The First Report of the International STEP Data Registry: Indications, Efficacy and Complications – A Good First Step Biren P. Modi MD, Patrick J. Javid MD, Tom Jaksic MD PhD, Hannah Piper MD, Monica Langer MD, and Heung Bae Kim MD on behalf of The International STEP Data Registry May 23, 2006

Serial Transverse Enteroplasty (STEP) Introduced in 2003 for intestinal lengthening and tapering in Short Bowel Syndrome No single center is likely to obtain sufficient experience Multicenter analysis needed

Demographics 38 patients have been enrolled through April, 2006 –Mean age at STEP 5.4 years (range DOL1 to 19.9 years) –Median Follow-up 11.5 months 19 pediatric surgery groups have submitted patients

Gastroschisis +/- volvulus 11 Segmental Volvulus 2 Congenital Shortened Bowel 1 Hirschsprung Disease 2 Intestinal Atresia 11 Necrotizing Enterocolitis 7 Malrotation with Volvulus 2

Outcomes Pre-STEP Post-STEP Mean 68cm Mean 115cm* * n=27 p<0.0001

Outcomes * n=20 p<0.01 Pre-STEP Post-STEP Mean 33% Mean 63%*

Complications *All mortality occurred from complications of liver failure after referral for transplant IntraoperativePostoperativeLate ComplicationN N N Staple line leak 2 Bowel obstruction 2 Mortality* 3 Aspiration 1 Hypertension 1 Transplantation 3 Hematoma 1 Abscess 1 Pleural effusion 1 Total n=38

Conclusions STEP has been performed successfully and safely around the world The indications for the procedure have broadened to include SBS, bacterial overgrowth and neonatal atresia STEP is effective in bowel lengthening, increasing enteral tolerance and treating persistent refractory bacterial overgrowth The complication profile of the STEP procedure is highly favorable

Conclusions Data provided by the Registry has helped to improve the operative technique and define the indications and outcomes of the STEP procedure With continued accrual, the goal of the International STEP Data Registry is to assist in the process of patient selection and patient counseling

International STEP Data Registry- Participating Groups Austin Pediatric Surgery Association Baylor College of Medicine Children’s Hospital Boston Children’s Hospital of Michigan Children’s Hospital of New Jersey Children’s Hospital of New York Columbus Children’s Hospital C.S. Mott Children’s Hospital, University of Michigan Hospital S Joao (Portugal) Hospital Universitario Valle de Hebron (Spain) Kapiolani Pediatric Surgery Northwest Permanente Pediatric Surgery of Idaho Pediatric Surgical Associates, Minneapolis Penn State University Schneider Children’s Hospital Seattle Children’s Hospital University of Utah Vanderbilt Children’s Hospital