下腿切断に至った下肢Parkes Weber症候群の1例

Surgical Treatment of Parkes Weber Syndrome in a Lower Extremity Leading to Below-Knee Amputation

はじめに

　Parkes Weber症候群（以下，PWS）は毛細血管奇形，患肢の腫大，患肢の動静脈奇形（arteriovenous malformation：以下，AVM）を主体とした脈管奇形病変を特徴とする症候群であり 1）2），多様な病変を有する脈管奇形病変の中でも特に難治性とされる疾患である。指定難病の1つであり，生涯にわたり断続的な治療を必要とすることが多い。今回，強い疼痛を有する下肢PWSの患者に対して，外科治療を施行したことにより下腿の血行が増悪し，下腿切断に至った症例を経験した。AVMの治療にあたっては，外科治療などの治療介入により病状を増悪させてしまうリスクがあることを常に念頭に置く必要がある 3）。

　Parkes Weber syndrome is a refractory disease accompanied by arteriovenous malformations (AVMs), and multiple AVM lesions are usually observed throughout the affected extremity. Interventional radiology (IVR) is thus generally preferred over surgical treatment. However, IVR also has limitations, and surgical treatment may be considered when the adequate control of manifestations of this disease cannot be managed. We provide the case details of a patient for whom a distal bypass procedure was considered for the preservation of the affected limb during lesion resection. The patient was a 15 -year-old male with Parkes Weber syndrome and an AVM at left lower extremity.

　A distal bypass procedure was performed before the resection of the main lesion, but the results of this procedure were insufficient due to changes in hemodynamics associated with the AVM. Necrosis of the patientʼs lower extremity was observed after the resection of the main lesion, and a below-knee amputation was required.

　The surgical treatment for Parkes Weber syndrome itself is difficult to avoid an amputation of an affected limb, and in cases similar to our patientʼs, IVR should be considered. In addition, even in a simple AVM resection in cases requiring revascularization during a lesionʼs resection, the resection and revascularization should be performed after careful consideration of the surrounding hemodynamics.