Bahan Pagets Disease of Maxilla

WJD
10.5005/jp-journals-10015-1053
CASE REPORT Pagets Disease of Maxilla
Pagets Disease of Maxilla

1
GC Rajkumar, 2Bindu Ramesh, 3R Shashikala, 2Manjunath
1
Professor, Department of Oral and Maxillofacial Surgery, MR Ambedkar Dental College and Hospital, Bengaluru, Karnataka, India
2
Senior Lecturer, Department of Oral and Maxillofacial Surgery, MR Ambedkar Dental College and Hospital, Bengaluru, Karnataka, India
3
Professor and Head, Department of Oral and Maxillofacial Surgery, MR Ambedkar Dental College and Hospital, Bengaluru, Karnataka, India
Correspondence: GC Rajkumar, Kshipra Dental and Implantology Center, No. 7, Sridevi Complex, NAT Street, Basavangudi
Bengaluru-4, Karnataka, India, e-mail: rajk23in@yahoo.co.in
ABSTRACT
Pagets disease is an idiopathic, non-neoplastic affliction characterized by disordered bone formation and resorption. It affects about 3%
of the population. Pagets disease affects mainly the skull, femur, tibia and pelvic bones. Facial skeleton is involved in about 17% of the
cases. We present a case of Pagets disease involving maxillary and mandibular skeleton which developed osteomyelitis following
extraction of maxillary right posterior teeth.
Keywords: Pagets disease, Maxilla, Osteomyelitis, Hereditary.
INTRODUCTION
Pagets disease (osteitis deformans) is an idiopathic,
non-neoplastic affliction characterized by disordered bone
formation and resorption.1 Nature of the disease is unknown,
although evidence suggests that it is a multicentric benign tumor
of the osteoclasts. The possibility of an infective viral etiology
is suggested by ultrastructural demonstration of intranuclear
inclusion bodies in abnormal osteoclasts found in these patients.2
Pagets affects about 3% of the population2-4 in the European
countries. There are no epidemiological studies or literature
showing incidence of Pagets disease in the Asian population.
Its occurrence is more common in males (78.6%) than females
(21.4%). It is found to be familial in about 40% of the patients.5
In all familial cases, the existence of atleast one first degree Fig. 1: Preoperative necrotic right maxilla
relative is present. In the following patient, chronic osteomyelitis
of the maxilla developed following extraction of right maxillary
posterior teeth.
CASE REPORT
A 45-year-old female patient reported to the department of oral
and maxillofacial surgery with a chief complaint of halitosis
and swelling in the right side of upper jaw. Patient gave a history
of extraction of three teeth, 6 months before in a private dental
clinic following which she developed halitosis and slight
discharge in the right side of maxilla. On examination extra-
orally, a diffuse swelling was present in the right maxillary Fig. 2: OPG showing the typical Cotton Wool appearance
region extending from the infraorbital region superiorly to line

joining the tragus to corner of the mouth inferiorly. On palpation, Orthopantomograph showed cotton wool appearance of
swelling had diffuse borders and was firm in consistency. upper and lower jaws in the tooth bearing region with loss of
Intraorally entire maxillary alveolus was enlarged and right lamina dura on all the teeth (Fig. 2). Further investigation with
maxillary alveolus extending from canine to second molar CT scan showed radiolucent and radiopaque areas in the maxilla
showed necrotic bone exposed into the oral cavity with and mandible with sequestration of the right maxillary alveolus
inflammation of the surrounding mucosa (Fig. 1). (Figs 3 and 4). Laboratory investigations showed an increase
World Journal of Dentistry, January-March 2011;2(1):49-51 49

GC Rajkumar et al
Fig. 3: Axial CT scan of the maxilla Fig. 4: 3D CT reconstruction showing the necrotic right maxilla
in serum alkaline phosphatase level 242 U/L. Calcium and

