329 results match your criteria Blue Rubber Bleb Nevus Syndrome


Blue rubber bleb nevus syndrome: endoscopic treatment with sclerotherapy during double-balloon enteroscopy in a 9-year-old boy.

Endoscopy 2019 Feb 12. Epub 2019 Feb 12.

Department of Endoscopy and Gastroenterology, Pavillon L, Edouard Herriot Hospital, Lyon, France.

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http://dx.doi.org/10.1055/a-0840-3214DOI Listing
February 2019
6 Reads

Magnetically Controlled Capsule Endoscopy in Children: A Single Center, Retrospective Cohort Study.

J Pediatr Gastroenterol Nutr 2019 Feb 7. Epub 2019 Feb 7.

Department of Gastroenterology, Hepatology and Nutrition, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai 200062, China.

Objective: Capsule endoscopy (CE) is a noninvasive diagnostic tool for the digestive tract. We aim to investigate the feasibility and safety of newly-developed magnetically controlled capsule endoscopy (MCE) in children.

Methods: A total of 129 children who underwent MCE in Shanghai Children's Hospital were retrospectively recruited between March 2016 and August 2018. Read More

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http://dx.doi.org/10.1097/MPG.0000000000002292DOI Listing
February 2019
2 Reads

Photoacoustic imaging as an innovative technique for the exploration of Blue Rubber Bleb Nevus.

Br J Dermatol 2019 Feb 11. Epub 2019 Feb 11.

Institute for Advanced Biosciences, Univ. of Grenoble Alpes, INSERM U1209 and CNRS UMR5309, 38000, Grenoble, France.

Blue rubber bleb nevus syndrome (BRBN, Bean syndrome) is a rare vascular malformation (VM) characterized by multiple cutaneous and mucous venous defects disseminated throughout the body. An infringement of mucous membranes is possible with potential complications as anemia or hemorrhage. Photoacoustic imaging (PAI) is an emerging technology combining the most compelling features of optical imaging and ultrasound, providing both high optical contrast and high ultrasound resolution at depth in living organisms . Read More

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http://doi.wiley.com/10.1111/bjd.17765
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http://dx.doi.org/10.1111/bjd.17765DOI Listing
February 2019
7 Reads

Glomuvenous malformations in a young man.

Dermatol Online J 2018 Oct 15;24(10). Epub 2018 Oct 15.

University of Mississippi Medical Center School of Medicine, Department of Dermatology, and Department of Pathology, Jackson, Mississippi.

A young man presented with blue nodules on the trunk, face, and extremities that gradually increased in number and size. His mother had similar lesions. Initially, blue rubber bleb nevus syndrome was suspected, but histological findings confirmed the diagnosis of hereditary glomuvenous malformations. Read More

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October 2018

Gastrointestinal: Endoscopic injection sclerotherapy for duodenal vascular malformation in blue rubber bleb nevus syndrome.

J Gastroenterol Hepatol 2019 Jan 14. Epub 2019 Jan 14.

Division of Gastroenterology, Department of Internal Medicine, School of Medicine, Iwate Medical University, Morioka, Japan.

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http://dx.doi.org/10.1111/jgh.14590DOI Listing
January 2019
1 Read

Blue Rubber Bleb Nevus Syndrome with Long-Term Follow-Up: A Case Report and Review of the Literature.

Case Rep Gastrointest Med 2018 25;2018:8087659. Epub 2018 Nov 25.

Department of Pediatric Surgery, Shizuoka Children's Hospital, 860 Urushiyama, Aoi-ku, Shizuoka 420-8660, Shizuoka, Japan.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease in which venous malformations (VMs) involve any body organ, most commonly the skin and the gastrointestinal (GI) tract. Treatment of BRBNS aims at preserving the GI tract as much as possible. Although there are several dozen case reports about BRBNS that describe short-term clinical courses, a few provide an account of long-term clinical course. Read More

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http://dx.doi.org/10.1155/2018/8087659DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6286777PMC
November 2018
2 Reads

Blue Rubber Bleb Nevus Syndrome: A Possible Cause for Growth Retardation and Pubertal Delay.

