Blue Rubber Bleb Nevus Syndrome

Fishman, Steven J.; Smithers, C. Jason; Folkman, Judah; Lund, Dennis P.; Burrows, Patricia E.; Mulliken, John B.; Fox, Victor L.

doi:10.1097/01.sla.0000154689.85629.93

Cited by 177 publications

(64 citation statements)

References 19 publications

(22 reference statements)

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“…The cutaneous VMs are multiple, small, rubbery often located on the palms and soles. 27, 28 There is often a large lesion present at birth, and an increasing number of multiple dark blue lesions disseminated all over the body. The multiple sessile lesions of the intestine, which cause chronic bleeding and anemia, are a diagnostic criterion.…”

Section: Clinical Presentation Of Combined and Syndromic Malformationsmentioning

confidence: 99%

Venous malformation: update on aetiopathogenesis, diagnosis and management

2010

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The aim of this review was to discuss the current knowledge on etiopathogenesis, diagnosis and therapeutic management of venous malformations. Venous malformations (VMs) are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneo-mucosal venous malformation or glomuvenous malformations), combined (e.g. capillaro-venous, capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, Blue Rubber Bleb Naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies. Those associated with pain are often responsive to Low Molecular Weight Heparin which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose–ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area.

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Section: Clinical Presentation Of Combined and Syndromic Malformationsmentioning

confidence: 99%

Venous malformation: update on aetiopathogenesis, diagnosis and management

2010

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“…However, the most important clinical concern is the high probability of fatal GI bleeding or chronic severe anemia [1] . The GI involvement in BRBNS is typically minimal, circumscribed, and multifocal [6] . The most common site of bowel involvement in BRBNS is the small intestine.…”

Section: Experiences and Lessonsmentioning

confidence: 99%

“…They consist of mature endothelial-lined channels with insufficient surrounding smooth muscle [6] . For convenience, we use the words "hemangioma" to describe both vascular malformations and hemangiomas in this case report.…”

Section: Experiences and Lessonsmentioning

confidence: 99%

Endoscopic therapy for esophageal hematoma with blue rubber bleb nevus syndrome

Takasumi¹,

Hikichi²,

Takagi³

et al. 2014

WJGE

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“…To date, the role of surgical resection in the management of GI BRBNS was deemed controversial. In Hasan Yuksekkaya's literature, surgical resection had been condemned as overly aggressive and unhelpful because of the belief that lesions would recur after removal [ 22 ] ; the risk of short bowel syndrome should be considered if the GI lesions were extensive. Conversely, Zhao-Hui Deng's [ 23 ] review described surgical wedge excision of angiomas or segmental resection of involved bowels was recommended in case of severe or recurrent hemorrhage, and surgery combined with intraoperative endoscopy intervention was proposed.…”

Section: Discussionmentioning

confidence: 99%

Blue rubber bleb nevus syndrome

Chen

Guo

et al. 2017

Medicine

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The aim of our study is to enhance the awareness of blue rubber bleb nevus syndrome (BRBNS) through the patients in our hospital and introduced a new measure of endoscopic intervention.A retrospective review of 5 patients, who were diagnosed as BRBNS in our hospital from January 2013 to January 2017, was conducted. Data were collected with regard to demographics, clinical presentation, endoscopic and imaging findings, management, and follow-up data.In total of 5 patients, the mean age was 28.8 years, range 16 to 44 years (male/female, 1/4) with the average initial age of onset 15.4 years. No family history was identified in our group. Physical examination showed multiple cutaneous lesions in 2 patients (40%, 2/5). All the 5 patients had gastrointestinal tract vascular malformations; stomach involved in 2 cases, large intestine in 2 cases, and small intestine involved in 3 cases. Lesions in the visceral organs and tissue were found in 1 patient. Gastrointestinal bleeding was its main symptom (3/5, 60%). Laboratory investigations revealed anemia in 4 patients and abnormality of coagulopathy in 2 patients with severe anemia. Conservative approach was recommended in 3 cases that included iron supplementation, drug hemostasis, and/or blood transfusion. An innovatively therapeutic approach with endoscopic submucosal dissection (ESD) procedure was used successfully in 1 patient with 2 polypoid BRBNS lesions in rectum.BRBNS is a very rare vascular malformation syndrome with unclear etiopathogenesis and noncurative treatments. ESD procedure was a feasible approach to remove the partial gastrointestinal lesions.

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Blue Rubber Bleb Nevus Syndrome

Abstract: We believe that an aggressive excisional approach is indicated for the venous anomalies that cause GI bleeding in BRBNS.

Cited by 177 publications

References 19 publications

Venous malformation: update on aetiopathogenesis, diagnosis and management

Venous malformation: update on aetiopathogenesis, diagnosis and management

Endoscopic therapy for esophageal hematoma with blue rubber bleb nevus syndrome

Blue rubber bleb nevus syndrome

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