Pediatric Low-Flow Vascular Malformations: Treatment Patterns and Outcomes.
Аннотация
Low-flow vascular malformations (LFVMs) are congenital vascular anomalies with heterogeneous clinical behavior and variable treatment response. This retrospective study described the clinical characteristics, treatment patterns, and modality-specific outcomes of children aged 0-18 years with LFVM evaluated at Hacettepe University between January 1999 and June 2020. Lesions were classified as capillary, venous, lymphatic, or combined LFVMs, and demographic, clinical, imaging, treatment, follow-up, and adverse event data were reviewed. Radiologic/clinical and symptomatic responses were assessed for sirolimus, image-guided sclerotherapy, and surgery. Among the patients, lesions were combined in 94 (40.5%), lymphatic in 80 (34.5%), and venous in 58 (25.0%). The mean age at diagnosis was 54.6 ± 55.2 months, the mean diagnostic delay was 37.6 ± 48.1 months, and the head and neck was the most frequent primary location (44.4%). Mean follow-up was 50.1 ± 42.3 months. Sirolimus was administered to 118 patients and achieved radiologic/clinical response in 55.1% and symptomatic improvement in 75.4%, with the highest symptomatic improvement observed in lymphatic malformations (84.2%). After sirolimus discontinuation, 19 patients experienced radiologic/clinical progression or symptom worsening. Sclerotherapy (n = 74) achieved radiologic/clinical and symptomatic response rates of 67.6% and 79.7%, respectively, whereas surgery (n = 54) achieved rates of 79.6% and 61.1%, respectively. Progressive disease at the last follow-up was rare (1.3%). Overall, multimodal therapy provided substantial disease control in this long-term pediatric LFVM cohort. Sirolimus primarily improved symptoms, particularly in lymphatic and combined lesions, while sclerotherapy and surgery were effective when selected according to lesion phenotype and treatment goals.
Предметные рубрики MeSH
Подготовка и перевод публикации
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
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