Osteitis deformans Report of a long-standing oral
case with
extensive
involvement
James B. Murphy, D.M.D., MS., * Allyn Segelman, D.M.D., ** and Chris Doku, D.M.D., M.S.D.,*** Boston, Mass. TUFTS
UNIVERSITY
SCHOOL
OF DENTAL
MEDICINE
Presented is a case of osteitis deformans (Paget’s disease of bone) with a 3%year history after diagnosis. The patient had nearly the entire spectrum of symptoms that can result from widespread involvement of the skeletal system with this disease. Diffuse involvement of the maxilla and mandible resulted in postextraction complications which are common in Paget’s disease of the bone.
0
steitis deformans was described by Sir James Paget in 1877.’ He thought that it representeda “chronic inflammation of the bone.” Pagetdescribedthe diseaseprocessas most often affecting the lower extremities and skull. The reported incidence of the diseasehas varied from 3 to 4 percent of the population over the age of 40, with a rise to 10 percent over the age of 80.2,2*3 The boneswhich are most affected are the sacrum, pelvis, skull, femur, vertebrae, tibia, humerus, and maxilla.4 Spontaneousfractures and collapse of the involved vertebrae may lead to bowing of the extremities and kyphosis. Other sequelaeof the disease include high-output cardiac failure, urinary calculi, and sarcomatouschangesin the bone.5 (This last complication probably occurs only in about 1 percent of the cases.6)Compression of various cranial nerves may result in blindness, loss of hearing, and facial pain.3, ‘3 8 The bony involvement is usually symmetrical. An interesting casewas reported by Akin and associate@in which only the right maxilla was involved, mimicking a fibrosseouslesion. Histologically, there is evidence of both bony apposition and resorption. There is an irregular deposition of new bone on both endostealand periosteal surfaceswhich results in a considerably increased thickness of poorly formed bones that may fracture spontane*Formerly Chief Resident, Department of Oral and Maxillofacial Surgery; Advanced Education Program in Oral and Maxillofacial Surgery. Now with the Kuhn Dental Clinic, Fort Campbell, Ky. **Graduate Student, Department of Immunology. ***Professor and Chairman, Department of Oral and Maxillofacial Surgery; Associate Dean for Hospital Affairs.
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1978
The C. V. Mosby Co.
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Fig. 1. Deformity resulting from fracture of right humerus in 942.
Fig. 2. Photograph revealing a noticeably enlarged calvarium with diffuse bony exostosis of facial bones
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Fig. 3. Lateral view of the skull demonstratesdiffuse cotton-wool appearancewith basilar invagination.
ously. This results, over a period of years, in numerousbone malformations which are often crippling. Radiographically, in advancedcases,there is a diffuse, irregular, radiopaqueappearance of the involved bone, often described as “cotton-wool.” The radiographic appearance, however, dependson the stageof the disease;and in early stages,osteolytic areas may dominate.lo The most useful laboratory test is that of serum alkaline phosphatase,which may be considerably elevated. Gee and co-workers” reported a casein which it was elevated to nine times its normal value, a common finding in advancedcases. Radiation alone and radiation combined with chemotherapyhave been used successfully in arresting the progression of the diseasein patients in whom the bony involvement has been life-threatening or caused severe pain. r2 Most recently, chemotherapeuticapproaches, including the use of animal calcitonins and mithramycin (a cytotoxic antibiotic), have been used with variable successin treating the disease.13,l4 With the use of mithramycin, Ryan and associates15were able to provide symptomatic relief in fifteen patients with Paget’s disease. The effect of the drug was also reflected in changes in urinary levels of hydroxyproline and serum levels of alkaline phosphatase.Ryan and associatesthought that the affects of the drug were probably secondaryto its cytotoxicity to the osteoclasts. Bone involvement in the oral cavity most frequently occurs in the maxilla. Other dental findings include hypercementosis, spreading of the teeth, and a loss of lamina dura.8,16,l’ Extraction sites heal slowly, and the incidence of osteomyelitis is significant. CASE REPORT The patient was a 77-year-old white woman who was confined to a wheelchair. Paget’s disease of the bone had been first diagnosed in 1939, subsequent to a fracture of the left shoulder. The
