11/42. Chronic subcutaneous octreotide decreases gastrointestinal blood loss in blue rubber-bleb nevus syndrome.BACKGROUND: A patient affected by blue rubber-bleb nevus syndrome had chronic gastrointestinal bleeding requiring weekly blood transfusions. Despite multiple surgical and endoscopic procedures to treat the venous malformations, the patient continued to bleed primarily from lesions in the small bowel. Therefore, this patient was treated with octreotide, a somatostatin analog known to decrease splanchnic blood flow and that is used for acute and chronic gastrointestinal bleeding. methods: octreotide therapy, 5.7 microg/kg subcutaneously twice daily, was initiated, and the patient was followed up clinically. Complete blood counts, blood glucose concentration, pancreatic enzyme concentration, liver function tests, and growth hormone concentration were monitored during treatments. RESULTS: During the 4 weeks after initiation of octreotide therapy, hemoglobin concentration was maintained without the need for transfusions. octreotide decreased the patient's monthly need for blood transfusion from 52 /- 7 mL. kg-1. mo-1 of packed red blood cells to 23 /- 7 mL. kg-1. mo-1. She had no detectable side effects or growth inhibition. Other medical interventions including -epsilonaminocaproic acid, nadolol, and total parenteral nutrition with bowel rest were not as effective as octreotide alone. CONCLUSION: octreotide decreased the patient's need for blood transfusions. Possible mechanisms include altering blood flow to the gastrointestinal tract and direct effects on the venous malformations.- - - - - - - - - - ranking = 1keywords = rubber (Clic here for more details about this article) |
12/42. Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome.PURPOSE: To report the clinical findings and surgical treatment of multifocal, bilateral orbital hemangiomas in a patient with the blue rubber bleb nevus syndrome, a rare vascular disorder characterized by distinctive cutaneous and visceral hemangiomas. DESIGN: Interventional case report. methods: review of clinical findings, radiologic studies, and treatment of the patient. RESULTS: A 36-year-old female with multifocal, bilateral orbital lesions was seen with severe proptosis and decreased visual acuity of the left eye. Surgical debulking of multiple cavernous hemangiomas of the orbit resulted in decreased proptosis and an improvement in visual acuity. Subsequent histopathologic analysis and the results of a systemic workup revealing multiple, distinctive, cutaneous and visceral lesions were found to be consistent with the blue rubber bleb nevus syndrome. CONCLUSIONS: Although hemangiomas of the orbit are relatively common occurrences, multifocal, bilateral lesions may exist in the setting of underlying systemic hemangiomatosis. Bilateral or multifocal hemangiomas associated with cutaneous or visceral lesions should alert the clinician to the possibility of a number of syndromes that may have life-threatening consequences.- - - - - - - - - - ranking = 1.2keywords = rubber (Clic here for more details about this article) |
13/42. Blue rubber bleb nevus syndrome in a patient with ataxia and dementia.Blue rubber bleb nevus syndrome (BRBNS), an uncommon disorder characterized by cavernous hemangiomas, most often of the skin and gastrointestinal tract, is usually diagnosed during childhood and young adulthood. We made this diagnosis in an octogenarian referred to a geriatric medicine clinic because of concerns about his ability to live independently. ataxia, dementia, focal neurologic signs, and bluish/purplish vascular nodules on his lips, buccal mucosa, tongue, chest, and neck were noted on physical examination. magnetic resonance imaging (MRI) revealed an old left parietal infarction, multiple cavernous hemangiomas most densely concentrated in the subcortical structures and cerebellum, and areas of hemosiderin deposition. skin biopsy findings were consistent with hemangioma. The physical examination, MRI, and skin biopsy made a diagnosis of BRBNS likely. The patient's ataxia, dementia, and other neurologic signs can be explained by previous hemorrhage from the vascular malformations in his brain. Blue rubber bleb nevus syndrome is an uncommon cause of a relatively common geriatric syndrome presentation.- - - - - - - - - - ranking = 1.2keywords = rubber (Clic here for more details about this article) |
14/42. Blue rubber bleb nevus syndrome and gastrointestinal haemorrhage: which treatment?PURPOSE: To describe a paediatric case of "Blue rubber Bleb nevus Syndrome" (BRBNS) or Bean's syndrome, a rare systemic disorder characterised by cutaneous and gastrointestinal vascular malformations that often lead to overt life-threatening gastrointestinal bleeding or occult blood loss with severe anaemia and iron deficiency. CASE REPORT: A 6-year-old girl with multiple characteristic cutaneous vascular lesions was admitted for a massive rectal bleeding. A few months previously she was endoscopically treated for gastric angiomas which developed into melaena. Preoperative investigations revealed the recurrence of gastric lesions. At laparotomy, more than 25 angiomas of the GI tract were found. Multiple intestinal resections were carried out. RESULTS: No intraoperative or postoperative problems occurred and the girl is completely healthy without further bleeding after a follow-up period of three years. CONCLUSIONS: BRBNS belongs to the group of vascular venous malformations. Most of the time it occurs sporadically, but it can be inherited as an autosomal dominant trait. Recent analysis identified a locus on chromosome 9 responsible for venous malformations. BRBNS patients present typical skin lesions, with some lesions having a rubber-like nipple appearance; the number of skin and GI lesions and the severity of anaemia are correlated. Treatment is dependent on the extent of gut involvement and the severity of the clinical picture. In the absence of massive bleeding, a conservative treatment will be sufficient; otherwise resections are mandatory, but additional lesions may subsequently develop. Management with electrocautery or laser photocoagulation are usually not effective even if some reports recommend them. Pharmacological treatment is useless. prognosis of BRBNS is unknown.- - - - - - - - - - ranking = 1keywords = rubber (Clic here for more details about this article) |
