有症状的Rathke’s裂隙囊肿的临床特点及手术治疗

Clinical features of Symptomatic Rathke＇ s cleft cyst and its surgical treatment

目的探讨有症状的Rathke’s裂隙囊肿术前术后内分泌功能紊乱情况及处理方法，以及术后复发问题。方法对11例经病理证实的Rathke’s裂隙囊肿患者的临床表现、影像学特点、内分泌改变及手术治疗方法进行回顾性分析。结果4例术前头痛患者术后均缓解，6例术前视力障碍术后缓解5例．9例术前性功能障碍及月经紊乱术后好转6例．而垂体功能低下及尿崩症不易恢复。在12～66月（平均34．5月）随访中，经颅全切除6例未见复发，而经蝶手术5例中2例大部切除者复发，复发2例病理上均合并慢性炎症浸润及局灶性鳞状上皮细胞。结论Rathke’s裂隙囊肿临床及影像学表现各异，术前确诊常常存在困难。术前术后患者的内分泌功能障碍需要进行全面的评价及激素替代治疗。术中尽量追求囊肿的全切除并取得足够的病理组织对明确诊断、判断预后有重要的意义，对于合并有鳞状细胞及炎性细胞浸润的患者术后应注意密切随访。

Objective To explore the management of endocrine disturbance related to Symptomatic Rathke＇ s cleft cysts （RCCs） in both pre- and post-operation, as well as the risk factors for the recurrence of RCCs after the operation. Methods The clinical manifestations, radiological and pathologic features, treatment, and surgical outcomes of 11 cases of symptomatic RCCs were reviewed retrospectively. Results Headache recovered in 100% of patients and visual disturbance improved in 83.3%, and Amenorrhea and/or galactorrhea recovered or improved in 66.7% of patients. However, diabetes insipidus and panhypopituitarism did not improve postoperatively. Transcranial surgery was performed in 6 patients and transsphenoidal surgery in 5 patients. No recurrence occurred in transcranial radical resected cases while 2 of 5 subtotally removed cases recurred with an average follow-up of 34.5 months. Inflammation was present in two of the recurrent cases. Conclusion RCCs is a rare pathology with a wide spectrum of clinical and radiological features. Accurate preoperative diagnosis can be difficult. Endocrine disturbance should be evaluated both pre- and post-operatively, and proper hormones replacement therapy is mandatory in these selected patients. Removing as much as possible and multiple biopsies around the cyst wall will be helpful in accurate diagnosis, especially of cases showing atypical pathologic findings, and special attention should be paid to follow-up examination.