Medicinski Glasnik (Aug 2007)
Diagnostic problems and surgical treatment of histiocytosis X
Abstract
A 16-year-old boy, with swelling and pain in the left submandibular region was treated at the Clinic for Maxillofacial Surgery. The x-ray examination showed destructive bone lesion of the mandibulae left side and oval lesions on the left hand bone and ribs. A biopsy showed characteristics of the Hand-Schueller-Christian disease. A surgical treatment consisted of a complete excision of the mandible lesion and reconstruction defect using a rib autologous transplant. After 3 years, osteolysis and extensive process of resorption of the autologous rib bone transplant occurred. Chemotherapy and radiotherapy stopped spreading of the disease for 4 years. However, progression with involvement of the cervical spine with limited motions, pain, and lesions of C5 and C7 appeared. An autologous bone transplant in the reconstruction of the postresection defects in this disease is not recommended.