Blue rubber bleb nevus syndrome

Krishnappa, Amita; Jeyachandran, Padmini

doi:10.4103/0377-4929.59219

Cited by 10 publications

(8 citation statements)

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“…[ 3 ] Cutaneous lesions may occur mostly in infants and adolescents in the early stage, and are common in the upper limbs and especially the trunk as reported in the literature. [ 4 , 17 , 18 ] Notably, cutaneous lesions associated with BRBNS are generally asymptomatic, although the number of lesions on the skin surface varies from several to more than 100. [ 4 , 19 ] GI lesions can affect the mucosa from the oral cavity to the anus, but are more common in the small intestine and are prone to bleeding, usually leading to chronic IDA.…”

Section: Discussionmentioning

confidence: 99%

Blue rubber bleb nevus syndrome with the complication of intussusception

Zhong

et al. 2020

Medicine

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Rationale: Blue rubber bleb nevus syndrome (BRBNS) is an extremely rare disorder characterized by multifocal venous malformations involving various organs such as the skin and gastrointestinal tract. Severe complications of BRBNS, such as intussusception, volvulus, and intestinal infarction are rarer and require surgery. This report describes a 33-year-old male of BRBNS complicated with intussusception that was successfully diagnosed and treated with surgery. Patient concerns: A 33-year-old Chinese man presented with persistent, colicky pain accompanied by nausea, abdominal distension, and dizziness. The patient presented with sporadic bluish nodules on his skin involving his head, neck, thorax, abdomen, and planta pedis. Diagnoses: BRBNS with the complication of intussusception. Interventions: An emergency laparotomy was performed, and postoperative management included blood transfusions and oral iron supplementation for 2 weeks. Outcomes: The patient's postoperative course of hospitalization was uneventful. During the 4-month follow-up, the patient showed no signs of intussusception recurrence. Lessons: Patients diagnosed with BRBNS who present with acute abdominal pain and distension should raise suspicion for the presence of intussusception, which requires emergent surgical intervention.

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Section: Discussionmentioning

confidence: 99%

Blue rubber bleb nevus syndrome with the complication of intussusception

Zhong

et al. 2020

Medicine

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“…Indian case reports are few in number and most of them are from North India. There have been two published reports from South India [2,3].The case reports from India all are sporadic and mostly in males. Most are associated with skeletal deformities in addition to cutaneous and GI vascular malformation.…”

Section: Discussionmentioning

confidence: 99%

Surgery for Blue Rubber Bleb Nevus Syndrome—a Case Report

Aravindan¹,

Ganesan²,

Kannan³

2017

Indian J Surg

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Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterised by multiple venous malformations and haemangioma in the skin and visceral organs. The lesion often involves the cutaneous and gastrointestinal tract. Most common visceral organ affected is the GIT. Most predominant region involved in the GIT is small bowel. However, vascular lesions can occur anywhere from oral mucosa to anal canal. GIT bleed is relatively slow, resulting in minor, chronic and occult blood loss. The syndrome in the GIT may also present with severe complications such as rupture, volvulus, intussusceptions and even death. Cutaneous malformations are usually asymptomatic and do not require treatment. The treatment of GIT lesions is determined by the extent of intestinal involvement and severity of the disease. Most patients respond to supportive therapy such as iron supplementation and blood transfusion. Surgical resection, endoscopic sclerosis and laser photocoagulation have been proposed for more significant haemorrhage and severe complications. Here, we present a case of BRBNS in a 13-year-old girl involving the GIT especially the large bowel, presenting with the complaints of bleeding per rectum and iron deficiency anaemia. Initially, endoscopic sclerotherapy was performed, but to no response. Hence, proceeded with surgical resection.

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“…Although most cases are spontaneous, there are a few reported cases associated with an autosomal dominant inheritance pattern with a locus found on chromosome 9p. 24 There are several symptoms that may manifest secondary to the blebs. The cutaneous blebs are usually painless, whereas GI lesions can spontaneously rupture and hemorrhage.…”

Section: Blue Rubber Bleb Nevus Syndromementioning

confidence: 99%

Medical Therapy for Pediatric Vascular Anomalies

Margolin

Soni

Pimpalwar

2014

Seminars in Plastic Surgery

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Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number of medical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascular malformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus syndrome (BRBNS). These therapies need to be combined with good supportive care, which often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain and symptom-relief strategies, as well as appropriate drug monitoring and management of side effects of medical treatment. The optimal care of these patients frequently involves close collaboration between surgeons, interventional and conventional radiologists, medical subspecialists, and nurses.

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Blue rubber bleb nevus syndrome

Cited by 10 publications

References 0 publications

Blue rubber bleb nevus syndrome with the complication of intussusception

Blue rubber bleb nevus syndrome with the complication of intussusception

Surgery for Blue Rubber Bleb Nevus Syndrome—a Case Report

Medical Therapy for Pediatric Vascular Anomalies

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