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Familial florid osseous dysplasia: A case report
636
FAMILIAL FLORID OSSEOUS DYSPLASIA
J Oral Maxillofac Surg 47:636-640,1989
Familial Florid Osseous Dysplasia: A Case Report ANTHONY E. MUSELLA, DDS,* AND LEE J. SLATER, DDSt
Introduction Florid osseous dysplasia is a benign fibro-osseous process typically affecting middle-aged black women, and resulting in pagetoid globular osteosclerosis involving more than one jaw quadrant. I The mandible is generally more severely affected than the maxilla; it may show cortical expansion of alveolar bone and multiple associated traumatic (simple) bone cysts. I Although florid osseous dysplasia is currently a commonly used term, cases exhibiting similar radiographic and clinical features have been previously reported under a wide variety of names. 2-20 Florid osseous dysplasia affecting multiple family members appears to be quite uncornmon.P'P The following is a case report of florid osseous dysplasia involving a white mother and daughter.
Report of a Case A 33-year-old white woman was referred to our clinic for evaluation of multiple radiopacities in all four quadrants (Fig I). She had her third molars removed in April 1982 and had dental radiographs showing multiple osteosclerotic masses throughout the alveolar bone of the maxilla and the mandible. She was asymptomatic, with no noticeable cortical expansion. Her medical history was remarkable only for a chronic frontal sinusitis requiring bilateral frontal sinus obliteration in February 1986. The immediate postoperative period was uncomplicated. However, the patient was readmitted to the hospital in July 1986, for work-up of a small subcutaneous air pocket that had developed at the surgical site in June, as well as periorbital headaches that accompanied it. The condition spontaneously resolved within a week. The patient's evaluation in July included a computed tomography (CT) scan with iodine contrast, and technetium and gallium
* Chief, Department of Oral and Maxillofacial Surgery, 56 Medical Group (TAC), MacDiII Air Force Base, Florida. t Chairman, Department of Oral Pathology, David Grant Medical Center, Travis Air Force Base, California. Address correspondence and reprint requests to Dr Musella: Department of Oral and Maxillofacial Surgery, 56 Medical Group (TAC), Mac Dill AFB, FL 33608. This is a US government work. There are no restrictions on its use. 0278-2391/89/4706-0017$0.00/0
bone scans. These showed no evidence of frontal sinus mucocele, air, or abnormal isotope uptake. However, the mandible showed marked increased isotope uptake. The panoramic radiographic findings were consistent with a diagnosis of florid osseous dysplasia. A biopsy was not performed. The patient's mother was 71 years old. An expansile osteosclerotic fibro-osseous process involving all four jaw quadrants (Figs 2 and 3) was first recognized in her in 1941 after routine dental radiographs, and a biopsy was obtained then because she complained "her gums began to grow after tooth extraction." Her medical history was significant for having cataracts, glaucoma, and osteoarthritis. She related that she had undergone multiple operations to recontour her enlarging jaws over a 30-year period, with difficulty in healing over the past 20 years. She subsequently developed a refractory osteomyelitis with a chronic draining fistulae in the maxilla. She took clindarnyacin, 150 rng, four times a day and had been treated unsuccessfully in the past with vibramyacin and erythromycin. In 1972,the patient underwent hyperbaric oxygen therapy in anticipation of further surgical recontouring and tooth extraction. She recalled no particular problems with the surgeries. However, she had difficulty healing postoperatively and related that the tooth extractions had been generally difficult. Laboratory studies, including serum levels of alkaline phosphatase, calcium, and phosphorus were normal for both mother and daughter. The mother stated that she had two paternal aunts with the same condition, but they were unavailable for questioning or examination.
Discussion Fibro-osseous lesions have been reported in the literature since the early 1950s with the exception of fibrous dysplasia, first reported in 1937 by Lichtenstein and Jaffe. 24 For at least three decades, this group of lesions has posed a problem in classification. Most fibro-osseous lesions can be delineated into one of the categories described by Waldron.i? given sufficient clinical, radiographic, and histologic information. However, the florid form of cemento-osseous dysplasia continues to be reported under a variety of names, each term selected to reflect the abundant radiopaque cemento-osseous product present in this fibro-osseous process. Although many authors feel this mineralized product is cementum, Melrose et all state that the use of the word cementum is inappropriate because the evi-
MUSELLA AND SLATER
637
FIGURE l. Panoramic radio gr aph of the daughter sho wing multiple flocculent radiopacities in all four jaw quadrants.
