ABSTRACT
Blue rubber bleb nevus syndrome (BRBNS) is a rare disease, which is characterized by multiple hemangiomas of the skin and gastrointestinal tract. We once treated a case with intussusception requiring surgery. The purpose of this article is to improve our understanding of this disease.
ABSTRACT
Blue rubber bleb nevus syndrome(BRBNS)is a rare disease, which is characterized by multiple hemangiomas of the skin and gastrointestinal tract. We once treated a case with intussusception requiring surgery. The purpose of this article is to improve our understanding of this disease.
ABSTRACT
Hemorrhagic anaemia caused by chronic gastrointestinal bleeding is a common complication of BRBNS that has the greatest impact on the patient′s quality of life, even life-threatening. Traditional treatments cannot slow down the overall progress of the disease. The curative effect is limited and has certain limitations. Sirolimus can effectively treat vascular malformations of the soft tissues, and it can control gastrointestinal lesions to correct iron deficiency anemia caused by bleeding, improve quality of life, and can inhibit disease progression and improve prognosis. This new emerging medication may become one of the powerful treatments for BRBNS in the future.
ABSTRACT
Gastrointestinal (GI) hemangiomas are relatively rare benign vascular tumors. The choice of an appropriate diagnostic method depends on patient age, anatomic location, and presenting symptoms. However, GI hemangiomas are not a common suspected cause of GI bleeding in children because of their rarity. Based on medical history, laboratory results, and imaging study findings, the patient could be treated with either medication or surgery. Herein, we report 3 cases of GI hemangioma found in the small bowel, rectum, and GI tract (multiple hemangiomas). Better knowledge and understanding of GI hemangioma could help reduce the delayed diagnosis rate and prevent inappropriate management. Although rare, GI hemangiomas should be considered in the differential diagnosis of GI bleeding.
Subject(s)
Child , Humans , Delayed Diagnosis , Diagnosis, Differential , Gastrointestinal Hemorrhage , Gastrointestinal Tract , Hemangioma , Hemangioma, Cavernous , Hemorrhage , Rectum , Vascular NeoplasmsABSTRACT
Objective The study was performed to analyze the diagnosis and treatment of blue rubber bleb nevus syndrome of Chinese patients by evaluating the researches in China.Methods Chinese Journal Full-text Database,Wangfang Data and Weipu Chinese Medical Journal Database were searched for the clinical data of the patients with blue rubber bled nevus syndrome.Results Among the 50 patients,31 cases were male,and 19 cases were female.All patients (100％) had the venous malformations of the skin.Thirty-five (70％) cases had melena,13 (26％) cases had bloody stool,and 35(70％) had dizziness and palpitation.Blue rubber bled nevus syndrome were diagnosed with gastroscopy (40/48,83.33 ％),coloscopy (30/41,73.17％) and small intestinal examination (23/25,92％).Fourteen cases received symptomatic treatment.Twelve cases received therapeutic endoscopy,which lesions were located in stomach and colon.Sixteen cases received operation,and 2 cases were treated by laparoscopy and endoscopy.Conclusions Blue rubber bleb nevus syndrome is a rare disease.The skin and gastrointestinal system are involved in all patients with blue rubber bleb nevus syndrome in China.The routine examination for diagnosis of blue rubber bleb nevus syndrome should include examination of the small intestine.It still needs further research about effective treatment of blue rubber bleb nevus syndrome.
ABSTRACT
Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic vascular disorder characterized by multiple venous malformations involving many organs. BRBNS can occur in various organs, but the most frequently involved organs are the skin and gastrointestinal (GI) tract. GI lesions of BRBNS can cause acute or chronic bleeding, and treatment is challenging. Herein, we report a case of GI BRBNS that was successfully treated with a combination of intraoperative endoscopy and radical resection.