Med Princ Pract 2018 Dec 10. Epub 2018 Dec 10.

Objective: To describe a rare case of Blue Rubber Bleb Nevus (BRBNS) with growth retardation and pubertal delay.

Clinical Presentation And Intervention: A 16-year-old boy with severe iron deficiency anemia was diagnosed with BRBNS, showing growth retardation and pubertal delay simultaneously. The patient was treated conservatively with intravenous iron therapy and his puberty advanced gradually. Read More

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http://dx.doi.org/10.1159/000496105DOI Listing
December 2018
8 Reads

Successful Management of Blue Rubber Bleb Nevus Syndrome (BRBNS) with Sirolimus.

Case Rep Pediatr 2018 8;2018:7654278. Epub 2018 Oct 8.

Division of Pediatric Hematology/Oncology, Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA, USA.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease with vascular malformations in several systems of the body, most commonly the skin and gastrointestinal tract. Bleeding from the gastrointestinal (GI) tract is a major complication, which may lead to chronic iron deficiency anemia and the need for frequent blood transfusions due to ongoing gastrointestinal blood loss. In this case report, we describe a now 19-year-old female with BRBNS who required six blood transfusions per year and after starting sirolimus is symptom- and transfusion-free. Read More

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http://dx.doi.org/10.1155/2018/7654278DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196891PMC
October 2018
11 Reads

Blue Rubber Bleb Nevus Syndrome.

Anesthesiology 2018 Dec;129(6):1169

From the Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, Massachusetts.

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http://dx.doi.org/10.1097/ALN.0000000000002414DOI Listing
December 2018
1 Read

Surgery for Blue Rubber Bleb Nevus Syndrome-a Case Report.

Indian J Surg 2018 Jun 19;80(3):272-274. Epub 2017 Dec 19.

Department of General Surgery ,TMCH, Thanjavur, India.

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. Read More

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http://dx.doi.org/10.1007/s12262-017-1715-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6014949PMC
June 2018
28 Reads

A 10-year delayed diagnosis of blue rubber bleb nevus syndrome characterized by refractory iron-deficiency anemia: A case report and literature review.

Medicine (Baltimore) 2018 Jun;97(22):e10873

Department of Pediatrics, West China Second University Hospital.

Rationale: Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular disorder consisting of multifocal venous malformations. Delayed diagnosis or misdiagnosis frequently occurs in patients without typical cutaneous lesions or gastrointestinal bleeding symptoms. This article reports a 10-year case of delayed diagnosis of BRBNS detected by capsule endoscopy. Read More

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http://dx.doi.org/10.1097/MD.0000000000010873DOI Listing
June 2018
7 Reads

An unusual cause of gastrointestinal bleeding in a hemodialysis patient.

Hemodial Int 2018 Oct 2;22(4):E60-E62. Epub 2018 Apr 2.

AURA Paris Plaisance, Paris, France.

Iron deficiency caused by gastrointestinal (GI) bleeding is a common cause of anemia in hemodialysis patient. Herein, we report the case of an 89-year-old hemodialysis patient who presented with acute anemia and melena. Endoscopy found gastritis and diverticulosis without active bleeding. Read More

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http://dx.doi.org/10.1111/hdi.12657DOI Listing
October 2018
27 Reads

[Blue rubber bleb naevus syndrome involving the pleura].

Rev Mal Respir 2018 Mar 28;35(3):333-337. Epub 2018 Mar 28.

