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继发于巨大颅咽管瘤的获得性Chiari-I畸形和脊髓空洞症。

Acquired Chiari-I malformation and hydromyelia secondary to a giant craniopharyngioma.

作者信息

Lee M, Rezai A R, Wisoff J H

机构信息

Division of Pediatric Neurosurgery, New York University Medical Center, NY 10016 USA.

出版信息

Pediatr Neurosurg. 1995;22(5):251-4. doi: 10.1159/000120910.

Abstract

Hydromyelia, or hydrosyringomyelia is frequently associated with the Chiari I malformation of the cerebellar tonsils. Descent of the cerebellar tonsils is considered a congenital anomaly with a few reports of 'acquired' Chiari I malformation. We report a patient with a giant craniopharyngioma and hydrocephalus who at presentation had a concomitant Chiari I malformation and hydromyelia. The patient underwent gross total resection of the tumor and, with no further treatment, demonstrated spontaneous resolution of the Chiari I malformation and hydromyelia during the postoperative period. This suggests that the Chiari I malformation and the resulting hydromyelia were 'acquired', and were caused by an intracranial mass effect. This provides further evidence for an associative mechanism of cerebellar tonsillar descent and the development of hydromyelia.

摘要

脊髓空洞症,或积水性脊髓空洞症常与小脑扁桃体的Chiari I畸形相关。小脑扁桃体下移被认为是一种先天性异常,仅有少数关于“后天性”Chiari I畸形的报道。我们报告了一名患有巨大颅咽管瘤和脑积水的患者,其在就诊时伴有Chiari I畸形和脊髓空洞症。该患者接受了肿瘤全切术,且未进行进一步治疗,术后Chiari I畸形和脊髓空洞症出现了自发缓解。这表明Chiari I畸形及由此导致的脊髓空洞症是“后天性的”,由颅内占位效应引起。这为小脑扁桃体下移与脊髓空洞症发生之间的关联机制提供了进一步证据。

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