Rathke’s cleft cysts (RCCs) are benign epithelial lesions of the sellar region typically treated via a transsphenoidal approach with cyst fenestration and drainage. At present, there is limited evidence to guide patient selection for operative treatment. Furthermore, there is minimal literature describing factors contributing to cyst recurrence.
The authors conducted a retrospective analysis of 109 consecutive cases of pathology-confirmed RCCs treated via a transsphenoidal approach at a single center from 1995 to 2016. The majority of cases (86.2%) involved cyst fenestration, drainage, and partial wall resection. Long-term outcomes were analyzed.
A total of 109 surgeries in 100 patients were included, with a mean follow-up duration of 67 months (range 3–220 months). The mean patient age was 44.6 years (range 12–82 years), and 73% were women. The mean maximal cyst diameter was 14.7 mm. Eighty-eight cases (80.7%) were primary operations, and 21 (19.3%) were reoperations. Intraoperative CSF leak repair was performed in 53% of cases and was more common in reoperation cases (71% vs 48%, p < 0.001). There were no new neurological deficits or perioperative deaths. Two patients (1.8%) developed postoperative CSF leaks. Transient diabetes insipidus (DI) developed in 24 cases (22%) and permanent DI developed in 6 (5.5%). Seven cases (6.4%) developed delayed postoperative hyponatremia. Of the 66 patients with preoperative headache, 27 (44.3%) of 61 reported postoperative improvement and 31 (50.8%) reported no change. Of 31 patients with preoperative vision loss, 13 (48.1%) reported subjective improvement and 12 (44.4%) reported unchanged vision. Initial postoperative MRI showed a residual cyst in 25% of cases and no evidence of RCC in 75% of cases. Imaging revealed evidence of RCC recurrence or progression in 29 cases (26.6%), with an average latency of 28.8 months. Of these, only 10 (9.2% of the total 109 cases) were symptomatic and underwent reoperation.
Transsphenoidal fenestration and drainage of RCCs is a safe and effective intervention for symptomatic lesions, with many patients experiencing improvement of headaches and vision. RCCs show an appreciable (although usually asymptomatic) recurrence rate, thereby mandating serial follow-up. Despite this, full RCC excision is typically not recommended due to risk of hypopituitarism, DI, and CSF leaks.
ABBREVIATIONSdDAVP = desmopressin acetate; DI = diabetes insipidus; RCC = Rathke’s cleft cyst; SIADH = syndrome of inappropriate antidiuretic hormone; USC = University of Southern California.
LilleheiKOWiddelLAsteteCAWiermanMEKleinschmidt-DeMastersBKKerrJM: Transsphenoidal resection of 82 Rathke cleft cysts: limited value of alcohol cauterization in reducing recurrence rates. J Neurosurg114:310–3172011
MukherjeeJJIslamNKaltsasGLoweDGCharlesworthMAfsharF: Clinical, radiological and pathological features of patients with Rathke’s cleft cysts: tumors that may recur. J Clin Endocrinol Metab82:2357–23621997
ShinJLAsaSLWoodhouseLJSmythHSEzzatS: Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke’s cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab84:3972–39821999
ZadaGLinNOjerholmERamkissoonSLawsER: Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships. Neurosurg Focus28(4):E42010