Service d'oncologie thoracique, maladies de la plèvre et pneumologie interventionnelle, hôpital Nord, chemin des Bourrely, 13015 Marseille, France; Aix-Marseille université, 13284 Marseille cedex 07, France. Electronic address:

Bean's syndrome ('blue rubber bleb nevus syndrome') is a rare disease characterized by venous malformations involving various organs. Most often these lesions are localized to the skin and the digestive system. Gastro-intestinal bleeding is the most frequent presentation. Read More

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http://dx.doi.org/10.1016/j.rmr.2017.06.006DOI Listing

Fern-shaped patch as a hallmark of blue rubber bleb nevus syndrome in neonatal venous malformations.

Eur J Pediatr 2018 Sep 8;177(9):1395-1398. Epub 2018 Mar 8.

Department of Pediatric Surgery, University Hospital La Paz, Madrid, Spain.

Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital vascular disease associated with important morbidity and non-insignificant risk of mortality in cases of severe gastrointestinal or neurological involvement. Early diagnosis in the neonatal period can be difficult as very often skin lesions appear progressively during childhood having no correlation with gastrointestinal lesion development. The appearance of one large venous malformation (VM) in the neonatal period has suggested a characteristic finding of this syndrome. Read More

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http://dx.doi.org/10.1007/s00431-018-3126-xDOI Listing
September 2018
7 Reads

[Bean's syndrome in children: about two cases].

Pan Afr Med J 2017 4;28:102. Epub 2017 Oct 4.

Service des Urgences Chirurgicales Pédiatriques, CHU Ibn Sina, Faculté de Médecine Mohammed V, Rabat, Maroc.

Diffuse angiomatosis or Bean's syndrome is a rare disease characterized by venous malformations mainly involving the skin and the digestive tract which can result in hemorrhage of variable severity. This study reports the case of two children aged 5 and 9 and a half years respectively with diffuse angiomatosis who had been treated in the Department of Emergency Paediatric Surgery over the years. The diagnosis was based on rectal bleeding and/or melenas causing severe anemia requiring regular transfusions in both patients as well as skin angiomas occurrence at the level of the limbs. Read More

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http://dx.doi.org/10.11604/pamj.2017.28.102.11109DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5837144PMC
March 2018
7 Reads

New therapies for vascular anomalies of the gastrointestinal tract.

Authors:
Victor L Fox

Minerva Pediatr 2018 Jun 23;70(3):303-307. Epub 2018 Feb 23.

Gastrointestinal Procedures and Endoscopy Unit, Division of Gastroenterology, Hepatology, and Nutrition, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA -

Vascular anomalies are a morphologically and biologically diverse group of vascular channel abnormalities that are often congenital but may evolve or change over time in the developing child. Classification is based on a combination of physical and biological properties and clinical behavior that differentiate primarily between tumors and malformations and includes a few provisionally unclassified lesions. Anomalies of the gastrointestinal (GI) tract may present clinically with GI bleeding, abdominal pain, high-output cardiac failure, and malabsorption. Read More

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http://dx.doi.org/10.23736/S0026-4946.18.05207-6DOI Listing
June 2018
32 Reads

Sirolimus alternative to blood transfusion as a life saver in blue rubber bleb nevus syndrome: A case report.

Medicine (Baltimore) 2018 Feb;97(8):e9453

Department of Pediatrics, Chinese PLA General Hospital, Beijing, China.

Rationale: Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by multiple venous malformations. The gastrointestinal bleeding and secondary iron deficiency anemia are the most common complications. There are currently no effective treatments for BRBNS. Read More

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http://Insights.ovid.com/crossref?an=00005792-201802230-0001
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http://dx.doi.org/10.1097/MD.0000000000009453DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5841973PMC
February 2018
19 Reads

An institution-wide algorithm for direct-stick embolization of peripheral venous malformations.

J Vasc Surg Venous Lymphat Disord 2018 05 1;6(3):351-357. Epub 2018 Feb 1.

Division of Hematology, Department of Medicine, Cancer Institute of New Jersey, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ.

