Clinical course of pediatric large vascular anomalies located in the extremities

Özet

Objective: Difficulties encountered in the diagnosis and treatment of vascular anomalies located in the extremities of the children. The most common vascular lesions are hemangiomas andvenous malformations. The complex malformations, such as, Klippel-Trenaunay Syndrome aremuch less commonly encountered lesions. Treatment of vascular malformations are variablebased on the etiology of the lesion and clinical presentation. In this study, we aimed to share ourexperience on the clinical features of vascular lesions in the extremities of the children.Material and Methods: The demographic, clinical and prognostic features of 330 children with vascular anomalies followed at IUC, Cerrahpasa Medical Faculty, Department of Pediatric Hematologyand Oncology were retrospectively reviewed. Fifty-one patients with lesions >5 cm in diameter wereincluded into the study. The diagnosis, age, sex, history of prematurity, lesion type and location, imaging and biopsy findings, complications, details of treatment, and follow-up were evaluated.Results: Twenty-nine (57%) of patients were female and 22 (43%) were male. The female tomale ratio was 1.3:1. The median age at admission was 15 months (10 days-180 months). Eightpatients (16%) had a history of premature birth. Thirty-one patients (61%) had lesions sincebirth, eight lesions (8%) appeared in the first month of life and 6 (12%) occurred after 1 year ofage. Sixteen of the patients (31%) had hemangioma, 11 (22%) had lymphangioma, 19 (37%) hadvenous malformation and 5 (10%) were diagnosed as Klippel Trenaunay Syndrome. The lesionswere in the upper extremity in 21 patients (41%), in the lower extremity in 27 patients (53%), andboth lower and upper extremities were affected in 3 patients (6%). Of all patients, six had intramuscular and two had intraarticular lesions. The diagnosis was made on clinical grounds inmost of the cases. In 22 children Magnetic Resonance Imaging was performed for differentialdiagnosis and to demonstrate the infrastructure of the lesion and the extent of local infiltration.Histopathologic examination by biopsy was done in four patients. Complications developed in19 patients as follows: Disseminated intravascular coagulation in 6, bleeding in 4, thrombosisin 3, and soft tissue infection in 6. Twenty-one patients were not given any treatment. Medicaltreatments were propranolol in 14 patients, sirolimus in 4 patients, propranolol and sirolimus in5 patients. Intralesional bleomycin injection was performed in 3 children.Conclusion: The diagnosis, classification and treatment of extremity located vascular malformations in children are complex. Treatment strategy should be defined as in accordance with a combination of the type of the vascular malformation, the age of the patient and the clinical picture.