dence that all the abnormal mineralized material actually represents cementum is far from convincing. They go on to cite Friedman and Goldman's report25 of identical calcifications in fibro-osseous lesions found in bones other than the jaws. Using plain and polarized light, Melrose et all found mineralized tissues resembling both bone and cementum in the same specimen. Bhaskar and Cutright" take the view that cementum is applicable only to the calcified tissue covering the roots of teeth and suggests that these "multiple enostoses" are analogous to intraosseous endophytic tori or exostoses. Part of the difficulty in classification is the remarkable histologic similarity of these fibroosseous lesions. Shafer et af6state that the clinical radiographic, and even microscopic finding: florid osseous dysplasia are virtually identical to those of sclerotic cemental masses and chronic diffuse sclerosing osteomyelitis. Melrose et all describe one feature that may distinguish florid osseous dysplasia from other benign fibro-osseous lesions: in a series of 34 patients, he found that florid osseous dysplasia occurs with simple bone cysts approximately 40% of the time. Analysis of the fluid from these cysts in patients with florid osseous dysplasia showed consistent elevations of alkaline and
acid phosphatase. The enzyme levels were higher than normal serum values, although calcium and phosphorus levels were slightly lower. Florid osseous dysplasia has been described by some authors as a chronic sclerosing osteomyelitis or a sclerosing osteitis .3.l3.l5.27 Low-grade osteomyelitis , particularly in edentulous areas, seems to be a complication of florid osseous dysplasia, which becomes symptomatic with denture wear. According to Waldron et al 17 and Melrose et ai, I the tissue has little or no capacity for physiologic resorption beneath a prosthesis due to fused cemental masses or dysplastic bone, which contain very few os teocytes and no marrow spaces. The reduced vasculature, as the bone becomes more sclerotic, contributes to nonhealing mucosal ulceration and consequential sequestration of bony fragments. I • 17 Waldron et al 17 further believe there is little evidence for an inflammatory or infectious etiology for this disease. Microscopic evidence of an inflammatory reaction was noted in 16 of 34 cases reviewed, but in each instance the lesions or masses were exposed to the oral cavity through mucosal ulceration or fistula. 17 Most symptomatic lesions were in edentulous areas with teeth having been extracted years previously. The inflammatory reaction appears su-
FIGURE 2. Panoramic radiograph of the mother showing generalized osteosclerotic areas throughout both jaws.
638
FAMILIAL FLORID OSSEOUS DYSPLASIA
FIGURE 3. Photomicrographs showing the predominance of sclerotic bone and small fibrous tissue component commonly found in the later stages of a fibro-osseous lesion. A, Low power. (Hematoxylin and eosin. Original magnification, xSO.) B, High power. (Hematoxylin and eosin. Original magnification, x t25.)
perimposed as a result of exposure of the masses, either by tooth proximity to lesions after extraction, or ulceration.F'?' As the osseous dysplasia lesions become progressively sclerotic, the risk of developing osteomyelitis subsequent to tooth extraction increases. Because clinical management of symptomatic lesions associated with ulceration or fistula is often difficult, tooth extraction and/or excision of masses should be avoided, if possible.'? For this reason, some authors 1 recommend retaining teeth with fixed, rather than removable, prosthesis, necessitating meticulous care and hygiene. CLINICAL FEATURES
Florid osseous dysplasia appears to be restricted to the jaws; all other benign fibro-osseous lesions can involve the extragnathic skeleton.'? Florid osseous dysplasia has a reported predilection for middle-aged black women. A review of several series'" revealed that 90% of florid osseous dysplasia cases occurred in women, 74% occurred in blacks, 24.5% in whites, and 1.5% in Orientals. The age range was 30 to 45 years old, with no patients reported younger than 20 years. t9 Melrose et al's' study 004 patients with florid osseous dysplasia showed an age at diagnosis ranging from 26 to 59 years, with 33 patients being women and 32 being black. The duration of the disease is generally unknown because initially the condition is usually asymptomatic and discovered only on routine radiographs. Some patients report a dull or intermittent aching sensation. Onset of symptoms does not necessarily correspond to buccal or lingual cortical expansion as the disease progresses. RADIOGRAPHIC FEATURES
The extent of the disease radiographically is variable, ranging from a few mullifocal lesions to dif-
fuse involvement of the alveolar processes. Although florid osseous dysplasia generally shows bilateral mandibular lesions, only about half of the cases demonstrate involvement of one or both maxillary quadrants. The anterior mandible is frequently affected with irregular, globular, mixed radiolucent/radiopaque masses diffusely distributed throughout the alveolar process tending to become more radiodense over time.'" Melrose et all describe a strikingly pagetoid appearance of the lesions in panoramic, lateral, and anterior-posterior radiographs. Radiographic features of florid osseous dysplasia are similar to that of Paget's disease and, if elevated serum levels of alkaline phosphatase and long-bone radiographs are positive, Paget's disease might be considered an appropriate diagnosis. 19.23 HISTOLOGIC ApPEARANCE
Histologically, irregular calcifications or mineralized deposits are found within a proliferating fibrous connective tissue stroma. This stroma can range from slightly mixoid to densely collagenous, and contain a prominent capillary network. The mineralized deposits within the stroma are irregularly shaped and variably sized; they consist of bony trabeculae with osteocytes or acellular cementum-like calcified masses.!? Melrose et all have found, in most cases, a mixture of cementum-like material and irregular trabeculae of bone, with some trabeculae rimmed by plump osteoblasts and others showing evidence of active resorption with apposed mullinucleated osteoclasts. Neville and Albenesius" indicate that the histologic features of the benign fibro-osseous lesion of florid osseous dysplasia may be histologically indistinguishable from those of a cernento-ossifying fibroma. Allhough certain radiographic and histologic
