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Published on: May 1, 2015
Multidisciplinary therapeutic strategy with appropriate timing and modalities for treating cervicofacial lymphatic
Kento Suzuki1, Shigehisa Fumino2, Masafumi Iguchi1
1Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan.
Insights
A multidisciplinary approach effectively treats pediatric cervicofacial lymphatic malformations (CFLMs). Strategies include watchful waiting, sclerotherapy, sirolimus, and surgery, significantly improving children's quality of life.
Area of Science:
- Pediatric Surgery
- Vascular Anomalies
- Lymphatic Malformations
Background:
- Cervicofacial lymphatic malformations (CFLMs) present unique challenges in pediatric treatment.
- A multidisciplinary approach is crucial for managing these complex vascular anomalies.
Purpose of the Study:
- To review a multidisciplinary treatment strategy for pediatric CFLMs.
- To evaluate the efficacy of various interventions including observation, sclerotherapy, sirolimus, and surgery.
Main Methods:
- Retrospective review of 53 children with CFLMs treated between 2007 and 2023.
- Prioritized airway management and "wait-and-see" policy for infants.
- Utilized OK-432 sclerotherapy, surgical resection, and sirolimus for residual or older-child lesions.
Main Results:
- Overall, 23 of 30 infants achieved moderate to excellent improvement.
- 16 of 23 older children improved spontaneously, with 7 showing good response to sclerotherapy.
- Sirolimus treatment led to moderate shrinkage or bleeding cessation in 6 patients.
Conclusions:
- Early sclerotherapy in infants, especially near the airway, carries risks; tracheostomy may be required.
- A comprehensive strategy combining "wait-and-see," sclerotherapy, sirolimus, and surgery improves pediatric CFLM outcomes.
- This integrated approach significantly enhances patients' quality of life.
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.
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