Background

Florid cemento-osseous dysplasia (FCOD) is a sclerosing disease characterised by intense opaque masses with many irregular lobules and is generally observed in the jaws and the alveolar process.1 FCOD was first identified by Melrose et al2 in 1976 as a subtype of cemento-osseous dysplasia. The current WHO classification (2005) recommends use of the term ‘florid osseous dysplasia’ (FOD) for this condition, which was previously known as sclerosing osteitis, multiple enostoses, gigantiform cementoma or FCOD.3

The disease is mainly observed in middle-aged black females. The ratio of males to females is 1:2.6.4 The aetiology of this disease and the reasons for the differences in prevalence between races and genders have not been completely explained.5 In many reports, the disease is said to originate from the periodontal ligament, based on its histopathological similarity and occurrence in nearby regions.4 However, some studies have argued that it may arise from the cementum remaining in the socket after an extraction.6 ,7

We describe a case of FOD that affected three family members, discuss its clinical, radiological and histological characteristics, and review the literature.

Case presentation

Case 1 (the mother)

A 45-year-old female patient was admitted to our oral and maxillofacial surgery department with regional pain around the socket of a tooth which had been extracted a month previously in another clinic. The patient had no systemic disease and her extra-oral appearance was normal. On intra-oral examination, edentulous regions in both jaws were seen together with a necrotic area of bone in the extraction socket. The patient stated that she had had teeth deficiency since birth. She did not complain of paresthesia. Orthopantomography revealed multiple impacted teeth, odontoma-like formations and common lobular, irregular radiopacities (‘cotton wool’ appearance) affecting the jaws (figure 1). Detailed evaluation of the patient using cone beam CT (CBCT) showed there was no root resorption or correlation between teeth and the lesion. After radiographic evaluation, the decision was made to perform curettage on the extraction socket under antibiotic pressure and simultaneously take a bone biopsy for histopathological diagnosis. The healing period was uneventful. Histopathology revealed large and small bone trabeculae with wide areas on the fibrocellular connective tissue and fibro-osseous lesions compatible with FOD. Osteoblastic rimming and a few osteoclasts were observed around the woven bone trabeculae (figure 2). The patient experienced no problems during the 6-month follow-up period.

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Figure 1

Orthopantomograms of the patients: (A) mother; (B) son; (C) mother's brother.

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Figure 2

Histologically woven bone trabeculae partly surrounded by osteoblasts(yellow arrows) were seen within fibrocellular connective tissue. A few osteoclasts(black arrows) were also located around the bone trabeculae. (H&E; A: ×100; B: ×200).

Differential diagnosis

The differential diagnosis is important as many lesions have radiographic features similar to those of FOD. Paget’'s disease, chronic diffuse sclerosing osteomyelitis (CDSO), periapical cemental dysplasia (PCD) and familial gigantiform cementoma (FGC) all have similarities with FOD on radiography.

CBCT is a valuable diagnostic tool as it can show the axial, sagittal and frontal sections. It can be used to differentiate FOD from lesions that exhibit a similar sclerotic appearance on conventional radiographs.8 In enostosis and exostosis, in which bone intensity increases, axial CBCT can distinguish between the lesion and the cortical layer, unlike occlusal radiography.

While CDSO is detected as a single irregular opaque mass on the jaw, FOD appears as many round or lobular opaque masses. CDSO can affect the entire region from the lower bound of the body of the mandible to the alveolar crest, and even the ramus.9 The absence of inflammation eliminates CDSO.

In radiographs, Paget's disease may show a ‘cotton wool’ type radiopaque appearance in association with hypercementosis. However, this polyostotic disease is generally observed in other bones in addition to the jaw bones. Biochemical analyses showing increased serum alkaline phosphatase, calcium and phosphor can differentiate Paget's disease from FOD.10 In our cases, biochemical analyses were only carried out for the mother and showed normal results.

FGC is an autosomal dominant disease mostly observed in young white individuals and causes explicit widening of the jaw. In bone dysplasia other than FGC, widening of the jaw is uncommon and FOD is very rarely observed.5 The clinical characteristics of our cases agreed with literature data describing FOD. No widening of the jaw bones was detected in our cases.

Thakkar et al11 and Waldron12 reported that the clinical, radiographic and histological differences between PCD, FOD and FGC can be attributed to changes in a single gene or to different clinical findings gathered from lesions belonging to the benign cement osseous dysplasia group.

Treatment

Treatment of FOD depends on current symptoms. Asymptomatic cases generally do not require treatment, but periodic clinical and radiological evaluation is recommended.5 If radiological and clinical findings are sufficient for diagnosis, biopsy is not advised so as to avoid infection, sequestrum formation and osteomyelitis; infection is usually associated with impaired vascularisation of the region. However, in symptomatic patients, treatment involves antibiotic therapy and sequestrectomy.13 No symptoms were found in our second and third cases, so periodic follow-ups were suggested to prevent possible infection, and information was given on oral hygiene. In our first case, a biopsy was performed as the tooth socket was close to the lesions. Waldron12 reported recovery of a bad socket and even sequestrum formation in FOD patients who had had a tooth extracted close to cemental masses. However, in our first case, the necrotic bone was removed from the extraction socket after an incisional biopsy, and recovery was uneventful after primary closure.