Retrospective evaluation of slow-flow vascular malformations

Vascular malformations are localized or generalized (focal or diffuse) congenital vascular lesions that occur as a result of abnormalities in stages of vasculogenesis and angiogenesis in the embryonic period. Correct classification of these lesions is very important in determining the appropriate treatment. Medical therapy, laser, surgical resection, endovascular therapy, or their combinations can be used in the treatment of vascular malformations. The aim of this study is to evaluate the demographic and clinical characteristics and treatment outcomes of patients with low-flow vascular malformation. Materials and Methods 236 patients who applied to the Department of Pediatrics, Division of Pediatric Oncology at Hacettepe University Faculty of Medicine between 01.01.1999-31.06.2020 were included in this study. The records of the patients were reviewed retrospectively. Patient's complaints, clinical examinations, and radiological findings were recorded, and their photographs were evaluated in chronological order. The efficacy of medical treatments, sclerotherapy, and surgical treatments applied to the patients were recorded as radiological/clinical and symptomatic response. Results Of the patients, 120 (50.8%) were girls, 116 (49.2%) were boys (Girl /Boy: 1.03). The mean age of diagnosis was 54.2±55.1 (0-216) months. Capillary malformation was found in 1.7% (n = 4), venous in 24.6% (n = 58), lymphatic in 33.9% (n: 80) and combined type vascular malformation in 39.8% (n = 94) of patients. The mean age at which symptoms were noticed by the family or the onset of complaints was 16.7±36.9 (0-200) months. The mean duration between the onset of symptoms and diagnosis was 36.6±46.3 (0-200) months. Vascular malformation was found in 29 patients as a component of syndromes such as Klippel-Trenaunay (n: 10), Gorham-Stout (n: 9), Blue Rubber Bleb (n: 5). Fifty-four (22.8%) patients were followed up without treatment, of these cases, 2 had capillary malformation 19 had venous malformation, 20 had combined and 13 had lymphatic malformation. Treatments applied to the patients were determined as 69 medical treatment, 32 sclerotherapy, 17 surgical treatment, 27 sclerotherapy and medical treatment, 22 medical treatment, and surgery, 1 sclerotherapy and surgery, 14 combinations of medical treatment, sclerotherapy, and surgery. In medical treatment, sirolimus was used in 120 patients, propranolol in 43 patients and interferon in 11 patients, and more than one drug was used in 48 patients. In 79.6% of the patients who underwent surgery alone or in combination with other treatments, a reduction in the size of the lesion was detected and in 61.1% of these cases improvement in the symptoms were detected. Radiological/clinical response and the symptomatic response of patients who underwent sclerotherapy were 67.5% and 79.7%, respectively. It was observed that 54.2% of the patients who received sirolimus have a decrease in the size of lesion radiologically or clinically and 74.2% of these cases had a clinical improvement in symptoms. Conclusion Medical treatment, surgery, and sclerotherapy could be applied to patients with vascular malformations as safe and effective treatment options with the correct classification and selection of the appropriate technique. Keywords:Vascular malformation, sclerotherapy, surgery, sirolimus