Multidisciplinary therapeutic strategy with appropriate timing and modalities for treating cervicofacial lymphatic malformations in children

• Published in: Pediatric surgery international 2024-12, Vol.41 (1), p.35
• Main Authors: Suzuki, Kento, Fumino, Shigehisa, Iguchi, Masafumi, Takayama, Shohei, Kim, Kiyokazu, Hirano, Shigeru, Ono, Shigeru
• Format: Article
• Language: English
• Subjects: Adolescent; Child; Child, Preschool; Combined Modality Therapy; Face - abnormalities; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Lymphatic Abnormalities - therapy; Male; Medicine; Medicine & Public Health; Neck - abnormalities; Original Article; Ostomy; Pediatric Surgery; Pediatrics; Picibanil - therapeutic use; Retrospective Studies; Sclerotherapy; Sclerotherapy - methods; Sirolimus - therapeutic use; Surgery; Tracheostomy; Tracheotomy; Treatment Outcome
• ISSN: 0179-0358; 1437-9813; 1437-9813
• DOI: 10.1007/s00383-024-05947-y

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.