Introduction:Martin Heinrich Rathke (1793-1860), the pioneer German embryologist, described the existence of Rathke’s pouch in 1839. This structure has subsequently become the target of endoscopic endonasal surgery when a Rathke cleft cyst becomes symptomatic. The clinical presentation, surgical techniques, and long-term outcomes continue to be of interest. Our goal is to highlight nuances in diagnosis, surgical philosophy and techniques, and long-term outcomes.
Methods: We retrospectively reviewed a single surgeon experience in a contemporary series of Rathke Cleft Cysts (RCC) from April 2008 through December 2019. The clinical presentation and diagnosis, radiographic studies, intraoperative findings, histopathological diagnosis, postoperative complications, and outcome data were recorded and analyzed. Patients were grouped into 3 main categories: patients who presented for initial surgery; patients who presented to our center but had prior surgery elsewhere; and patients with recurrent lesions.
Results: Our search yielded a total of 131 endoscopic endonasal operations performed on 116 patients (30 male, 86 female). The average age of patients was 38.4 years (range 12-78.2 years). We identified 102 patients in whom this was the first operation (primary surgery group), and 14 patients who had prior surgical intervention. The most common clinical presentations included: headache (n=93, 80%), visual changes (n=42, 36%), hypopituitarism (n=18, 16%), symptoms of hyperprolactinemia (n=16, 14%), and apoplexy (n=4, 3%). Eight patients (6.8%) had their RCC diagnosed incidentally. The average maximal cyst diameter was 1.3 cm, and suprasellar extension was observed in 50 patients (43%). Typical histopathological RCC criteria (cyst wall and contents) were proven in 75 patients (65%). Postoperative complications (in the primary surgery group) included transient diabetes insipidus (DI) (n=21, 20.5%), permanent DI (n=5, 4.9%), SIADH (n=6, 5.8%), epistaxis (n=6, 5.8%), postoperative CSF leak (n=1, <1%), postoperative infection (n=3, 2.9%), intrasellar hematoma (n=1, <1%), and a small thalamic stroke (n=1, <1%). The average duration of follow-up was 38.8 months (1.3-141 months). Complete follow up data were available in 87 patients, and the median duration of follow-up was 15.2 months. Visual improvement was observed in 74% (n= 31/42) of patients, and headache improvement (severity and/or frequency) in 84% (n= 78/93). Recurrence rate (in the primary surgery group) was 10.7% and the overall recurrence rate was 11.2%. Average time to recurrence was 31.6 months (8.4-72.7 months).
Conclusion: The philosophy behind a surgical approach to Rathke cleft cysts represents a controversy. Our results reflect the philosophy of a conservative surgical approach, designed to preserve normal pituitary function, avoid DI, while achieving the maximal feasible resection. Evacuation of the cyst contents and meticulous sampling of the cyst wall when possible for histopathological diagnosis are crucial. The postoperative relief of symptoms and signs is gratifying, as are the overall rates of recurrence and long-term outcomes.