Connolly E S, Winfree C J, Carmel P W
Department of Neurological Surgery, Columbia-Presbyterian Medical Center, New York, New York, USA.
Surg Neurol. 1997 Mar;47(3):291-9. doi: 10.1016/s0090-3019(96)00253-4.
Autopsy studies and recent reviews report that 4% of craniopharyngiomas have posterior fossa extension at initial operation, and 12% subsequently develop this extension during their course. However, only two patients in the literature have been shown to present with deafness, which preceded the more typical suprasellar signs and symptoms of increased intracranial pressure, endocrine disturbance, altered mentation, and visual deterioration.
The authors report three cases of giant cystic posterior fossa craniopharyngioma initially presenting in childhood with either unilateral or bilateral deafness. These cases are presented, the literature on posterior fossa craniopharyngioma is reviewed, and staged operative management is discussed.
Despite being rarely reported, 10% to 20% of giant cystic craniopharyngiomas with posterior fossa extension at presentation may have unilateral or bilateral deafness as their first symptom. Deafness as a presenting symptom is much less common in patients presenting with recurrent tumors in this location than with primary tumors. Pterional or bifrontal craniotomy is appropriate for management of the parasellar component and should be undertaken first when visual symptoms are present. Suboccipital craniectomy is appropriate for management of the posterior fossa component and should be undertaken first when brain stem compressive symptoms make it necessary. Skull-base techniques may have value in certain settings, but patient's morbidity when using these techniques must be carefully considered.
Our results indicate that staged operations with the goal of achieving gross total resection can yield excellent results. Adjuvant radiation is indicated for those with residual tumor seen on magnetic resonance imaging (MRI), but in young children without residual tumors by MRI, we prefer to follow carefully with serial scans. Since the loss of hearing in children can compromise language development, we suggest referral of these children to a specialist in language rehabilitation.
尸检研究及近期综述报告显示，4%的颅咽管瘤在初次手术时即有后颅窝扩展，12%在病程中会出现这种扩展。然而，文献中仅报道过两例以耳聋为首发症状的患者，且耳聋症状先于更为典型的鞍上症状出现，如颅内压升高、内分泌紊乱、精神状态改变及视力减退。
作者报告了3例巨大囊性后颅窝颅咽管瘤病例，这些病例均在儿童期发病，首发症状为单侧或双侧耳聋。本文呈现了这些病例，回顾了后颅窝颅咽管瘤的相关文献，并讨论了分期手术治疗方法。
尽管报道较少，但10%至20%的表现为后颅窝扩展的巨大囊性颅咽管瘤可能以单侧或双侧耳聋为首发症状。与原发性肿瘤相比，在该部位出现复发性肿瘤的患者中，以耳聋为首发症状的情况要少见得多。翼点或双额开颅术适用于鞍旁部分的处理，当出现视觉症状时应首先进行。枕下颅骨切除术适用于后颅窝部分的处理，当出现脑干压迫症状时必须首先进行。颅底技术在某些情况下可能有价值，但使用这些技术时患者的发病率必须仔细考虑。
我们的结果表明，以实现大体全切为目标的分期手术可取得良好效果。对于磁共振成像（MRI）显示有残留肿瘤的患者，应进行辅助放疗，但对于MRI未显示残留肿瘤的幼儿，我们更倾向于通过系列扫描进行密切随访。由于儿童听力丧失会影响语言发育，我们建议将这些儿童转诊至语言康复专家处。
