Paget's disease of the mandible — a review and report of a case
5
Casereport Paget's disease of the mandibie - a review and report of a case , i Barnett F, Elfenbein L. Paget's disease of the mandible - a review and report of a case. Endod Dent Traumatol 1985; 1: 39-42. Abstract - Paget's disease is a chronic progressive disease of bone of unknown etiology. Jaw involvement is seen in approximately 1 7 % of cases, and usually it is the maxilla that is involved. This is a report of a case of Paget's disease involving the mandible. An apparently uncommon observation was severe resorption of the roots of t h e 2nd molar tooth. Clinically the tooth appeared ankylo- sed. After surgical removal, the tooth was fixed, demineralized and paraffin sections were made. The microscopic evaluation re- vealed that the root structures had been leplaced by osseous tissue by a mechanism of replacement resorption. Frederic Barnett and Leslie Elfenbein Department of Endodontics, University ot Pennsyl- vania, School ot Dental Medicine, Philadelphia, Pa, USA Key words: Paget's disease, mandibular involve- ment, root resorption. Frederic Barnett, D.M.D., University ot Pennsylvania School ot Dental Medicine, Department ot Endo- dontics, 4001 Spruce St, Philadelphia, PA 19104, USA. Accepted tor publication 24 September 1984. Paget's disease, or osteitis defor?nans, is a chronic pro- gressive disease of bone of unknown etiology. It has been reported to be a familial disease transmitted as an autosomal dominant trait, seldom becoming evident before 40 years of age (1). However, a spe- cific genetic transmission has not yet been revealed (2). The prevalence of this disease has been reported to be between 0.01% and 3% in the general popu- lation after the 4th decade, and in about 10% of patients over the age of 70, provided the frequency is determined by systematic pathological examin- ation of the skeleton (3). With the use of skeletal scintimaging techniques and measurements of plas- ma alkaline phosphatase, the occurrence of asymptomatic lesions is even greater (4). Pagct first described this disorder in 1877 as a chronic, noninfectious inflammatory disease that affects bone (5). There is some evidence, however, that circulatory disturbances are the cause of the disease (6). It is well recognized that the bone in osteitis deformans is excessively vascular, and it has been suggested that the vessels are similar to ar- teriovenous aneurysms (6). Also, there is a sizeable group of other vascular alterations that occur con- comitanlly with Paget's disease, such as increased cardiac output, cardiac enlargement, and arterio- sclerosis (7). Hyperperfusion of diseased bone, rather than arteriovenous shunts associated with Paget's disease, was shown by Rhodes et al. (8), while Gafe et. a l . (9) postulated a metabolic disturb- ance as the etiology of the disorder. It has also been considered to be an inborn error of connective tissue metabolism, a hormone disorder, or an autoimmune state (10). Recently a viral etiology was proposed, and osteoclastic intranuclear inclusions resembling the nucleocapsids of viruses have been demonstrated (11). The primary early feature of this disease is the increase in bone resorption and replacement with poorly mineralized, less dense, fibrotic bone with disorganized trabecular patterns (1, 12). The dis- ease process overcompensates with bulk for the re- placed bone, and most commonly affects the weight- bearing bones, especially the sacrum, spine, femur, and tibia (13). Stafne & Austin (14) reported 20 cases involving the maxilla and 3 cases involving the mandible in a series of 138 cases of generalized osteitis deformans, that is 17% jaw involvement. The disease may occur in 3 stages: 1 . an early osteoclastic stage, 2 . an intermediate stage which demonstrates both osteoclastic and osteoblastic activity, and 3 . a mature stage, in which osteoblastic activity predominates (1). Chnical symptoms develop slowly due to the cluonicity of the disease. Symptoms most commonly encountered are bone pain, severe headaches, dizzi- ness, deafness, visual disturbances, facial paralysis, weakness, and mental disturbances (7). The signs of the disease are not always obvious until it has become relatively far advanced. They include pro- gressive enlargement of the skull, deformities of the
Paget's disease of the mandible — a review and report of a case
