Background: Congenital short bowel syndrome (SBS) associated with malrotation, gut volvulus and jejuno-ileal atresia is a very rare condition. It is a severe challenge for surgeons to preserve residual ischemic bowel segment in the management of short bowel syndrome,especially in neonates.
Case summary: We report a newborn baby with gut malrotation associated with jejuno-ileal atresia, congenital SBS and jejunal volvulus. Hematemesis and abdominal distention were noted. At laparotomy, malrotation associated with jejuno-ileal atresia, congenital SBS and jenunal volvulus was confirmed. The total length of the small bowel was 63 cm with proximal jejunal bowel segment measuring 38 cm, including 18 cm necrotic segment below the Treitz's ligament and 20 cm severe ischemic segment. The distal part of the small bowel was 25 cm in length and only about 0.8 cm in diameter. Ladd's procedure, necrotic segment resection and end-to-back duodeno-ileal anastomosis were performed. The residual severe ischemic jejunum was preserved with single proximal stoma and distal end closure. Three months later, to restore the continuity of the isolated gut segment, end-to-end duodeno-jejunal and jejuno-ileal anastomosis was performed. The entire functional small bowel length increased to 80 cm. Intravenous fluid therapy and parenteral nutrition were discontinued on the 10th day postoperatively. Twelve months later, her body weight was 9.5 kg.
Conclusion: Isolation of severe ischemic bowel segment and staged anastomosis to restore the gut continuity for infants with SBS are safe and feasible.
Keywords: Bowel isolation technique; Case report; Ischemic bowel segment; Short bowel syndrome; Staged salvaging procedure.
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