Blue rubber bleb nevus syndrome (BRBNS), also called Bean syndrome, is a rare disease associated with multiple venous malformations essentially in the skin and the gastrointestinal (GI) tract [1]
[2]. It commonly presents with anemia and patients respond to supportive measures, but severe symptoms may be treated by surgical resection, endoscopic sclerotherapy, and laser photocoagulation [3]
[4].
We report here a case of BRBNS presenting with GI bleeding in a 9-year-old boy who was first diagnosed at the age of 1, with a venous angioma in the knee. The onset of melena led to a complete exploration of the upper and lower GI tract, and capsule endoscopy, which revealed several typical blue lesions in the stomach ([Fig. 1 a, b]), small bowel ([Fig. 2]), and colon ([Fig. 1 c]).
Fig. 1 Endoscopic view of the angiomas. a Gastric antral lesion. b Gastric pre-pyloric lesion. c Colonic lesion.
Fig. 2 Capsule endoscopic views of angiomas in the small bowel.
Gastric and colonic lesions were first treated with argon plasma coagulation ([Fig. 3]) in the pediatric hospital. The patient was then referred to our unit for double-balloon enteroscopy (EN-580T; Fujifilm, Tokyo, Japan), through which we could identify five pedunculated lesions and four flat friable ones. The progression of the endoscope was difficult owing to the small size of the intestinal loops; however, successful sclerotherapy of the lesions was achieved by injecting aetoxisclerol ([Video 1]). Apart from these angiomas, no further lesions required treatment, according to capsule endoscopy. The patient did not experience any new bleeding after this treatment.
Fig. 3 The lesions after treatment with argon plasma coagulation. a Gastric antral lesion. b Gastric pre-pyloric lesion. c Colonic lesion.
Video 1 Video capsule endoscopy diagnosis and double-balloon endoscopic sclerotherapy of gastrointestinal angiomas.
In conclusion, BRBNS is rare but the diagnosis has to be considerd when typical lesions are seen in different areas. Double-balloon enteroscopy using an adult enteroscope appears to be feasible in children, but progression is probably more difficult in a small-diameter bowel. As previously demonstrated, aetoxisclerol seems to be effective for the treatment of lesions with low risk of perforation.
Endoscopy_UCTN_Code_TTT_1AP_2AD
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