|Year : 2017 | Volume
| Issue : 3 | Page : 227-230
Atypical presentation of Paget's disease with secondary osteomyelitis of mandible
Sadaksharam Jayachandran, Ramiah Vasudevi, Luis Kayal
Department of Oral Medicine and Radiology, Tamilnadu Government Dental College and Hospital, Chennai, India
|Date of Submission||13-Jun-2017|
|Date of Acceptance||09-Nov-2017|
|Date of Web Publication||20-Nov-2017|
Department of Oral Medicine and Radiology, Tamilnadu Government Dental College and Hospital, Chennai
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Paget's disease (PD) is a chronic progressive disease of the bone characterized by abnormal bone resorption and deposition affecting either single bone (monostotic) or many bones (polyostotic). The precise etiology of the disease is unknown but it is likely that the disease is a result of a viral infection of the osteoclasts in a genetically susceptible host. It is inherited as an autosomal dominant trait with high penetrance. The patients affected are usually over forty years of age, and disease is often asymptomatic. The diagnosis is mainly based on biochemical markers of bone turnover and radiological examinations. Normal levels of calcium phosphates and markedly elevated serum alkaline phosphatase (SAP) is a constant feature of this disease. It is regarded as uncommon in Asians, Scandinavians, and black Africans. In India, Paget's disease is rare. Here, we report a rare case of PD with secondary osteomyelitis of mandible with biochemical, radiological, and dual X-ray absortiometry findings. The patient is currently undergoing treatment successfully using bisphosphonates (Alendronate), an anti-resorptive drug to decrease the morbidity associated with the disease.
Keywords: Alendronate, bisphosphonates, cotton wool appearance, osteitis deformans, serum alkaline phosphatase
|How to cite this article:|
Jayachandran S, Vasudevi R, Kayal L. Atypical presentation of Paget's disease with secondary osteomyelitis of mandible. J Indian Acad Oral Med Radiol 2017;29:227-30
|How to cite this URL:|
Jayachandran S, Vasudevi R, Kayal L. Atypical presentation of Paget's disease with secondary osteomyelitis of mandible. J Indian Acad Oral Med Radiol [serial online] 2017 [cited 2021 Oct 17];29:227-30. Available from: https://www.jiaomr.in/text.asp?2017/29/3/227/218723
| Introduction|| |
Paget's disease (PD) of bone is a chronic disease of the adult skeleton characterized by focal areas of excessive bone resorption followed by bone formation. First described by a British surgeon Sir James Paget in 1877, he named the condition “osteitis deformans”. PD can be monostotic or polyostotic; the disease process is one of the disordered bone metabolism. Basically the osteoclastic resorptive process is overactive, with a compensatory increase in osteoblastic action attempting to maintain strong bone. The bones most likely to be involved in decreasing order are pelvis, femur, skull, vertebrae, clavicle, and humerus. The jaws are involved in 17% of cases; the maxilla is involved more often than mandible. Here, we report a case of PD with generalized involvement with secondary osteomyelitis of mandible and its dental considerations.
| Case Report|| |
A 66-year-old male presented with five years of progressively increasing pain in lower jaw region and pus discharge since six months. Past medical history and family history were non contributory. On examination, long, narrow face with frontal bossing was present. The mandible was placed forward than maxilla and there was a diffuse swelling present in right side parasymphysis region of mandible crossing the midline. There was a healed extraoral sinus present in left side parasymphysis region [Figure 1]a. Intraorally uniform enlargement of mandible with spaced anteriors was present. Pus discharge was present in relation to 46 and 47 region [Figure 1]b. All maxillary teeth and mandibular anteriors were vital, in posteriors 34 showed delayed response to stimuli and 34, 35, 36, 37, 44, 45, 46 were nonvital.
|Figure 1: (a) Extraoral photograph showing swelling in the symphysis region with healed sinus. (b)Intraoral photograph showing intraoral sinus with pus discharge in 46 and 47 region|
Click here to view
Streptococcus species was detected on pus culture, which was resistant to penicillin and sensitive to ciprofloxacin and amikacin. Complete bloodcount, renal function test, liver function test, and serum electrolytes levels were in normal range, but serum alkaline phosphatase level increased to 1485 U/L. Serum calcium, serum phosphorous, vitamin D total, paratharmone level, and serum glutamic oxaloacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT) levels were normal. To confirm the raised SAP due to bone condition, bone fraction of alkaline phosphatase test was done. Bone alkaline phosphatase range was above 120 where normal range is between 6–30 mg/l. With these results, patient was sent for skeletal survey to ruleout generalized bone involvement and dual energy X-ray absortiometry (DEXA) for assessment of bone density.
