目的:鞍区Rathke裂囊肿(RCC)的传统治疗通常涉及经鞍区引流;然而,鞍上RCC对适当的管理和技术复杂性提出了独特的挑战。关于这种病理的内镜经鼻途径(EEA)的总体结果的报告有限。用于RCC的EEA允许三种手术技术:袋状化,开窗术,和囊肿壁切除开窗术。
方法:作者对2004年1月至2021年5月在单一机构通过EEA治疗的连续RCC患者进行了回顾性研究。有袋化需要去除囊肿内容物,同时保持进入蝶窦的引流途径。开窗术包括去除囊肿内容物,然后从蝶窦分离,通常带有游离的粘膜移植物或血管化的鼻中隔皮瓣。囊肿壁切除术,部分或完整,已添加到选择案例中。
结果:共有148例患者因RCC接受了EEA。88例(59.5%)进行了囊袋化或开窗术,60例(40.5%)进行了囊肿壁切除术。囊肿被归类为纯鞍源(43.2%),鞍上延伸的鞍源(37.8%),纯鞍上起源(18.9%)。22例(14.9%)在平均39.7个月的随访(中位数45个月,范围0.5-99个月),包括13例(8.8%)有症状的病例。囊肿壁切除的病例复发率无明显差异(11.7%vs15.9%,p=0.48)或术后永久性垂体前叶功能障碍(21.6%vs12.5%,p=0.29)与有窗和有袋的病例相比。术后永久性垂体后叶功能障碍的技术差异无统计学意义,尽管这种功能障碍在囊肿壁切除后趋于恶化(13.6%vs4.0%,p=0.09)。根据囊肿的位置,单纯鞍上囊肿比鞍上囊肿(12.5%)和单纯鞍囊囊肿(9.4%;p=0.008)更可能发生放射学复发(28.6%).最值得注意的是,在28个纯粹的鞍上囊肿中,选择性囊壁切除术与单纯开窗术相比,显著改善了长期(10年)复发风险(17.4%vs80.0%,p=0.0005),无任何明显的内分泌病风险。
结论:内镜下鼻内有袋化或开窗术可能是理想的治疗策略,而单纯鞍上囊肿受益于部分囊肿壁切除术以防止复发。选择性囊壁切除术可降低长期复发率,而不会显着增加垂体功能减退症的发生率。
OBJECTIVE: The traditional treatment of sellar Rathke cleft cysts (RCCs) generally involves transsellar drainage; however, suprasellar RCCs present unique challenges to appropriate management and technical complexity. Reports on overall outcomes for the endoscopic endonasal approach (EEA) for this pathology are limited. The EEA for RCCs allows three surgical techniques: marsupialization, fenestration, and fenestration with cyst wall resection.
METHODS: The authors performed a retrospective review of consecutive patients with RCCs that had been treated via an EEA at a single institution between January 2004 and May 2021. Marsupialization entailed the removal of cyst contents while maintaining a drainage pathway into the sphenoid sinus. Fenestration involved the removal of cyst contents, followed by separation from the sphenoid sinus, often with a free mucosal graft or vascularized nasoseptal flap. Cyst wall resection, either partial or complete, was added to select cases.
RESULTS: A total of 148 patients underwent an EEA for RCC. Marsupialization or fenestration was performed in 88 cases (59.5%) and cyst wall resection in 60 (40.5%). Cysts were classified as having a purely sellar origin (43.2%), sellar origin with
suprasellar extension (37.8%), and purely
suprasellar origin (18.9%). Radiological recurrence was demonstrated in 22 cases (14.9%) at an average 39.7 months\' follow-up (median 45 months, range 0.5-99 months), including 13 symptomatic cases (8.8%). Cases with cyst wall resection had no significantly different rate of recurrence (11.7% vs 15.9%, p = 0.48) or postoperative permanent anterior pituitary dysfunction (21.6% vs 12.5%, p = 0.29) compared to those of fenestrated and marsupialized cases. There was no significant difference in postoperative permanent posterior pituitary dysfunction based on technique, although such dysfunction tended to worsen with cyst wall resection (13.6% vs 4.0%, p = 0.09). Based on cyst location, purely
suprasellar cysts were more likely to have a radiological recurrence (28.6%) than sellar cysts with
suprasellar extension (12.5%) and purely sellar cysts (9.4%; p = 0.008). Most notably, of the 28 purely
suprasellar cysts, selective cyst wall resection significantly improved the long-term (10-year) recurrence risk compared to fenestration alone (17.4% vs 80.0%, p = 0.0005) without any significant added risk of endocrinopathy.
CONCLUSIONS: Endoscopic endonasal marsupialization or fenestration of sellar RCCs may be the ideal treatment strategy, whereas purely suprasellar cysts benefit from partial cyst wall resection to prevent recurrence. Selective cyst wall resection reduced long-term recurrence rates without significantly increasing rates of hypopituitarism.