Long-Term Outcomes of Short Bowel Syndrome Requiring Long-Term/Home Intravenous Nutrition Compared in Children With Gastroschisis and Those With Volvulus

Abstract 

Introduction

Short bowel syndrome (SBS) is a malabsorptive state that occurs following extensive small intestinal resection. The most severely affected children require intravenous feeding/parenteral nutrition (PN). Two common causes in infancy/early childhood are intestinal resection for volvulus and resection of necrotic small intestine in infants with gastroschisis. The aim of this study was to review the long-term outcomes of children with severe intestinal failure who remained dependent on PN for many months or years. We evaluated the outcomes among gastroschisis cases versus those following resection of mid-gut volvulus or congenital short gut.

Methods

We reviewed the case records over a 10-year period from 1997 of children presenting by 5 years of age with volvulus and/or congenital short gut or gastroschisis and who were dependent on long-term/home PN. We obtained data regarding the diagnosis, the small intestinal length, the presence of the ileo-cecal valve, the survivals, and the incidences of weaning from PN.

Results

Six children underwent resection following volvulus or congenital short gut syndromes and 7 had gastroschisis. Ten of the 13 children underwent surgery as neonates and 3 from 2–5 years of age. The residual small intestinal length was <10 cm in 1, 10–25 cm in 4, 25–50 cm in 5, 50–100 cm in 2, and >100 cm in 1 case. The ileo-cecal valve was removed in 8 children. All children were treated with PN after surgery for at least 7 months. All 7 children with gastroschisis and 1 with <10 cm small intestine after intestinal resection for volvulus still required PN after 14–120 months. Three with gastroschisis died. All 5 children with volvulus and >10 cm small intestine were weaned from PN, thriving at our review 7 months–7 years later.

Conclusions

Infants and young children with short gut and >10 cm small intestine gain intestinal autonomy. In contrast, those with gastroschisis have poor outcomes and should be referred for intestinal transplantation assessment more readily than those with after intestinal resection for volvulus short gut.