Comprehensive therapy for hemangioma presenting with Kasabach-Merritt syndrome in the maxillofacial region.

PURPOSE: To summarize the characteristics of Kasabach-Merritt syndrome (KMS) and to evaluate the therapeutic effect of drug therapy combined with transarterial embolization.

PATIENTS AND

METHODS

From 2007 to 2011, 6 cases of KMS that underwent drug therapy and transarterial embolization were retrospectively analyzed, comprising of 3 male and 3 female patients; the ages of the patients ranged from 3 to 40 days. The lesions were located in the temporal region (1 of 6, 16.7%), parotid region (2 of 6, 33.3%), or submandibular region and neck (3 of 6, 50%). All the patients were followed for 12 to 18 months. Therapeutic outcomes were assessed by evaluating the platelet count, coagulation parameters, and size of the lesion.

RESULTS

Positive responses were visible shrinkage of the hemangioma or lightening of the skin color of the cutaneous tumor (or both) within 8 to 72 hours in 4 patients (66.67%). These occurred within 1 week in 5 patients (83.33%) and within 2 weeks in all patients (100%). The mean platelet count before treatment was 18,000/L (range, 8,000 to 33,000/L). After the first week of medical treatment, the mean platelet count increased to above 80,000/L in 5 patients (83.33%); it began to increase on the second day after embolization and reached 102,000/L in 1 patient (16.67%). All 6 cases (100%) showed good results with treatment. A 12- to 18-month follow-up evaluation was obtained for all treated patients, and no rebound growth or platelet count decreases were observed. No severe or obvious adverse complications were noted during all treatment courses.

CONCLUSIONS

For most hemangiomas presenting with KMS, good results can be obtained with systemic medical treatment. Transcatheter arterial embolization with polyvinyl alcohol particles combined with systemic medical treatment should be considered an efficacious and important therapeutic option for challenging cases.