Objective: No standardized therapeutic algorithm or embolic agent of choice has yet been identified for management of congenital peripheral venous malformations (VMs). Treatment options and reported outcomes therefore vary widely. Herein, we present an institution-wide algorithm for management of symptomatic congenital peripheral VMs using a single embolotherapeutic modality. Read More

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http://dx.doi.org/10.1016/j.jvsv.2017.12.011DOI Listing
May 2018
34 Reads

A Rare Cause of Chronic Anemia and Recurrent Bowel Obstruction.

Gastroenterology 2018 Feb 18;154(3):e4-e7. Epub 2018 Jan 18.

Department of Surgery, Chung Shan Medical University Hospital, Taichung, Taiwan; Institute of Medicine, Chung Shan Medical University, Taichung, Taiwan.

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http://dx.doi.org/10.1053/j.gastro.2017.05.025DOI Listing
February 2018
8 Reads

Nevus-like lesions on the lip and the foot.

Eur J Intern Med 2018 07 12;53:e6-e7. Epub 2018 Jan 12.

Ege University, Faculty of Medicine, Dermatology Department, Izmir, Turkey. Electronic address:

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http://dx.doi.org/10.1016/j.ejim.2017.12.017DOI Listing
July 2018
3 Reads

Sirolimus Experience in Blue Rubber Bleb Nevus Syndrome.

J Pediatr Hematol Oncol 2018 Mar;40(2):168-169

Division of Pediatric Hematology-Oncology Cerrahpasa Faculty of Medicine Istanbul University.

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http://dx.doi.org/10.1097/MPH.0000000000001070DOI Listing
March 2018
46 Reads

Images in Vascular Medicine: Blue rubber bleb nevus syndrome: A rare cause of gastrointestinal bleeding and vascular anomaly in children.

Vasc Med 2018 02 6;23(1):82-83. Epub 2017 Oct 6.

2 Department of Medicine, University Hospital Centre Sisters of Charity, Zagreb, Croatia.

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http://dx.doi.org/10.1177/1358863X17735596DOI Listing
February 2018
14 Reads

Successful Endoscopic Resection of Blue Rubber Bleb Nevus Syndrome in the Stomach Using a Snare.

Am J Gastroenterol 2017 Oct;112(10):1494

Department of Gastroenterology, West China Hospital, Sichuan University, China.

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http://dx.doi.org/10.1038/ajg.2017.169DOI Listing
October 2017
6 Reads

Surgical Emergencies in Intestinal Venous Malformations.

Eur J Pediatr Surg 2018 Feb 25;28(1):101-104. Epub 2017 Sep 25.

Division of Vascular Anomalies, Pediatric Surgery, La Paz Children's Hospital, Madrid, Spain.

Background:  Venous malformations (VMs) can occur in any part of the body; however, the gastrointestinal tract is a frequent location. These are usually asymptomatic, thus, representing a challenge to diagnosis. Intestinal location of VMs can be associated with severe complications that ultimately require an emergency surgery. Read More

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http://dx.doi.org/10.1055/s-0037-1606846DOI Listing
February 2018
22 Reads

Postoperative disseminated intravascular coagulation in a pregnant patient with Blue Rubber Bleb Nevus Syndrome presenting with acute intestinal obstruction: Case report and literature review.

Int J Surg Case Rep 2017 23;39:235-238. Epub 2017 Aug 23.

Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas of the University of Sao Paulo School of Medicine, Brazil. Electronic address:

Background: Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare condition which usually manifests as multiple hemangioma-like skin and gastrointestinal lesions. The latter often present with chronic bleeding. There is no consensus regarding the optimal management of such patients. Read More

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http://dx.doi.org/10.1016/j.ijscr.2017.08.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5581376PMC
August 2017
4 Reads

Blue Rubber Bleb Nevus Syndrome: A Delayed Diagnosis.

GE Port J Gastroenterol 2017 Mar 12;24(2):101-103. Epub 2016 Nov 12.

Gastroenterology Department, Centro Hospitalar do Algarve, EPE, Faro, Portugal.

