Pediatric Langerhans Cell Histiocytosis presents with skull involvement in 48.8% of casesNew data clarifies treatment outcomes for children with LCH

Langerhans Cell Histiocytosis (LCH) is a rare clonal disorder that frequently involves the head and neck in pediatric patients, often presenting with nonspecific otolaryngologic symptoms that can delay diagnosis. This systematic review and meta-analysis aimed to characterize the otolaryngologic manifestations of pediatric LCH and evaluate current treatment strategies and clinical outcomes. A comprehensive search of Ovid MEDLINE, Embase, Cochrane CENTRAL, and Web of Science was conducted through June 2025. Studies were included if they involved patients under 18 years of age with head and neck manifestations of LCH and reported treatment modalities and outcomes. Twenty studies comprising 885 pediatric patients met inclusion criteria. The most common head and neck manifestation was skull involvement (48.8%, 95% CI: 38.1%-59.8%), with temporal bone lesions frequently reported. Otologic involvement occurred in 36.8% (95% CI: 21.8%-54.8%) of patients, and otorrhea was the most common presenting symptom (22.8%, 95% CI: 10.6%-42.6%). These findings highlight the potential for LCH to mimic chronic or refractory ear infections. Systemic chemotherapy was the most commonly utilized treatment modality. Overall remission was achieved in 55.2% (95% CI: 36.8%-72.2%), while recurrence occurred in 20% of patients and mortality attributable to LCH was 11.5%. Pediatric LCH frequently presents with nonspecific otologic complaints, particularly persistent otorrhea, warranting heightened clinical suspicion in refractory cases. Although many patients achieve remission, recurrence and mortality remain significant, warranting further investigation into effective treatment protocols.