A Fatal Case of a Ruptured Posterior Communicating Artery Aneurysm in a Patient With Suspected Klippel-Feil Syndrome

一例疑似患有克利佩尔-费尔综合征的患者发生后交通动脉瘤破裂致死的病例报告

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Abstract

Klippel-Feil syndrome (KFS) is a rare congenital disorder characterized by cervical vertebral fusion and often associated with multisystem anomalies. Although primarily skeletal, KFS has been linked to vascular abnormalities, including arterial dissections, agenesis, and aneurysms. We present the case of a 33-year-old previously healthy woman who presented with diffuse subarachnoid hemorrhage. Imaging revealed a ruptured 5.5 mm posterior communicating artery aneurysm arising from the left supraclinoid internal carotid artery, along with congenital cervical vertebral fusion consistent with undiagnosed KFS. The patient underwent emergency craniotomy with clip ligation complicated by intraoperative rupture and cerebral edema, ultimately requiring decompressive hemicraniectomy. Despite maximal surgical and medical management, she progressed to brain death. This case suggests a possible association between KFS and cerebrovascular anomalies and highlights the importance of considering vascular imaging in patients with KFS who present with acute neurological deterioration. Increased awareness of these potential complications may aid in earlier recognition and improved outcomes.

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