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 17% 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 the 2nd molar tooth. Clinically the tooth appeared ankylosed. After surgical removal, the tooth was fixed, demineralized and paraffin sections were made. The microscopic evaluation revealed 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 Pennsylvania, School ot Dental Medicine, Philadelphia, Pa, USA

Key words: Paget's disease, mandibular involvement, root resorption. Frederic Barnett, D.M.D., University ot Pennsylvania School ot Dental Medicine, Department ot Endodontics, 4001 Spruce St, Philadelphia, PA 19104, USA. Accepted tor publication 24 September 1984.

Paget's disease, or osteitis defor?nans, is a chronic progressive 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 specific 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 population after the 4th decade, and in about 10% of patients over the age of 70, provided the frequency is determined by systematic pathological examination of the skeleton (3). With the use of skeletal scintimaging techniques and measurements of plasma 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 arteriovenous aneurysms (6). Also, there is a sizeable group of other vascular alterations that occur concomitanlly with Paget's disease, such as increased cardiac output, cardiac enlargement, and arteriosclerosis (7). Hyperperfusion of diseased bone, rather than arteriovenous shunts associated with Paget's disease, was shown by Rhodes et al. (8), while Gafe et. al. (9) postulated a metabolic disturbance 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 disease process overcompensates with bulk for the replaced bone, and most commonly affects the weightbearing 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, dizziness, 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 progressive enlargement of the skull, deformities of the

Barnett and Elfenbein spine, femur and tibia so that the patient actually becomes shorter (7). Involvement of the facial bones
is called leontiasis ossea (5). , - '-

The radiographic features vary with the stage of the disease (1). In the early osteolytic phase, radiolucent lesions are seen. The areas of decreased radiodensity have a flne trabecular pattern and resemble ground glass. In the osteoblastic phase, there are areas of increased bone formation lliat tend to be patchy in distribution, resulting in the typical cotton-wool appearance associated with the disease (7). When the jaws are involved, the maxilla is more commonly affected than the mandible (7). The involved jaw can exhibit progressive enlargement, with widening of the alveolar ridge. If teeth are present, they may become loose and migrate, producing interdental spacing. Dental radiographs may reveal the typical cotton-wool appearance of the bone as well as areas of hypercementosis or clublike appearance at numerous root apices. The lamina dura may be absent either in all the mandibular or all the maxillary teeth, but never in both. Occasionally, one or two teeth may display a loss of lamina dura in either, or both jaws. This flnding is not unusual and is consistent with the disease process in that only certain parts of the skeletal system are involved (15). Root resorption with subsequent replacement with pagetoid bone has been reported, but is a rare finding (16). The microscopic appearance of ihc bone varies with the stage of the disease (1). As the disease is characterized by resorption and apposition, corresponding osteoclastic and osteoblastic activities are evident. These processes occur somewhat haphazardly, producing a mosaic ]5attern of bone formation. Deeply sLaining hematoxyphilic reversal lines

indicate the alternation between the resorptive and formative phases of the disease, yielding a jigsaw pattern. The marrow varies from fatty to flbrotic with vascular proliferation (1, 7, 17). A differcnlial blood count, scrum calcium and scrum phospliorus levels are usually within normal limits (7). Serum alkaline phosphatase and urinary hydroxyprolinc are elevated and can usually be correlated with the extent and activity of the disease (18). Normal alkaline phosphatase values have been noted in 15%, and normal hydroxypioliiic excretion in more tlian 50% of patients witii early active di.seasc detected by radioisotopic scintimaging (2). There is no specific Uxatniciil (or o.sk-itis deformans. Limited improvement was observed with glucocorticoid, aspirin, and sodium fluoride therapy, but the sicle-cffccls were excessive. More recently, calcitonin, mithramycin, and disodium-ethane-1-hydroxy, 1-diphosphonate (EHDP) have all been considered effective therapeutic agents. Calcitonin treatment by injection resulted in symptomatic and biochemical improvement and a decrease in bone blood flow (19). Mithramycin, an inhibitor of DNAdirected RNA synthesis, resulted in prompt improvement of pain, reversal of biochemical abnormalities, and radiologic improvement (20). However, serious side-elfects such as nephrotoxicity, thrombocytopenia and hepalotoxicity have occurred in some patients, suggesting that intravenous mithramycin therapy be reserved fbr patients with severe disease who do not respond to safer agents (20). EHDP has also proven eflective in reducing the serum alkaline phosphatase levels and urinary hydroxyproline excretion. However, with therapeutic doses, osteomalacia with bone pain and increased incidence of fractures can occur willi this treatment (19).

