Hong-Zhao Lei1, Bin Sun2, Yu-Chun Ma2, Miao-Miao Li2, Li-Fu Wang3, Shi-Wen Jiang4, Ran Huo5, Chang-Xian Dong6. 1. Department of Aesthetic, Plastic, and Burn Surgery, Shandong Provincial Hospital, Shandong University, 324, Jingwu Road, Jinan, Shandong 250021, China; Department of Hemangioma and Vascular Malformation Surgery, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, 7 Weiwu Road, Zhengzhou 450003, China. 2. Department of Hemangioma and Vascular Malformation Surgery, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, 7 Weiwu Road, Zhengzhou 450003, China. 3. Departments of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, 7 Weiwu Road, Zhengzhou 450003, China. 4. Center for Reproductive Medicine, Wuxi Maternity and Child Health Care Hospital Affiliated to Nanjing Medical University, Wuxi 214002, China. 5. Department of Aesthetic, Plastic, and Burn Surgery, Shandong Provincial Hospital, Shandong University, 324, Jingwu Road, Jinan, Shandong 250021, China. Electronic address: huoran@medmail.com.cn. 6. Department of Hemangioma and Vascular Malformation Surgery, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, 7 Weiwu Road, Zhengzhou 450003, China. Electronic address: xueguanliu@126.com.
Abstract
OBJECTIVE: To analyze the clinical characteristics and treatment of pediatric tufted angiomas(TA)complicated by Kasabach-Merritt Phenomenon (KMP). METHOD: A retrospective analysis was conducted on the clinical data and follow-up data of 13 patients diagnosed with TA complicated by KMP. Five male and 8 female patients with an average age of 5.7 months (range, 29 days to 1 year) were treated with surgery between January 2009 and June 2012. According to the size and location of lesions and the degree of thrombocytopenia, complete or subtotal resection was performed. The median follow-up period was 3.4 years (range, 1.7 years to 5.2 years). Therapeutic outcomes were evaluated by platelet count and lesion size. RESULTS: Curative treatment of KMP is defined as restoration of normal hemostasis and elimination of tumor cells. Twelve patients achieved curative treatment and one died of multiple organ failure after operation. Ten patients received complete resection and three patients received incomplete resection. Thrombocyte count, hemoglobin and blood coagulation were respectively restored to normal levels within 1-3 days and 1-2 weeks post complete resection operation. One of the three patients who received subtotal resection operation died. In the other two patients, the platelet count fluctuated over time but remained above 60 × 109 /L, a significantly higher level than the preoperational level. Residual lesions slowly disappeared after continuous medication 3-6 months post operation. CONCLUSION: Early surgical treatment of patients with TA complicated with KMP resulted in significantly higher curative rate and reduced side-effects of drugs.
OBJECTIVE: To analyze the clinical characteristics and treatment of pediatric tufted angiomas(TA)complicated by Kasabach-Merritt Phenomenon (KMP). METHOD: A retrospective analysis was conducted on the clinical data and follow-up data of 13 patients diagnosed with TA complicated by KMP. Five male and 8 female patients with an average age of 5.7 months (range, 29 days to 1 year) were treated with surgery between January 2009 and June 2012. According to the size and location of lesions and the degree of thrombocytopenia, complete or subtotal resection was performed. The median follow-up period was 3.4 years (range, 1.7 years to 5.2 years). Therapeutic outcomes were evaluated by platelet count and lesion size. RESULTS: Curative treatment of KMP is defined as restoration of normal hemostasis and elimination of tumor cells. Twelve patients achieved curative treatment and one died of multiple organ failure after operation. Ten patients received complete resection and three patients received incomplete resection. Thrombocyte count, hemoglobin and blood coagulation were respectively restored to normal levels within 1-3 days and 1-2 weeks post complete resection operation. One of the three patients who received subtotal resection operation died. In the other two patients, the platelet count fluctuated over time but remained above 60 × 109 /L, a significantly higher level than the preoperational level. Residual lesions slowly disappeared after continuous medication 3-6 months post operation. CONCLUSION: Early surgical treatment of patients with TA complicated with KMP resulted in significantly higher curative rate and reduced side-effects of drugs.