Features of the managing boys with congenital lymphatic malformations of the urogenital region
DOI:
https://doi.org/10.15574/PS.2024.4(85).5964Keywords:
lymphatic malformation, boys, sclerotherapy, surgical treatment, urogenital regionAbstract
Lymphatic malformations (LM) of the urogenital region are rare but complex conditions causing cosmetic defects, hygiene difficulties, recurrent infections, and lymphorrhea, leading to significant patient distress.
Aim - to assess the clinical course, complications, and treatment outcomes of LM in the urogenital region in boys to optimize patient management.
Materials and methods. A retrospective analysis was conducted on the medical records of boys with LM of the urogenital region treated in inpatient settings between 2014 and 2023. The analysis covered the age of onset, clinical manifestations, anatomical localization, type of malformation, treatment methods, surgical interventions, and their outcomes.
Results. Among 259 patients, LM of the urogenital region was diagnosed in 17 (6.6%). Symptoms were congenital in 3 (17.6%) patients, appeared between 1-10 years in 7 (41.2%), and during adolescence in 7 (41.2%). LM types: lymphedema in 10 (58.8%) patients, with 7 (41.2%) also having lower limb lymphedema; cystic LM in 4 (23.5%) patients, including 2 (11.8%) cases with involvement of multiple anatomical regions; and central lymphatic channel anomalies in 2 (11.8%) cases. Conservative management of lower limb lymphedema was performed in 8 (47.1%) patients. Sclerotherapy for cystic LM of the perineal region had excellent (n=2) and good (n=2) outcomes. Surgical resection was performed in 9 (52.9%) patients (penile surgery in 2, scrotal surgery in 3, combined surgery in 4), with an age range of 11-17 (15±1.55) years. mTOR inhibitor therapy was given to 2 patients, yielding temporary improvement.
Conclusions. Main clinical manifestations included swelling 11 (64.7%), anatomical deformity 9 (52.9%), pain 2 (11.8%), lymphorrhea 2 (11.8%), and protein-losing enteropathy with hypoproteinemia 2 (11.8%). Cystic LM was treated with sclerotherapy, while penile and scrotal lymphedema required surgical resection, often combined with sclerotherapy or mTOR inhibitors.
The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the local ethics committee of the institutions mentioned in the work. Parents' informed consent was obtained for children's participation in the study.
The authors declare no conflict of interest.
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