Abstract
We report a case of a neonatal patient with ultrashort bowel syndrome with 0 cm of residual small intestine, on whom we performed an initial surgery and nutritional management, considering the future use of teduglutide. The patient was an 8-day-old female infant. Owing to intestinal necrosis caused by midgut volvulus associated with intestinal malrotation, the entire small intestine and the right side of the colon were resected. The duodenum was left as a blind end, and a mucous fistula was created with the remaining colon. Decompression of the duodenal blind end was achieved using a gastric tube. At 2 months of age, a reoperation revealed the dilation and extension of the duodenum, and a duodenum–colon side-to-end anastomosis was performed. Additionally, a central venous catheter was placed, alternating every 3 months between the right and left internal jugular veins. Her energy quotient was managed at a relatively low level of 60–70 kcal/kg/day. At 6 months of age, the patient transitioned to home care, with an average weight gain of 6.0 g/day. However, after starting teduglutide administration at 1 year and 3 months, the average weight gain increased to 18.0 g/day. In this case, good weight gain was achieved through surgical innovation and the use of teduglutide. A continued long-term follow-up will be necessary.
