心不全症状を来たしたParkes Weber症候群に対して塞栓-硬化療法を用いて疼痛と心機能の改善が見られた1例

Successful Embolo-Sclerotherapy for a Parkes Weber Syndrome Patient with Severe Pain and Heart Failure Symptoms

はじめに

　動静脈奇形（arteriovenous malformation：以下，AVM）は，病勢の進行に伴い疼痛の増強や組織壊死および心不全などに至る疾患である。Parkes Weber症候群（以下，PWS）は皮膚の毛細血管奇形に加えAVMや動静脈瘻（arteriovenous fistula：以下，AVF）などの高流速血管奇形を含み，四肢の片側肥大を来たす先天性血管奇形である 1）。今回著者らは，上肢AVM病変を有し疼痛と心不全症状を生じていたPWSの患者を経験した。塞栓-硬化療法を施行することで，臨床症状および心機能の改善を認め，良好な経過を得たので文献的考察を加え報告する。

　Parkes Weber syndrome is a rare congenital vascular disorder consisting of high-flow vascular malformation and the enlargement of affected limb(s) that may cause cardiac failure or limb amputation in severe cases. A 24-year-old man who had been diagnosed with Parkes Weber syndrome in childhood and had been living without therapy presented to our clinic due to an aggravation of his symptoms. Severe pain inhibited the elevation of his left arm, and he had recently become aware of breathlessness. Chest radiography revealed heart enlargement due to heart failure, and we diagnosed stage 4 Parkes Weber syndrome with arteriovenous malformation (AVM). Planned embolization-sclerotherapy in cooperation with interventional radiologists was scheduled. Two days later he underwent four cycles of embolization with n-butyl 2-cyanoacrylate and intraosseous sclerotherapy with ethanol for the arm. After treatment, a limb radiograph revealed ossification of aneurysmal bone cysts caused by the AVM. The ensuing pain enabled the patient to return to daily life. Although a complete cure was not possible in this patientʼs case, the AVM was well controlled and the patient escaped cardiac failure and upper-limb amputation.