Radiographic changes of the oral and maxillofacial hard tissues can be an indication of an underlying systemic disease. In this article, the range of individual disease entities that have both systemic and dental manifestations are reviewed. Images for many conditions are provided to illustrate the radiographic changes. A summary of the most common jaw affected, radiographic and pathognomonic findings, and management aspects is listed in a table format within this article for quick reference.
Key points
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Systemic diseases can have oral and maxillofacial radiographic changes.
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General dentists are likely to encounter one of many systemic diseases that present with oral and maxillofacial radiologic changes.
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The scope of differential diagnoses of radiographic changes should involve systemic as well as traditional dental etiologies.
Systemic disease can be manifested in the jaws in multiple ways ( Table 1 ). The manifestations can be limited to the teeth or may extend to involve the soft and hard tissues that form the oral cavity. Mandibular and maxillary bone often is the target. In this article, individual disease entities that have both systemic and dental manifestations and a summary of the most common jaw affected, radiographic and pathognomonic findings, and management aspects is listed in a table format within this article. Images for many conditions are provided to illustrate the jaw involvement.
| Disorder | Commonly Affected Part (Jaws) | Radiographic Category | Radiopathognomic Sign | Treatment Strategy |
|---|---|---|---|---|
| Gorlin syndrome | Mandible >> maxilla | Decreased bone density | Multiple radiolucent lesions | Surgical intervention |
| Cherubism | Mandible and maxilla | Decrease bone density | Symmetric radiolucent lesions | Spontaneous regression |
| Brown tumor of primary hyperparathyroidism | Mandible >> maxilla | Radiolucent bone cavities | Radiolucent lesion | Surgical removal of affected parathyroid gland. Resection of affected bone may be necessary. |
| MRONJ | Mandible >> maxilla | Radiolucency (plain images); osteolytic lesions (CT) | Osteomyelitic-like lesions surrounded by dense sclerotic bone | Stage specific treatment- débridement and antibiotics |
| Sickle cell anemia | Mandible >> maxilla | Altered trabeculation | Extreme enlargement of bone marrow spaces | Prevention; blood transfusions |
| Thalassemia | Maxilla | Altered trabeculation | Extreme enlargement of bone marrow spaces | Prevention; blood transfusions |
| Osteoporosis | Mandible >> maxilla | Decreased bone density (lucent) | Absence or reduction of trabeculae with thinning of inferior cortex | Medication to increase bone density |
| Gardner syndrome | Both mandible and maxilla are affected | Increased bone density | Osteomas, supernumerary teeth | Surgical and symptomatic intervention |
| FD | Both maxilla and mandible | Mixed radiolucent/radiopaque density | Jaw expansion, dental malocclusion | Surgery and medications to control bone pain |
| Renal osteodystrophy | Both maxilla and mandible | Osteolytic (lucent) lesions | Subperiosteal bone resorption, decreased bone trabeculation, reduced cortical bone thickness; ground-glass opacities; jaw enlargement, increased interdental spaces | Medical correction of hyperphosphatemia and lowering of PHT levels |
| Osteopetrosis | Both maxilla and mandible | Generalized increased bone density | Prognathic mandible, increased skull size, intracranial calcifications | Calcitriol, aesthetic, and functional treatment |
| Paget disease of bone | Both maxilla and mandible | Radiolucent | Cotton wool–like irregular opaque lesions, loss of lamina dura, hypercementosis | Surgery for early lesions, drug therapy |
| Acromegaly | Mandible | Increased BMD | Mandibular prognathism, dental malocclusion, dense skull bones, enlarged pituitary fossa | Both surgical (transsphenoidal resection) and medical |
| CCD | Mandible and maxilla | Mixed densities | Hypoplastic or absent clavicles, supernumerary teeth and missing teeth | Symptomatic, aesthetic, and functional |
| Papillon-Lefèvre syndrome | Maxilla and mandible | Periapical radiolucency, alveolar resorption | Free floating teeth, premature loss of primary and permanent dentition | Periodontal therapy, medications |
| AI | Maxilla and mandible | Alterations to enamel thickness and density | Thinning of enamel and/or decreased radiodensity of enamel | Multidisciplinary approach for preventative measures, malocclusion, and aesthetics |
| Dentinogenesis imperfecta types II and III | Maxilla and mandible | Alterations in tooth morphology and density | Type II: bulbous crowns and obliteration of pulp and root canals Type III: hypoplastic dentin, described as shell teeth |
Treatment of infections and restoring aesthetics |
| Dentin dysplasia type II | Maxilla and mandible | Alterations in tooth morphology and density | Primary teeth resemble features seen in dentinogenesis types II and III. Permanent teeth: may appear normal, but with pulpal chamber abnormalities, described as thistle/flame-shaped |
Treatment of infections and restoring aesthetics |
| Regional odontodysplasia | All or part of maxillary quadrant | Decreased density of enamel and dentin with enlarged pulp chamber | Ghostlike teeth | Early extraction or conservative management of teeth |
Amelogenesis imperfecta
Amelogenesis imperfecta is a group of hereditary enamel defects that occur in the absence of a generalized syndrome.
