347 Management of Rathke Cleft Cysts in Children: The Rady Children's Hospital Approach. (April 2023)

• Record Type: Journal Article
• Authors: Brandel, Michael G.; Plonsker, Jillian Hannah; Rennert, Robert; Khan, Usman; Nation, Javan; Crawford, John R.; Levy, Michael L.
• Is Part Of: Neurosurgery. Volume 69(2023)Supplement 1
• Page Start: 55
• Page End: 56
• Publication Date: 2023-04
• Subjects: Nervous system -- Surgery -- Periodicals; 617.48005
• DOI: 10.1227/neu.0000000000002375_347
• Languages: English
• ISSNs: 0148-396X

Abstract

Abstract : INTRODUCTION: Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and management of growing or symptomatic RCCs. METHODS: We performed a retrospective study of RCC patients age ≤18 years treated between 2006-2022 at our institution. RESULTS: 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 underwent surgery and had a histopathological diagnosis of RCC, 58% female and 42% male. Mean age was 13.2 +/- 4.2 years. Presenting symptoms included headache (58%), visual changes (35%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and grew significantly on serial imaging. Six percent presented with intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients after surgery. New pituitary axis deficits were seen in 9.7%. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging. Mean follow-up was 62.9 +/- 58.4 months. Recurrence requiring reoperation occurred in 32% of patients (transsphenoidal n = 8, pterional n = 2). Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up. CONCLUSIONS: Due to their secretory epithelium, RCCs may demonstrate rapid growth and recurrence. Incidental RCCs can be managed conservatively, whereas symptomatic or growing lesions may be fenestrated or resected. Due to their dynamic and variable nature, careful patient selection and close postoperative surveillance are critical to optimizing patient outcomes.