Rapidly recurrent recalcitrant Rathke Cleft Cyst: Case report and review of the literature

Abstract

BackgroundRathke Cleft Cysts (RCC) are cystic sellar or suprasellar nonneoplastic lesions that are thought to originate from remnants of the rathke pouch. Postoperatively, RCCs have a tendency to reaccumulate, after which preoperative symptoms may recur. However, there exists very little evidence on which treatments are most effective in these patients. Case-descriptionIn this report, we present a unique case of a symptomatic RCC that recurred four times after initial transsphenoidal surgery. Following each surgery, the patient had significant visual improvement with post-op imaging displaying decompression of the neural elements. However, RCC reaccumulated in strikingly rapid time intervals of 1.5 months, 0.5 months, 1.5 years, and 5 months after each respective prior surgery. Repeat interventions with transsphenoidal, pterional and supraorbital approaches were unsuccessful in providing a durable treatment response. The patient ultimately underwent radiotherapy after a final surgical marsupialization of the cyst and has since displayed stable imaging with improved vision. This patient represented a ‘perfect storm’ of factors that may contribute to cyst recurrence, including substantial visual field deficits, large cyst size, peripheral wall enhancements on MRI, an intraoperative CSF leak, use of a fat graft, subtotal resection of the portion of cyst wall that adhered to important suprasellar structures, squamous metaplasia noted in cyst wall, and suprasellar extension. ConclusionsWe demonstrate that different surgical approaches through repeat surgeries may not assist in prevention of further recurrence; instead, we propose that radiotherapy should be offered early in the treatment course of recurrent cases that have additional risk factors for further reoccurrence.