KEGG   DISEASE: 末梢性動静脈奇形
エントリ  
H01849                                                             
名称    
末梢性動静脈奇形;
巨大動静脈奇形 (頚部顔面又は四肢病変)
概要    
Arteriovenous malformation (AVM) is a congenital vascular malformation with direct communication from arteries to veins (arteriovenous shunting) and lack of a normal capillary network. The behavior of peripheral (extracranial) AVM is locally aggressive. AVMs are high-flow vascular malformations, and have a wide range of clinical presentations. Some patients may present with an asymptomatic birthmark, while in some patients, the condition may be even life-threatening, leading to congestive heart failure. Typically during puberty or adolescence, the initially quiescent lesion progresses to an expansive mass with cosmetic and functional disturbance. With later progression, the AVM destroys normal tissues and eventually leads to complications such as severe disfigurement, uncontrollable bleeding, ulceration, pain, and cardiac volume overload. A surgical approach is indicated in well-circumscribed malformations of small size. However, the surgical treatment of larger lesions can often lead to excessive blood loss, in addition to serious complications. Ligation or embolization of the arteries supplying the AVM is usually followed by recurrence.
カテゴリ  
先天奇形
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  主に1つの体系に影響する構造的発達異常
   循環器系の構造的発達異常
    LA90  末梢血管系の構造的発達異常
     H01849  末梢性動静脈奇形
指定難病 [jp08407.html]
 H01849
リンク   
ICD-11: LA90.3
ICD-10: Q27.3
文献    
  著者
Pekkola J, Lappalainen K, Vuola P, Klockars T, Salminen P, Pitkaranta A
  タイトル
Head and neck arteriovenous malformations: results of ethanol sclerotherapy.
  雑誌
AJNR Am J Neuroradiol 34:198-204 (2013)
DOI:10.3174/ajnr.A3180
文献    
  著者
Ergun O, Atli E, Gulek B, Ciftci T, Cil B, Vargel I, Peynircioglu B
  タイトル
Intraarterial polidocanol injection for the treatment of peripheral arteriovenous malformations.
  雑誌
Surg Today 44:1232-41 (2014)
DOI:10.1007/s00595-013-0680-8
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