Fig. 1. Periapieal radiograph of lower left quadrant. Severe root resorption of the distal root of the 2nd molar is seen. Note the cotton-wool appearance of the osseous tissues and ihc absence of a lamina dura of both molar teeth.

Eig. 2. Panoramic radiograph. Signs of Paget's disease are observed only in the mandible. Hypereementosis (o]X:n arrows), loss of lamina dura and a cotton-wool appearance of the osseotis tissue aie seen. Note the root resorption of the niandibniar right and lelt 2iul molars (closed arrows).

40

Paget's disease of tiie mandibie

There are several complications that may arise in patients M'ith Paget's disease: renal calculi, hypercalcemia, osteomyelitis and osteogenic sarcoma (7, 21). Sarcoma should be suspected in those patients who experience a marked increase in intensity of bone pain or a marked elevation in alkaline phosphatase levels (22). The risk of osteogenic sarcoma in pagetic individuals over the age of 40 years is 30 times greater than in healthy persons (7). Paget's disease must be dincrciitiatcd from osteitis fibrosa cystica (hyperparatliyroidism), osteotiiyclitis, fibrous dysplasia, osteosarcoma, and other disseminated nco]:)lastns. A definitive diagnosis depends oti a correlation ofthe radiographic, clinical, blood chemistry, and biopsy findings (1, 4, 7, 15,
23). .: . ,

mm. The tooth was tender to palpation and percussion. A periapical radiograph was taken of the mandibular left molar region (Fig. 1). This revealed severe root resorption of the distal root of the 2nd molar. Other findings included a cotton-wool appearance of the bony tissues and the absence of the lamina dm a of both molar teeth. A panoramic and lull mouth series of radiographs and a posterioranterior skull film were taken for evaluation (Fig. 2). The radiographs revealed signs typical of Paget's disease in the mandible. The maxilla appeared uninvolved. Hypcrccmcntosis, loss of lamina dura, radiopaque and radiolucent areas were observed associated with the mandibular teeth. An interesting finding was severe external root resorption associated with the mandibular left and right 2nd molars
only. '-"^ :, .. . : ' - r "i , - "pv^sj-v'-j'fB

Case report

An 82-year-old black female presented to our emergency clinic with the chief complaint of pain and swelling of 2 weeks duration associated with teeth in the lower left jaw. The medical history revealed generalized arthritis and Paget's disease diagnosed in 1954. A thorough review of systems revealed no known abnormalities. Vital signs were within normal limits. Consultation with the patient's physician revealed that the pagctoid involvement was limited to the tibia and the mandiljlc. There were no cardiac abnormalities. Blood studies revealed an elevated serum alkaline phos]3hatase level. The clinical examination revealed a soft tissue swelling ofthe buccal mucosa adjacent to the mandibular left 2nd molar. The marginal gingiva appeared reddish in color and was markedly edcmatous. A suppurative exudate was draining from the sulcus of the 2nd molar. Probing depths were 5-7

The decision was made to extract the mandibular left 2nd molar, and the patient was referred to the Oral Surgery Clinic. During the extraction procedure it was evident that this tooth was ankylosed, and a surgical extraction was performed. The patient was placed on penicillin-VK in order to prevent the development of osteomyelitis (1). Subsequent healing was uneventful and the symptoms had resolved by 7 d. Portions ofthe tooth and surrounding tissue were fixed in 10% neutral buffered formalin, decalcified, and subjected to routine histologic procedures. The sections were stained with hematoxylin and eosin. Microscopic evaluation ofthe osseous tissue revealed the typical jig-saw pattern consistent with Paget's disease (Fig. 3). Another specimen containing root structure and bone revealed replacement root resorption (Fig. 4). The osseous tissue was in direct contact with the root dentin. No multinucleated osteoclasts were observed in any of the sections.

Fig. 3. Histologic .section of hard and soft tissue curetted from the extraction site. Observe the typical jig-saw pattern of the osseotis tissue. (Original magnification x 45; hematoxylin & eosin). , . . .

Fig. 4. Histologic section of hard tissue curetted from the extraction site. Observe the direct contact between the osseous ti.s.sue (OT) and the dentin (D). (Original magnifieation x 100; hematoxylin & eosin). . .

