SURGICAL TREATMENT OF RATHKE CLEFT CYSTS IN CHILDREN

OBJECTIVE: Rathke cleft cysts (RCCs) are cystic epithelial lesions in the sellar and suprasellar regions that are often discovered incidentally. They require surgical fenestration and drainage in a small proportion of patients who develop symptoms or demonstrate progressive enlargement. Our aim was to review our experience with pediatric patients treated surgically for RCCs. METHODS: A retrospective review was conducted of all patients treated surgically for RCCs at Childrens Hospital Los Angeles between 1999 and 2007 after approval by the institutional review board. Clinical notes, operative reports, radiological studies, and pathology reports were reviewed. The median follow-up period was 34 months. RESULTS: Ten patients undergoing surgical treatment of an RCC were identified, making up 20% of the 51 patients with RCCs followed clinically over the same time period. The mean age was 13 years (age range, 2-17 years). There were 6 females and 4 males. Patients requiring surgery presented with the following clinical symptoms: headache (8 patients, 80%), endocrine insufficiency (6 patients, 60%), meningitis followed by visual loss (1 patient, 10%), and incidental finding 0 patient, 10%). The mean cyst diameter was 13.6 mm (range, 8-18 mm). Four patients had strictly sellar lesions, 4 patients had suprasellar extension of an RCC, and 2 patients had primarily suprasellar RCCs. Nine of 10 patients underwent transsphenoidal surgery, and I patient underwent a pterional craniotomy. Complete cyst drainage on radiography was noted in 9 of 10 patients (90%), all of whom underwent transsphenoidal surgery. One patient experienced a symptomatic recurrence 6 years after complete surgical drainage. Headaches improved in 7 of 8 patients after surgery. Two patients had complete resolution of a hormonal axis deficit, whereas 3 patients developed new anterior pituitary axis deficits. Two patients developed persistent diabetes insipidus after surgery. CONCLUSION: RCCs are an infrequent cause of symptoms in pediatric patients. The transsphenoidal approach offers an effective means of achieving complete cyst drainage for lesions requiring surgery. Fenestration and aspiration of the cyst are usually sufficient to achieve total resolution of symptoms and signs caused by RCCs. Clinical symptoms such as headaches improved in the majority of patients, whereas hormonal disturbances typically persisted. Patient selection remains of paramount importance when considering surgery for pediatric patients with RCCs.