Congenital Small Bowel Syndrome

Congenital short bowel syndrome (CSBS) is a rare entity of short bowel syndrome, with only 37 cases reported in literature till this date, with numerous reports of its high morbidity and mortality rate.

We present a case of a 7-week-old boy who presented with abdominal distension, recurrent vomiting, and chronic diarrhea. The diagnosis of CSBS was confirmed by exploratory laparotomy, and was suggested by imaging studies of the abdomen and upper gastrointestinal tract. Post-operatively, the patient was started on total parenteral nutrition for a month, but subsequently passed away as a result of disseminated sepsis.