Chiari I Malformation in Patients with Pfeiffer Syndrome: Important Aspects of Preoperative Imaging
JJK Ip, PKT Hui, WWM Lam, MT Chau
Hong Kong J Radiol 2012;15:247-51
Chiari I malformation may not be congenital, but may be acquired as a consequence of skull deformities and other associated intracranial factors in patients with craniosynostosis. Pfeiffer syndrome is one of the many conditions associated with Chiari I malformation. Premature fusion of multiple cranial sutures and cloverleaf skull (kleeblattschädel deformity) are often observed in the calvaria of patients with Pfeiffer syndrome. This report is of a male infant, with Pfeiffer syndrome who was noted to have progressive Chiari I malformation, with classical imaging features illustrated. Important aspects of preoperative imaging will be discussed, with a brief review of literature.
中文摘要
Pfeiffer綜合症患者的Chiari I型畸形：術前影像的重要性
葉精勤、許其達、林慧文、周明德
Chiari I型畸形不一定是先天性的病患，它可以是頭骨畸形以及因顱縫早閉而引致有關顱內其他相 關症狀的結果。Pfeiffer綜合症是與Chiari I型畸形眾多相關病症的其中一種。Pfeiffer綜合症患者的顱 蓋骨普遍出現多個顱縫的提早融合與三葉草顱綜合症（kleeblattschädel畸形）。本文報告一名患有 Pfeiffer綜合症的男嬰出現進行性Chiari I型畸形的典型影像，並會討論術前的影像及簡要回顧文獻。