What Is Chronic Non-suppurative Sclerosing Osteomyelitis?
Chronic non-suppurative sclerosing osteomyelitis is exclusively found in children and young adults. It is a type of chronic osteomyelitis. Carl Garre, a researcher, first described the disease in 1893. The pathology is characterized by a focal thickening of the periosteum that may be induced due to irritation. It is a non-suppurative inflammatory reaction with peripheral subperiosteal bone deposition caused due to mild irritation and infection. The mandible is more commonly affected than the maxilla.
When the jaw is affected, the disease generally develops from a low-virulence infection, such as dental decay, mild periodontitis (inflammation of the gums and adjoining bone), dental eruption, or previous tooth extraction in the affected area. This reactive proliferative process results in the hard swelling of the affected jaw, thereby causing subsequent facial asymmetry in the patients. The lesion does not exhibit any symptoms with no accompanying signs of inflammation.
Chronic non-suppurative sclerosing osteomyelitis presents a characteristic radiographic feature, showing new periosteal bone proliferation deposited in successive layers to the condensed cortical bone.
What Are the Other Synonyms of Chronic Non-suppurative Sclerosing Osteomyelitis?
It is also known as:
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Garre’s sclerosing osteomyelitis.
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Chronic osteomyelitis with proliferative periostitis.
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Ossifying periostitis.
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Non-suppurative chronic sclerosing osteomyelitis.
What Is the Cause of Chronic Non-suppurative Sclerosing Osteomyelitis?
It is the cause of odontogenic infection resulting from tooth decay (dental caries). The condition may result from pulp and periapical infection in relation to permanent teeth.
Garre, who originally described the disease, suggested that a sequestrum (a piece of dead bone tissue that is formed within a diseased bone) might serve as a chronic irritant, thereby stimulating bone formation without pus formation, and that this piece of dead bone tissue could be taken care of by the tissues of the host without fistulation (to create an artificial channel by surgical means).
Another scientist, Peters, however, reported that in some cases, sequestrate and fistulae are developed within months or years after the acute symptoms have subsided.
The etiology is still not clear. A bacterial infection is usually suspected, but cultures obtained are generally negative, and the chronic state of the disease may be maintained through a low-virulence bacterial infection. The main causative organisms proposed are the anaerobic bacterium Propionibacterium acnes, Actinomycosis naeslundii, and Actinomyces israelii.
What Are the Clinical Symptoms of Chronic Non-suppurative Sclerosing Osteomyelitis?
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The clinical feature is an insidious sudden onset of localized pain with episodic nonprogressive nature.
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There can be trismus (difficulty in opening the mouth).
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The disease may last for a variable duration, persist for several months or years, and can also affect multiple sites in the jaw. However, the function of the affected jaw bone is preserved.
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It is characterized by a rigid bony hard swelling that is usually present at the periphery of the jaw.
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There is no suppuration and sinus formation. Pain is not present but may occur if the lesion is secondarily infected.
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Other symptoms include high fever, lymphadenopathy (enlarged lymph nodes), and leukocytosis (increased white blood cell count).
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The size of the swelling usually varies from one to two centimeters, but it can involve the entire length of the jaw on the affected side. The thickness of the cortical bone can reach up to two to three centimeters.
What Are the Radiographic Features of Chronic Non-suppurative Sclerosing Osteomyelitis?
The lesion's radiographic appearance varies with the lesion's duration and the degree of calcification. In the early stages of the disease, a convex layer which looks like a thin crust appears over the cortex. As the event continues, the cortex becomes thickened due to the successive deposition of new bone. This lamellar deposition of bone is known as “onion skin” on radiographs.
What Are the Histopathological Features of Chronic Non-suppurative Sclerosing Osteomyelitis?
Biopsy of the bone lesion is usually performed to exclude malignancy and other disease entities that have almost similar competing radiographic and clinical differential diagnostic features.
Microscopic findings include:
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The chronic phase shows scarce inflammation with predominant sclerosis and fibrosis.
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Reactive and remodeling bone with periosteal thickening, particularly in a region of periosteal reaction.
What is the Differential Diagnosis of Chronic Non-suppurative Sclerosing Osteomyelitis?
The most important differential diagnosis is fibrous dysplasia, where the signs and symptoms of fibrous dysplasia and Garre’s osteomyelitis are almost clinically indistinguishable. It is differentiated from fibrous dysplasia based on the typical radiographic feature of “onion skin” due to periosteal new bone formation.
Cancerous conditions such as osteoid osteoma (benign bone tumor), Ewing’s sarcoma (malignant tumor), osteosarcoma (a malignant tumor of the bones), and eosinophilic granuloma should be excluded from the diagnosis. Additional differential diagnoses include syphilis, ulcerative recto colitis, Crohn’s disease, SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome, and Paget’s disease.
What Is the Treatment of Chronic Non-suppurative Sclerosing Osteomyelitis?
The main treatment goal for chronic non-suppurative sclerosing osteomyelitis is to eliminate the etiologic factor. Extraction of the causative decayed tooth is carried out to relieve the symptoms. With the help of root canal therapy and the administration of antibiotics, the size of periapical lesions can be reduced.
Some patients even respond to nonsteroidal anti-inflammatory drugs or analgesics to relieve the symptoms of inflammation. If the symptoms become uncontrollable, surgical treatment should be recommended. Bone curettage of the affected site should be performed. Very rarely, a larger section of the bone is resected with some amount of bone reconstruction. Long-term oral antibiotics and hyperbaric oxygen treatment are given to eliminate the infection.
Conclusion:
Chronic non-suppurative sclerosing osteomyelitis's a rare disease entity in occurrence since its development depends on the occurrence of a set of a few critical conditions, which includes chronic infection in a young individual with a periosteum capable of rapid osteoblastic activity, and there should be an equilibrium between the bacterial virulence and the resistance of the host. Endodontic therapy is generally considered the main treatment goal for the disease. Sclerosing osteomyelitis of Garre's is very confusing to diagnose because the other diseases have similar clinical and radiographic features, and its symptomatic control is difficult. Bone curettage, drilling with biopsy, and antibiotics should be considered as treatment options. Rarely, bone reconstruction may be required after the resection of the larger pieces of bones.