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http://dx.doi.org/10.1159/000450876DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5553358PMC
March 2017
4 Reads

Blue rubber bleb nevus syndrome: our experience and new endoscopic management.

Medicine (Baltimore) 2017 Aug;96(33):e7792

aDepartment of Gastroenterology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province bDepartment of Nephrology, Jilin City Central Hospital, Jilin, Jilin Province, China.

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. Read More

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http://dx.doi.org/10.1097/MD.0000000000007792DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5571702PMC
August 2017
8 Reads

An Unusual Case of Iron Deficiency and Cardiac Murmur.

Gastroenterology 2017 Jun 4;152(8):e1-e2. Epub 2017 May 4.

Division of Gastroenterology, University of Southern California Schools of Medicine, Los Angeles, California.

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http://dx.doi.org/10.1053/j.gastro.2016.09.058DOI Listing
June 2017
5 Reads

Endoscopic management of blue rubber bleb nevus syndrome in the colon with hemostatic clamp and snare.

Endoscopy 2017 06 2;49(6):E149-E150. Epub 2017 May 2.

Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China.

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http://dx.doi.org/10.1055/s-0043-106044DOI Listing
June 2017
60 Reads

Blue Rubber Bleb Nevus Syndrome: Promising Response To Sirolimus.

Indian Pediatr 2017 Jan;54(1):53-54

Department of Pediatric Oncology, Hacettepe University, Cancer Institute, 06100 Ankara-Turkey. Correspondence to: Dr Burca Aydin, Department of Pediatric Oncology, Hacettepe University, Cancer Institute, 06100 Ankara-Turkey.

Background: Blue rubber bleb nevus syndrome is a rare disease involving venous malformations.

Case Characteristics: We present a 6-year-old female with the syndrome, and consumptive coagulopathy.

Intervention/outcome: After management with sirolimus, symptoms resolved. Read More

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January 2017
7 Reads

Unilateral blue rubber bleb naevus syndrome with Chiari malformation.

Clin Exp Dermatol 2017 Mar 5;42(2):210-212. Epub 2017 Jan 5.

Department of Dermatology, Sisli Hamidiye Etfal Training and Research Hospital, Halaskargazi Cad. Etfal Sk., 34371, Şişli Istanbul, Turkey.

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http://doi.wiley.com/10.1111/ced.13012
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http://dx.doi.org/10.1111/ced.13012DOI Listing
March 2017
2 Reads

Double Jeopardy: The Rubber Ball Bounces Twice.

J Invest Dermatol 2017 01;137(1):15-17

Department of Dermatology, Emory University School of Medicine, Atlanta Veterans Administration Medical Center, Winship Cancer Institute, Atlanta, Georgia, USA.

Soblet et al. describe cis mutations in TEK/Tie-2 in blue rubber bleb nevus and sporadic vascular malformations. This suggests that the remaining normal allele is required for the phenotype. Read More

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http://dx.doi.org/10.1016/j.jid.2016.08.011DOI Listing
January 2017
3 Reads

Blue Rubber Bleb Nevus Syndrome Diagnosed Prenatally as an Epignathus.

J Craniofac Surg 2016 Nov;27(8):e737-e738

*I Department of Obstetrics and Gynaecology, The Centre of Postgraduate Medical Education†Center for Craniofacial Disorders, Institute of Mother and Child, Warsaw, Poland.

The authors present a clinical report of the giant fetal tumor protruding from the oral cavity diagnosed sonographically at 32 weeks of gestation as an epignathus. After delivery, tumor proved to be a presentation of the blue rubber bleb nevus syndrome. To the best of our knowledge, the literature offers no reports on similar cases. Read More

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http://dx.doi.org/10.1097/SCS.0000000000003069DOI Listing
November 2016
15 Reads

Blue rubber bleb naevus syndrome: a rare cause of chronic occult blood loss and iron deficiency anaemia.

BMJ Case Rep 2016 Dec 20;2016. Epub 2016 Dec 20.