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Fig. 4. Ulcerated lesion in area of extraction site of right maxillary canine patient later suffered fractures of the right humerus (1942), right hip (1955), and lateral three fingers of the left hand (1963). All of her fractures were associated with severe deformities in spite of diligent orthopedic management (Fig. 1). In 1960, the patient underwent a right mastectomy for cancer secondary to Paget’s disease of the breast. The calvarium and face were noticably enlarged and irregular in appearance (Fig. 2). Radiographs revealed a diffuse cotton-wool appearance with basilar invagination (Fig. 3). Associated findings included high-output cardiac failure, first-degree A-V block, and angina pectoris. The cardiovascular condition was being treated with digitalis and diuretics. The patient had not had acute congestive heart failure. Since 1973, she had suffered a decrease in hearing acuity, bilaterally, and this had progressed to a total loss of hearing. She had suffered from diplopia since 1965. All the associated findings were thought to be secondary to Paget’s disease. In May, 1974, the patient had had the two remaining maxillary teeth extracted under local anesthesia. The immediate postoperative period was unremarkable, but in August, 1974, she developed pain and swelling over the extraction site of the right maxillary canine. She was placed on a regimen of tetracycline by her physician, and the pain and swelling resolved within 10 days. Again in October, 1974, the patient developed pain and swelling in the same area (right anterior maxilla). On this occasion the patient was given Cleocin, 150 mg. four times daily, and again the swelling and pain subsided within 10 days, only to recur within 1 month. She came to our outpatient department at this time with an ulcerated lesion in the area of the extraction site of the right maxillary canine (Fig. 4). The mandibular right premolars were in traumatic occlusion (Fig. 5). The area was debrided and a specimen for biopsy was taken. The patient was placed on erythromycin, 500 mg. orally q.i.d. Differential diagnosis at this time included: osteomyelitis of the bone’“, i9; osteogenic sarcoma2”, 21; carcinoma”“; giant-cell tumor.13 The biopsy report was consistent with acute osteomyelitis. Cultured from the biopsy specimen were alpha streptococcus and a Neisseria species sensitive to erythromycin. The patient was continued on erythromycin, and the pain and swelling resolved. In February, 1975, the patient again reported to the outpatient department with right maxillary pain and swelling associated with a draining fistula. She was placed on erythromycin, and plans
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Fig. 5. Area of swelling resulting from traumatic occlusion of mandibular right premolars. were made to admit her for sequestrectomy. Radiographs at this time revealed complete obliteration of the right maxillary sinus (Fig. 6). In March, 1975, a sequestrectomy of the right maxilla was performed, without complications. The patient was maintained on parenteral cephalothin for 4 weeks postoperatively, until complete healing of the surgical site had taken place. There has been no further evidence of osteomyelitis to date. In April, 1977, the patient presented to the oral surgery outpatient department with a chief complaint of trauma to the maxilla from the remaining mandibular teeth. A panoramic roentgenogram revealed extensive cotton-wool radiopacities of the maxilla and mandible. Because of the gross bony deformities of the maxilla and mandible, and because of the patient’s own wishes, no plans were made for prosthetic replacement of the maxillary or mandibular teeth and she was scheduled for admission for extraction of the remaining mandibular teeth. In May, 1977, the patient was admitted, the physical findings were essentially unchanged from those enumerated in her past history. The serum alkaline phosphatase value was 8 Bodansky units (normal, 2-4). The remainder of the laboratory values were unremarkable. One hour preoperatively the patient was given 500 mg. of erythromycin lactobionate intravenously. The remaining mandibular teeth were extracted under general anesthesia, without complications. She was maintained on erythromycin 250 mg. intravenously for 24 hours, and then this regimen was changed to oral erythromycin 250 mg. four times a day. Her hospital course was uneventful and she was discharged on oral erythromycin, 250 mg. four times a day for 1 week. The mandibular extraction sites healed uneventfully.
DISCUSSION AND CONCLUSION Osteitis deformans is a relatively common disease. Between 3 and 4 percent of the population over the age of 40 is affected. The case presented is unusual because of the extent of involvement. Virtually the entire spectrum of symptoms of Paget’s disease was present. Deposition of bone along the auditory canal and optic foramen resulted in loss of hearing and diplopia. The develop-
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Fig. 6. Radiograph taken in February, 1975, reveals complete obliteration of right maxillary sinus.
