Position Paper

Chronic fibrosing osteomyelitis: a position statement William Adams1, Christopher R. Brown2, Arthur Roberts1,3, Michael Gossweiler1, Richard Halstead4, Kenneth Spolnik1, Kevin Deardorf1, Gaston Dana4, Lawrence I Goldblatt1, Richard Biggerstaff5, Steven Neucks5 1

Indiana University School of Dentistry, private practice, Indianapolis Indiana, 2Private Practice, Versailles, IN, USA, 3College of Medicine and Veterinary Medicine, University of Edinborough, Private Practice, Indianapolis, IN, USA, 4Marion College School of Osteopathic Medicine, private practice, Mooresville, Indiana, 5Indiana University School of Medicine, Private practice, Indianapolis, Indiana Chronic Fibrosing Osteomyletis (CFO) is a commonly found condition which is often undiagnosed because of its co-morbidities with other systemic conditions. Clinicians should consider CFO in their differential diagnosis when confronted with complex head, neck and facial pain patients who present with multisymptom/systems overlay. A multi-disciplinary approach is often required for proper patient diagnosis and treatment. Keywords: chronic fibrosing osteomyletis, multi-disciplinary, histological findings, contributing factors

Introduction In 2012, the Indiana Craniofacial Group, a multidisciplinary consortium of practitioners was formed, consisting of practitioners from the fields of Oral Medicine, Endodontics, Oral Pathology, Neurology, Rheumatology, Otolaryngology, Periodontology, Psychiatry, Oral and Maxillofacial Radiology, Anesthesia, General Dentistry, Internal Medicine, and Pain Management. The purpose of this professional group was to provide a matrix for multiple opinions and treatment of inter-connecting co-morbidities associated with head, neck, and facial pain to help establish inter-disciplinary continuity of care built upon clinical knowledge, expertise, and experience. This group of practitioners has been working together for the betterment of the patient in an effort to improve the patient’s overall care, and to reduce or eliminate the patient’s symptoms. After reviewing collective complex head, neck, and facial pain cases, a recurring aggregate of symptoms was noticed across all licensures, often baffling the individual treating doctor, leading to mis-diagnosis and misdirected

Correspondence to: C. R. Brown, 823 S. Adams, Hwy. 421 S., Versailles, IN 47042, USA. Email: [email protected] ß W. S. Maney & Son Ltd 2014 DOI 10.1179/0886963414Z.00000000057

treatment. Through the collective efforts of the Craniofacial Group, extensive literature searches and patient interviews, a distinct clinical pattern began to emerge, leading to a discernable, predictably diagnosed, evidenced-based entity. This condition is currently referred to as chronic fibrosing osteomyelitis (CFO). Chronic fibrosing osteomyelitis is a condition that often requires a multi-disciplinary approach to diagnose and treat. The CFO symptoms often cross the various aspects of the medical and dental professions and is often described as a ‘medical condition treated by dentists’. Our American system is not inherently conducive to multi-disciplinary communication and comprehensive care. Third party reimbursement further complicates the situation by shifting coverage back and forth between ‘dental’ and ‘medical’ policies, often leaving suffering patients with little or no coverage. As practitioners who work in the field of pain management can attest, patients in pain often seek care without prejudice of professional degrees, simply seeking relief from their condition. Unfortunately, due to the diversity of symptoms and lack of a proper working diagnosis, they often end up wandering from doctor to doctor seeking care, further compounding their pain with frustration, exhaustion, and financial burdens. With the advent of emerging technology and the ability of practitioners to actively mine data and

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interconnect, diagnoses that once depended upon ‘hunches’ and intuition are now evidence based. Advancement within a multi-disciplinary field, however, is still often dependent upon clinicians educating themselves, in order to be able to listen to their patients with complex regional head, neck, and facial pain complaints, and to be knowledgeable enough to include CFO in their differential diagnosis. The following position paper is the first collective, multi-disciplinary attempt to combine the current literature with collective years of advanced clinical experience into an understandable format to help educate clinicians to better serve our patients. Chronic fibrosing osteomyelitis of the jaw was first described in the literature by Black1 in 1915. This disease of bone has been called by several names through the years, including: Ratner Bone Cavity, Robert’s Bone Cavity, Alveolar Cavitational Osteopathosis, and ischemic or avascular necrosis.2 The most current name for this condition, based upon histopathologic findings is CFO. Chronic fibrosing osteomyelitis is a non-suppurative condition, which occurs in both the mandible and maxilla. Chronic fibrosing osteomyelitis is a condition found in most other bones in the body, but is often known by different names in the literature, depending upon the site of occurrence.3–9 Chronic fibrosing osteomyelitis can occur in bone surrounding both vital and non-vital teeth, edentulous areas including rami, and deep body of the mandible, and the condylar heads. This chronically painful condition occurs in both sexes but is predominately found in females by approximately an 8 : 1 ratio.