Subject(s)
Blister , Endoscopy , Gastrointestinal Neoplasms , Hemorrhage , Intestines , Nevus , Nevus, Blue , Rubber , Skin , Skin NeoplasmsABSTRACT
Blue rubber bleb nevus syndrome is a rare disorder characterized by multiple cutaneous venous malformations in association with visceral lesions, most commonly affecting the gastrointestinal tract. Oral cavity lesions occur in 59 to 64% of cases. We report a unique presentation of this syndrome in a 25-year-old male patient with prominent oral findings. This is a sporadic case, started during early childhood, progressively increasing in number and size. Oral vascular lesions were part of gastrointestinal involvement. Associated cardiac abnormalities were also observed. An early diagnosis of this syndrome is required as it gets complicated with bleeding, anemia and other systemic complications.
ABSTRACT
Blue rubber bleb nevus syndrome is a rare disorder that's characterized by multiple venous malformations of the skin and gastrointestinal tract, and these lesions usually cause episodes of occult gastrointestinal hemorrhage and iron deficiency anemia. We report here on a case of a 57-year-old woman who presented with intermittent melena and multiple cutaneous venous malformations. The endoscopic and radiologic examinations show multiple bluish polypoid venous malformations on the gastrointestinal tract, retroperitoneum, liver, mediastinum and lung. We were able to diagnose her as suffering from blue rubber bleb nevus syndrome and we treated her with iron supplementation for anemia. We report this case along with a brief review of the relevant literature.
Subject(s)
Female , Humans , Middle Aged , Anemia , Anemia, Iron-Deficiency , Blister , Gastrointestinal Hemorrhage , Gastrointestinal Neoplasms , Gastrointestinal Tract , Hemangioma , Iron , Liver , Lung , Mediastinum , Melena , Nevus , Nevus, Blue , Rubber , Skin , Skin Neoplasms , Stress, PsychologicalABSTRACT
Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder that is characterized by multiple cutaneous vascular malformations in the skin and gastrointestinal tract and is associated with intestinal hemorrhage and iron deficiency anemia. This syndrome may induce severe or fatal bleeding; however, the causes of this syndrome are unknown. We report here a case of BRBNS that was diagnosed in a 20-year-old male with chronic anemia and a past history of surgery due to cutaneous hemangioma on the shoulder and thigh. One purplish hemangioma on the stomach and multiple bluish hemangiomas on the colon were noted on the endoscopic examination. We successfully treated the GI hemangioma with endoscopic band ligation and endoscopic polypectomy.
Subject(s)
Humans , Male , Young Adult , Anemia , Anemia, Iron-Deficiency , Blister , Colon , Gastrointestinal Neoplasms , Gastrointestinal Tract , Hemangioma , Hemorrhage , Ligation , Nevus , Nevus, Blue , Rubber , Shoulder , Skin , Skin Neoplasms , Stomach , Thigh , Vascular MalformationsABSTRACT
We report a 10-year-old girl with the blue rubber bleb nevus syndrome (BRBNS) who had chronic severe anemia caused by chronic occult bleeding in the gastrointestinal (GI) tract. The patient was admitted to the hospital frequently for recurrent pallor and fatigue since the age of 7 years. Gastroduodenoscopy and capsule endoscopy revealed multiple venous malformations with blood oozing in the stomach, small bowel and colon. The patient was treated by aggressive surgical resection of the 23 vascular malformations in the GI tract. The patient is well without anemia 15 months post surgery.
Subject(s)
Child , Humans , Anemia , Blister , Capsule Endoscopy , Colon , Fatigue , Gastrointestinal Neoplasms , Gastrointestinal Tract , Hemorrhage , Nevus , Nevus, Blue , Pallor , Rubber , Skin Neoplasms , Stomach , Vascular MalformationsABSTRACT
We report a 10-year-old girl with the blue rubber bleb nevus syndrome (BRBNS) who had chronic severe anemia caused by chronic occult bleeding in the gastrointestinal (GI) tract. The patient was admitted to the hospital frequently for recurrent pallor and fatigue since the age of 7 years. Gastroduodenoscopy and capsule endoscopy revealed multiple venous malformations with blood oozing in the stomach, small bowel and colon. The patient was treated by aggressive surgical resection of the 23 vascular malformations in the GI tract. The patient is well without anemia 15 months post surgery.
