Treatment of lymphatic malformations: How, to whom, and when should be made?


Akçali Y. F. , Atabey R.

15th International Congress of Update in Cardiology and Cardiovascular Surgery, Antalya, Türkiye, 27 - 30 Mart 2019

  • Basıldığı Şehir: Antalya
  • Basıldığı Ülke: Türkiye

Özet

OBJECTIVE. Lymphatic malformations (LMs), result from abnormal development of the lymphatic channels, are one of the most common forms of congenital vascular malformations (CVMs) when you should include both truncular (T-LMs) and extratruncular (ET-LMs) lesions according to the new Hamburg classification (Table I). Both are consequences of a developmental arrest at the different stages of embryonal life as a result of defective genes. The aim of this study was to evaluate the current treatment results of LMs.

METHODS. A retrospective review of the clinical data of 186 patients (female/male=96/90) with a median age of 9 years (range, 2 days to 80 years) who had been treated with the diagnosis of LM in the last decade was performed. Ultrasound, lymphoscintigraphy, and MRI were frequently used diagnostic tests. The patients with ET-LM were treated with sclerotherapy and/or surgical excision. Combinations of complex decongestive physiotherapy (CDP) and/or compressotherapy were used to treat all the T-LM patients (Figure 1). The results of LM management were evaluated twice a year.

RESULTS. Among total 1312 patients with CVM, 186 were confirmed to have LMs, either as the T-LM (n=114, 64.2%) or the ET-LM (n=72, 35.8%) (Table II). Eleven patients had both truncal and ET forms (Figure 2). Another 38 (20.3%) LMs were diagnosed with hemolymphatic malformations (HLM) (Figure 3). The T-LMs were located to the extremities, mostly to the lower extremity (86.8%), and they included lymphatic aplasia and/or obstruction (76.3%). Most of the ET-LMs were the cystic type (91.6%). The ET-LMs were most frequent in the extremities and axillary- cervicofacial region (75.6%). ET-LMs were treated with sclerotherapy (~37%) and/or surgical excision (30.1%)(Figure 4). The ET-LMs in patients undergoing surgery located to extremity (72.7%), trunk (22.7%), axillary fossa (13.6%), buttocks (4.5%), and retroperitoneum (4.5%)(Video 1). Transcutaneous radiofrequency ablation (TRFA) was performed in three patients. A patient with HLM was followed by aspiration and compression. The clinical response of the T-LMs at the extremity to CDP was good (Figure 5).

CONCLUSION. Multimodal treatment of LMs continues to develop as new knowledge about the molecular biology and genetics of these complicated lesions is discovered, along with data obtained from clinical practice. A patient-centered multidisciplinary medical and surgical team approach should guide modality and timing of treatment, which includes surgery, sclerotherapy with various scleroagents, TRFA, CDP, drug therapies such sirolimus, and observation. The main objective of all these treatments is to support and optimize LM patients' quality of life.