[A retrospective study of hemophilic pseudotumor in maxillofacial region]

Objective: To provide clinical references for the diagnosis and treatment of hemophilic pseudotumor (HPT) in maxillofacial region. Methods: Fourteen cases of HPT in maxillofacial region from the Department of Stomatology, Beijing Children's Hospital from Jan 2009 to Jan 2019 were collected. Two cases were lost for follow-up and 12 patient,all boys, were finally followed up and included in the study. The patients aged from 13 months to 10 years old. The medical history, clinic manefestitions and the features of the radiology examination were recorded. The patients were treated by using replacement treatment first. If the conservative treatment was not effective, the patients then received operation combined with pereoperation replacement thearapy. The patients were followed up for 13 months to 10 years.There were 11 cases of hemophilia A, and 1 case of hemophilia B. Two cases were severe type, the others (10/12) were mild and moderate types. Only 1 case was diagnosed as hemophilia initially. Nine cases (9/12) were misdiagnosed as malignant tumors, 1 case was misdiagnosed as osteomyelitis and 1 case was misdiagnosed as hemangioma. Only 3 cases had identified history of trauma before. Results: All cases were treated with replacement therapy first, among which 10 cases were effective, 8 cases were cured by conservative therapy, 1 case had residual soft tissue fistula after conservative treatment and 1 case recurrented after conservative treatment for 8 months. Two patients with poor efficacy to the replacement treatment were performed operations and finally were cured. Conclusions: The misdiagnosis rate of HPT in maxillofacial region was high. The conservative factor replacement therapy could achieve good results in most children and could be used as the preferred treatment. If the conservative treatment was not effective, the surgical treatment was also a safe option.