Rathke’s Cleft Cyst Presenting Synchronously with a Diaphragma Sellae Meningioma

Background Diaphragma sellae meningiomas have been known to mimic pituitary adenomas; however, they have not been seen synchronously with a Rathke’s cleft cyst (RCC). The authors present the case of a patient who presented with vision loss and an unusual magnetic resonance imaging (MRI) to discuss the impact of both lesions on patient outcomes.

Case Report A 44-year-old woman presented with 8 months of vision changes and left abducens palsy. MRI revealed a cystic, T2 hyperintense sellar, suprasellar mass with bilateral cavernous sinus invasion, more prominent on the left side, with peripheral enhancement. After undergoing an endoscopic, endonasal transsphenoidal approach (EEA), pathology confirmed WHO Grade I meningioma with an entrapped RCC. Postoperatively, that patient’s vision improved and her sixth nerve palsy resolved. Her postoperative MRI revealed rightward deviation of the infundibulum with abnormal thickening of the stalk, involvement of the bilateral cavernous sinuses, and capsular retraction of the meningioma medially toward the sella. Approximately 5 months after surgery, she was found to have normal visual fields and did not complete her 6-month, follow-up MRI. She presented about 1 year from surgery with recurrent headache and left orbit pain. She had an MRI of the brain that showed interval increase of the RCC with upward displacement of the optic chiasm and persistent enhancement. Her ophthalmologist confirmed visual deterioration. She subsequently underwent, an EEA for fenestration and drainage of her RCC.

Discussion The synchronous presentation of a RCC and diaphragma sellae meningioma presents a clinical dilemma for the skull base surgeon. The management of tuberculum sellae and diaphragma sellae meningiomas is controversial because experienced surgeons disagree as to whether an open approach or EEA is most appropriate for their extirpation. However, the earlier patient presented with vision loss related to mass effect from the proteinaceous contents of the RCC, which the authors felt was most appropriately treated with an EEA. The removal of the meningioma was secondary to fenestration of the RCC for the purpose of decompression and marsupialization.

Conclusion Synchronous tumors of the central nervous system can be challenging to manage because they can cause simultaneous neurologic deficit. The RCC was the primary cause for the patient’s vision deterioration due to the fact that her symptoms improved with fenestration and then worsened reaccumulation of the cyst. This concurrent set of lesions should be treated with an EEA to decompress the optic apparatus as well as marsupialize the cyst to the sphenoid sinus to prevent recurrence.