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复发性脑干内表皮样囊肿

Recurrent intrinsic brain stem epidermoid cyst.

作者信息

Fournier D, Mercier P, Menei P, Pouplard F, Rizk T, Guy G

机构信息

Service de Neurochirurgie, Centre Hospitalier Régional et Universitaire d'Angers, France.

出版信息

Childs Nerv Syst. 1992 Dec;8(8):471-4. doi: 10.1007/BF00274412.

Abstract

The authors report the case of a 14-month-old baby boy with an epidermoid cyst located entirely within the pons and medulla, without an exophytic component. The lesion was examined by computed tomography and magnetic resonance imaging. The child was operated upon three times after two recurrences of the lesion. A suboccipital, subtonsillar approach was used for the first and second procedures and a transtemporal approach for the last one. Excision was thought to be complete the first time, since a solid tumor was found and removed in a large cyst. The cyst wall was not identified. No tumor was found during the second procedure despite recurrence of the cyst, which was drained without an attempt to remove the cyst wall. Finally the cyst recurred with a large tumor in the cyst wall which was again totally removed. Consistent with the high mortality of brain stem epidermoid cysts in the literature, the child eventually died. The therapeutic problems, surgical options, and consequences are discussed.

摘要

作者报告了一例14个月大男婴的病例,其表皮样囊肿完全位于脑桥和延髓内,无外生性成分。通过计算机断层扫描和磁共振成像对该病变进行了检查。该病变两次复发后,患儿接受了三次手术。第一次和第二次手术采用枕下、扁桃体下入路,最后一次采用经颞入路。第一次手术时认为切除彻底,因为在一个大囊肿内发现并切除了一个实体瘤。未发现囊肿壁。第二次手术时尽管囊肿复发,但未发现肿瘤,仅对囊肿进行了引流,未尝试切除囊肿壁。最后囊肿再次复发,囊肿壁出现一个大肿瘤,再次将其完全切除。与文献中脑干表皮样囊肿的高死亡率一致,患儿最终死亡。文中讨论了治疗问题、手术选择及后果。

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