Rathke cleft cyst apoplexy: a newly characterized distinct clinical entity

Abstract

Although most Rathke cleft cysts (RCCs) remain asymptomatic, some present with compression of surrounding structures and pituitary hormone dysfunction. A rare, but distinct presentation of the RCC includes hemorrhage into the cyst--a presentation that mimics the clinical syndrome of pituitary tumor apoplexy. The objective of this article is to present the authors' experience on the prevalence and the clinical, biochemical, pathological, and imaging characteristics of hemorrhage into an RCC and review published reports on this entity. An institutional database for patients with pituitary masses was reviewed and data on cases involving surgery for a subsequently documented RCC were identified. Patients with documented hemorrhage within the RCC were included in the analysis. Documentation included intraoperative visualization of hemorrhage by the surgeon and/or pathological confirmation of hemorrhage within the RCC. The clinical, biochemical, pathological, and imaging characteristics of these patients were extracted from the database. All published data on RCC were also reviewed and confirmed cases of hemorrhage were extracted and included in the review. A total of 11 cases with hemorrhage within an RCC were identified to have been managed at the authors' institution over a 10-year span. This figure represented 20% of all surgically treated cases of RCC from that period. Among published reports, a total of 10 confirmed cases were evaluated. The clinical presentation of patients with hemorrhage within an RCC are classical and include sudden onset of severe headache--or a sudden increase in headache severity--associated with visual disturbances, impairment in pituitary function, and occasionally alterations in mental status. Leakage of the cystic contents into the subarachnoid space can occur rarely and may lead to associated chemical meningitis. There are no known precipitating factors for this phenomenon. The clinical presentation of such patients is difficult to distinguish from that of patients with pituitary tumor apoplexy. However, patients with RCC apoplexy often present with less severe symptoms, have a lower prevalence of pituitary dysfunction, and generally have smaller mass lesions than those with pituitary tumor apoplexy. Management of patients with RCC apoplexy is similar to that of those with pituitary tumor apoplexy. Surgery can provide definitive diagnosis and relief of symptoms and often leads to recovery from hypopituitarism. All patients with this syndrome require long-term follow-up to treat any residual sequelae. This article provides the first thorough review and characterization of patients with hemorrhage within an RCC. Based on the clinical manifestations of this presentation, we recommend using the term "Rathke cleft cyst apoplexy" to describe the syndrome.