Rathke cleft cyst apoplexy: a newly characterized distinct clinical entity

Abstract

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.

A Hemorrhagic Pineal Cyst with a Bacterial Meningitis-like Manifestation and Benign Outcome

“…These tumors include craniopharyngioma cysts, dermoid or epidermoid cysts (14), and Rathke cleft cysts (15). Furthermore, there are several reports of patients with hemorrhagic pituitary adenoma (pituitary adenoma apoplexy) with symptoms mimicking bacterial meningitis (16)(17)(18)(19)(20).…”

Bilateral acute visual loss from Rathke’s cleft cyst apoplexy in a patient with dengue fever

“…RCCs are found in 13-22% of autopsies of patients with normal pituitary. Infrequently, when the cysts become larger and compress the pituitary and surrounding structures, patients may develop headaches, endocrinopathies, and visual disturbances secondary to lesion extension (6) . A rare presentation of symptomatic RCCs is hemorrhage into the cyst, which mimics the clinical syndrome of pituitary tumor apoplexy, with acute worsening or new onset of the described symptoms.…”

“…A rare presentation of symptomatic RCCs is hemorrhage into the cyst, which mimics the clinical syndrome of pituitary tumor apoplexy, with acute worsening or new onset of the described symptoms. Recently, Chaiban et al (6) reviewed the database at their institution and the PubMed database with regard to RCC apoplexy. At their institution, RCC represented 7% of surgically treated sellar lesions, and RCC apoplexy was the first manifestation in 20% of these.…”

“…At their institution, RCC represented 7% of surgically treated sellar lesions, and RCC apoplexy was the first manifestation in 20% of these. The mean age of presentation was 40.2 years (range 16-70 years), with a female/ male ratio of 15:5 (6) . The clinical presentation of RCC apoplexy includes acute headaches, VF loss, decreased visual acuity, cranial nerve palsies, pituitary dysfunction, and occasionally altered mental status.…”

“…Yet, hemorrhage into the cyst most often appears as an area of signal hyperintensity on T1-weighted noncontrast images. Without the aid of surgical and histopathological findings, this condition is very difficult to distinguish from pituitary tumor apoplexy (unless the patient is known to have a RCC) (6,7) . In cases of RCC apoplexy with visual impairment or hypopituitarism, transsphenoidal surgery is the treatment of choice.…”