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- W64618972 abstract "1 April 2009 Dear Editor, BLUE RUBBER BLEB NEVUS DIAGNOSED BY WIRELESS CAPSULE ENDOSCOPY Blue rubber bleb nevus syndrome (BRBNS) was first described by Gascoyen in 1860 and characterised by Bean in 1958.1 BRBNS is a rare entity consisting of cutaneous and gastrointestinal tract cavernous hemangiomas in the skin, gastrointestinal tract and less often in other organs.1 Until 1993, less than 150 cases were found by Moodley and Ramdial, but nearly 50 further cases have been reported in the literature since then (MEDLINE).2 Chronic iron deficiency anaemia, with or without overt intestinal bleeding, is because of gastrointestinal tract location. Capsule endoscopy (CE) is a videoendoscopic procedure available for paediatric use from the last 5 years, and is a promising and well-tolerated method to assess the entire length of the small bowel.3 An 8-year-old boy was referred with a history of microcytic and hypochromic anaemia diagnosed on a routine analysis. He was born with a cervical lymphangioma resected when he was 3 months old. Physical examination showed pallor, no evidence of growth retardation and three nodular rubbery bluish vascular malformation, slightly elevated over feet plantar surfaces and in the dorsal region of the left lower extremity. The mucous membranes were not affected. The laboratory findings confirmed the anaemia (haemoglobin: 6 g/dL (11.5–15.5 g/dL); VCM: 59.9 fl (77–95 fl); HCM: 17.4 pg (25–33 pg); ferritin level was 2 ng/mL (15–170 ng/mL)) and revealed a positive stool occult blood test. Upper gastrointestinal endoscopy to the ligament of Treitz showed two 5 × 5 mm sessile bluish vascular malformations in the stomach. Five more vascular malformations (5 × 6 mm) were found using CE in the jejunum and ileum (Fig. 1). Push enteroscopy was performed after CE reaching up to distal jejunum and it failed to show the lesions already seen on CE. Wireless capsule endoscopy findings. Vascular malformation located in the jejunum. Histological examination of skin lesions revealed cavernous venous dilatation in submucosa and mucosa. Chronic oral iron supplementation was administered for the anaemia, and after 5 years of follow-up, CE was performed again and showed multiple sessile and pedunculated bluish vascular malformations located in the jejunum and ileum. The patient was diagnosed of BRBNS based on the association of skin and gastrointestinal venous malformations and skin histological findings. BRBNS is a rare disorder characterised by distinctive cutaneous and gastrointestinal cavernous hemangiomas. This syndrome is commonly associated with occult or life-threatening gastrointestinal bleeding and severe iron deficiency anaemia.1,4,5 Patients must be evaluated individually according to clinical presentation, organ systems involved and the severity of bleeding episodes. Endoscopy is useful to diagnose patients and perform treatment modalities such as band ligation, laser photocoagulation or sclerotherapy.6 CE in paediatric age has focused in the study of gastrointestinal bleeding and occult Crohn's disease.3,4 Whereas most reported cases concern adult patients, lesions may present in childhood.4,5 Our report describes one 13-year-old boy in which CE demonstrated venous malformations that push enteroscopy failed to show. In our patient, CE was well tolerated and showed the small bowel extent of the lesions. CE allows the exploration of the whole small bowel and can be proposed as a diagnostic tool in many small bowel diseases in children. In conclusion, CE is an effective and well-tolerated method for evaluating small bowel pathology in patients with occult gastrointestinal bleeding, and should be considered for the detection of gastrointestinal venous malformations and for surveillance procedure in these patients." @default.
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- W64618972 date "2010-01-01" @default.
- W64618972 modified "2023-10-18" @default.
- W64618972 title "Letter to the Editor" @default.
- W64618972 cites W1983696078 @default.
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- W64618972 doi "https://doi.org/10.1111/j.1440-1754.2009.01668.x" @default.
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