Endoscopic Endonasal Transsphenoidal Fenestration of Rathke Cleft Cysts in Children

Object: With improved access to advanced neuroimaging, Rathke’s cleft cysts (RCCs) are increasingly being diagnosed in the pediatric population. For children with medically refractory headaches, visual changes or progressive enlargement, surgical intervention may be appropriate to reduce symptoms and prevent visual decline. We present our experience with endoscopic transsphenoidal fenestration in the pediatric population.

Methods: We retrospectively reviewed the neuroimaging, operative notes and pathology reports of all pediatric patients with a RCC treated with an endoscopic transsphenoidal approach at the University of Virginia since 2005.

Results: We identified 29 pediatric patients with a RCC on the basis of MRI who were evaluated at the Neuroendocrine clinic at the University of Virginia. Twenty patients (70%) were recommended for conservative management and no growth on serial imaging was detected with a mean follow up of 32 months. The remaining 9 patients underwent endoscopic transsphenoidal cyst fenestration. One was excluded from this study due to lack of follow up. The mean age of the 5 females and 3 males was 15 years (range 8–18 years) and median follow-up was 81 months (range 2–108 months). Primary symptomatology was medically refractory headache in all patients and two also presented with visual deficits. Three patients had mild elevations in prolactin levels and one was found to have adrenal insufficiency at presentation. Mean cyst diameter was 12 mm (range 5–18 mm). The RCCs were completely intrasellar in 5 patients; the remaining three had suprasellar extension. All patients underwent an endoscopic transsphenoidal fenestration and cyst wall biopsy. Headaches improved in 7 of 8 patients postoperatively. The visual deficits in the two patients completely resolved. One patient developed diabetes insipidus postoperatively but none of the patients experienced a new anterior pituitary deficit. There was radiographic evidence of complete cyst drainage in 5 patients. In the remaining 3, residual cyst contents remained stable in size during follow up imaging. One patient whose initial postoperative imaging at 2 months showed complete drainage; experienced a symptomatic recurrence after 6 months and underwent a second fenestration.

Conclusions: The endoscopic transsphenoidal fenestration of RCCs provides symptomatic relief in the majority of properly selected children with a low incidence of new endocrinopathy.