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.