639
MUSELLA AND SLATER
characteristics have been ascribed to certain lesions within the fibro-osseous group, ie, "ground glass" radiographic appearance and C-shaped histologic trabeculae of fibrous dysplasia, and "cotton wool" radiographic appearance and mosaic histologic pattern of Paget's disease, radiographic features are probably more important than histologic features in determining the most appropriate diagnosis.i? GENETIC PREDISPOSITION
Reports substantiating a familial relationship or particular inheritance pattern to florid osseous dysplasia are so few that authorities find inadequate grounds to substantiate any apparent genetic predisposition for this disease. Familial gigantiform cementoma, reported by Agazzi and Belloni in 1953,21 described multiple benign cementomas occurring in two members of the same family. Cannon et al22 reported a case of mother and son with gigantiform cementoma in 1980. A third series was reported by Sedano et al23 in 1982 describing autosomal dominant cemental dysplasia in ten members of the same family. Our review of the English literature reoccurrence of florid osvealed no other familial . . seous dysplasia. Although florid osseous dysplasia is prevalent in black women, it is interesting to note that the af-· fected individuals in this case are of Italian descent, as were those reported with familial gigantiform cementomas by Agazzi and Belloni. 21 An autosomaldominant pattern has been reported previously.P and the presence of the disease in females of three consecutive generations in our report is consistent with such a mode of inheritance. Summary
Most cases of florid osseous dysplasia are asymptomatic and are found during routine radiographic examination. The radiographic spectrum is similar to that of other benign fibro-osseous lesions, beginning as a radiolucent lesion and becoming more radiopaque as the involved bone becomes more sclerotic. Many benign fibro-osseous lesions demonstrate similar histologic features, and without other radiographic and clinical information, a definitive diagnosis may be impossible. The dense bone of florid osseous dysplasia is very susceptible to infection and osteomyelitis, particularly with advancing years and in response to trauma. The disease may be self-limiting as reported by many authors, but as in this case, can be symptomatic for most of the person's lifetime. This presents a diagnostic and treatment dilemma for the clinician. Although this
entity has a distinct predilection for black women, more reported cases should be evaluated before the mode of inheritance can be established and before a tendency for familial cases in individuals of Italian descent can be confirmed.
Acknowledgment We wish to thank Dr Dennis DeWitt, who first recognized these familial osseous dysplasia cases and referred the family members to our institution for evaluation.
References 1. Melrose RJ, Abrams AM, Mills RG: Florid osseous dysplasia. Oral Surg 41:62, 1976 2. Hamner JE III, Scofield HH, Cornyn J: Benign fibroosseous jaw lesions of periodontal membrane origin: An analysis of 249 cases. Cancer 22:861, 1968 3. Laband PG, Leacock AG: Sclerosing osteitis of the jaws. J Oral Surg 25:23, 1967 4. Bell WH: Sclerosing osteomyelitis of the mandible and maxilla. Oral Surg 12:391, 1959 5. Bhaskar DN, Cutright DE: Multiple enostosis: Report of 16 cases. J Oral Surg 26:321, 1968 6. Lyons DC: Multiple osteomas of the mandible and maxilla. Oral Surg 8:738, 1955 7. Morgan GA, Poynton GH: Periapical cementoblastoma. Oral Surg 12:1344, 1959 8. Uthman A, AI-Shawaf M: Paget's disease of the mandible. Oral Surg 28:866, 1969 9. Pindborg JJ, Kramer IR, Torlini H: Histological typing of odontogenic tumors, jaw cysts, and allied lesions, in International Histological Classification of Tumors (ed 5). Geneva, World Health Organization, 1971, pp 332-334 10. Winer HJ, Goepp RA, Olson RE: Gigantiform cementomas resembling Paget's disease: Report of a case. J Oral Surg 30:517, 1972 11. Vanderwall I, Vanderkwast WAM: A case of gigantiforrn cementoma. 1nt J Oral Surg 3:440, 1974 12. Gorlin RJ, Chaudhry AP, Pindborg JJ: Odontogenic tumors, classification, histopathology, and clinical behavior in man and domesticated animals. Cancer 14:73, 1961 13. EI-Mofty S: Chronic diffuse sclerosing osteomyelitis. Oral Surg 36:898, 1973 14. Keen EG, Sammartino CA, Johnson ES: Chronic sclerosing osteomyelitis of the mandible. J Am Dent Assoc 76:597, 1968 15. Shafer WG: Chronic sclerosing osteomyelitis. J Oral Surg 15:138, 1957 16. Towns TL: Chronic diffuse sclerosing osteomyelitis of the maxilla and mandible. J Oral Surg 30:903, 1972 17. Waldron CA, Giansanti JS, Browand BC: Sclerotic cemental masses of the jaws (so called chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostoses, and gigantiform-cementoma). Oral Surg 39:590, 1975 18. Morgan GA, Morgan PR: Cysts and cyst-like lesions of the mandible. Dent Radiogr Photog 47:47, 1974 19. Ellis GL, Lewis DM, Carleton AS: Multiple osteosclerotic masses of the jaws. J Am Dent Assoc 114:678, 1987 20. Waldron CA: Fibro-osseous lesions of the jaws. J Oral MaxiIIofac Surg 43:249, 1985 21. Agazzi C, Belloni L: Gli Odontomi Duri Dei Mascellari. Arch Ital Otolaryngol 64:3, 1953 (suppl 16) 22. Cannon JS, Keller EE, Dahlin DC: Gigantiforrn cementoma: Report of two cases (mother and son). J Oral Surg 38:65, 1980 23. Sedano HO, Kuba R, Gorlin RJ: Autosomal dominant cemento-dysplasia. Oral Surg 54:642, 1982 24. Lichtenstein L, Jaffe HL: Fibrous dysplasia of the bone. Arch Pathol 33:777, 1942
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FACIAL TRAUMA AFTER ORTHOGNATHIC SURGERY
