Osler-Weber-Rendu Syndrome
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چکیده
Presentation Age-related penetrance is seen in HHT. [2] It does not present at birth but commonly presents with recurrent epistaxis, usually in the teenage years. People with the condition develop mucocutaneous lesions, usually involving the nasal mucosa, lips and tongue. These lesions are sharply demarcated red-purple macules, papules or spider-like lesions comprising a mat of tortuous vessels. These can also occur in the conjunctiva, upper respiratory tract, gastrointestinal (GI) tract, bladder, vagina, bronchi, brain and liver. Cutaneous telangiectasias are often not evident until between 20 and 30 years of age. Unfortunately, due to the relatively low prevalence of the condition, it is frequently underdiagnosed. [2]
منابع مشابه
[The use of superselective embolization of the maxillary artery in treatment of bleedings in the Rendu-Osler-Weber syndrome].
Rendu-Osler-Weber syndrome is a rare genetically determined disorder that affects blood vessels throughout the body and results in a tendency for bleeding. Authors describe the case of superselective embolization of the left maxillary artery with polyvinyl alcohol particles in a patient with the Rendu-Osler-Weber syndrome hospitalized and treated in the Department of Otolaryngology and the Depa...
متن کاملOsler-Weber-Rendu syndrome: a case report with familial clustering.
Osler-Weber-Rendu syndrome, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant disorder manifested by telangiectases of the skin and mucous membranes and arteriovenous malformations of various organ systems. We present a case of Osler-Weber-Rendu syndrome with 11 affected members in her family.
متن کاملIntraoperative choroidal hemorrhage in the Osler-Rendu-Weber syndrome.
PURPOSE To describe a patient with Osler-Rendu-Weber syndrome who developed a nonsimultaneous intraoperative choroidal hemorrhage in each eye. METHOD Interventional case report. A 65-year-old Caucasian woman with Osler-Rendu-Weber syndrome developed a choroidal hemorrhage in the left eye during vitrectomy for a complicated retinal detachment with a poor visual outcome. Fifteen years later, sh...
متن کاملOsler-Weber-Rendu syndrome during pregnancy.
Osler-Weber-Rendu syndrome is a very rare systemic fibrovascular dysplasia. Rupture of angiomas can cause haemorrhages, which sometimes can be severe with difficult bleeding control. The main manifestation is recurrent epistaxis. Treatment of this disorder is symptomatic. During pregnancy, there may be an increased risk of complications. We describe a case of a pregnant woman with Osler-Weber-R...
متن کاملOsler-Weber-Rendu syndrome.
Osler–Weber–Rendu syndrome, also known as Hereditary Hemorrhagic Telangiectasia (HHT), is an autosomal dominant disorder. Telangiectasias and Arterio-Venous Malformations (AVMs) are vascular lesions present in HHT, most commonly causing epistaxis and gastrointestinal bleeding. While epistaxis presents as early as childhood, the gastrointestinal manifestations of HHT develop with increasing age.
متن کامل[Maxillary artery embolisation as a method of treatment of hemorrhages in Rendu-Osler-Weber Syndrome--our experience].
The authors have described case of patient suffering from Rendu-Osler-Weber Syndrome for many years, after many unsuccessful attempts to treat massive hemorrhages from the nose. This was what made them use maxillary artery embolisation method. It was done using Seldinger technique. Radiologic visualisation of blood vessels was obtained by use of C-arm. The use of this method in that case has be...
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