ment of arteriovenousshunts within the poorly formed bone probably led to the patient’s high-output cardiac failure. Spontaneousbone fractures resulted in crippling deformities. The patient’s remarkable ability to survive her diseasefor so many years allowed the gradual evolution of these symptoms. The developement of osteomyelitis after the first oral surgical procedure is not uncommon. It is difficult to say whether the use of antibiotics before, during, and after the secondprocedureaverted this complication, but we recommendtheir use in patients with such extensive jaw involvement who are to undergo oral surgical procedures. REFERENCES 1, Paget, J.: On a Form of Chronic Inflammation of Bones (Osteitis Deformans), Med. Chir. Tr. (Lond.) 60: 37-63, 1877. 2. Collins, D. H., and Hunter, J.: Paget’s Disease of Bone: Incidence and Subclinical Forms, Lancet 2: 51, 1956. 3. de Deuxchaimes, C. N., and Krane, S. M.: Paget’s Disease of Bone: Clinical and Metabolic Observations, Medicine 43: 233-266, 1964. 4. Anderson, W. A. D., and Scott, T. M.: Synopsis of Pathology, ed. 7, St. Louis, 1968, The C. V. Mosby Co., p. 836. 5. Rosenkrantz, J. A., Wolf, J., and Kaicher, J. J.: Paget’s Disease (Osteitis Deformans). Review of One Hundred Eleven Cases, Arch. Intern. Med. 90: 610-633, 1952. 6. Akin, R. K., Barton, K., and Walters, P. J., Paget’s Disease of Bone: A Case Report, ORAL SURG. 39: 5, 707-712, 1975. 7. Halazonetis, J. A., and Darling, A. 1.: Paget’s Disease of the Maxilla: Report of a Case Treated Surgically, Br. Dent. J. 122: 425-429, 1967. 8. Tillman, H. H.: Paget’s Disease of Bone: A Clinical Radiographic and Histopathologic Study of Twentyfour Cases Involving the Jaws, ORAL SURG. 15: 12251237, 1962. 9. Lichenstein, L.: Disease of Bone and Joints, ed. 1, St. Louis, 1970, The C. V. Mosby Company, pp. 122-234.
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10.Pindborg, J.J.,andHjorting-Hanson, E,:AtlasofDiseases oftheJaws, Philadelphia, 1974, W,B.
W.,Catho,A. F., and Lumerman, II.: Paget’s Disease (Osteitis Deformans) of the Mandible: Report of a Case, J. Oral Surg. 30: 223-227, 1972. 12. Levison, V.: The Treatment of Paget’s Diseaseof Bone by Radiotherapy,Ann. Physiol. Med. 10: 230-233,
1I. GE, J. K., Zambito,R. F., Agrentiere,G. 1970.
13. DeRose, et al.: Response of Paget’s Disease to Porcine and Salmon Calcitonins: Effect of Long-term Treatment, Am. J. Med. 56: 858, 1974. 14. Lebbin, D., et al.: Outpatient Treatment of Paget’s Diseaseof Bone With Mithramycin, Ann. Intern. Med. 81: 635, 1974.
15. Ryan, W. G., Schwartz, T. B., and Northrop, G.: Experiencesin the Treatment of Paget’s DiseaseWith Mithramycin, J.A.M.A. 213: 1153-1157, 1970. 16. Weinberger, A.: The Clinical Significance of Hypercementosis,ORAL SURG. 7: 79-87, 1954. 17. Spilka, C. J., and Callahan, K. R.: A Review of the Differential Diagnosis of the Oral Manifestationsin Early Osteitis Deformans, ORAL SURG. 11: 809-826, 1958. 18. Feig, H. I.: Chronic Osteomyelitis of the Maxilla Secondaryto Paget’s Disease:A Complication Following Dental Extractions, ORAL SURG. 28: 320-325, 1969. 19. Ripp, G. A.: A Complication After Extractions in a Patient With Advanced Paget’s Disease,ORAL SURG. 33: 35-40, 1972. 20. Kragh, L. V., Dahlin, D. C., and Erich, J. B.: OsteogenicSarcomaof the Jaws and Facial Bones, Am. J.
Surg. 96: 496, 1958. 21. Caron, A. S., Hajdu, S. I., and Strong, E. W.: OsteogenicSarcomaof the Facial and Cranial Bones: A Review of Forty-three Cases, Am. J. Surg. 122: 719-725, 1971. 22. Taylor, R., and Shklar, G.: Carcinoma of the Maxillary Antrum in a Patient With Osteitis Deformans (Paget’s Diseaseof Bone). Report of a Case, J. Oral Surg. 22: 428-433, 1964. 23. Shklar, G., and Meyer, I.: A Giant Cell Tumor of the Maxilla in an Area of Osteitis Deformans(Paget’s Diseaseof Bone), ORAL SURG. 11: 835-842, 1958. Reprint
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Dr. J. B. Murphy Kuhn Dental Clinic Fort Campbell, Ky. 42223