Anesthetic considerations for the patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).
نویسنده
چکیده
Hereditary hemorrhagic telangiectasia (HHT), Osler-Weber-Rendu Syndrome, is an uncommon disease but may be present in many people who remain undiagnosed. It is an autosomal dominant disorder characterized by multiple arteriovenous malformations (AVMs) and telangiectases that affect multiple organ systems. Hereditary hemorrhagic telangiectasia patients have a propensity for bleeding, especially from the oropharynx, nasopharynx and gastrointestinal tract, as well as from rupture of AVMs of other organ systems. Anesthetic care of patients with HHT involves very specific interventions with regard to control of bleeding, maintaining adequate oxygenation, and balancing hemodynamic values to optimize perfusion without compromising anesthetic depth.
منابع مشابه
Anesthetic Considerations for a Patient With Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Syndrome) Undergoing a Five-Box Thoracoscopic Maze Procedure for Atrial Fibrillation
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder involving the abnormal communication of vascular structures. HHT typically presents with recurrent epistaxis and telangiectasis of the nasal and buccal mucosa, tongue, and lips. More serious manifestations of this disease include cerebral, pulmonary, gastrointestinal, and hepatic arteriovenous malformations. T...
متن کامل[Ophthalmologic diagnosis of hereditary hemorrhagic telangiectasia or Rendu-Osler-Weber disease].
CASE REPORT Our patient was a 45-year-old woman who had recurrent episodes of hematic epiphora, repeated epistaxes for which no cause was found and a family history of gastric hemorrhage. One of her daughters also suffered from spontaneous hemorrhages. DISCUSSION Hereditary hemorrhagic telangiectasia is rarely diagnosed by an ophthalmologist; however the occurrence of bloody tears occurring s...
متن کاملCongestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report
Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary hemorrhagic telangiectasia), although symptomatic liver disease is quite infreque...
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 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 کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- AANA journal
دوره 77 2 شماره
صفحات -
تاریخ انتشار 2009