線維筋異形成症による頭蓋内動脈瘤破裂による致死的くも膜下出血を伴う出血性網膜症および視神経鞘出血

Hemorrhagic Retinopathy and Optic Nerve Sheath Hemorrhage Associated With Fatal Subarachnoid Hemorrhage from a Ruptured Intracranial Aneurysm Due to Segmental Fibromuscular Dysplasia.

• Elizabeth O Silloca-Cabana
• Vivian M Hathuc
• William T Harrison
• Patrick E Lantz

抄録

線維筋形成不全症は，腎動脈と頸動脈を侵すことが最も多い特発性，非異形成性，非炎症性の動脈疾患である．線維筋異形成による動脈瘤を伴うくも膜下出血と眼球出血を呈した小児を報告する。倒れた後のCT検査でびまん性くも膜下出血と重度の脳浮腫を認めた．剖検ではX線所見を確認し，両側網膜出血，視神経鞘出血，頭蓋内右椎骨動脈の線維筋異形成による嚢状動脈瘤破裂を認めた．本症例は、線維筋異形成による頭蓋内嚢状動脈瘤を発症した小児のくも膜下出血が致命的なものであった。関連する網膜出血は、死後の単眼間接眼鏡検査で容易に検出できる。

Fibromuscular dysplasia is an idiopathic, nonatheromatous, and noninflammatory arterial disease that most commonly affects the renal and carotid arteries. We report a child with subarachnoid and ocular hemorrhage associated with an aneurysm due to fibromuscular dysplasia. Computed tomography following a witnessed collapse revealed diffuse subarachnoid hemorrhage and severe cerebral edema. An autopsy confirmed the radiographic findings and detected bilateral retinal hemorrhages, optic nerve sheath hemorrhages, and a ruptured saccular aneurysm due to focal fibromuscular dysplasia involving the intracranial right vertebral artery. This case documents a fatal subarachnoid hemorrhage in a child with an intracranial saccular aneurysm caused by fibromuscular dysplasia. The associated retinal hemorrhages are easily detected by postmortem monocular indirect ophthalmoscopy.

© 2019 American Academy of Forensic Sciences.