Blue Rubber Bleb Nevus Syndrome

Abstract

Blue rubber bleb nevus syndrome is a rare disorder characterized by distinctive cutaneous and gastrointestinal venous malformations that usually cause massive or occult gastrointestinal hemorrhage and iron deficiency anemia secondary to the bleeding episodes. It is even a rare cause of gastrointestinal hemorrhage during childhood. We describe a 6-year-old boy who had multiple venous malformations all over his body. He also suffered from several episodes of melena, chronic anemia, and growth retardation. The endoscopic examination of the gastrointestinal tract revealed multiple bluish-black sessile and polypoid venous malformations in various sizes. It was possible to remove the largest venous malformations causing massive bleeding during colonoscopy.

Blue Rubber Bleb Nevus Syndrome: Immunohistochemical Study

“…In other cases, bleeding is acute and is associated with hematemesis or melena. [2][3][4] Less frequently, the angiomas are found in other organs, such as the eye, lung, liver, spleen, skeletal muscle, and central nervous system.> The syndrome is usually present from birth or early childhood, and the lesions tend to increase in size and frequency with age. The pathogenesis is unknown.…”

Blue rubber bleb naevus syndrome: A rare cause of gastrointestinal bleeding in an African child

“…Persistent bleeding from residual untreated small bowel disease and recurrent disease has lead the reliability and consistency of results using this approach to be questioned. [3][4][5][6] As BRBNS most commonly affects small bowel, some surgeons argue for early surgical intervention. [5] Fishman et al [5] reported a series of 10 BRBNS patients presenting with symptomatic gastrointestinal lesions which were treated with operative excision.…”

Blue Rubber Bleb Nevus Syndrome

“…Surgical excision has been discouraged because of the frequent multitudes of lesions and the belief that resected lesions would recur. 9,[17][18][19][20][21] Given that there is no currently available pharmacologic agent to effectively treat the GI bleeding and the likelihood that resected venous malformations would not recur, we undertook a strategy of aggressive removal of all the gastrointestinal lesions of BRBNS. If the rationale were sound, all lesions identified, and technical success possible, we believed that the bleeding could be permanently controlled.…”

“…Surgical resection has been condemned as overly aggressive and unhelpful because of the belief that lesions would recur after removal. 9,[17][18][19][20][21] Confusing terminology has historically obscured the diagnosis and treatment of vascular anomalies, whereas the biologic classification of these anomalies as either vascular tumors or malformations has greatly advanced this field. 22 Because the vascular lesions of BRBNS are consistent with congenital vascular malformations rather than proliferative tumors, it is not surprising that antiangiogenic agents such as corticosteroids and interferon are not effective.…”