Blue rubber bleb nevus: an uncommon cause of intestinal intussusception

The blue rubber bleb nevus syndrome or Beansyndrome is a rare disorder characterized by cutaneous andgastrointestinal vascular malformations. A 5-year-old girlwith Bean syndrome hospitalized in a pediatric unit cameunder our observation with abdominal pain and vomiting.An X-ray of the abdomen showed an intestinal occlusionand an ultrasonography showed a suspected intestinal invagination.She underwent emergency laparoscopic surgeryusing three trocars. Laparoscopy revealed a huge ascitis andmultiple vascular lesions located on the loops and on theparietal peritoneum, and we identified also an ileo-ilealinvagination. We performed a laparoscopic disinvaginationthat showed one huge vascular lesion producing the invaginationand causing a stenosis of intestinal lumen. We performedan intestinal resection after exteriorizing the loopsthrough the umbilicus as well as a termino-terminal ilealanastomosis. Conclusions: Our case shows that an intestinalinvagination due to Bean syndrome is extremely rare inpediatric patients but possible. In the emergency, laparoscopyseems to be a safe and effective procedure to confirm thediagnosis and to perform the disinvagination miniinvasivally.In addition, laparoscopy permits to have a clearpicture of other intra-abdominal lesions linked to Beansyndrome.