phosphorous levels were within normal limit. Bone scan of the
entire skeleton showed increased mitotic activity only in maxilla
and mandible with no other involvement of the skeleton. After
clinical and radiographic examination, the case was diagnosed
as osteomyelitis of right maxilla due to underlying Pagets
disease.
Under local anesthesia, a buccal mucoperiosteal flap was
raised extending from the canine to second molar region on
right side, necrotic bony sequestrum in the region was removed,
curettage of the remaining bone was done. Hemostasis was
achieved and a primary closure was done. Patient was prescribed Fig. 5: The right maxilla after curettage 4 weeks postoperative
antibiotics and analgesics. Postoperatively, the wound healed
uneventfully (Fig. 5). Based on biochemical investigations, it
was decided not to put the patient any medication to inhibit. generally appear in later decades of life. Radiograph, CT scan
and bone scan reports show involvement of only the maxilla
DISCUSSION and mandible indicating milder form of the disease, which is
Pagets disease of the bone is a chronic disease of adult skeleton confirmed by her blood investigations showing mild increase
characterized by focal areas of excessive bone resorption in alkaline phosphatase level. Patient is in the burned out stage
followed by bone formation. Radiographically, one sees of the disease resulting in increased density and decreased
predominantly osteolytic areas; as the disease progresses there vascularity, which has led to fracture of alveolus during
is compensatory almost exuberant overgrowth of bone. The extraction. Decreased vascularity has further led to impaired
rapidly laid-down bone is soft and extremely vascular healing of the extraction wound and necrosis.
responding poorly to stress and resulting deformity, especially It has been stated by many authors that bone involved in
in the weight bearing bones. Finally, the disease progresses to Pagets disease is highly susceptible to infection when exposed
the burned out stage, where the enlarged bones become well to the oral flora.3 When there is a necessity for extraction,
mineralized with decreased vascularity.6, 7 surgical removal of teeth is recommended along with adequate
Significant observations made in our patient are that she suturing of the mucosa, followed by antibiotic therapy. In
has a family history of Pagets disease with patients father and Pagets disease, medical line of treatment mainly includes bone
a sibling suffering with similar condition. It is stated by Morales resorption inhibitors, such as calcitonin and bisphosphonates
et al that in familial cases, age at diagnosis is earlier than that alter the bone metabolism and biomechanical pattern.
sporadic cases as our patient was 45-year-old at the time of Surgical treatment is limited to nerve decompression, arthro-
diagnosis and already in the burned out stage, sporadic cases plasty and correction of deformities.8
50
JAYPEE
WJD
Pagets Disease of Maxilla
REFERENCES 5. Morales-Piga AA, Rey-Rey JS, Corres Gonzalez J. Frequency

and characteristics of familial aggregation of Pagets Disease of
1. Kasper, Braunwald, Fauci. Harrisons Principals of Internal
bone. Journal of Bone and Mineral Research 1995;10(4).
Medicine (16th ed), McGraw Hill Book Company Inc.
6. Howard I Feig, Walter R Edmunds, Richard beaubien. Chronic
New York 1966;2279-81. osteomyelitis of the maxilla secondary to Pagets disease: A
2. Wood NK, Goaz PW. Differential diagnosis of oral lesions complication following dental extraction. J of Oral Surg Oral
(5th ed). St Louis: Mosby Year Book, 1997:407-08,439-40. Med and Oral Pathology Sep 1969;28(3):320-25.
3. Martin S Greenberg, Micheal Glick. Burkets Oral Medicine 7. Woo TS, Schwartz HC. Unusual presentation of Pagets disease
Diagnosis and Treatment (10th ed). BC Becker Inc, Hamilton of the maxilla. British Journal of Oral and Maxillofacial Surgery
Ontorio, Canada. Apr 1995;33(2):98-100.
4. Wen Chen Wang, Yi Shing Lisa Cheng, Chung Ho Chen. Pagets 8. Juan L Martinez, Fernando Almeida, Manue Picon.
disease of bone in a Chinese patient: A case report and review Maxillomandibular monoblock removal, reshaping, and
of literature. Oral Surg Oral Med Oral Oral Pathol Oral Radiol reinsertion in Pagets disease: 15-year follow-up. J Oral
Endo 2005;99:727-33. Maxillofac Surg 2005;63:1680-85.
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Pagets Disease of Maxilla

region extending from the infraorbital region superiorly to line

World Journal of Dentistry, January-March 2011;2(1):49-51 49

in serum alkaline phosphatase level 242 U/L. Calcium and

Pagets Disease of Maxilla

REFERENCES 5. Morales-Piga AA, Rey-Rey JS, Corres Gonzalez J. Frequency

World Journal of Dentistry, January-March 2011;2(1):49-51 51

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