Endoscopy 2019; 51(05): E98-E100
DOI: 10.1055/a-0840-3214
E-Videos
© Georg Thieme Verlag KG Stuttgart · New York

Blue rubber bleb nevus syndrome: endoscopic treatment with sclerotherapy during double-balloon enteroscopy in a 9-year-old boy

Authors

  • Maria Carolina Malafaia

    1   Department of General and Digestive Surgery, Professor Alberto Antunes University Hospital, Maceió, Alagoas, Brazil
  • Sophie Heissat

    2   Department of pediatric gastroenterology, Hospital Femme Mere Enfant, Lyon
  • Philippe Jacob

    2   Department of pediatric gastroenterology, Hospital Femme Mere Enfant, Lyon
  • Catherine Le Gall

    2   Department of pediatric gastroenterology, Hospital Femme Mere Enfant, Lyon
  • Mathias Ruiz

    2   Department of pediatric gastroenterology, Hospital Femme Mere Enfant, Lyon
  • Jean-Christophe Saurin

    3   Department of Endoscopy and Gastroenterology, Pavillon L, Edouard Herriot Hospital, Lyon, France
  • Mathieu Pioche

    3   Department of Endoscopy and Gastroenterology, Pavillon L, Edouard Herriot Hospital, Lyon, France
    4   Inserm U1032 LabTau, Lyon, France
Further Information

Publication History

Publication Date:
12 February 2019 (online)

Preview

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]).

Zoom
Fig. 1 Endoscopic view of the angiomas. a Gastric antral lesion. b Gastric pre-pyloric lesion. c Colonic lesion.
Zoom
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.

Zoom
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

Endoscopy E-Videos is a free access online section, reporting on interesting cases and new techniques in gastroenterological endoscopy. All papers include a high quality video and all contributions are freely accessible online.
This section has its own submission website at https://mc.manuscriptcentral.com/e-videos