Abstract
Blue rubber bleb naevus syndrome is a rare vascular disorder associated with multiple gastrointestinal haemangiomas that have the potential for life-threatening haemorrhage. These may be difficult to diagnose, and have classically been described using computed tomographic studies and/or mesenteric angiography. Resected surgical specimens of these lesions, especially in the small bowel, have often been extensive and poorly localized. The recent advent and progressive development of double balloon enteroscopy has allowed the direct visualization and marking of these enteric lesions and serves as a valuable adjunct not only in diagnosis but also planning prior to surgery to allow accurate estimate of the extent of resection.
Highlights
Blue rubber bleb naevus syndrome, known as Bean Syndrome was first described in 1958 on the basis of 1-2 cm violaceous cutaneous lesions, is a rare disorder consisting of gastrointestinal vascular malformations, which carry a significant potential for serious haemorrhage
Double balloon enteroscopy was developed in 2001 as a technique to endoscopically visualize the small bowel in real time and allow therapeutic intervention such as biopsy, dilatation and placement of stents, and it has largely supplanted Computed tomography (CT) enteroclysis and angiography in the diagnosis of small bowel sources of blood loss [2]
In one of few prospective studies on blue rubber bleb naevus syndrome, Fishman et al demonstrated the surgical resection resulted in elimination of bleeding in nine out of ten patients without recurrence over a five year follow-up period [6]
Summary
Blue rubber bleb naevus syndrome, known as Bean Syndrome was first described in 1958 on the basis of 1-2 cm violaceous cutaneous lesions, is a rare disorder consisting of gastrointestinal vascular malformations, which carry a significant potential for serious haemorrhage. Case presentation A 58-year-old female, Irish, Caucasian patient presented, on a background history of chronic anaemia, with fatigue, palpitations, dyspnoea and haemoglobin of 5.4 g/dL She had received intermittent blood transfusions during the previous three years. The patient underwent a mini laparotomy via a 5 cm incision, with segmental jejunal resection and primary hand-sewn anastomosis. Histology demonstrated sections of jejunal polyps showing multiple cavernous haemangiomas, many of which showed organizing thrombus. Given the multiplicity, these were consistent with the blue rubber bleb naevus syndrome
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