Management of macrocystic lymphatic malformation at uncommon site with aqueous bleomycin sclerotherapy

Introduction: Lymphangioma are rare vascular malformation that results from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. Atypical site of cystic hygroma in pediatric age group are usually difficult to diagnose clinically but can be diagnosed easily by ultrasound. The aim of the study was to evaluate the result of the intralesional bleomycin for macrocystic lymphatic malformation (LM) presenting at atypical site Material and Method: All patients of LM of other than head& neck, axilla and abdomen presenting in pediatric age group were included in the study. Mainstay of diagnosis was ultrasound and was supplemented by CT scan wherever required. All patients were managed with intralesional bleomycin (ILB) and surgical excision was done only if primary therapy failed. Result: Total 15 cases of LM presenting at atypical sites were included in the study. Series include two case of cystic hygroma of breast, 4 cases of cystic hygroma of anterior chest wall, two case of substernal LM, three cases of LM of parotid gland, one case of inguinal region cystic hygroma and 4 cases involving submandicular area. Complete resolution was observed in 13 out of 15 cases, and two cases had less than 50% reduction in size and were managed with surgical excision after second session of ILB. Conclusion: Aqueous Intralesional bleomycin is a cost effective alternative to surgery even at rare sites of LM which provide better aesthetic outcome, and avoids complication associated with surgery.