Blue rubber bleb nevus syndrome: case report

Teixeira, Magaly Gêmio; Perini, Marcos Vinícius; Marques, Carlos Frederico Sparapan; Habr‐Gama, Angelita; Kiss, Desidério Roberto; Gama-Rodrigues, Joaquim

doi:10.1590/s0041-87812003000200009

Cited by 10 publications

(8 citation statements)

References 8 publications

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“…Choi KK, et al5, de un total de 39 pacientes, indica que la piel se vio afectada en 39 y el tracto GI en 31, de la misma forma, como reportado por Chen W, et al (7) , uno de los sistemas más afectados es el tracto GI, pues de un total de 5 pacientes, todos presentaron afección de este aparato, estas revisiones coinciden con la descripción del síndrome reportado en la bibliografía médica. (1,2) Las MV pueden desarrollarse desde la boca hasta el ano y por lo general están localizadas en el intestino delgado (ID), teniendo mayor relevancia clínica por el riesgo de sangrado masivo que puede generar, (3) Texeira MG, et al (10) , coinciden pues el ID fue el lugar donde mayor número de MV se encontraron, en su reporte de caso. A diferencia del nuestro, la localización mayoritaria fue en colon con predominio en recto, coincidiendo con el trabajo de Martínez CA, et al (9) por esta razón consideramos que el diagnóstico inicial de trombosis colónica fue erróneo, probablemente por la observación de MV que dio a la mucosa del colon un aspecto de trombosis.…”

Section: Discussionunclassified

“…No existe tratamiento curativo descrito para esta patología hasta el momento, el manejo es conservador, con vigilancia de complicaciones adyacentes, las MV pueden ser tratadas precisando el riesgo de sangrado y localización a través de láser, escisión quirúrgica o escleroterapia, (1,3) no se recomienda la resección de MV del aparato GI ya que es poco eficaz, debido a la cantidad de lesiones, riesgo hemorrágico y la probabilidad de recurrencia, este tipo de procedimiento está indicado en caso de sangrados masivos que comprometan la estabilidad hemodinámica del paciente, (1,3,13) Choi KK, et al (5) , Texeira ME, et al (10) , y Fishman SJ, et al (4) , han reportado excelentes resultados del tratamiento quirúrgico, en cuanto al control posterior del sangrado y anemia, nuestra paciente no requirió tratamiento quirúrgico y recibió tratamiento conservador.…”

Section: Discussionunclassified

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Síndrome de Bean y albinismo: reporte del primer caso en Ecuador

Ortiz-Culcay¹,

Hernández-Lojano²,

Zurita-Alvarado³

et al. 2019

RMV

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Blue Rubber Bleb Nevus syndrome (BRBNS) or its acronym Bean syndrome is characterized by venous malformations (MV) distributed in multiple anatomical areas, predominantly in the skin and gastrointestinal (GI) tract. Symptomatology is commonly manifested with hemorrhagic episodes and consequent chronic secondary anemia due to deficit. We present the clinical case of a female albino patient of 68 years of age, who came for acute abdominal pain to this we add a chronic rectorrhagia, when performing the physical examination, the presence of venous malformations (MV) characterized by bluish, compressible and heterogeneous nodules, located in the lower lip, chin, perianal region and extremities, as well as pain on palpation in the right hypochondrium. The laboratory analysis revealed low hemoglobin and hematocrit values and other parameters suggestive of iron deficiency anemia, in the colonoscopy report multiple venous malformations (MV) were disseminated, disseminated with rectal predominance, the diagnosis was compatible with Bean Syndrome by the association of MV in skin and Gastrointestinal tract and secondary chronic anemia. The management was clinical symptomatic and palliative surgery, with a favorable evolution. The association of albinism and Bean syndrome are not reported in the international medical literature, considering this the first case in Ecuador as a not very frequent pathology but with important secondary complications, this syndrome should be included as a cause of harmless venous malformations, digestive bleeding, chronic anemia and occlusive effects of hollow viscera

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Section: Discussionunclassified

Síndrome de Bean y albinismo: reporte del primer caso en Ecuador

Ortiz-Culcay¹,

Hernández-Lojano²,

Zurita-Alvarado³

et al. 2019

RMV

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“…Most are associated with skeletal deformities in addition to cutaneous and GI vascular malformation. Few case reports show involvement of GIT alone [4].…”

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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“…BRNS is characterized by the appearance of venous malformations of the skin that are present from birth or childhood and increase in size and number with increasing age [10, 11]. The majority of the lesions are asymptomatic, but some may be painful upon compression.…”

Section: Discussionmentioning

confidence: 99%

Blue Rubber Bleb Nevus Syndrome as a Cause of Lower Digestive Bleeding

Rodrigues

Sato

Júnior

et al. 2014

Case Reports in Surgery

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Introduction. Blue rubber bleb nevus syndrome is a rare disorder that is characterized by multiple recurrent vascular malformations that involve the skin and the gastrointestinal tract. The disease can present chronic anemia and severe episodes of gastrointestinal bleeding. Case Report. A 41-year-old man was admitted with recurrent episodes of lower gastrointestinal bleeding and anemia that had worsened over the last 3 months. The physical examination showed soft, diffuse, compressible, bluish nodules on all of the skin surfaces of the body. A biopsy from one of these skin lesions allowed a histological diagnosis of cavernous hemangioma. He submitted to a colonoscopy, which showed hemorrhoids and a plane vascular lesion mainly located on the right colon with recent signs of bleeding; this lesion was treated by local excision and sclerosis. The pathological study of the colon specimens also reflected the presence of cavernous hemangioma. The cutaneous hemangiomas and the presence of colonic venous malformations were compatible with blue rubber bleb nevus syndrome. The patient presented a favorable follow-up with clinical control of the anemia and without relapse of the gastrointestinal bleeding two years after the procedure. Conclusion. Although rarely diagnosed, blue rubber bleb nevus syndrome may be responsible for lower digestive bleeding.

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Blue rubber bleb nevus syndrome: case report

Cited by 10 publications

References 8 publications

Síndrome de Bean y albinismo: reporte del primer caso en Ecuador

Síndrome de Bean y albinismo: reporte del primer caso en Ecuador

Surgery for Blue Rubber Bleb Nevus Syndrome—a Case Report

Blue Rubber Bleb Nevus Syndrome as a Cause of Lower Digestive Bleeding

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