel caliber reached 5 cm. STEP was performed and continuity established to the colonic remnant. Small bowel length (SBL) and enteral caloric intake were tabulated.

RESULTS: Patients were born with a mean residual SBL of 19 ± 7.6 cm (14.8% of expected). Median duration of sham feeds prior to STEP was 108 (range 27-232)days. Mean SBL at STEP was 47 ± 12.1cm, which increased post-STEP to 70 ± 12.7 cm (a mean increase of 296% from birth, representing 36.4% ± 13.1% of expected gut length). With a median follow-up time of 20 months (range 8-28), 4/5 achieved >50% enteral calories and have normal liver function. One has undergone liver transplantation.

CONCLUSIONS: In USBS patients, delayed surgical correction with sham feeds accelerates gut growth, optimizing potential for autologous reconstruction. This approach may offer greater opportunity for intestinal adaptation than traditional options.