弾力線維性仮性黄色腫を合併したcarotid rete mirabileの1例—症例報告

A CASE OF CAROTID RETE MIRABILE ASSOCIATED WITH PSEUDOXANTHOMA ELASTICUM:A CASE REPORT

　抄録　Carotid rete mirabile （頸動脈奇異網症，以下CRM）は内頸動脈の海綿静脈洞部分と外頸動脈系との間に認められる血管網であり，下等哺乳動物では正常の構造物である。また，弾力線維性仮性黄色腫（pseudoxanthoma elasticum，以下PXE）は，皮膚症状，眼症状，全身血管系症状を3主徴とする全身の結合織疾患である。我々はこの両者を合併し，クモ膜下出血にて発症し，約10年後に左不全片麻痺を来した55歳女性の1例を経験した。頭部CTにて右内包後脚にラクーナを認め，脳血管写にては両側とも内頸動脈が広汎に狭窄しており，海綿静脈洞部と外頸動脈分枝の内上顎動脈との間に血管網が形成されており，CRMと診断した。さらに，眼底，右側頸部皮膚生検より，網膜色素線条およびPXEの特徴である真皮の弾性線維の断裂およびCaの沈着が証明され，PXEを合併していることが判明した。現在までにCRMの報告は5例であり，その内1例にPXEの合併がみられた。また，PXEの10％には中枢神経症状を来すといわれ，今後，この両者の因果関係につき検討を要すと思われた。

A case of carotid rete mirabile (CRM) with pseudoxanthoma elasticum (PXE) was reported. A 55-year-old woman was admittd to our hospital with complain of left motor weakness. Plain CT scan showed a lacuna in the right internal capsule. Right retrograde brachial angiography and left carotid angiography demonstrated that internal carotid arteries were hypoplastic and winding,and there were abnormal networks between ICA in the cavernous portion and internal maxillary artery, which was a branch of external carotid artery. Diagnosis of CRM was made. Fundoscopy showed bilateral angioid streaks and histological examination of a eruption in her right neck re-vealed degenaration of elastic fiber and calcium deposits in the derm, which findings were verified to be PXE. So this case was diagnosed of CRM combined with PXE. She got better by conserva-tive therapy in 3 weeks and discharged on foot. On previous literatures 5 cases of CRM were reported and one of which was associated with PXE. CRM is a normal anatomical structure noted lower mammals like cat, sheep and pig. 10% of PXE, which is systemic disorder of elastic fiber, showed CNS lesions. Our case seems to be CRM combined with PXE by chance. But two of 6 cases of CRM were with PXE, so it is necessary that a case of PXE should be examined angiographically.