Orthopantomograph revealed generalized rarefaction of bone with altered trabeculae pattern and overall density. Multiple areas showed periapical radiopacities giving cottonwool appearance in both quadrants of the mandible. In mandible, roots of 34, 35, 36, 37, and 44, 45, 46, 47, were bulbous suggestive of hypercementosis [Figure 2]. Cone beam computed tomography axial section taken at the level of root revealed well-defined multipe hyperdense masses surrounding the apex of the root [Figure 3]a. Coronal section of CBCT at the level of anterior teeth and sagital section at the level of premolar revealed irregular hyperdense and hypodense masses and hyperdense masses attached to the root [Figure 3]b and [Figure 3]c. Computed tomographic 3D picture of skull showed multiple osteolytic regions in skull and other facial bones [Figure 3]d. Based on the clinical, biochemical, and radiographic findings, this case was provisionally diagnosed as PD of the mandible with secondary osteomyelitis.
|Figure 2: Multiple areas showing periapical radiopacities giving cottonwool appearance in both quadrants of the mandibular roots of 34, 35, 36, 37, and 44, 45, 46, 47 showing hypercementosis|
Click here to view
|Figure 3: (a) CBCT axial section at the level of root showing well-defined multipe hyperdense masses surrounding the apex of the root. (b and c) Coronal section of CBCT at the level of anterior teeth and sagital section at the level of premolar showing irregular hyperdense and hypodense masses and hyperdense masses attached to the root. (d) Computed tomographic 3D picture of skull showing multiple osteolytic regions in skull and other facial bones|
Click here to view
On further evaluation of axial skeleton, lateral skull view revealed frontal bone enlargement and increased width of diploic spaces, increased bone density of the inner bone and cortex of the skull, and area of radiopaque masses in the skull and mandible were present giving a characteristic cotton wool appearance [Figure 4]a. In lateral view of lumbosacral radiograph, vertebral involvement demonstrated dense sclerotic bone in the periphery and greater radiolucency in the center, which is also characteristic of early changes of PD  [Figure 4]b. Frontal view of pelvic radiograph revealed cortical thickening and sclerosis of ileopectinal and ischio pubic lines showing brim sign of paget's [Figure 4]c.
|Figure 4: (a) Lateral skull view showing increased width of diploic spaces, increased bone density of the inner bone and cortex of the skull, and area of radiopaque masses in the skull and mandible giving charecteristic cotton wool appearance. (b) Lumbosacral radiograph (lateral view), showing vertebral involvement demonstrating dense sclerotic bone in the periphery and greater radiolucency in the center. (c) Pelvic radiograph (frontal view) revealing cortical thickening and sclerosis of ileopectinal and ischio pubic lines: brim sign of paget's|
Click here to view
DEXA was also done to evaluate the density of the bone, which revealed that the patient was in osteopenic stage. Local debridement was done in mandible. Antibiotics, ciprofloxacin 500 mg, and metronidazole 400 mg were prescribed for osteomyelitis. After one week of antibiotics, pain and pus discharge was reduced. This case had been discussed with general radiologists and orthopedicians and the treatment started through interdisciplinary approach. Alendronate 70 mg/wk was prescribed. The patient is under regular follow up. After 3 months follow-up, the patient's alkaline phosphatase level had decreased from 1485 to 948 IU/L. After 2 months follow-up, SAP further reduced to746 IU/L.
| Discussion|| |
PD is a geriatric disease reported in the late adult age with onset above 5th to 6th decades of life; occurs both in men and women, with male predominance of approximately 3:2. It is characterized by excessive resorption in focal areas followed by abundant bone formation with eventual replacement of normal bone marrow by vascular and fibrous tissue. Genetic factors may play an important role in PD. One-third of patients with PD have a familial form transmitted in an autosomal dominant pattern of inheritance with incomplete penetrance but overall etiology remains unclear. Studies have suggested an association with viral infection.,
In PD, all bones of the craniofacial complex can be affected to varying degrees. Tubular bones show bowing and spinal curvature with vertebral collapse occuring in the later stages of the disease. Polyostotic disease (65–90%) is more frequent than monostotic disease. Monostotic PD appears to predominate in the axial skeleton, although every bone in the skeleton can be the sole site of involvement. Affected bones in the same person will not show the same stage. The radiographic features itself are virtually diagnostic including initial osteolytic and subsequent osteosclerosis phase.
