Professor Rathke's Legacy to Neurosurgery: The Cyst, Its Diagnosis, Surgical Management, and Outcomes

Introduction: Martin Heinrich Rathke (1793–1860), the pioneer German embryologist, reported the evolutionary existence of Rathke's pouch in 1839. Subsequently, this structure has become the target of endoscopic endonasal surgery when a Rathke's cleft cyst becomes symptomatic. The clinical presentation, surgical techniques, and long-term outcomes continue to be of interest. We aim to highlight the nuances in diagnosis, surgical philosophy and techniques, and long-term clinical and radiographic outcomes.

Methods: We retrospectively reviewed a single surgeon experience in a contemporary series of Rathke's 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 three 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 (31 males and 85 females). The average age of patients was 38.4 years (range: 12–78.2 years). We identified 102 patients in whom this was the first surgery, and 14 patients who had prior surgical intervention. The most common clinical presentations included: headache (n = 93, 80.1%), visual changes (n = 42, 36.2%), hypopituitarism (n = 18, 15.5%), and symptoms of hyperprolactinemia (n = 16, 13.7%). Eight patients (6.8%) had their RCC incidentally diagnosed. The maximal cyst diameter was 13 ± 6 mm, and suprasellar extension was observed in 50 patients (43.1%). Typical histopathological RCC criteria (cyst wall and contents) were proven in 75 patients (64.6%). Postoperative complications (in the initial surgery group) included transient diabetes insipidus (DI; n = 21, 20.5%), permanent DI (n = 5, 4.9%), transient SIADH (n = 6, 5.8%), epistaxis (n = 6, 5.8%), infection (n = 3, 2.9%), CSF leak (n = 1, <1%), hematoma (n = 1, <1%), and stroke (n = 1, <1%). The average duration of follow-up was 38.8 months (range: 1.3–141 months). Complete follow-up data were available in 87 patients. Visual improvement was observed in 83.3% of patients, and headache improvement (severity and/or frequency) in 91.3%. Recurrence rate (in the initial surgery group) was 10.7% and the overall recurrence rate was 11.2%. Time to recurrence was 31.6 months (8.4–72.7 months; Table 1).

Conclusion: These results reflect the philosophy of a conservative surgical approach, designed to preserve normal pituitary function and 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 clinical and radiological outcomes.

Table 1

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021