Surgical Management of Recurrent Rathke's Cleft Cysts

Objective Rathke's cleft cysts (RCCs) are benign epithelial lesions of the sellar region, frequently treated with cyst fenestration and drainage, via a transsphenoidal craniotomy (TSC) approach. While these lesions are benign, they have an appreciable recurrence rate. At present, the literature contains limited data regarding factors contributing to recurrence in patients treated for recurrent cysts.

Methods We conducted a retrospective analysis of pathology-confirmed RCCs from 1995 to 2016 in our institutional database. TSC for cyst fenestration and drainage was our first-line therapy for symptomatic patients with open craniotomy or radiation as a salvage therapy.

Results We identified 112 surgeries for RCC in 101 patients. The mean age of patients presenting for reoperation was significantly higher than for patients presenting for their first operation (42.4 ± 17 years vs. 58.6 ± 17 years, p < 0.001). Ninety-one surgeries (80.5%) were primary operations and 21 surgeries in 18 patients (19.5%) were reoperations. Of the 91 patients who underwent their first operation at our institution, eight (8.7%) required reoperation for recurrence. Intraoperative cerebrospinal fluid (CSF) leak repair was performed in 53% of cases and was more common in reoperation cases (71 vs. 48%, p < 0.04). There were no new neurological deficits or perioperative deaths; however, one patient developed an internal carotid artery pseudoaneurysm requiring endovascular treatment. Two patients (9.5%) in the reoperation group developed a postoperative CSF leak, one of which resolved with lumbar drain and one of which required a reoperation. Of the 16 reoperation patients for whom data were available, 11/16 (68.6%) were on pituitary hormone supplementation prior to their reoperations, and 6/16 (37.5%) had diabetes insipidus (DI) requiring desmopressin acetate (DDAVP) at home. Of the 10 reoperation patients without DI prior to revision, the rates of transient hyponatremia (3/10 vs. 4/91, p < 0.04) and transient DI (5/10 vs. 17/91, p = 0.04) were significantly higher in the reoperation group. The rate of new permanent DI was not higher for reoperation patients (2/10 vs. 4/91, p < 0.001). At the last clinical follow-up, 15% of patients reported worsened headaches, 52% reported unchanged headaches, and 33% reported improvement. Of the patients with visual deficits prior to surgery, 61.5% of patients reported improved vision, 30.8% of patients reported stable vision, and 7.7% of patients reported worsening of vision. RCC recurrence developed in 7/45 patients (15.6%) <40 years of age, compared with 22/64 patients (34.4%) ≥40 years of age (p < 0.04). Recurrence was observed in 4/10 RCCs (40%) with squamous metaplasia compared with 25/99 (25.2%) patients without squamous metaplasia (p = 0.45). Recurrence was observed in 4/12 RCCs (33.3%) with inflammation which was not statistically different from the rates of recurrence in patients without inflammation 25/97 (25.7%, p = 0.73).

Conclusion Reoperation for recurrent RCC is a safe and effective intervention for patients with recurrent visual symptoms or headaches. Many patients present with hypopituitarism, thus care should be taken to ensure adequate hormonal supplementation in the perioperative period. Additionally, patients should be counseled that hormonal recovery is unlikely and additional hormonal supplementation may be required following reoperation. RCCs show an appreciable recurrence rate and require fastidious long-term follow-up.