Clinical and Radiographic Presentation
In general, enamel malformations fall into 3 categories: hypoplastic, hypomature, and hypocalcified. Hypoplastic enamel presents as pathologically thin and rough, and there is lack of contact between teeth that are yellowish. The most severe form of hypoplastic amelogenesis imperfecta (AI) is enamel agenesis. Hypomature forms of AI present with dental crowns that are normal in size and contact adjacent teeth, but the mottled, brownish-yellow enamel is soft and has a radiodensity similar to dentin. Lastly, in hypocalcified teeth, the enamel layer may be of normal thickness but is rough and soft and wears away quickly following tooth eruption. Radiographically, mineral deficient enamel is marked by the lack of contrast between enamel and dentin ( Fig. 1 ).
Diagnostic Approach
Environmental factors and systemic diseases, such as fever, can be associated with enamel disorders that do not represent isolated AI. Thus, diagnosis requires family history, pedigree plotting, and clinical observations for a diagnosis of AI.
Management
Treatment of teeth usually depends on the state of enamel and likely requires a multidisciplinary approach. Crowns may be used to improve the appearance of teeth and protect from damage. Malocclusions occur in AI, so a treatment plan includes orthodontic consultation.
Dentinogenesis imperfecta types II and III
Dentinogenesis imperfecta describes a group of nonsyndromic, hereditary defects in dentin formation and pulp morphology.
Clinical and Radiographic Presentation
In general, both types II and III can involve primary and permanent teeth, and teeth are marked by a bluish to opalescent amber/brown discoloration. Radiographically, teeth are bulbous with cervical constriction, with complete pulpal obliteration, including the root canals , ( Fig. 2 ). Specifically, for type III, there may be multiple pulp exposures and periapical radiolucencies in noncarious teeth. Moreover, type III teeth present with hypotrophy of the dentin, and this appearance has been described as “shell” teeth.
Diagnostic Approach
Historically, the Shields classification has divided inherited dentin defects in 5 types. , Despite overlap in clinical features, however, genetic studies have revealed distinctions within these groupings. Specifically, mutations in dentin sialophosphoproprotein ( DSPP ) have been mapped in dentinogenesis imperfecta types II and III and also in dentin dysplasia type II, , whereas dentinogenesis type I (osteogenesis imperfecta) demonstrates mutations in COL1A1 and presents with skeletal abnormalities not seen in types II and III. Notwithstanding the classification systems, diagnosis is based on history, clinical examination, and radiographic features.
Management
Aims of treatment are to remove sources of infection or pain, restore aesthetics, and protect posterior teeth from wear.
Dentin dysplasia type II
Dentin dysplasia type II is a nonsyndromic, hereditary defect that affects dentin formation and pulp morphology.
Clinical and Radiographic Presentation
Clinical features for dentin dysplasia type II overlap features seen in dentinogenesis imperfecta types II and III. In the permanent teeth, however, the teeth are normal in color, and on radiographs, the pulp chamber has a thistle/flame-shaped morphology and contains pulp stones. ,
Diagnostic Approach
Based on a shared DSPP mutation between dentin dysplasia type II and dentinogenesis imperfecta types II and III, the clinical features seen may represent a spectrum along the same disease. Thus, dentin dysplasia type II may represent a milder clinical phenotype compared with dentinogenesis imperfecta type III. Nevertheless, diagnosis is based on history, clinical examination, and radiographic features.
Management
Aims of treatment are to remove sources of infection or pain, restore aesthetics, and protect posterior teeth from wear.
Regional odontodysplasia
Regional odontodysplasia is a nonhereditary disorder of root development that is associated with enamel and dentin dysplasia.
Clinical and Radiographic Presentation
Clinically, teeth are rough with a discolored crown surface, and all or part of a quadrant is affected, more often the maxilla than the mandible. , Both deciduous and/or permanent teeth can be affected. Often, the most common clinical presentation is failure or delay of dental eruption. , Radiographically, affected teeth are hypoplastic with reduced radiodensity and enlarged pulp chamber, which have been described as “ghostlike” , ( Fig. 3 ).