41

Barnett and Eifeniiein Paget's disease of the mandible is an uncommon finding. Even more unusual is root resorption occurring with this disease, as was reported by Smith (16). However, Rushton (24) has suggested that root resorption might precede and accompany the hypercementosis that is commonly observed in this disease. Also, a pathologic resorption of the root cementum and dentin might occur by a process similar to the osteoclastic resorption of bone that occurs during the ostcolytic stage of Paget's disease (25). However, in the present case osteoclasts were not seen. The root structures had been replaced by osseous tissue by a mechanism of replacement resorption. This was consistent with the clinical situation encountered during the surgical procedure where the tooth was felt to be ankylosed to bone. Reterences
1. WOOD N K , GOAZ P W . (eds.) Differential diagnosis of oral lesions. 2nd ed. St. Louis: GV Mosby Co., 1980; 598 601. 2. BoNDY PK, ROSENBERG LE. (eds.) Metabolic control and disease. Philadelphia; WB Saunders Co., 1980; 1779-86. 3. JAFFE HL. Paget's disease of bone. Arch Pathol 1933; 1583-131.
4. WILLIAM HN, SCHAUWECKER D, ROBU JA, KHAIRI MR,

686-9.
9. GAFE SM, STEIN M , RADCLIFFE RB. Paget's di.sease, report

of a ease involving the maxilla. Oral Surg 1965; 20: 616 23. 10. SINGER FR. Pagel's disease oft/one. New York and London: Plenum Press, 1977. 11. MILLS BG, SINGER FR. Nuclear inclu.sioiis in Paget's di.sease of bone. Science 1976; 194: 201-3. 12. RoBHiNS S. (ed.) Patholoi>y. Philadelphia: WB Saunder.s Co., 1967; 1336-8. 13. SGHMORI. G . Uber osteiti.s deformans paget. Virchow Arch Path Anat 1932; 283: 694-751. 14. STAFNE EC, AUSTIN LT. Study of dental roentgenograms in eases of Paget's disease (o.stcitis deformans), osteitis librosa cystica and osteoma. J^y.)/f 1938; 25; 1212 4. 15. BENDER IB. Roentgenographie signilieanee of the lamina dura in systemic disease. J Albert Einstein Med Ctr 1961; .9; 82-9. 16. SMITH N H H . Monostatic Paget's disease of the mandible presenting with progressive resorption ofthe teeth. Orat Surg 1978; 46: 246-53. 17. THOMA K H . Oral Pathology. St. Louis: GV Mo.sby Go., 1950; 736.
18. HENNEMAN PH, DtiLi. TA, Avion LV, BASTOMSKY C15,

LYNCH TN. ElVeets of aspirin and corticosteroids on Paget's disease of bone. Tram Stud Colt Ptiy.sicians Phila 1963; 31: 6 25.
19. WooTTON R, REEVE J, SPELI.ACY E , TELLEZ-YUDILEVICH M .

JOHNSTON CC. Skeletal .seintimaging and radiography in ihe diagnosis and managemeni of Paget's disease. Clin Orlhop 1977; 127: 55 63. 5. PAGETJ. On a form ofehronie inflammation of bones. Trans Royal Med Chir Soc (London) 1877; 60: 37 63.
6. EDHOLM O G , HOWARTII S, MCMICHAEL J . Heart failure

Skeletal blood (low in Paget's disease of bone and its response to ealeitonin therapy. Ctin Sei Mot Med 1978; 54: 69-74. 20. RYAN WA. Treatment of Paget's disease of bone with mithramycin. Clin Orthop 1977; 127: 106 I I . 21. RosENMER'rz S, ScHARE H. Ostcogenic sarcoma arising In Paget's disease ofthe mandible. Oral Surg 1969; 28: 304 9. 22. JOLLY DE, HARRIS TB. Osteitis deformans, a ca.se of late involvement ofthe maxilla. J Oral Med 1979; 34: 54 9.
23. WADE GW, HAYES RL, GRIFFITHS NH. Osteitis deformans,

and bone and blood tlow in osetitis deformans. Ctin Sci 19455: 249-60. 7. SnAFER WG, HiNE MK, LEVY BM. (eds.) A textbook of orat pathotogy. Philadelphia: WB Saunders Co., 1974; 635-40.
8. RHODES BA, GREYSON NO, HAMILTON CR, WHITE R I , GIAR-

a systemic disease with oral manifestations: report of a case. JADA t965; 71: 43-50. 24. RUSHTON MA. Some disorders involving tooth and bone. Dent Practit 1956; 6"; 244 50.
25. RUSHTON MA, GOOKE BED, DUCKWORTH R . (eds.) Oral

GiANA FA, WAGNER HN. Absenee of anatomic arteriovenous shunts in Paget's disease of bone. jV Engl J Med 1972; 28:

Histopathology. 2nd ed. Edinburgh: Livingstone, 1970; 86.

42