25. Friedman NB, Goldman RL: Cementoma oflong bones-An extragnathic odontogenic tumor. Clin Orthop 67:243, 1969 26. Shafer WG, Hine MK, Levy BM: A Textbook of Oral Pathology (ed 4). Philadelphia, Saunders, 1983, p 505 27. Jacobsen S, Heyden G: Chronic sclerosing osteomyelitis of the mandible. Oral Surg 43:357, 1977
28. Neville BW, Albenesius RJ: The prevalence of benign fibroosseous lesions of periodontal ligament origin in black women: A radiographic survey. Oral Surg Oral Med Oral Pathol 62:340, 1986 29. Eversole LR, Sabes WR, Rovin S: Fibrous dysplasia: Anasologic problem in the diagnosis of fibro-osseous lesions of the jaws. J Oral Patholl:189, 1972
J Oral Maxillefao Surg 47:640-642,1989
Severe Facial Trauma After Orthognathic Surgery With Rigid Fixation GREGG W. WILSON, DMD* AND WILLIAM M. IOVINO, DMDt The use of rigid internal fixation has become widespread in the stabilization of osteotomy sites during orthognathic surgery. The advantages of rigid fixation and other various treatments currently in use have received much attention in the literature. These include a greatly decreased period of maxillomandibular fixation (MMF), decreased risk of postoperative aspiration, less skeletal relapse, better postoperative nutrition and oral hygiene, and increased patient comfort.ll'Also mentioned are an increased range of manidibular motion, and faster, stronger bony union. 6 , 15, 17, 18 These benefits of using rigid fixation have contributed to making orthognathic surgery more acceptable to patients and practitioners alike. This article describes a case in which a patient who had undergone orthognathic surgery with multiple rigidly fixed osteotomies was involved in a motor vehicle accident 6 weeks postoperatively, sustaining severe facial trauma. The findings in this case give clinical support to the advantages of rigid fixation. Report of a Case A 21-year-old white man was initially evaluated for surgical correction of skeletal facial deformity. He subsequently underwent a three-piece Lefort I osteotomy with impaction and transverse widening of the maxilla, bilateral sagittal ramus osteotomies of the mandible, and an advancement genioplasty. The maxillary segments were
* Attending Surgeon, University of Pittsburgh Hospitals; private practice, Indiana, Pennsylvania. t Attending Surgeon, University of Pittsburgh Hospitals; Private Practice, Monroeville, Pennsylvania. Address correspondence and reprint requests to Dr Wilson: 502 Airport Office and Professional Center, Indiana, PA 15701. © 1989
American Association of Oral and Maxillofacial Sur-
geons 0278-2391/89/4706-0018$3.00/0
fixed using Luhr (Howmedica Inc, Rutherford, NJ) fourhole miniplates and self-tapping screws. Two plates were positioned near the pyriform rims, and two were placed posteriorly in the zygomatic buttress regions (Fig 1). The sagittal ramus osteotomies were fixed with three Synthes (Synthes Ltd, Paoli, PA) screws placed from an extraoral approach on each side. The advancement genioplasty was fixed with 24-gauge stainless steel wires. Maxillomandibular fixation was applied, and all incisions closed. The patient's initial postoperative course was unremarkable. He was discharged on the third day, and MMF was removed on postoperative day 5. Because the maxilla had been segmented, the occlusal splint was retained on the maxillary arch for approximately 5 weeks. On removal of the splint, results of the procedure were quite good and the teeth were in the proper occlusion. On July 6, 1987, approximately 6 weeks after surgery, the patient was involved in a motor vehicle accident and sustained severe facial trauma with loss of conciousness. Clinical and radiographic examination revealed multiple facial fractures with displacement at several of the osteotomy sites. Physical examination revealed moderate facial edema and ecchymosis. The left eye showed evidence of entrapment of the extraocular muscles, with deficient upward gaze. A step defect was palpable at the left infraorbital rim. The maxilla was displaced in an inferior and posterior position causing an anterior open bite. There was obvious mobility and a step defect in the incisor region of the mandible. Also apparent was a nasal septal deviation to the left and some crepitus of the nasal bridge. There were no other apparent injuries, and the patient was neurologically stable. Radiographic studies showed the extent of the facial injuries (Figs 2 and 3). The maxilla was separted across the previous Lefort I site, with the bone plates and screws of the left side tearing through the lateral maxillary wall, causing gross comminution. In addition, the inferior and lateral orbital rims were torn by the posterior bone plate, and the periorbita was entrapped in several small bone fragments. The right maxillary bone plates also tore through the fragile antral wall, but without the degree of comminution seen on the left side. A slightly displaced nasal bone fracture was also noted. The mandible was fractured through the superior portion of the parasyrnphysis to the level of the genioplasty cut. The genial segment also was separated, creating a full-thickness man-
FAMILIAL FLORID OSSEOUS DYSPLASIA
J Oral Maxillofac Surg 47:636-640,1989
Familial Florid Osseous Dysplasia: A Case Report ANTHONY E. MUSELLA, DDS,* AND LEE J. SLATER, DDSt
Introduction Florid osseous dysplasia is a benign fibro-osseous process typically affecting middle-aged black women, and resulting in pagetoid globular osteosclerosis involving more than one jaw quadrant. I The mandible is generally more severely affected than the maxilla; it may show cortical expansion of alveolar bone and multiple associated traumatic (simple) bone cysts. I Although florid osseous dysplasia is currently a commonly used term, cases exhibiting similar radiographic and clinical features have been previously reported under a wide variety of names. 2-20 Florid osseous dysplasia affecting multiple family members appears to be quite uncornmon.P'P The following is a case report of florid osseous dysplasia involving a white mother and daughter.
