Intrasphenoidal Rathke’s Cleft Cyst: An Uncommon Feat

Abstract

Usually occurring entirely intrasellarly or extending suprasellarly (intra-suprasellar), Rathke's cleft cysts (RCCs) can present with an intrasphenoidal location. Extrasellar positions are rare. To date, only seven patients with intrasphenoidal RCC have been reported in the literature. Despite the rarity of the condition and the lack of pathognomonic radiological features, preoperative diagnosis remains challenging. A transsphenoidal approach can be adopted to treat this type of cyst, which has great clinical relevance. Awareness of this different presentation of RCC before respective management may be of value in its approach. Intrasphenoidal RCC should be diagnosed preoperatively and the surgical approach should be changed accordingly by aspiration and partial removal before the histological examination.

Spontaneous and asymptomatic rupture of an RCC with resolution of symptoms

“…Thus, patients can present with visual impairment, endocrine dysfunction, obstructive hydrocephalus and/or reversible Korsakoff syndrome, should the SSC progress in a retrochiasmatic fashion 6. However, the most common presentation for RCCs is visual defects, which were not immediately apparent in our patient 7…”

“…Therefore, it is difficult to differentiate clinical presentations of SACs versus RCCs/craniopharyngiomas. We experienced similar difficulty with our patient’s suprasellar cyst, ultimately relying on imaging findings to help guide us, as RCCs are known to have a well-defined, homogeneous appearance on imaging 7. We believed the mass was an RCC based on the cystic, midline and homogenous appearance of it noted on MRI 8 9.…”

“… 6 However, the most common presentation for RCCs is visual defects, which were not immediately apparent in our patient. 7 …”

“…We experienced similar difficulty with our patient’s suprasellar cyst, ultimately relying on imaging findings to help guide us, as RCCs are known to have a well-defined, homogeneous appearance on imaging. 7 We believed the mass was an RCC based on the cystic, midline and homogenous appearance of it noted on MRI. 8 9 Additionally, the appearance of a cyst without evidence of a mass leads us to prioritise the diagnosis of RCC over that of craniopharyngioma, which would reflect mass components.…”