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First STEPs: serial transverse enteroplasty as a primary procedure in neonates with congenital short bowel
Gwendolyn M Garnett1, Kuang H Kang1, Tom Jaksic1
1Boston Children's Hospital, Boston, MA, USA.
Insights
Primary serial transverse enteroplasty (STEP) is a safe and feasible surgical option for congenital short bowel syndrome (SBS), increasing intestinal length. Further research is needed to evaluate long-term outcomes with evolving SBS management.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Regenerative Medicine
Background:
- Serial transverse enteroplasty (STEP) is an established intestinal lengthening technique for short bowel syndrome (SBS).
- Primary STEP for congenital SBS was proposed but a staged approach was often emphasized.
- Outcomes of primary STEP for congenital SBS have not been comprehensively reviewed by the International STEP Data Registry.
Purpose of the Study:
- To review the outcomes of primary serial transverse enteroplasty (STEP) in patients with congenital short bowel syndrome (SBS).
- To assess the safety and efficacy of primary STEP in increasing intestinal length in neonates with congenital SBS.
Main Methods:
- Retrospective review of the International STEP Data Registry for patients undergoing primary STEP for congenital SBS.
- Paired t-tests were used to assess changes in pre- and post-STEP intestinal lengths (p<0.05).
- Data presented as mean ± standard deviation.
Main Results:
- Fifteen patients underwent primary STEP for congenital SBS; 13 had follow-up.
- Average intestinal length increased from 32 cm to 47 cm (50.4% increase, p<0.001).
- Complications included obstruction and re-dilatation; one death from intestinal failure-associated liver disease (IFALD).
Conclusions:
- Primary STEP is a feasible and safe option for congenital SBS, establishing early bowel continuity and creating intestinal length.
- This technique may avoid interval stomas and associated bowel length loss in neonates.
- Long-term adaptation requires further study, especially with evolving SBS management strategies.
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 3months 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.
