Multidisciplinary management of pediatric head and neck lymphatic malformations: a 10-year single-institution experience

儿童头颈部淋巴管畸形的多学科管理:一家机构10年的经验

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Abstract

INTRODUCTION: Pediatric head and neck lymphatic malformations (HNLM) are heterogeneous lesions that often require multimodal care. Contemporary real-world data incorporating evolving interventional techniques and targeted systemic therapy are limited. We describe patient characteristics, imaging utilization, treatment utilization/sequencing, and 90-day treatment-related complications within a multidisciplinary vascular anomalies program. METHODS: Retrospective single-institution chart review of patients <18 years with head and neck lymphatic malformations through the Birthmarks and Vascular Anomalies Program (BVAP) from 2014 to 2024 (n = 59). Demographics, lesion characteristics, imaging, treatment sequencing, and 90-day treatment-related complications were abstracted from the medical record. Analyses were descriptive; procedure-level complication rates were reported with exact 95% confidence intervals (CIs). RESULTS: Morphology was 42.4% microcystic, 37.3% macrocystic, and 20.3% mixed; 39.0% were multiregional. Median lesion diameter was 4.0 cm (IQR 2.1-6.4). MRI was obtained in 93.2% of patients. Sclerotherapy was used in 67.8% (86 sessions), most commonly doxycycline (45.8%) and bleomycin (35.6%). Resection without embolization occurred in 45.8%, laser therapy in 15.3%, and glue embolization surgery in 5.1%. Systemic therapy included sirolimus in 25.4%, with 4 patients transitioning to alpelisib. Eight patients (13.6%) underwent molecular testing; all tested had pathogenic PIK3CA variants. Ten patients (16.9%) experienced ≥1 complication. Procedure-level complications occurred after 5/86 sclerotherapy procedures (5.8%; 95% CI 1.9-13.0), 6/53 resections without embolization (11.3%; 95% CI 4.3-23.0), and 0/3 glue embolization procedures (0.0%; 95% CI 0.0-70.8). CONCLUSION: Pediatric HNLM often required multimodal, staged treatment with high MRI utilization and repeat procedures. Prospective multicenter studies with standardized outcomes and systematic molecular testing are needed to guide lesion-specific care.

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