First STEPs: Serial transverse enteroplasty as a primary procedure in neonates with congenital short bowel

Background: Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty (STEP) has been increasingly used as the surgical treatment of choice for patients with refractory short bowel syndrome (SBS). While primary STEP for the treatment of congenital conditions was proposed in the original description of the procedure, emphasis was placed on a delayed or staged approach to these patients. To date, a comprehensive review of the outcomes from this sub-population has not been reported by the International STEP Data Registry. Methods: A retrospective review of the International STEP Data Registry was performed to identify all patients who underwent STEP as a primary operative procedure for the treatment of congenital SBS. Changes in pre- and post-STEP values were assessed using paired t-tests with significance set at p < 0.05. Data are presented as mean +/- standard deviation. Results: Fifteen patients underwent primary STEP for congenital SBS between September 1, 2004, and April 10, 2012. Thirteen patients had follow-up information available. Causes of congenital SBS included closing gastroschisis, small bowel atresia, and midgut volvulus. Twelve patients had pre- and post-STEP bowel measurements taken. Average pre- and post-STEP bowel lengths were 32 +/- 16 cm and 47 +/- 22 cm, respectively. Intestinal length was increased by a mean of 15 +/- 12 cm for a relative small bowel length increase of 50.4 +/- 27.3% (p < 0.001). Only one patient required an ostomy at the time of primary STEP. A second patient required a temporary ostomy at 3 months of age that was later closed. There was one death from intestinal failure associated liver disease (IFALD). Another patient experienced IFALD progression and required liver and intestinal transplantation. The most commonly reported complication following primary STEP was obstruction or bowel re-dilatation requiring additional operative interventions. Nine patients underwent second STEP procedures under these circumstances. Eight patients remain dependent on parenteral nutrition, while three patients achieved enteral autonomy. Conclusions: Primary STEP is a feasible and safe surgical option for the treatment of congenital conditions resulting in SBS. Primary STEP establishes early bowel continuity, creates intestinal length from congenitally dilated bowel, and appears to obviate the need for interval stomas and their associated loss of bowel length in neonates with congenital SBS. However, with recent changes in SBS management emphasizing intestinal rehabilitation, additional studies are needed to assess the long-term impact on intestinal adaptation of STEP performed in the neonatal period prior to adoption of this technique. (C) 2014 Elsevier Inc. All rights reserved.