Management of pediatric posterior fossa dermoid cysts: a systematic review and illustrative case report

Background Dermoid cysts of the posterior fossa are less common congenital lesions that arise from ectodermal inclusions during neural tube closure. Their deep location in certain instances, as well as their slow growth, often delays diagnosis until symptoms from mass effect or rupture emerge. However, some posterior fossa dermoid cysts associated with dermal sinus tracts may present earlier, typically with recurrent infections. Materials and methods We describe a child with a surgically managed posterior fossa dermoid cyst and present a systematic review of PubMed, ScienceDirect, and Scopus. Pediatric case reports and series were analyzed for demographics, presentation, imaging, surgical strategies, and outcomes. Results Fifty-five studies (96 pediatric cases) met inclusion criteria. Of 86 patients with sex data, 54 (63%) were female. Common symptoms were hydrocephalus (49.6%, n = 34), headache (44.2%, n = 35), vomiting (38.2%, n = 34), and ataxia (24.5%, n = 22). CT (74.1%, n = 61) and MRI (65.2%, n = 47) were the main diagnostic tools, while earlier reports used radiographs (17.8%, n = 21). Suboccipital craniotomy was performed in 58 patients (84.7%). Gross total resection was achieved in 73.9% ( n = 55), subtotal in 24.1% ( n = 13). Cyst rupture occurred in 7 patients (10.9%). We report a 33-month-old female with vomiting, progressive ataxia, and dysphagia. MRI showed a midline posterior fossa lesion compressing the fourth ventricle and causing hydrocephalus. Gross total resection was achieved via suboccipital craniotomy, with uneventful recovery and symptom resolution at 3 months. Conclusion Pediatric posterior fossa dermoid cysts, while less common, should be considered in the differential diagnosis of posterior fossa masses. Early recognition through characteristic imaging and meticulous surgical planning is vital for optimal outcomes. This review summarizes current evidence on the clinical features, imaging findings, surgical management and outcomes of pediatric posterior fossa dermoid cysts.