異所性交叉性融合腎と一側椎骨動脈閉塞を伴ったKlippel-Feil症候群の1例

A Case of Klippel-Feil Syndrome with Crossed Renal Ectopia with Fusion and Unilateral Vertebral Artery Occlusion

I．はじめに

　Klippel-Feil症候群においては中枢神経系，骨格系，心血管系，泌尿生殖器系などほぼ全身にわたる合併奇形が報告されている^5,7）．今回稀な腎奇形である異所性交叉性融合腎（crossed renal ectopia with fusion）と左椎骨動脈閉塞を伴ったKlippel-Feil症候群の1例を経験したので文献的考察を加え報告する．

Abstract

A case of Klippel-Feil syndrome with crossed renal ectopia with fusion and unilateral vertebral artery occlusion is reported. A 61-year-old female was admit-ted because of having developed myelopathy, gait and sensory disturbance. The physical examination on admission revealed spastic gait and hypesthesia of the lower extremities. A hand size, elastic hard tumor mass was palpable at the right lower abdomen. The cervical radiogram showed fused 5th, 6th and 7th cervical ver-tebrae and severe spondylotic changes.

Laminectomy was performed because of severe com-pressions of the cervical cord, evaluated by Metriza-mide CT scans and MRI. The left subclavian angio-gram showed occlusion of the left vertebral artery, and excretory pyelogram and abdominal CT scans showed crossed renal ectopia with fusion. 99mTc-DTPA reno-scintigram revealed poor RI uptake and low glomerular filtrating rate of ectopic kidney.

The patients with Klippel-Feil syndrome are at grea-ter risk of having a renal anomaly and a vascular acci-dent of the vertebral artery. Examinations of urogeni-tary organs and vertebral arteries are necessary for the treatment of this syndrome.