Congenital short bowel syndrome with malrotation

Congenital short bowel syndrome (SBS) associated with malrotation and malabsorption is a very rare condition. We report on an infant girl with congenital SBS associated with malrotation and malabsorption. No polyhydraminos was noted during the regular prenatal examination. Protracted postnatal postprandial vomiting with progressive failure to thrive was noted. A laparotomy showed the small bowel was only about 20 cm in length. She eventually survived with short-term parenteral nutrition and use of oral L-glutamine supplementation. To our knowledge, this might be the shortest length of bowel loop ever reported. Currently, she is 15 months of age with a body weight of about 7 kg and good development.