Common complications of PD include osseous deformities, fractures, osteoarthrosis, basilar impression, spinal stenosis, osteomyelits, and neurologic abnormalities. Jaw involvement is seen in maxilla to mandible with the ratio of 2:1. In maxilla, progressive enlargement, widened alveolar ridge and flattened palate can be observed. Teeth may become mobile and migrate, producing some spacing. Edentulous patients with dentures commonly complain of difficulty in using dentures due to increasing tightness as a result of expansion of the jaws. Dental concerns in PD include difficult extractions, poor healing of extraction sites, and excessive post surgical bleeding from the highly vascular bone. PD patients are prone to oseomyelitis due to decreased vascularity of bone leading persistence of various infections.
The bones of the maxillofacial complex manifest a ground glass trabecular pattern in the early stage disease, and with progression, diffuse sclerosis is seen radiographically as ''cotton wool'' appearance of opacifications. Similar changes are identifiable in other bones of the craniofacial complex including the calvarium. In this present case, cotton wool appearance is classical in skull and mandible, which denoted that the regions were in osteolytic phase and it is associated with generalized hypercementosis, which is most advanced on premolar and molar teeth in both the arches.
Biochemical studies of PD have revealed that elevation of SAP levels is secondary to intense osteoblastic activity. It has been considered an important parameter, reflecting overall disease activity, in the assessment and follow-up of patients with PD. Other useful indices of bone resorption are the urinary excretion of total hydroxyproline and, more recently, of pyridinium cross links. In the present case, the diagnosis was made based on clinical features, classic radiographical signs of polyostotic bone disease, the abnormally elevated SAP and serum bone specific alkaline phosphatase. Selby PL et al. stated that serum total alkaline phosphatase is the most commonly used method of monitoring disease activity and it should be measured every three months for the first six months after therapy and every six months thereafter. Retreatment is usually indicated when there are persistent symptoms of PD or biochemical relapse.
The main aim of therapeutic intervention would eliminate bone pain, normalize serum total alkaline phosphatase with prolonged remission, heal radiographic osteolytic lesions, restore normal lamellar bone, and prevent recurrence and complications., Therapeutic agents commonly used include calcitonin, bisphosphonate, and mithramycin. Nitrogen-containing bisphosphonates are the treatment of choice for PD. Among bisphosphonates, amidronate is given intravenously, and etidronate, tiludronate, alendronate, and risedronate, all of which are taken orally. A retrospective study was done using 51 paget's patients medical records in Southern India (1995–2003) by Anjali et al. suggests that lower doses of alendronate might work well in Indian patients. This present case has been prescribed alendronate 70 mg/wk and is on regular observation, showing gradual prognosis.
| Conclusion|| |
We have presented a rare case of PD with secondary osteomyelitis of mandible with biochemical, radiological, and dual X-ray absortiometry investigations. This case presented with unique clinical, biochemical, and radiological findings. Interdisciplinary and careful treatment planning and monitoring are essential for these patients to improve the quality of life.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Theodorou DJ, Theodorou SJ, Kakitsubata Y. Imaging of Paget disease of bone and its musculoskeletal complications: Review. AJR Am J Roentgenol 2011;196:S64-75.
Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex: A review. Head Neck Pathol 2008;2:177-202.
Karunakaran K, Murugesan P, Rajeshwar G, Babu S. Paget's disease of the mandible. J Oral Maxillofac Pathol 2012;16:107-9. [Full text]
Roodman GD, Windle JJ. Paget disease of bone. J Clin Invest 2005;115:2008.
Michou L, Brown JP. Emerging strategies and therapies for treatment of Paget's disease of bone. Drug Des Devel Ther 2011;5:225-39.
Sekar B, Augustine D, Murali S. Paget's disease of Mandible - A case report and review of literature. J Orofac Sci 2010:2:13-7.
Shankar Y, Misra S, Vineet D, Baskaran P. Paget disease of bone: A classic case report. Contemp Clin Dent 2013;4:227-30.
] [Full text]
Pincus DJ, Armstrong MB, Thaller SR. Osteomyelitis of the craniofacial skeleton. Semin Plast Surg 2009;23:73-9.
Selby PL, Davie MW, Ralston SH, Stone MD. Guidelines on the management of Paget's disease of bone. Bone 2002;31:366-73.
Anjali, Thomas N, Rajaratnam S, Shanthly N, Oommen R, Seshadri MS. Paget's disease of bone: Experience from a centre on southern India. J Assoc Physicians India 2006;54:525-9.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]