Blue rubber bleb nevus: an uncommon cause of intestinal intussusception

• Published in: European journal of pediatrics 2012-07, Vol.171 (7), p.1139-1140
• Main Authors: Esposito, C., Giurin, I., Farina, A., Ascione, G., Miele, E., Staiano, A., Di Benedetto, V., Settimi, A.
• Format: Article
• Language: English
• Subjects: Abdomen; Anemia; Biological and medical sciences; Case Report; Case reports; Child, Preschool; Dermatology; Digestive system. Abdomen; Endoscopy; Female; Gastrointestinal Neoplasms - complications; General aspects; Hereditary diseases of the skin. Congenital diseases of the skin. Haemangioma of the skin, of mucosae and of soft tissue; Hospitalization; Humans; Ileal Diseases - diagnosis; Ileal Diseases - etiology; Intestinal obstruction; Intussusception - diagnosis; Intussusception - etiology; Investigative techniques, diagnostic techniques (general aspects); Iron; Laparoscopy; Medical sciences; Medicine; Medicine & Public Health; Nevus, Blue - complications; Patients; Pediatrics; Rubber; Skin Neoplasms - complications; Surgery; Ultrasonic imaging
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s00431-012-1706-8

The blue rubber bleb nevus syndrome or Bean syndrome is a rare disorder characterized by cutaneous and gastrointestinal vascular malformations. A 5-year-old girl with Bean syndrome hospitalized in a pediatric unit came under our observation with abdominal pain and vomiting. An X-ray of the abdomen showed an intestinal occlusion and an ultrasonography showed a suspected intestinal invagination. She underwent emergency laparoscopic surgery using three trocars. Laparoscopy revealed a huge ascitis and multiple vascular lesions located on the loops and on the parietal peritoneum, and we identified also an ileo-ileal invagination. We performed a laparoscopic disinvagination that showed one huge vascular lesion producing the invagination and causing a stenosis of intestinal lumen. We performed an intestinal resection after exteriorizing the loops through the umbilicus as well as a termino-terminal ileal anastomosis. Conclusions: Our case shows that an intestinal invagination due to Bean syndrome is extremely rare in pediatric patients but possible. In the emergency, laparoscopy seems to be a safe and effective procedure to confirm the diagnosis and to perform the disinvagination mini-invasivally. In addition, laparoscopy permits to have a clear picture of other intra-abdominal lesions linked to Bean syndrome.