Report of a Case A 33-year-old white woman was referred to our clinic for evaluation of multiple radiopacities in all four quadrants (Fig I). She had her third molars removed in April 1982 and had dental radiographs showing multiple osteosclerotic masses throughout the alveolar bone of the maxilla and the mandible. She was asymptomatic, with no noticeable cortical expansion. Her medical history was remarkable only for a chronic frontal sinusitis requiring bilateral frontal sinus obliteration in February 1986. The immediate postoperative period was uncomplicated. However, the patient was readmitted to the hospital in July 1986, for work-up of a small subcutaneous air pocket that had developed at the surgical site in June, as well as periorbital headaches that accompanied it. The condition spontaneously resolved within a week. The patient's evaluation in July included a computed tomography (CT) scan with iodine contrast, and technetium and gallium
* Chief, Department of Oral and Maxillofacial Surgery, 56 Medical Group (TAC), MacDiII Air Force Base, Florida. t Chairman, Department of Oral Pathology, David Grant Medical Center, Travis Air Force Base, California. Address correspondence and reprint requests to Dr Musella: Department of Oral and Maxillofacial Surgery, 56 Medical Group (TAC), Mac Dill AFB, FL 33608. This is a US government work. There are no restrictions on its use. 0278-2391/89/4706-0017$0.00/0
bone scans. These showed no evidence of frontal sinus mucocele, air, or abnormal isotope uptake. However, the mandible showed marked increased isotope uptake. The panoramic radiographic findings were consistent with a diagnosis of florid osseous dysplasia. A biopsy was not performed. The patient's mother was 71 years old. An expansile osteosclerotic fibro-osseous process involving all four jaw quadrants (Figs 2 and 3) was first recognized in her in 1941 after routine dental radiographs, and a biopsy was obtained then because she complained "her gums began to grow after tooth extraction." Her medical history was significant for having cataracts, glaucoma, and osteoarthritis. She related that she had undergone multiple operations to recontour her enlarging jaws over a 30-year period, with difficulty in healing over the past 20 years. She subsequently developed a refractory osteomyelitis with a chronic draining fistulae in the maxilla. She took clindarnyacin, 150 rng, four times a day and had been treated unsuccessfully in the past with vibramyacin and erythromycin. In 1972,the patient underwent hyperbaric oxygen therapy in anticipation of further surgical recontouring and tooth extraction. She recalled no particular problems with the surgeries. However, she had difficulty healing postoperatively and related that the tooth extractions had been generally difficult. Laboratory studies, including serum levels of alkaline phosphatase, calcium, and phosphorus were normal for both mother and daughter. The mother stated that she had two paternal aunts with the same condition, but they were unavailable for questioning or examination.
Discussion Fibro-osseous lesions have been reported in the literature since the early 1950s with the exception of fibrous dysplasia, first reported in 1937 by Lichtenstein and Jaffe. 24 For at least three decades, this group of lesions has posed a problem in classification. Most fibro-osseous lesions can be delineated into one of the categories described by Waldron.i? given sufficient clinical, radiographic, and histologic information. However, the florid form of cemento-osseous dysplasia continues to be reported under a variety of names, each term selected to reflect the abundant radiopaque cemento-osseous product present in this fibro-osseous process. Although many authors feel this mineralized product is cementum, Melrose et all state that the use of the word cementum is inappropriate because the evi-
MUSELLA AND SLATER
637
FIGURE l. Panoramic radio gr aph of the daughter sho wing multiple flocculent radiopacities in all four jaw quadrants.
