内镜下鼻内切除颅咽管瘤在23个月的病人:说明性病例。

Jessica Eaton, Spencer Raub, Rebecca Ronsley, Christian L Roth, Ralph Ermoian, Seth D Friedman, Samuel N Emerson, Manuel Ferreira, Amy Lee, Randall A Bly, Jacob Ruzevick
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摘要

背景:儿童颅咽管瘤的治疗需要多学科的方法来咨询患者和家属的治疗选择范围,包括活检,放疗和/或切除。全切除可避免放射及其长期合并症。在非常年轻的患者中,这是特别重要的,但由于解剖学的考虑,尤其具有挑战性。观察:一个23个月大的男孩被发现有部分钙化和囊性鞍上肿块。经鼻内经脑膜及鼻鞍入路行横膈膜下硬瘤性颅咽管瘤全切除术。术后,患者被诊断为垂体功能减退伴尿崩症,但无其他下丘脑功能障碍或新的视觉缺陷。到目前为止,没有肿瘤复发的证据。经验教训:在一个拥有经验丰富的多学科颅底团队的大容量中心,对2岁以下儿童的鞍和鞍上病理进行内窥镜鼻内入路是安全的。较小的鼻腔和缺乏鼻窦通气需要通过骨切除和鼻窦完全开放进行广泛暴露,以提供足够的工作通道,不仅可以进行肿瘤切除,还可以进行重建。安全的全切可以避免,或至少延迟放射及其在发育中的儿童中的长期发病率。https://thejns.org/doi/10.3171/CASE24209。
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Endoscopic endonasal resection of a craniopharyngioma in a 23-month-old patient: illustrative case.

Background: Treatment of pediatric craniopharyngioma requires a multidisciplinary approach to counsel patients and families on the spectrum of treatment options, including biopsy, radiation, and/or resection. Gross-total resection can avoid radiation and its long-term comorbidities. In very young patients, this is of particular importance but is especially challenging because of anatomical considerations.

Observations: A 23-month-old boy was found to have a partially calcified and cystic sellar and suprasellar mass. A fully endoscopic endonasal transtuberculum and transsellar approach was performed for gross-total resection of a subdiaphragmatic adamantinomatous craniopharyngioma. Postoperatively, the patient was diagnosed with panhypopituitarism with diabetes insipidus, though without other hypothalamic dysfunction or new visual deficits. To date, there is no evidence of tumor recurrence.

Lessons: In a high-volume center with an experienced multidisciplinary skull base team, endoscopic endonasal approaches to sellar and suprasellar pathology in children younger than 2 years can be safely performed. A smaller nasal cavity and lack of sinus aeration necessitate wide exposure via bone removal and complete opening of the sinuses to enable an adequate working corridor to perform not only tumor resection but also reconstruction. Safe gross-total resection can avoid, or at least delay, radiation and its long-term morbidity when performed in a developing child. https://thejns.org/doi/10.3171/CASE24209.

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