Hemorrhagic Rathke's Cleft Cyst: Clinical Presentation and Transsphenoidal Surgical Outcomes in a Series of Six Patients

Background Rathke's cleft cysts (RCCs) are benign cystic lesions of the sellar and suprasellar space, are often found incidentally, and are typically asymptomatic. Due to their anatomical location, when symptomatic they can present with visual deficits, headaches, and endocrinopathies. Occasionally, RCCs can present clinically with an acute onset signs and symptoms mimicking pituitary apoplexy. The purpose of this study was to review our experience treating patients with confirmed hemorrhagic Rathke's cleft cysts.

Methods We performed a single-center retrospective review and identified six patients presenting with acute clinical onset of hemorrhagic Rathke's cleft cysts who underwent transsphenoidal surgical management at the University of Southern California Pituitary Center. Lesions were pathologically confirmed hemorrhagic RCCs. Surgical treatment typically involved transsphenoidal fenestration and drainage. Clinical, endocrine, and imaging outcomes were reviewed.

Results A total of six patients with a mean age of 42.5 years were identified and included. There were three men and three women with a mean follow-up time of 38.4 months (range: 3−66). All six patients were referred to for clinical symptoms of apoplexy with findings consistent with hemorrhage on outside imaging. Five of six patients (83.3%) presented with acute onset headaches as their presenting symptom. Two patients (33.3%) presented with vision loss. All three female patients presented with amenorrhea. Two patients (33.3%) presented with hypothyroidism. Two patients presented with panhypopituitarism including one with profound hyponatremia (sodium 116 mmol/L). The average RCC diameter was 16 mm (range: 14−20 mm). Imaging typically showed a T1 hyperintense lesion consistent with internal hemorrhage. Patients were taken for elective transsphenoidal fenestration and drainage of the RCC with partial wall resection. Two patients (33.3%) had intraoperative CSF leaks that were repaired, and both showed no postoperative CSF rhinorrhea. At latest follow-up, two of five (40%) patients with headaches had improvement and the two patients with vision loss had improved vision. While three of three women had resolution of amenorrhea, all patients who required hormone supplementation preoperatively continue on their regimens postoperatively. Follow-up MRI showed no instances of RCC recurrence.

Conclusion Rathke's cleft cysts can occasionally present with internal hemorrhage and clinical signs and symptoms mimicking pituitary apoplexy. Outcomes for hemorrhagic RCCs are similar to those patients with standard RCCs. Although hyperprolactinemia tends to improve following surgery, possibly secondary to decompression of the stalk, other pituitary axes typically do not.

Learning Objectives By the conclusion of this session, participants should be able to (1) describe common presenting symptoms of hemorrhagic Rathke's cysts, (2) understand the uniqueness of this type of symptomatic Rathke's complicated by hemorrhage, and (3) understand the safety and success rate of surgically resecting the lesions.