Blue Rubber-Bleb Nevus Syndrome which was Associated with an Atrial Septal Defect: A Case Report

Aroor, Shrikiran; Varma, Chaitanya; Mundkur, Suneel C

doi:10.7860/jcdr/2012/4197.2564

Cited by 3 publications

(3 citation statements)

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“…Rarely, complications like intussusception, volvulus, infarction or hemorrhage can occur. There are few reports of BRBNS with associated cardiac defects like atrial and ventricular septal defects and pulmonary hypertension secondary to thromboembolic events [5,6]. Our patient had pulmonary stenosis, an association hitherto undescribed.…”

Section: Discussionmentioning

confidence: 80%

Blue rubber bleb nevus syndrome with musculo-skeletal involvement and pulmonary stenosis

et al. 2016

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Section: Discussionmentioning

confidence: 80%

Blue rubber bleb nevus syndrome with musculo-skeletal involvement and pulmonary stenosis

et al. 2016

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“…In addition, previous studies have shown some very uncommonly involved tissues. For example, Aroor et al described a case of BRBNS with an atrial septal defect [3]. Huang et al reported a case of BRBNS with both GI and central nervous system involvement.…”

Section: Discussionmentioning

confidence: 99%

“…The management of BRBNS remains considerably challenging, and the lesions in the skin rarely bleed. In contrast, GI lesions manifest throughout the GI tract and begin to bleed at an early age, persisting for the rest of a person's life [3]. The treatment plan is dependent on the extent of the GI involvement and severity of the clinical condition.…”

Section: Discussionmentioning

confidence: 99%

A combination of single-balloon enteroscopy-assisted laparoscopy and endoscopic mucosal resection for treating gastrointestinal venous malformations in blue rubber bleb nevus syndrome: a case report

Dai

et al. 2020

BMC Gastroenterol

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Background: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital disease characterized by multifocal venous malformations. It remains a considerable challenge in treating the gastrointestinal (GI) venous malformations due to multiple lesions throughout the GI tract, and the likelihood of recurrence. We report a case study of BRBNS in the GI tract, in which GI venous malformations and related GI bleeding were successfully treated with a combination of multiple endoscopic procedures. Case presentation: A 17-year-old man was admitted to our hospital for dizziness and hypodynamia. The symptoms persisted for nearly 1 year. The laboratory tests revealed iron-deficiency anemia with abnormally low hemoglobin (Hb), and a strong positive fecal occult blood test. A total of four hemangiomas were detected: one in the stomach, one in the descending colon, and two in the small intestines with a high risk of hemorrhage. Under gastroendoscopy, enteroscopy, and video capsule endoscopy (VCE) throughout the GI tract, the patient underwent surgical treatment. Endoscopic mucosal resection was initially performed in the stomach and colon, and the lesions in the small intestine were resected with laparoscopy auxiliaried by single-balloon enteroscopy (SBE), during which SBE assisted in identifying the lesions. The patient well-tolerated the procedures, and had a favorable prognosis. Conclusion: The combination of single-balloon enteroscopy-assisted laparoscopy and endoscopic mucosal resection was effective for the present case, which could be considered for patients with similar clinical conditions.

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Blue Rubber Bleb Nevus Syndrome in a Child

Srinivas

Premalatha

2015

JCDR

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A 13-year-old female, child born of non consanguineous marriage was referred to Department of Pediatric Dermatology for multiple nevi on trunk and extremities from birth. It was slowly progressive with occasional bleeding from these lesions. She had generalized weakness, fatigue and anaemia from past three years. She was evaluated at various hospitals for anaemia but the cause was not known. There was no history of fever, nausea, vomiting, pain abdomen, melena, haematemesis, epistaxis or recurrent infections. She had received multiple blood transfusions but had recurrent anaemia. Her developmental milestones were normal. Family history was non contributory.On examination, the child had pallor. Vital parameters were normal. Cutaneous examination showed multiple well defined soft compressible bluish-black, verrucous, protuberant bleb like nevi on the dorsum of left hand, left palm, chest, thighs, both soles and medial aspect of left lower leg [Table/ Fig-1,2]. Oral mucosa was normal. Laboratory investigations revealed severe anaemia (haemoglobin-3.7gm/dl), thrombocytopenia (platelet count -89000/ mm 3 ), peripheral smear showed microcytic hypochromic anaemia, with anisopikilocytosis and few target cells. Serum ferritin levels were very low (2.22ng/ml). Stool for occult blood was negative. Ultrasound showed mild hepatosplenomegaly with no focal lesions. Upper GI endoscopy was normal. Colonoscopy showed multiple nodular bluish nevi measuring 3x2 cm in the sigmoid colon. Other investigations like chest X-ray, liver functions test, haemoglobin electrophoresis and bone marrow examination was normal. Skin biopsy revealed epidermis with papillomatosis and orthohyperkeratosis, papillary dermis and reticular dermis showed large ectatic spaces lined by endothelial cells filled with blood [ ABSTRACTBlue rubber bleb nevus syndrome (BRBNS) is a rare vascular disorder with multiple haemangiomas in the skin, gastrointestinal tract and other visceral organs often associated with fatal bleeding and anaemia. We report a 13-year-old female child with multiple haemangiomas on skin and gastrointestinal tract with refractory anaemia. Awareness of this entity is necessary to prevent complications.[ Table/

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Blue Rubber-Bleb Nevus Syndrome which was Associated with an Atrial Septal Defect: A Case Report

Abstract: The Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare cause of persisting gastrointestinal bleeding. We are presenting a case which was associated with an atrial septal defect.

Cited by 3 publications

References 4 publications

Blue rubber bleb nevus syndrome with musculo-skeletal involvement and pulmonary stenosis

Blue rubber bleb nevus syndrome with musculo-skeletal involvement and pulmonary stenosis

A combination of single-balloon enteroscopy-assisted laparoscopy and endoscopic mucosal resection for treating gastrointestinal venous malformations in blue rubber bleb nevus syndrome: a case report

Blue Rubber Bleb Nevus Syndrome in a Child

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