dence that all the abnormal mineralized material actually represents cementum is far from convincing. They go on to cite Friedman and Goldman's report25 of identical calcifications in fibro-osseous lesions found in bones other than the jaws. Using plain and polarized light, Melrose et all found mineralized tissues resembling both bone and cementum in the same specimen. Bhaskar and Cutright" take the view that cementum is applicable only to the calcified tissue covering the roots of teeth and suggests that these "multiple enostoses" are analogous to intraosseous endophytic tori or exostoses. Part of the difficulty in classification is the remarkable histologic similarity of these fibroosseous lesions. Shafer et af6state that the clinical radiographic, and even microscopic finding: florid osseous dysplasia are virtually identical to those of sclerotic cemental masses and chronic diffuse sclerosing osteomyelitis. Melrose et all describe one feature that may distinguish florid osseous dysplasia from other benign fibro-osseous lesions: in a series of 34 patients, he found that florid osseous dysplasia occurs with simple bone cysts approximately 40% of the time. Analysis of the fluid from these cysts in patients with florid osseous dysplasia showed consistent elevations of alkaline and
acid phosphatase. The enzyme levels were higher than normal serum values, although calcium and phosphorus levels were slightly lower. Florid osseous dysplasia has been described by some authors as a chronic sclerosing osteomyelitis or a sclerosing osteitis .3.l3.l5.27 Low-grade osteomyelitis , particularly in edentulous areas, seems to be a complication of florid osseous dysplasia, which becomes symptomatic with denture wear. According to Waldron et al 17 and Melrose et ai, I the tissue has little or no capacity for physiologic resorption beneath a prosthesis due to fused cemental masses or dysplastic bone, which contain very few os teocytes and no marrow spaces. The reduced vasculature, as the bone becomes more sclerotic, contributes to nonhealing mucosal ulceration and consequential sequestration of bony fragments. I • 17 Waldron et al 17 further believe there is little evidence for an inflammatory or infectious etiology for this disease. Microscopic evidence of an inflammatory reaction was noted in 16 of 34 cases reviewed, but in each instance the lesions or masses were exposed to the oral cavity through mucosal ulceration or fistula. 17 Most symptomatic lesions were in edentulous areas with teeth having been extracted years previously. The inflammatory reaction appears su-
FIGURE 2. Panoramic radiograph of the mother showing generalized osteosclerotic areas throughout both jaws.
638
FAMILIAL FLORID OSSEOUS DYSPLASIA
FIGURE 3. Photomicrographs showing the predominance of sclerotic bone and small fibrous tissue component commonly found in the later stages of a fibro-osseous lesion. A, Low power. (Hematoxylin and eosin. Original magnification, xSO.) B, High power. (Hematoxylin and eosin. Original magnification, x t25.)
perimposed as a result of exposure of the masses, either by tooth proximity to lesions after extraction, or ulceration.F'?' As the osseous dysplasia lesions become progressively sclerotic, the risk of developing osteomyelitis subsequent to tooth extraction increases. Because clinical management of symptomatic lesions associated with ulceration or fistula is often difficult, tooth extraction and/or excision of masses should be avoided, if possible.'? For this reason, some authors 1 recommend retaining teeth with fixed, rather than removable, prosthesis, necessitating meticulous care and hygiene. CLINICAL FEATURES
Florid osseous dysplasia appears to be restricted to the jaws; all other benign fibro-osseous lesions can involve the extragnathic skeleton.'? Florid osseous dysplasia has a reported predilection for middle-aged black women. A review of several series'" revealed that 90% of florid osseous dysplasia cases occurred in women, 74% occurred in blacks, 24.5% in whites, and 1.5% in Orientals. The age range was 30 to 45 years old, with no patients reported younger than 20 years. t9 Melrose et al's' study 004 patients with florid osseous dysplasia showed an age at diagnosis ranging from 26 to 59 years, with 33 patients being women and 32 being black. The duration of the disease is generally unknown because initially the condition is usually asymptomatic and discovered only on routine radiographs. Some patients report a dull or intermittent aching sensation. Onset of symptoms does not necessarily correspond to buccal or lingual cortical expansion as the disease progresses. RADIOGRAPHIC FEATURES
The extent of the disease radiographically is variable, ranging from a few mullifocal lesions to dif-
fuse involvement of the alveolar processes. Although florid osseous dysplasia generally shows bilateral mandibular lesions, only about half of the cases demonstrate involvement of one or both maxillary quadrants. The anterior mandible is frequently affected with irregular, globular, mixed radiolucent/radiopaque masses diffusely distributed throughout the alveolar process tending to become more radiodense over time.'" Melrose et all describe a strikingly pagetoid appearance of the lesions in panoramic, lateral, and anterior-posterior radiographs. Radiographic features of florid osseous dysplasia are similar to that of Paget's disease and, if elevated serum levels of alkaline phosphatase and long-bone radiographs are positive, Paget's disease might be considered an appropriate diagnosis. 19.23 HISTOLOGIC ApPEARANCE
