Blue rubber bleb nevus: an uncommon cause of intestinal intussusception.
Eur J Pediatr
; 171(7): 1139-40, 2012 Jul.
Article
in En
| MEDLINE
| ID: mdl-22374252
ABSTRACT
UNLABELLED 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.
Full text:
1
Database:
MEDLINE
Main subject:
Skin Neoplasms
/
Nevus, Blue
/
Gastrointestinal Neoplasms
/
Ileal Diseases
/
Intussusception
Type of study:
Etiology_studies
Limits:
Child, preschool
/
Female
/
Humans
Language:
En
Year:
2012
Type:
Article