Department of Pediatrics, Aarhus Universitetshospital, Aarhus, Denmark.

Blue rubber bleb naevus syndrome (BRBNS) is a rare vascular disorder with malformed veins, or blebs, appearing in the skin or internal organs. Gastrointestinal tract involvement is the most common feature and often subject to bleeding, potentially resulting in chronic occult blood loss and iron deficiency anaemia. We present the case of a 10-year-old boy with venous malformations on the feet and severe anaemia. Read More

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http://dx.doi.org/10.1136/bcr-2016-216963DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5174816PMC
December 2016
12 Reads

[Blue rubber bleb nevus syndrome complicated by a ventricular septal defect: a case report].

Nan Fang Yi Ke Da Xue Xue Bao 2016 Dec;36(12):1599-1601

Department of Nuclear Medicine, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China. E-mail:

Objective: The co-occurrence of blue rubber bleb nevus syndrome (BRBNS) and ventricular septal defects is rare. Here we present a case of BRBNS in a 15-year-old boy who was born with multiple cavernous hemangiomas and a ventricular septal defect. Examinations revealed the presence of hemangioma lesions in the subcutaneous and mucosal tissues as well as in the cerebrum, nasopharynx, tongue, esophagus, gastric body, sigmoid colon and adrenal gland. Read More

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December 2016
6 Reads

Diffuse Cavernous Hemangioma of the Colon.

Acta Gastroenterol Belg 2016 Jul-Sep;79(3):393-394

A 70-year-old man was admitted to our clinic with a history of rectal bleeding and constipation, his colonoscopy revealed varicosities and bluish nodular lesions of the rectum (Figure 1). Abdominal CT showed multiple nodular lesions beginning from the distal descending colon and extending to the rectum, calcifications suggesting phleboliths were also seen in these lesions. A contrast enhanced pelvic MRI demonstrated multiple tubular lesions showing hyperintensity on T2-weighted images and hypointensity on T1-weighted images, consistent with the affected areas on the CT scan (Figure 2). Read More

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January 2017
4 Reads

Favorable Response to Sirolimus in a Child With Blue Rubber Bleb Nevus Syndrome in the Gastrointestinal Tract.

J Pediatr Hematol Oncol 2017 03;39(2):147-149

Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Gazi University Faculty of Medicine, Ankara, Turkey.

Recently, sirolimus was demonstrated to be effective in treating vascular lesions and lessening the frequency of bleeding and secondary iron deficiency anemia. We present a child with blue rubber bleb nevus syndrome who had prolonged history of iron deficiency anemia secondary to unrecognized gastrointestinal bleeding. Treatment with propranolol, omeprazole and iron had failed. Read More

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http://dx.doi.org/10.1097/MPH.0000000000000681DOI Listing
March 2017
46 Reads

Abdominal vascular syndromes: characteristic imaging findings.

Radiol Bras 2016 Jul-Aug;49(4):257-263

Tenured Associate Professor in the Department of Diagnostic Imaging of the Escola Paulista de Medicina da Universidade Federal de São Paulo (EPM-Unifesp), São Paulo, SP, Brazil.

Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital-including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)-or compressive-including "nutcracker" syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. Read More

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http://dx.doi.org/10.1590/0100-3984.2015.0136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5073393PMC
October 2016
32 Reads

Multifocal vascular lesions.

Semin Cutan Med Surg 2016 Sep;35(3):153-60

Departments of Dermatology and Pediatrics, Columbia University Medical Center, New York, New York, USA.

Multifocal vascular lesions are important to recognize and appropriately diagnose. Generally first noticed on the skin, multifocal vascular lesions may have systemic involvement. Distinguishing among the different types of multifocal vascular lesions is often based on clinical features; however, radiological imaging and/or biopsy are frequently needed to identify distinct features and guide treatment. Read More

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http://dx.doi.org/10.12788/j.sder.2016.054DOI Listing
September 2016
14 Reads

Genetic basis for vascular anomalies.

