Eaton Jessica, Raub Spencer, Ronsley Rebecca, Roth Christian L, Ermoian Ralph, Friedman Seth D, Emerson Samuel N, Ferreira Manuel, Lee Amy, Bly Randall A, Ruzevick Jacob
Departments of Neurological Surgery, University of Washington, Seattle, Washington.
Department of Pediatrics, Division of Hematology, Oncology & BMT, Seattle Children's Hospital and the University of Washington, Seattle, Washington.
J Neurosurg Case Lessons. 2024 Dec 9;8(24). doi: 10.3171/CASE24209.
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.
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.
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.
小儿颅咽管瘤的治疗需要多学科方法，以便就包括活检、放疗和/或手术切除在内的一系列治疗选择向患者及其家属提供咨询。全切除可避免放疗及其长期并发症。在非常年幼的患者中，这一点尤为重要，但由于解剖学因素，实施起来特别具有挑战性。
一名23个月大的男孩被发现蝶鞍和鞍上有部分钙化的囊性肿块。采用全内镜经鼻结节和经蝶入路对膈下造釉细胞瘤型颅咽管瘤进行全切除。术后，患者被诊断为垂体功能减退合并尿崩症，但无其他下丘脑功能障碍或新的视力缺陷。迄今为止，尚无肿瘤复发的证据。
在一个拥有经验丰富的多学科颅底团队的大型中心，可以安全地对2岁以下儿童的蝶鞍和鞍上病变采用内镜经鼻入路。较小的鼻腔和鼻窦未充气需要通过去除骨质进行广泛暴露并完全打开鼻窦，以形成足够的工作通道，不仅能够进行肿瘤切除，还能进行重建。在发育中的儿童中进行安全的全切除可以避免或至少延迟放疗及其长期并发症。https://thejns.org/doi/10.3171/CASE24209 。
