Rathke's cleft cysts (RCCs) are benign, cystic lesions that account for less than 5% of cases in the pediatric population. While asymptomatic RCCs often require only conservative management, symptomatic cases may necessitate surgical intervention. Advances in surgical techniques have improved the safety of these procedures. This comprehensive review and single-arm meta-analysis evaluates the outcomes of surgical management in pediatric patients. PubMed, Embase, and Web of Science were systematically searched for studies documenting RCC resection in pediatric patients (< 18 years). Outcomes of interest included symptomatic improvement (headache, visual impairment, endocrinopathy), postoperative complications, postoperative diabetes insipidus (DI), recurrence, and reoperation rate. Heterogeneity was assessed using I2 statistics, and a random-effects model was adopted. Twelve studies were included in the final analysis. The pooled proportion of headache improvement was 84% (95% CI: 73%-91%), with 65% of patients achieving complete resolution. Improvement of visual symptoms occurred in 87% of cases (95% CI: 63%-96%). The rate of improvement of endocrinopathy after surgery had an overall pooled rate of 48% (95% CI: 29%-69%). The rate of postoperative complications was 8% (95% CI: 4%-13%), and the incidence of new postoperative DI was 28% (95% CI: 18%-40%). The pooled incidence of cyst recurrence was 16% (95% CI: 11%-23%), while the incidence of reoperation was 14% (95% CI: 8%-24%).Surgery for RCC in pediatric patients offers high rates of symptomatic improvement for headaches and visual impairments, though improvement for endocrinopathy tends to be moderate. The procedure generally has a low risk of postoperative complications, but the incidence of new-onset DI is notable. While many patients experienced complete headache resolution, surgery should not be solely indicated for headache management. Surgical decisions must prioritize objective clinical factors, such as cyst characteristics, neurological impact, and endocrine dysfunction. Additionally, recurrence and reoperation rates are observed, highlighting the need for careful consideration of potential risks and individualized treatment planning. Future research should focus on refining surgical techniques and assessing long-term outcomes to optimize treatment strategies for pediatric RCC patients.
Keywords: Diabetes insipidus; Endocrinopathy; Pediatric; Rathke’s cleft cyst; Sellar; Surgery.
© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.