Semin Cutan Med Surg 2016 Sep;35(3):128-36

Johns Hopkins School of Medicine, Department of Dermatology, Baltimore, Maryland, USA.

The fundamental genetics of many isolated vascular anomalies and syndromes associated with vascular anomalies have been elucidated. The rate of discovery continues to increase, expanding our understanding of the underlying interconnected molecular pathways. This review summarizes genetic and clinical information on the following diagnoses: capillary malformation, venous malformation, lymphatic malformation, arteriovenous malformation, PIK3CA-related overgrowth spectrum (PROS), Proteus syndrome, SOLAMEN syndrome, Sturge-Weber syndrome, phakomatosis pigmentovascularis, congenital hemangioma, verrucous venous malformation, cutaneomucosal venous malformation, blue rubber bleb nevus syndrome, capillary malformation-arteriovenous malformation syndrome, Parkes-Weber syndrome, and Maffucci syndrome. Read More

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http://dx.doi.org/10.12788/j.sder.2016.051DOI Listing
September 2016
21 Reads

Blue Rubber Bleb Nevus (BRBN) Syndrome Is Caused by Somatic TEK (TIE2) Mutations.

J Invest Dermatol 2017 01 9;137(1):207-216. Epub 2016 Aug 9.

Human Molecular Genetics, de Duve Institute, Université catholique de Louvain, Brussels, Belgium. Electronic address:

Blue rubber bleb nevus syndrome (Bean syndrome) is a rare, severe disorder of unknown cause, characterized by numerous cutaneous and internal venous malformations; gastrointestinal lesions are pathognomonic. We discovered somatic mutations in TEK, the gene encoding TIE2, in 15 of 17 individuals with blue rubber bleb nevus syndrome. Somatic mutations were also identified in five of six individuals with sporadically occurring multifocal venous malformations. Read More

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http://dx.doi.org/10.1016/j.jid.2016.07.034DOI Listing
January 2017
36 Reads

Blue Rubber Bleb Nevus Syndrome with Musculo-skeletal Involvement and Pulmonary Stenosis.

Indian Pediatr 2016 Jun;53(6):525-7

Departments of Dermatology and STD, and *Radiology, University College of Medical Sciences and GTB Hospital (University of Delhi), Dilshad Garden, Delhi, India. Correspondence to: Dr (Prof) Archana Singal, University College of Medical Sciences and GTB Hospital, Delhi 110 095, India.

Background: Blue rubber bleb nevus syndrome is a rare clinical entity.

Case Characteristics: A 13-year-old Indian boy presented with characteristic cutaneous lesions, gastrointestinal malformations, skeletal involvement and pulmonary stenosis.

Observations: Diagnosis was confirmed on skin biopsy, radiographic evaluation, colonoscopy and echocardiography. Read More

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June 2016
5 Reads

An atypical presentation of blue rubber bleb nevus syndrome.

Proc (Bayl Univ Med Cent) 2016 Jul;29(3):323-4

Department of Surgery (Gonzalez-Hernandez) and the Division of Gastroenterology, Department of Internal Medicine (Lizardo-Sanchez), Baylor University Medical Center at Dallas.

A 69-year-old white man presented with several episodes of hematochezia. Colonoscopy demonstrated multiple colonic blebs localized mainly in the distal transverse colon. Esophagogastroduodenoscopy, capsule endoscopy, and computed tomography of the abdomen did not reveal any abnormalities. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4900785PMC
July 2016
4 Reads

Response of Blue Rubber Bleb Nevus Syndrome to Sirolimus Treatment.

Pediatr Blood Cancer 2016 11 8;63(11):1911-4. Epub 2016 Jun 8.

Vascular Anomalies Center, Boston Children's Hospital, Boston, Massachusetts.