Clinical Symptoms Clinical symptoms may be confusing and can occur over an extended period of time.10 Symptoms often mimic other clinical conditions and may include but not be limited to:

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Jaw pain (either jaw), often cyclic, varying in intensity from mild to debilitating Feeling of pressure in mandible, maxilla, and/or sinus area Foul taste (‘metallic, rotten, sour’, etc.) Headaches Temporal tenderness Occipital tenderness TMD type symptoms (masticatory muscle tenderness, limited mandibular range of motion, mandibular deviation/deflection, joint noise) Trigeminal neuralgia type symptoms (divisions I, II, III), including electric shock type of pain, palpable trigger zones, pain with or without provocation Neck pain Otalgia Co-morbid anxiety/depression

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Psychosocial dysfunction and/or coping mechanisms Alteration of chewing patterns Functional disorders such as bruxing, clenching, mandibular dyskinesia Occlusal changes.

Clinical Findings Symptoms of CFO are often elusive, and an accurate diagnosis must be developed with a thorough medical/dental history, proper visualization, selective anesthesia, and histological examination. The clinical findings may include but not be limited to:

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Teeth in area are often tender to percussion (but will pulp test normal) Alveolar process in area is very tender to palpation Soft tissues appear visually and periodontally normal Absence of visible swelling, exudate Unilateral presence of extensive dental work (root canals, extractions, or implants) on the painful side without evidence of inadequate or inappropriate treatment.

Radiographic Findings Radiographic findings are very commonly absent in the traditional plane films (periapical or panoramic) taken in the dental office (whether they be regular film or digital). In some cases, a panograph will show radiolucency in the area compared to the unaffected side. Asymptomatic radiolucent areas may also be visible at the apices of endodontically treated teeth. If the condylar heads are affected, radiolucencies and changes to condylar morphology similar to osteoarthritis and/or adaptive remodeling may be visible. Cone beam computed tomography will often reveal a radiolucent medullary bone defect. Technetium-99 radioisotope uptake procedures, properly done, may also be considered in an attempt to visualize an effected area and determine blood flow or lack thereof in the affected bones.

Contributing Factors Contributing factors can occur as primary, secondary or tertiary precipitators singularly or co-morbidly, and may include, but not be limited to:

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Coagulopathies (hypercoagulable or hypofibrinolytic)11 Corticosteroids Autoimmune diseases Hyperlipidemias Uncontrolled diabetes History of dry socket in area Multiple invasive or extensive dental procedures, including: endodontics (both successful and failed), dental extractions, failed implants, and extensive dental restorations Multiple uses of dental anesthetics containing vasoconstrictors12

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Co-morbid chronic central sensitization syndromes (fibromyalgia, chronic fatigue syndrome, irritable bowel, restless leg, multiple chemical sensitivities, etc.) Physical or chemical insults Use of oral or IV bisphosphonates Uncontrolled high blood pressure.

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The bone is present in the form of multiple, mostly irregular trabeculae which:

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Clinical Diagnosis Achieving a differential diagnosis is important and should be made in a progressive fashion. To do so, the following diagnostic evaluations may include, but not be limited to:

Past medical/dental history History should be taken with special attention to chronic pain issues, central sensitization syndrome, endocrine disorders, past or current use of oral or IV bisphosphonates, physical or chemical trauma, extensive dental work (including extractions and endodontics with or without resolution of pain, chronic tooth pain, tooth sensitivity, pain in edentulous areas, TMD pain/ dysfunction, and perceived occlusal changes. Patients often have an extensive history of copious visits, diagnostic testing, and/or multiple prescription medications, including NSAIDS, SSRIs, and central acting opioids from various types of practitioners, with little to no resolution of their pain. They are often labeled as malingerers or drug seekers, and may describe themselves as frustrated, discouraged, and sometimes desperate.

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Visual exam (magnification is helpful) Firm palpation of alveolar process Radiographic examination (old films may be helpful, especially panographs) Cone beam computed tomography of the maxilla/ mandible Technetium-99 bone scan (The scan labels areas of osteoblastic activity such as inflammation, neoplasm, or recent surgery) Selective inter-osseous anesthesia of radiolucent, palpation-tender, bone using vaso-constrictor free anesthetic13 Lipid panel, coagulation panel, and genetic markers are often useful14 Bone biopsy.

Histological Findings For a definitive diagnosis, a histological examination is required. A specimen of hard and soft tissue IS recommended, based upon a complete medical/dental history, clinical examination, and proper visualization. Histology findings reveal:

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Decalcified specimen of hard and soft tissue consisting of:

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viable sclerotic bone focally fibrosed bone marrow extravasated erythrocytes.

Chronic fibrosing osteomyelitis: a position statement

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exhibit a lamellar bone and containing varying numbers of osteocytes, each within its respective lacuna exhibit prominent basophilic cement lines indicative of past remodeling activity exhibit distinctly rounded external contours exhibit delaminated or splayed ends.

