Comprehensive Therapy for Hemangioma Presenting With Kasabach-Merritt Syndrome in the Maxillofacial Region

• Published in: Journal of oral and maxillofacial surgery Vol. 73; no. 1; pp. 92 - 98
• Main Authors: Su, Lixin, DDS, PhD, Wang, Deming, MD, Fan, Xindong, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 2015
• Subjects: Blood Coagulation Tests; Combined Modality Therapy; Dentistry; Embolization, Therapeutic - methods; Female; Follow-Up Studies; Glucocorticoids - therapeutic use; Head and Neck Neoplasms - drug therapy; Head and Neck Neoplasms - pathology; Head and Neck Neoplasms - therapy; Hemangioma - drug therapy; Hemangioma - pathology; Hemangioma - therapy; Humans; Infant; Infant, Newborn; Kasabach-Merritt Syndrome - drug therapy; Kasabach-Merritt Syndrome - pathology; Kasabach-Merritt Syndrome - therapy; Male; Methylprednisolone - therapeutic use; Platelet Count; Polyvinyl Alcohol - therapeutic use; Propranolol - therapeutic use; Retrospective Studies; Skin Neoplasms - drug therapy; Skin Neoplasms - pathology; Skin Neoplasms - therapy; Skin Pigmentation - drug effects; Surgery; Treatment Outcome; Vasodilator Agents - therapeutic use
• ISSN: 0278-2391; 1531-5053
• DOI: 10.1016/j.joms.2014.07.037

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.