Subject(s)
Child , Humans , Anemia , Blister , Capsule Endoscopy , Colon , Fatigue , Gastrointestinal Neoplasms , Gastrointestinal Tract , Hemorrhage , Nevus , Nevus, Blue , Pallor , Rubber , Skin Neoplasms , Stomach , Vascular MalformationsABSTRACT
Blue rubber bleb nevus syndrome is a rare disorder that is characterized by multiple recurrent vascular malformations, such as hemangioma, and these primarily involve the skin and the gastrointestinal tract. It may also involve the brain, liver, lungs, and skeletal muscles. A 14-year-old female visited our hospital with a chief complaint of dizziness; upon examination, we found multiple recurrent hemangiomas on the skin and gastrointestinal tract. We were able to diagnose her as suffering from blue rubber bleb nevus syndrome and we treated her with methylprednisolone (2 mg/kg/day for 1 month and 1 mg/kg/day for additional 3 months). We report on this case along with a review of the literature.
Subject(s)
Adolescent , Female , Humans , Gastrointestinal Neoplasms/diagnosis , Hemangioma/diagnosis , Nevus, Blue/diagnosis , Skin Neoplasms/diagnosis , SyndromeABSTRACT
The blue rubber bleb nevus syndrome(CRBNS) is a rare disorder characterized by distinctive vascular malformations of the skin and gastrointestinal tract. The syndrome is associated commonly with iron deficiency anemia due to gastrointestinal bleeding. Most cases are sporadic, although a few have been reported with autosomal dominant inheritance. We report a case with clinical characteristics of the blue rubber bleb nevus syndrome. The patient had typical skin manifestations of the BRBNS and chronic iron deficiency anemia. However, we could not find any gastrointestinal vascular malformations radiologically.
Subject(s)
Humans , Anemia, Iron-Deficiency , Blister , Gastrointestinal Tract , Hemorrhage , Nevus , Rubber , Skin , Skin Manifestations , Vascular Malformations , WillsABSTRACT
Blue Rubber Bleb Nevus syndrome, or Bean's syndrome is a very rare disease characterized by an association of 1) hemangioma cutis in which the nevi feel like rubber blebs; 2) hemangiomas of the digestive organs; 3) iron-deficiency anemia due to hemorrhage from the digestive tract. Sometimes, the bleeding is so severe and massive that the patient needs blood transfusions and/or emergency operations. We present a 29-year-old man with Blue Rubber Bleb Nevus syndrome. This patient was admitted to out hospital due to recently aggrevated dizziness and intermittent rectal bleeding. Multiple bluish tumors were noted on the palm, sole and glans penis. Laboratory examination revealed severe iron-deficiency anemia. Barium studies revealed multiple polypoid masses in the stomach, small bowel and colorectum. On esophagogastroduodenoscopy and colonoscopy, three or four dozens of hemangiomas with variable size and shape were noted in the stomach, duodenum at or around the ampulla of Vater and colorectum. This patient was of particular interest because rectal bleeding occurred from rectal lesion which protruded out of anus and caused bleeding during defecation, and which showed as a 2.0 cm Yamada type III polypoid lesion. Endoscopic ultrasonography confirmed us that this lesion was confined to the mucosa and submucosa without extension into muscle layer. Endoscopic polypectomy of this lesion was performed because which considered as the main focus of current rectal bleeding. He was discharged without any complication and 4 months later, he was well without rectal bleeding and his hemoglobin level increased upto 13.5 g/dl. To our knowledge, this is the first case of Blue Rubber Bleb Nevus syndrome in Korea.