Volvulus of a jejunal lymphatic vascular malformation presenting with bilious vomiting and the radiological appearances of malrotation

Abstract 

Bile-stained vomiting in infancy is abnormal. Malrotation with small bowel volvulus is probably the most worrying of possible causes because if untreated for too long, it can result in massive bowel necrosis. As a consequence, if examination or plain radiography does not suggest another cause, an upper gastrointestinal (GI) series is often performed to exclude malrotation. If the examination is normal however and symptoms settle, should investigation stop, or how far should it be pursued?

Small bowel lymphatic vascular malformations in children are extremely rare but carry a risk of obstruction, volvulus, and loss of small bowel. We present a case of a 5-year-old boy referred to our unit with bilious vomiting and the radiological findings of malrotation on upper GI series. Interestingly, a previous upper GI series undertaken during the first year of life for intermittent bilious vomiting was normal. Operative findings were of a 16-cm cystic lymphatic vascular malformation of the jejunal wall causing a 360° volvulus. The mesentery was derotated, and the cyst removed with the attached section of jejunum. This case highlights the need to reinvestigate patients with bilious vomiting despite a previous normal upper GI series. The case raises the issue of whether bilious vomiting, with a normal upper GI series, should be investigated further with an abdominal ultrasound scan to identify more unusual causes such as jejunal lymphatic vascular malformations.