Successful CO2 laser ablation of true vocal fold microaneurysm in a patient with Ehlers-Danlos syndrome.
نویسندگان
چکیده
E hlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders composed of 6 major forms under the Villefranche classification: classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, and dermatoparaxis. Vascular type, or type IV, is diagnosed clinically by easy bruising, characteristic facial features, visible veins, and arterial/visceral rupture. It is an autosomal dominant disorder due to a mutation in the gene for type III procollagen (COL3A1). Affected patients have a decreased life expectancy, with a median survival of 48 years. Most mortalities result from arterial or visceral rupture. Although not a main feature of EDS, dysphonia has been reported in all subtypes of the disease at a significantly higher frequency than the general population. Dysphonia in this population may be secondary to impairment of vocal fold mobility or hemorrhagic infiltration of Reinke space. We present a patient with vascular EDS with recurrent vocal fold hemorrhage who underwent successful treatment by microflap excision and CO2 laser ablation of true vocal fold (TVF) microaneurysm. This is the first reported successful therapeutic intervention for laryngeal abnormality in a patient with EDS demonstrating that additional care should be considered when addressing hemorrhagic polyps in such patients. This case report was exempt from institutional review board approval.
منابع مشابه
Ehlers-Danlos syndrome: type VI A – kyphoscoliosis; a case report
Ehlers-Danlos syndrome (EDS) is a clinically heterogeneous groupof inherited disorders with ten different types, all involving agenetic defect in collagen and connective-tissue synthesis andstructure that affecting the skin, joints, and blood vessels. EDStype VIA, a very rare kyphoscoliotic type, is autosomal recessiveand clinically characterized by soft extensible skin, laxity ofjoints and kyp...
متن کاملAn Iranian family with cutis laxa and classic Ehlers-Danlos syndrome
Ehlers-Danlos syndrome (EDS) is a heterogenous group of inherited disorders of connective tissue characterized by fragility of the skin and blood vessels, hyperextensibility of the skin and joint hypermobility. Cutis laxa is characteized clinically by lax, pendulous skin and histologically by loss of elastic tissue in the dermis. There are some reports of coexistence of cutis laxa with ot...
متن کاملEhlers-Danlos Syndrome (TYPEI) with Mental Retardation -an Unusual Association (Reports on Two Brothers)
Ehlers Danlos syndrome (EDS) is an inherited connective tissue disease due to impaired collagen metabolism. Joint hypermobility and skin hyper extensibility are the major findings. Six types of EDS are recognized. Type I or Gravis type is characterized by skin hyperextensibility, joint hypermobility, skin splitting autosoml dominancy inheritance, preterm premature rupture of membrane (PPROM) an...
متن کاملاهمیت شناخت سندرم Ehlers Danlos بوسیله دندانپزشکان: گزارش یک مورد
This syndrome is a genetical disorder with symptoms result from deficit in the formation of connective tissue, especially collagen fiber biosynthesis. In these patients, there is hyperelasticity and fragility of the skin and mucosa. Injuries sever bleeding, internal bleeding and hemartrosis may be seen in these patients. Wounds leave scars after healing. There is recurrent dislocation of the jo...
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:
- Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
دوره 149 6 شماره
صفحات -
تاریخ انتشار 2013