Histologically, irregular calcifications or mineralized deposits are found within a proliferating fibrous connective tissue stroma. This stroma can range from slightly mixoid to densely collagenous, and contain a prominent capillary network. The mineralized deposits within the stroma are irregularly shaped and variably sized; they consist of bony trabeculae with osteocytes or acellular cementum-like calcified masses.!? Melrose et all have found, in most cases, a mixture of cementum-like material and irregular trabeculae of bone, with some trabeculae rimmed by plump osteoblasts and others showing evidence of active resorption with apposed mullinucleated osteoclasts. Neville and Albenesius" indicate that the histologic features of the benign fibro-osseous lesion of florid osseous dysplasia may be histologically indistinguishable from those of a cernento-ossifying fibroma. Allhough certain radiographic and histologic
639
MUSELLA AND SLATER
characteristics have been ascribed to certain lesions within the fibro-osseous group, ie, "ground glass" radiographic appearance and C-shaped histologic trabeculae of fibrous dysplasia, and "cotton wool" radiographic appearance and mosaic histologic pattern of Paget's disease, radiographic features are probably more important than histologic features in determining the most appropriate diagnosis.i? GENETIC PREDISPOSITION
Reports substantiating a familial relationship or particular inheritance pattern to florid osseous dysplasia are so few that authorities find inadequate grounds to substantiate any apparent genetic predisposition for this disease. Familial gigantiform cementoma, reported by Agazzi and Belloni in 1953,21 described multiple benign cementomas occurring in two members of the same family. Cannon et al22 reported a case of mother and son with gigantiform cementoma in 1980. A third series was reported by Sedano et al23 in 1982 describing autosomal dominant cemental dysplasia in ten members of the same family. Our review of the English literature reoccurrence of florid osvealed no other familial . . seous dysplasia. Although florid osseous dysplasia is prevalent in black women, it is interesting to note that the af-· fected individuals in this case are of Italian descent, as were those reported with familial gigantiform cementomas by Agazzi and Belloni. 21 An autosomaldominant pattern has been reported previously.P and the presence of the disease in females of three consecutive generations in our report is consistent with such a mode of inheritance. Summary
Most cases of florid osseous dysplasia are asymptomatic and are found during routine radiographic examination. The radiographic spectrum is similar to that of other benign fibro-osseous lesions, beginning as a radiolucent lesion and becoming more radiopaque as the involved bone becomes more sclerotic. Many benign fibro-osseous lesions demonstrate similar histologic features, and without other radiographic and clinical information, a definitive diagnosis may be impossible. The dense bone of florid osseous dysplasia is very susceptible to infection and osteomyelitis, particularly with advancing years and in response to trauma. The disease may be self-limiting as reported by many authors, but as in this case, can be symptomatic for most of the person's lifetime. This presents a diagnostic and treatment dilemma for the clinician. Although this
entity has a distinct predilection for black women, more reported cases should be evaluated before the mode of inheritance can be established and before a tendency for familial cases in individuals of Italian descent can be confirmed.
Acknowledgment We wish to thank Dr Dennis DeWitt, who first recognized these familial osseous dysplasia cases and referred the family members to our institution for evaluation.
References 1. Melrose RJ, Abrams AM, Mills RG: Florid osseous dysplasia. Oral Surg 41:62, 1976 2. Hamner JE III, Scofield HH, Cornyn J: Benign fibroosseous jaw lesions of periodontal membrane origin: An analysis of 249 cases. Cancer 22:861, 1968 3. Laband PG, Leacock AG: Sclerosing osteitis of the jaws. J Oral Surg 25:23, 1967 4. Bell WH: Sclerosing osteomyelitis of the mandible and maxilla. Oral Surg 12:391, 1959 5. Bhaskar DN, Cutright DE: Multiple enostosis: Report of 16 cases. J Oral Surg 26:321, 1968 6. Lyons DC: Multiple osteomas of the mandible and maxilla. Oral Surg 8:738, 1955 7. Morgan GA, Poynton GH: Periapical cementoblastoma. Oral Surg 12:1344, 1959 8. Uthman A, AI-Shawaf M: Paget's disease of the mandible. Oral Surg 28:866, 1969 9. Pindborg JJ, Kramer IR, Torlini H: Histological typing of odontogenic tumors, jaw cysts, and allied lesions, in International Histological Classification of Tumors (ed 5). Geneva, World Health Organization, 1971, pp 332-334 10. Winer HJ, Goepp RA, Olson RE: Gigantiform cementomas resembling Paget's disease: Report of a case. J Oral Surg 30:517, 1972 11. Vanderwall I, Vanderkwast WAM: A case of gigantiforrn cementoma. 1nt J Oral Surg 3:440, 1974 12. Gorlin RJ, Chaudhry AP, Pindborg JJ: Odontogenic tumors, classification, histopathology, and clinical behavior in man and domesticated animals. Cancer 14:73, 1961 13. EI-Mofty S: Chronic diffuse sclerosing osteomyelitis. Oral Surg 36:898, 1973 14. Keen EG, Sammartino CA, Johnson ES: Chronic sclerosing osteomyelitis of the mandible. J Am Dent Assoc 76:597, 1968 15. Shafer WG: Chronic sclerosing osteomyelitis. J Oral Surg 15:138, 1957 16. Towns TL: Chronic diffuse sclerosing osteomyelitis of the maxilla and mandible. J Oral Surg 30:903, 1972 17. Waldron CA, Giansanti JS, Browand BC: Sclerotic cemental masses of the jaws (so called chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostoses, and gigantiform-cementoma). Oral Surg 39:590, 1975 18. Morgan GA, Morgan PR: Cysts and cyst-like lesions of the mandible. Dent Radiogr Photog 47:47, 1974 19. Ellis GL, Lewis DM, Carleton AS: Multiple osteosclerotic masses of the jaws. J Am Dent Assoc 114:678, 1987 20. Waldron CA: Fibro-osseous lesions of the jaws. J Oral MaxiIIofac Surg 43:249, 1985 21. Agazzi C, Belloni L: Gli Odontomi Duri Dei Mascellari. Arch Ital Otolaryngol 64:3, 1953 (suppl 16) 22. Cannon JS, Keller EE, Dahlin DC: Gigantiforrn cementoma: Report of two cases (mother and son). J Oral Surg 38:65, 1980 23. Sedano HO, Kuba R, Gorlin RJ: Autosomal dominant cemento-dysplasia. Oral Surg 54:642, 1982 24. Lichtenstein L, Jaffe HL: Fibrous dysplasia of the bone. Arch Pathol 33:777, 1942
