Multidisciplinary therapeutic strategy with appropriate timing and modalities for treating cervicofacial lymphatic malformations in children
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In brief
In brief
This study describes a multidisciplinary treatment approach for cervicofacial lymphatic malformations in 53 pediatric patients, emphasizing airway management and watchful waiting in infants, followed by OK-432 sclerotherapy, sirolimus therapy, and selective surgery for persistent lesions. The strategy achieved moderate to excellent outcomes in most patients while minimizing early intervention risks.
- Infants with cervicofacial lymphatic malformations benefit from airway-first management with wait-and-see approach for spontaneous regression
- OK-432 sclerotherapy after age 1 year combined with selective surgery achieves moderate-to-excellent outcomes in majority of pediatric cases
- Sirolimus demonstrates efficacy for residual lesions showing moderate shrinkage and bleeding control in refractory cases
- Early sclerotherapy in infants carries significant airway risks; tracheostomy may be required but can often be reversed after treatment
- Multidisciplinary timing-based strategy (observation, sclerotherapy, sirolimus, surgery) significantly improves quality of life in pediatric CFLMs
Written by the GCMD Library team from the article.
Abstract
Purpose
The study reviewed a multidisciplinary approach to treating cervicofacial lymphatic malformations (CFLMs) in children.
Methods
Between 2007 and 2023, 53 children with CFLMs were treated with the median on-set age of 5 months (0–165) at our institute. For infants, airway management, including possible tracheotomy was prioritized, and a “wait-and-see” policy was adopted to expect spontaneous regression. Once children reached one year of age or diagnosed after infancy, OK-432 sclerotherapy and surgical treatment with/without sirolimus were considered for residual lesions.
Results
The median follow-up period was 38 months (0–169). Among 30 infants, tracheostomy was performed in 4 patients, with 3 successfully closed after treatment. Thirteen patients showed excellent improvement without treatment. Sclerotherapy was performed in 15 patients, and partial resection in 5. Six patients were treated with sirolimus and showed moderate shrinkage or cessation of bleeding. Overall, 23 of 30 infants showed moderate to excellent improvement. For the 23 patients diagnosed after infancy, 16 improved without treatment, and 7 showed moderate to excellent improvement with sclerotherapy.
Conclusions
The study concluded that early sclerotherapy for infants, particularly around the airway, poses risks, and tracheostomy might be necessary. The comprehensive strategy including "wait-and-see," sclerotherapy, sirolimus treatment, and timely surgery significantly improved the patients' quality of life.