In addition, multiple zones of narrow longitudinal fragments of bone lie free within the tissue. Associated with the bone are multiple segments of fatty and/or hematopoietic bone marrow. This marrow notably exhibits varying degrees of fibrosis. In some zones, there appear multiple variably sized round to oval clear spaces often surrounded by extravasated erythrocytes. These spaces are interpreted as lipid globules resulting from the degeneration and fusion of marrow lipocytes. Some cases are also accompanied by inflamed granulation tissue, which is probably residual from previous periapical or periodontal infection. In addition, very occasional cases will include bacterial colonies and/or prominent sections of peripheral nerve. (In the authors’ experience, the incidence of both of these is very low.)

Treatment Treatment for CFO is determined by the degree and location of the condition. Treatment should begin as conservatively as possible and progress according to the patient’s particular condition, response, or lack thereof. The following treatments may be considered when treating CFO, either singularly or in combination:15,16

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Medications (oral, IV) Intra-oral orthotic Periauricular PNS/PENFS (percutaneous nerve stimulation/percutaneous electrical nerve field stimulation) when properly applied by patent pending application technique [Innovative health Solutions (829 S. Adams St., Versailles Indiana has patented a peri-auricular PNS/PNFS ambulatory, physician applied device called the NSS, designed to be implanted only by using their patent pending application technique] Trephination of the affected bone Surgical debridement Hyperbaric oxygen therapy Genetic counseling.

Scope of Practice Licensed medical and osteopathic physicians and dentists, according to state licensure, individual education, clinical skills, and experience should consider treatment of CFO within the scope of their practice. Multi-disciplinary approaches should often be considered to properly address all contributing co-morbidities.

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Disclaimer Statements Contributors Written sections of the articles were contributed by Drs. Adams, Brown, Roberts and Goldblatt. Final manuscript was approved by all authors. Funding none. Conflicts of interest none. Ethics approval Ethics approval: Content was approved by all authors by unanimous consensus.

References 1 Adams WR, Spolnik KJ, Bouquot JE. Maxillofacial osteonecrosis in a patient with multiple facial pains. J Oral Pathol Med. 1999;28:423–32. 2 Arlet J, Mazieres B, editors. Bone circulation and bone necrosis. Heidelberg: Springer-Verlag; 1990. 3 Black GV. A work on special dental pathology. Chicago, IL: Medico-Dental Publ Co.; 1915. 4 Bouguot J, Martin W, Wribleski G. Computer-based thrutransmisson sonography (CTS) imaging of ischemic osteonecrosis of the jaws - a preliminary investigation of cadaver jaws and 15 pain patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;92:550. 5 Bouquot JE, Roberts AM, Person P, Christian J. The histopathology of neuragia-inducing cavitational osteonecrosis (NICO). J Dent Res. 1989;68:952.

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6 Bouquot JE, Christian J. Long-term effects of jawbone curettage on the pain of facial neuralgia; treatment results in neuralgia-inducing cavitational osteonecrosis. Oral Surg Oral Med Oral Pathol. 1991;72:582. 7 Jiao X, Meng Q. The influence of pathologic bone cavity of jaw bone on the etiopathology of trigeminal neuralgia. Acta Acad Med Sichuan. 1981;12:243–7. 8 McMahon RE, Griep J, Marfurt CP. Local anesthetic effects in the presence of chronic osteomyelitis of the mandible. Anesth Prog. 1991;38:189. 9 McMahon RE, Adams W, Spolnik K. Diagnostic anesthesia for referred trigeminal pain, part I. Compendium Cont Educ Dent. 1992;11:870–81. 10 Bouquot JE, LaMarche M. Subpontic osteonecrosis: imaging and microscopic features in 38 patients with ‘idiopathic’ chronic pain. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;86:209–10. 11 Glueck CJ, Frelberg R, Glueck HI, Henderson C, Welch M, Tracy T, et al. Hypofibrinolysis: a common, major cause of osteonecrosis. Am J Hemotol. 1994;45:156–66. 12 Bouquot JE, McMahan RE. Neuropathic pain in maxillofacial osteonecrosis. J Oral Maxillofac Surg. 2000;58:1003– 20. 13 Ratner EJ, Langer B, Evins ML. Alveolar cavitational osteopathosis – manifestations of an infectious process and its implication in the causation of chronic pain. J Periodontol. 1986;57:593–603. 14 Roberts AM, Person P. Etiology treatment of idiopathic trigeminal and atypical facial neuralgias. Oral Surg. 1979;48:298–308. 15 Neville BW, Damm DD, Allen C, Bouquot J. Oral and maxillofacial pathology. 2008: Philadelphia, PA: W.B. Saunders; 2008.

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Chronic Fibrosing Osteomyletis (CFO) is a commonly found condition which is often undiagnosed because of its co-morbidities with other systemic condit...
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