Suzuki Kento, Fumino Shigehisa, Iguchi Masafumi, Takayama Shohei, Kim Kiyokazu, Hirano Shigeru, Ono Shigeru
Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan.
Department of Otolaryngology-Head and Neck Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Pediatr Surg Int. 2024 Dec 19;41(1):35. doi: 10.1007/s00383-024-05947-y.
The study reviewed a multidisciplinary approach to treating cervicofacial lymphatic malformations (CFLMs) in children.
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.
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.
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.
本研究回顾了一种多学科方法治疗儿童颈面部淋巴管畸形（CFLM）。
2007年至2023年期间，我院对53例CFLM患儿进行了治疗，中位发病年龄为5个月（0 - 165个月）。对于婴儿，优先进行气道管理，包括可能的气管切开术，并采取“观察等待”策略以期自发消退。一旦儿童满一岁或在婴儿期后被诊断，对于残留病变考虑采用OK - 432硬化治疗以及联合或不联合西罗莫司的手术治疗。
中位随访期为38个月（0 - 169个月）。在30例婴儿中，4例患者进行了气管切开术，3例治疗后成功闭合。13例患者未经治疗显示出极佳的改善。15例患者接受了硬化治疗，5例进行了部分切除术。6例患者接受西罗莫司治疗，显示出中度缩小或出血停止。总体而言，30例婴儿中有23例显示出中度至极佳的改善。对于23例婴儿期后诊断的患者，16例未经治疗得到改善，7例通过硬化治疗显示出中度至极佳的改善。
该研究得出结论，婴儿早期硬化治疗，尤其是气道周围的硬化治疗存在风险，可能需要进行气管切开术。包括“观察等待”、硬化治疗、西罗莫司治疗和及时手术在内的综合策略显著改善了患者的生活质量。
