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PMID: 41830665 已发表 · ppublish 英语

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

Singh S, Chun RH, Carlberg VM, Schloemer NJ, Adams J, Friedland DR, Puccia R

摘要

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. 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). 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). 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.

关键词
Alpelisib Head and neck lymphatic malformations Multidisciplinary care PIK3CA Pediatric vascular anomalies Sclerotherapy n-butyl cyanoacrylate embolization
文献信息
期刊
International journal of pediatric otorhinolaryngology
期刊简称
Int J Pediatr Otorhinolaryngol
ISSN
1872-8464
发表日期
2026-05-00
语言
英语
国家/地区
Ireland
NLM ID
8003603
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