Introduction: Gastroschisis (GS) is the most common abdominal wall defect requiring neonatal operative intervention and it represents one of the most costly congenital defects. It is associated with short and long-term morbidity associated with complications due to preterm birth, sepsis, need for small bowel resections, necrotizing enterocolitis (NEC), associated anomalies such as intestinal atresia (in 10 to 20% of patients with GS) and other birth defects. We report all patients with GS who were surgically treated or underwent intestinal rehabilitation (IR) at our institution in the past 10 years describing features, management and outcome.

Methods: We retrospectively reviewed patients with diagnosis of GS treated at our institution from July 2007 to January 2018.

Results: In this study a total of 91 patients with GS were identified (62% male). Median gestational age was 36 weeks (interquartile range [IQR]: 33-38).

Sixty-four patients (71%) had uncomplicated GS. Primary surgical closure in these patients was associated with earlier intestinal autonomy and shorter duration of PN in 31% of patients (p<0.05). In this group, introduction of feeds occurred at a median age of 23 days of life (IQR: 13-35), 90% of them received breast milk with supplemental standard infant formula.

Twenty-seven patients (29%) had complex GS; their complications include: intestinal atresia (n=12), intestinal necrosis at birth requiring bowel resection (n=10), medical NEC (n=18), bacteremia (n=14) and severe intestinal dysmotility requiring promotility medications (n=13).

All patients with complex GS initiated treatment by our Intestinal Rehabilitation Program (IRP) at a median age of 2.5 months. Upon initial evaluation, 88% of patients (n=24) were on parenteral nutrition (PN) that provided a mean of 97% of their daily calorie goal. 17 patients (71%) were weaned off PN after a mean of 7 months of IR. 7 patients (29%) remain PN dependent with a median bowel length of 34 cm, their mean daily energy requirement from PN is presently 48%, down from 90% at IRP enrollment.

Conclusion: Multiple prenatal and postnatal events affect outcomes in patients with GS. Patients with complex GS are at higher risk of morbidity. A specialized multidisciplinary IRP is crucial in the management of these patients to optimize their health related outcomes.