Background: Blue rubber bleb nevus syndrome (BRBNS) is a rare multifocal venous malformation syndrome involving predominantly the skin and gastrointestinal tract. Traditional treatment modalities include corticosteroids, interferon-α, sclerotherapy, and aggressive surgical resection. Sirolimus has been used in several single case reports. Read More

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http://dx.doi.org/10.1002/pbc.26049DOI Listing
November 2016
48 Reads

[Anesthetic Management of a Pediatric Case of Blue Rubber Bleb Nevi Syndrome Combined with Small-intestinal Intussusception].

Masui 2016 Apr;65(4):384-6

We report the anesthetic management of a pediatric case of blue rubber bleb nevi syndrome combined with small-intestinal intussusception. A 2-year-old girl was transferred to our hospital for small-intestinal intussusception. Emergent ablation of the upper gastrointestinal tract nevus under general anesthesia was planned. Read More

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April 2016
10 Reads

Blue rubber bleb nevus syndrome with knee joint disorder.

Pediatr Int 2016 Aug 26;58(8):740-3. Epub 2016 Apr 26.

Department of Pediatrics, National Hospital Organization, Fukuoka-Higashi Medical Center, Koga City, Fukuoka, Japan.

Blue rubber bleb nevus syndrome (BRBNS) involves cutaneous vascular malformation characterized by multiple venous malformations. This commonly affects the skin and gastrointestinal tract. BRBNS is associated with anemia and occasionally involves orthopedic manifestations. Read More

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http://dx.doi.org/10.1111/ped.12929DOI Listing
August 2016
12 Reads

Proptosis Reduction Using Sirolimus in a Child With an Orbital Vascular Malformation and Blue Rubber Bleb Nevus Syndrome.

Ophthalmic Plast Reconstr Surg 2017 May/Jun;33(3S Suppl 1):S143-S146

Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, U.S.A.

A 15-month-old boy with left congenital proptosis presented to the emergency department with melena. Upper GI endoscopy and magnetic resonance angiography revealed vascular lesions, consistent with gastrointestinal tract manifestations of blue rubber bleb nevus syndrome. MRI revealed vascular malformations in both orbits, with mass effect on the left side. Read More

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http://dx.doi.org/10.1097/IOP.0000000000000692DOI Listing
September 2017
8 Reads

Anemia Due to Inflammation in an Anti-Coagulated Patient with Blue Rubber Bleb Nevus Syndrome.

Clin Lab 2016 ;62(1-2):241-3

Background: Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by vascular malformations mostly involving skin and gastrointestinal tract. This disease is often associated with sideropenic anemia and occult bleeding.

Methods: We report the case of chronic severe anemia in an old patient under oral anticoagulation treatment for chronic atrial fibrillation. Read More

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April 2016
7 Reads

Blue rubber bleb nevus syndrome: a rare presentation of late-onset anemia and lower gastrointestinal bleeding without cutaneous manifestations.

J Community Hosp Intern Med Perspect 2016 17;6(1):29966. Epub 2016 Feb 17.

Department of Pulmonary and Critical care Medicine, MedStar Franklin Square Hospital Center, Baltimore, MD, USA.

Blue rubber bleb nevus syndrome (BRBNS) is a congenital disorder with characteristic venous anomalies that can present with varying degree of blood loss. The most clinically significant symptoms in adults include gastrointestinal (GI) bleeding and iron deficiency anemia. Severe complications can include intestinal torsion, intussusception, and even perforation, with each leading to significant morbidity and mortality. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4763553PMC
http://dx.doi.org/10.3402/jchimp.v6.29966DOI Listing
April 2016
12 Reads

The surgery for blue rubber bleb nevus syndrome.

Ann Med Surg (Lond) 2016 Feb 30;5:93-6. Epub 2015 Dec 30.

Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China.

Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular anomaly syndrome consisting of multifocal venous malformations (VM). The malformations are most. prominent in the skin, soft tissues and gastrointestinal (GI) tract. Read More

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http://dx.doi.org/10.1016/j.amsu.2015.12.062DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4724023PMC
February 2016
5 Reads