15/42. Blue rubber-bleb nevus syndrome: a case report.Blue rubber-bleb nevus syndrome (BRBNS) is an uncommon systemic disorder characterized by cutaneous and visceral cavernous hemangiomas. The characteristic rubbery textured and easily compressible lesions usually present in childhood and predominate cutaneously over the trunk and extremities. Gastrointestinal foci appear most commonly in the small bowel, a site that appears to dominate visceral involvement. We review the case of a 23-year-old white woman, whose numerous lesions on her trunk, extremities, and oral mucosa had been present since childhood.- - - - - - - - - - ranking = 1.2keywords = rubber (Clic here for more details about this article) |
16/42. argon plasma coagulation for blue rubber bleb nevus syndrome in a female infant.We report on the case of an 8-month-old baby with blue rubber bleb nevus syndrome (BRBNS) presenting with recurrent bleeding from multiple haemangiomas in the colon and rectum. Treatment with argon plasma coagulation resulted in long-term success. Our case is unique in that 1) it is the first documented case treated by argon plasma coagulation; 2) it is only the second case in the world literature with involvement of the genital tract; 3) it is the youngest patient treated endoscopically to date; 4) it is the endoscopically treated case with the longest follow-up; 5) it represents the second case of BRBNS reported from this part of the world; and 6) it expands the spectrum of mucosal lesions amenable to this new form of treatment. argon plasma coagulation is a simple, inexpensive and effective treatment for selected lesions in BRBNS.- - - - - - - - - - ranking = 1keywords = rubber (Clic here for more details about this article) |
17/42. Blue rubber bleb nevus syndrome: case report.The case of a patient with blue rubber bleb nevus syndrome who is infected by acquired immunodeficiency syndrome virus due to multiple blood transfusions is presented. This case shows that although it is a rare systemic disorder, blue rubber bleb nevus syndrome has to be considered in the differential diagnosis of chronic anemia or gastrointestinal bleeding. patients should be investigated by endoscopy, which is the most reliable method for detecting these lesions. The patient underwent gastroscopy and enteroscopy via enterotomy with identification of all lesions. Minimal resection of the larger lesions and string-purse suture of the smaller ones involving all the layers of the intestine were performed. The string-purse suture of the lesions detected by enteroscopy proved to be an effective technique for handling these lesions, avoiding extensive intestinal resection and stopping the bleeding. Effective management of these patients demands aggressive treatment and should be initiated as soon as possible to avoid risks involved in blood transfusions, as occurred in this case.- - - - - - - - - - ranking = 1.2keywords = rubber (Clic here for more details about this article) |
18/42. Blue rubber bleb nevus syndrome: immunohistochemical study.Blue rubber bleb nevus is a rare entity consisting of distinctive angiomas in the skin and gastrointestinal tract, leading to occult or profound gastrointestinal bleeding and chronic anemia. The efficacy has been documented of systemic treatment with corticoids, interferon, vincristine, and, more recently, subcutaneous octreotide in the presence of active lesion proliferation or disseminated intravascular coagulation. A case of blue rubber bleb nevus syndrome with oral hemangiomas in a 24-year-old woman is reported. The surgical specimens were subjected to immunohistochemical study, which showed all of the hemangiomas to be in an inactive phase.- - - - - - - - - - ranking = 1.2keywords = rubber (Clic here for more details about this article) |
19/42. Blue rubber bleb nevus syndrome: endobronchial involvement presenting as chronic cough.This case report describes a rare presentation of chronic cough secondary to endobronchial involvement with blue rubber bleb nevus syndrome (BRBNS) lesions. BRNBS is a rare syndrome characterized with cavernous hemangiomas involving the skin and GI tract. We report the case of a 37-year-old woman, with known BRBNS, who acquired an intractable cough with a radiograph revealing multiple pulmonary nodules. A bronchoscopy demonstrated various bluish, raised, and hypervascular lesions characteristic for BRBNS involving the right mainstem bronchus and segmental bronchi. To our knowledge, this is the first report of endobronchial involvement with BRBNS.- - - - - - - - - - ranking = 1keywords = rubber (Clic here for more details about this article) |
20/42. Developmental venous anomalies and sinus pericranii in the blue rubber-bleb nevus syndrome. Case report.Blue rubber-bleb nevus syndrome (BRBNS) is a developmental disorder that originally was identified by the presence of distinctive cutaneous and gastrointestinal hemangiomas. More recently it has been recognized that the number of affected organs is larger and that BRBNS includes central nervous system vascular malformations. A 52-year-old woman in whom intracranial vascular malformations had been diagnosed earlier presented for evaluation. At birth, several blue nevi had been noted on her tongue, lips, and neck. cerebral angiography demonstrated an extensive network of developmental venous anomalies and a left anterior sinus pericranii. The literature on BRBNS and developmental venous anomalies is reviewed.- - - - - - - - - - ranking = 1keywords = rubber (Clic here for more details about this article) |
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