Diagnostic Approach
Although clinical and radiographic features, described previously, are diagnostic, histologic examination of affected teeth can be further supportive for diagnosis.
Management
Treatment of regional odontodysplasia involves early extraction of teeth, the rationale being that affected teeth are nonrestorable and susceptible to dental abscess formation after eruption. , Conversely, conservative management entails retaining noninfected teeth to help maintain alveolar bone.
Cleidocranial dysplasia
Cleidocranial dysplasia (CCD) is a rare genetic disorder, reported in 1 in every 100,0000 cases, and is inherited as an autosomal dominant genetic trait. CCD represents several skeletal abnormalities.
Clinical and Radiographic Presentation
Also known as cleidocranial dysostosis, this is a rare skeletal disorder with defective or absent clavicles causing sloping shoulders, moderately short stature, delayed eruption of teeth, incomplete development or absence of teeth, hypoplastic enamel, and supernumerary teeth ( Figs. 4 and 5 ) apart from other features like delayed closure of fontanels, deformations of chest, and abnormal pelvic and pubic bones, which are common among other skeletal deformities. Individuals with CCD have increased risk for recurrent ear and sinus infections and upper respiratory tract problems as well as hearing loss.
Diagnostic Approach and Management
Failure of resorption of overlying alveolar bone as well as mechanical interference from impacted supernumerary teeth leads to noneruption of many teeth. Serial uncovering of teeth is suggested that would accelerate the eruption of these teeth. Interceptive and traditional orthodontics and selective osteotomies, all are part of the management of these patients.
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Genetic disorder inherited as an autosomal dominant trait
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Absent or defective clavicles, delayed eruption of teeth, supernumerary teeth
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Chest deformation, abnormal pelvis, short stature
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Recurrent ear and sinus infections
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Serial uncovering of teeth inducing eruption, selective osteotomies, orthodontics when possible are the treatments of choice,
Osteoporosis
Osteoporosis is a degenerative or metabolic bone disease associated with increased fracture risk, if left untreated or undiagnosed for long periods of time. Patients with this condition present with low bone mineral density (BMD) as well as changes in bony tissue structure. Osteoporosis is more prevalent in populations with deficiency in sex hormone, estrogen depletion (usually in postmenopausal women), and advanced age.
Clinical and Radiographic Findings
Patients who have low trauma fractures in bones not easily susceptible to fracture raise a suspicion for osteoporosis. The exception, however, is those with other previously diagnosed bone diseases like multiple myeloma. Postmenopausal women over the age 50 years also are more likely to have osteoporosis as are men and women over the age of 65 years. Other clinical risk factor, include alcoholism, smoking, and low body weight (<57.6 kg). Panoramic radiographic findings include an alteration in trabecular pattern of the bone and thinning of the mandibular cortex ( Fig. 6 ).
Diagnosis
Diagnosis of osteoporosis is attained by assessing BMD. The most accurate assessment of BMD is with the use of dual-energy x-ray absorptiometry (DXA) scan. A T-score of less than −2.5, which compares the BMD of the patient to that of healthy adults, is diagnostic for osteoporosis. When BMD is evaluated via computed tomography (CT), a procedure called quantitative CT is performed for BMD in lumbar spine, hip, and limbs. This is considered more specific compared with DXA.
Management
Management of osteoporosis is dependent on disease severity. The goal of treatment is to reduce fracture risk as well as maintain quality of life for patients with osteoporosis. Pharmacologic management includes the consecutive use of bone-building agents, such as teriparatide with bisphosphonates (oral or intravenous) or denosumab. Supplements like calcium and vitamin D as well as exercises, including those that focus on weight bearing and improving balance, can be used as preventive measures.
Medication-related osteonecrosis of the jaw
Medication-related osteonecrosis of the jaw (MRONJ) can be described as persistent nonspecific odontogenic symptoms without exposed bone or exposed bone in the jaws of at least 8 weeks’ duration in a patient treated with antiresorptives or antiangiogenic agents. Exclusion criteria for an MRONJ diagnosis include previous radiation treatment and primary or metastatic cancer to the head and neck.
Clinical and Radiographic Findings
Clinical findings can vary from nonspecific presentation, such as periodontitis and mobile teeth, to more specific findings of fistulas, exposed bone, infection with purulent discharge, and pathologic fractures in severe cases. Radiologic findings also vary depending on stage of disease ( Table 2 ), with CT being able to accurately identify disease extent at an early stage compared with panoramic radiographs ( Fig. 7 ). Although MRONJ can affect either the maxilla or mandible, it is reported more commonly in the mandible.
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