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28. Neville BW, Albenesius RJ: The prevalence of benign fibroosseous lesions of periodontal ligament origin in black women: A radiographic survey. Oral Surg Oral Med Oral Pathol 62:340, 1986 29. Eversole LR, Sabes WR, Rovin S: Fibrous dysplasia: Anasologic problem in the diagnosis of fibro-osseous lesions of the jaws. J Oral Patholl:189, 1972
J Oral Maxillefao Surg 47:640-642,1989
Severe Facial Trauma After Orthognathic Surgery With Rigid Fixation GREGG W. WILSON, DMD* AND WILLIAM M. IOVINO, DMDt The use of rigid internal fixation has become widespread in the stabilization of osteotomy sites during orthognathic surgery. The advantages of rigid fixation and other various treatments currently in use have received much attention in the literature. These include a greatly decreased period of maxillomandibular fixation (MMF), decreased risk of postoperative aspiration, less skeletal relapse, better postoperative nutrition and oral hygiene, and increased patient comfort.ll'Also mentioned are an increased range of manidibular motion, and faster, stronger bony union. 6 , 15, 17, 18 These benefits of using rigid fixation have contributed to making orthognathic surgery more acceptable to patients and practitioners alike. This article describes a case in which a patient who had undergone orthognathic surgery with multiple rigidly fixed osteotomies was involved in a motor vehicle accident 6 weeks postoperatively, sustaining severe facial trauma. The findings in this case give clinical support to the advantages of rigid fixation. Report of a Case A 21-year-old white man was initially evaluated for surgical correction of skeletal facial deformity. He subsequently underwent a three-piece Lefort I osteotomy with impaction and transverse widening of the maxilla, bilateral sagittal ramus osteotomies of the mandible, and an advancement genioplasty. The maxillary segments were
* Attending Surgeon, University of Pittsburgh Hospitals; private practice, Indiana, Pennsylvania. t Attending Surgeon, University of Pittsburgh Hospitals; Private Practice, Monroeville, Pennsylvania. Address correspondence and reprint requests to Dr Wilson: 502 Airport Office and Professional Center, Indiana, PA 15701. © 1989
American Association of Oral and Maxillofacial Sur-
geons 0278-2391/89/4706-0018$3.00/0
fixed using Luhr (Howmedica Inc, Rutherford, NJ) fourhole miniplates and self-tapping screws. Two plates were positioned near the pyriform rims, and two were placed posteriorly in the zygomatic buttress regions (Fig 1). The sagittal ramus osteotomies were fixed with three Synthes (Synthes Ltd, Paoli, PA) screws placed from an extraoral approach on each side. The advancement genioplasty was fixed with 24-gauge stainless steel wires. Maxillomandibular fixation was applied, and all incisions closed. The patient's initial postoperative course was unremarkable. He was discharged on the third day, and MMF was removed on postoperative day 5. Because the maxilla had been segmented, the occlusal splint was retained on the maxillary arch for approximately 5 weeks. On removal of the splint, results of the procedure were quite good and the teeth were in the proper occlusion. On July 6, 1987, approximately 6 weeks after surgery, the patient was involved in a motor vehicle accident and sustained severe facial trauma with loss of conciousness. Clinical and radiographic examination revealed multiple facial fractures with displacement at several of the osteotomy sites. Physical examination revealed moderate facial edema and ecchymosis. The left eye showed evidence of entrapment of the extraocular muscles, with deficient upward gaze. A step defect was palpable at the left infraorbital rim. The maxilla was displaced in an inferior and posterior position causing an anterior open bite. There was obvious mobility and a step defect in the incisor region of the mandible. Also apparent was a nasal septal deviation to the left and some crepitus of the nasal bridge. There were no other apparent injuries, and the patient was neurologically stable. Radiographic studies showed the extent of the facial injuries (Figs 2 and 3). The maxilla was separted across the previous Lefort I site, with the bone plates and screws of the left side tearing through the lateral maxillary wall, causing gross comminution. In addition, the inferior and lateral orbital rims were torn by the posterior bone plate, and the periorbita was entrapped in several small bone fragments. The right maxillary bone plates also tore through the fragile antral wall, but without the degree of comminution seen on the left side. A slightly displaced nasal bone fracture was also noted. The mandible was fractured through the superior portion of the parasyrnphysis to the level of the genioplasty cut. The genial segment also was separated, creating a full-thickness man-