高血圧性網膜症にて発見された若年性褐色細胞腫の1例

A case of juvenile pheochromocytoma with hypertensive retinopathy as the initial manifestation

要約　1か月前からの霧視を契機として乳頭浮腫が発見された17歳女性が紹介され受診した。矯正視力は右0.5，左1.2で，両眼に滲出性網膜病変，網膜浅層と網膜下出血，乳頭周囲の浮腫があった。頭蓋内病変と血液疾患は否定された。拡張期血圧が110mmHgであり，血中ノルアドレナリン値の上昇とレジチンテスト陽性が発見され，褐色細胞腫による二次性高血圧症の診断が確定した。血圧管理を行ったが不安定で，網膜症が増悪した。初診から6週後に腫瘍を摘出した。血圧の正常化に伴って網膜症と視力が改善した。若年者であるにもかかわらず高血圧性網膜症が発症した原因として，一時的な血圧上昇による末梢循環不全に加え，代謝亢進による代謝産物の血中濃度上昇が考えられた。

Abstract.　A 17-year-old female was referred to us for choked disk. She had had blurring of vision since one month before. Her corrected visual acuity was 0.5 right and 1.2 left. Both eyes showed exudative retinal lesions,superficial and subretinal hemorrhages,and peripapillary edema. Intracranial lesions and hematological disorders were ruled out. She had diastolic blood pressure of 110 mmHg. Detection of elevated serum noradrenaline and positive regitine test led to the diagnosis of pheochromocytoma with secondary hypertension. Attempted control of blood pressure was unstable and the retinopathy deteriorated further. The tumor was surgically removed 6 weeks after her initial visit. The blood pressure normalized followed by improved retinopathy and visual acuity. Hypertensive retinopathy in this young patient appeared to be due to insuffcient peripheral circulation secondary to temporary rise in blood pressure and to hypermetabolic state resulting in accumulation of metabolic products in the serum.