Department of Neurosurgery, University of California, San Diego-Rady Children's Hospital, San Diego, CA, 92123, USA.
Department of Neurosurgery, University of Utah, Salt Lake City, UT, 84112, USA.
Childs Nerv Syst. 2024 May;40(5):1367-1375. doi: 10.1007/s00381-024-06277-z. Epub 2024 Jan 19.
Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and optimal management of growing or symptomatic RCCs. We present our institutional experience with the surgical management of RCCs.
We performed a retrospective study of consecutive RCC patients ≤ 18 years old treated surgically at our institution between 2006 and 2022.
Overall, 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 had a histopathological diagnosis of RCC, 58% female and 42% male. The mean age was 13.2 ± 4.2 years. Presenting symptoms included headache (58%), visual changes (32%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and subsequently demonstrated growth on serial imaging. Six percent presented with symptomatic intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients and preoperative visual deficits improvement in 55% after surgery. New pituitary axis deficits were seen in 9.7% of patients. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging at a mean follow-up was 62.9 ± 58.4 months. Recurrence requiring reoperation occurred in 32% of patients. Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up.
Due to their secretory epithelium, pediatric RCCs may demonstrate rapid growth and can cause symptoms due to local mass effect. Surgical management of symptomatic or growing pediatric RCCs via cyst fenestration or partial resection of the cyst wall can be performed safely, with good neurologic outcomes. There is a nontrivial risk of endocrinologic injury, and long-term follow up is needed due to high recurrence rates.
Rathke 裂隙囊肿（RCC）是一种良性、上皮衬里的鞍内病变，起源于颅咽管的残余物。由于在儿科人群中罕见，因此关于生长或有症状的 RCC 的自然病史和最佳治疗的数据有限。我们介绍了我们在机构中治疗 RCC 的经验。
我们对 2006 年至 2022 年期间在我们机构接受手术治疗的≤18 岁 RCC 连续患者进行了回顾性研究。
共有 567 例诊断为垂体肿块或囊肿的患者，其中 31 例有 RCC 的组织病理学诊断，女性占 58%，男性占 42%。平均年龄为 13.2±4.2 岁。主要症状包括头痛（58%）、视力改变（32%）和内分泌或生长迟缓（26%）；13%为偶然发现，随后在连续影像学检查中显示生长。6%的患者表现为有症状的瘤内出血。90%的患者采用经蝶窦入路，10%的患者采用眶颧入路。61%的患者术前头痛缓解，55%的患者术前视力减退术后改善。9.7%的患者出现新的垂体轴缺陷。首次手术仅发生 2 例并发症：1 例脑脊液漏需放置腰大池引流，1 例鼻出血需烧灼。没有患者出现新的视力或神经功能缺损。患者术后接受了平均 62.9±58.4 个月的连续影像学随访。32%的患者需要再次手术以治疗复发。5 年无进展生存率为 47.9%。除 1 例因并发脑顶盖胶质瘤而出现多种神经功能缺损的患者外，所有患者末次随访时的改良 Rankin 量表评分为 0 或 1（良好结局）。
由于其分泌上皮，小儿 RCC 可能会迅速生长，并因局部肿块效应而引起症状。通过囊壁开窗或部分切除囊肿的方法治疗有症状或生长的小儿 RCC 可以安全进行，神经功能预后良好。存在相当大的内分泌损伤风险，由于复发率高，需要长期随访。
