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Osteomyelitis of the mandible in a patient with dysosteosclerosis
Osteomyelitis of the mandible in a patient with dysosteosclerosis Report of a case Garnet V. Packota, DMD. MSc,” Jack ShijYman, BSc, DDS,b and J. Michael Hail, DDS,’ Saskatoon, Saskatchewan, Canada DEPARTMENT
OF DIAGNOSTIC
AND SURGICAL
SCIENCES,
COLLEGE
OF DENTISTRY,
UNIVERSITY
OF SASKATCHEWAN Dysosteosclerosis is a rare bone dysplasia that has radiographic, histopathologic, and clinical similarities to osteopetrosis. This article reviews the dental findings reported in patients with dysosteosclerosis and presents the first reported case of osteomyelitis of the mandible in a patient with this disease. This is also the first report that demonstrates ankylosis of impacted teeth in dysosteosclerosis. (ORAL SURC ORAL MED ORAL PATHOL 1991;71:144-7)
D
ysosteosclerosisis a rare bone dysplasia characterized clinically by small stature, blindness, increasedbone fragility, and occasionally other neurologic and skin manifestations.’ Radiologic findings in the skeleton include thickening and sclerosis of the baseof the skull, flattening, dorsal wedging, and sclerosis of the vertebral bodies, sclerosis of numerous other bones, and Erlenmeyer flask-like metaphyseal flaring of the tubular bones.le3The progressive thickening and sclerosis of the base of the skull often impinges on the optic canals, leading to blindness.2The diseaseusually manifests itself in early childhood.’ Dysosteosclerosisis believed to be transmitted asan autosomal recessive trait, but four caseshave been shown to have sex-linked recessive inheritance.4 Dental anomalies reported include delay in or lack of eruption of deciduous and permanent teeth,‘, 2.4-6 rudimentary development of teeth,5 enamel hypoplasia,‘, 2,4 malalignment of teeth,4,6 caries,4,6 and premature loss of teeth.4,7 There was apparently normal development of the dentition in one case.3 This article presents the first case history of a patient with dysosteosclerosisin whom osteomyelitis of the mandible developed.His clinical and radiographic findings have been previously reported.2
aAssociate Professor, Oral Radiology. bClinicaI Associate Professor, Oral Surgery. ‘Assistant Professor, Oral Pathology. 7/12/15676 144
1.
Panoramic radiograph.
CASEREPORT
A blind 27-year-old white man, short in stature, had pain and swelling of the right mandibular alveolus of 3 weeks’ duration. The pain and swelling arose shortly after he noticed a small, hard object penetrating the mucosaof the alveolar ridge in the mandibular right premolar area. On examination, the jaws were clinically edentulous and atrophic. There was a fluctuant swelling of the right mandibular alveolar ridge extending lingually in the canine region and buccally in the molar region. A yellow-white “toothlike” structure was seen penetrating the mucosa in the canine region. Pus could be expressedthrough the mucosal defect by digital pressure. A panoramic radiograph (Fig. 1) showed numerous impacted malformed teeth with hypoplastic enamel caps in both jaws. There was an area of radiolucency in the alveolar bone of the mandible at the site of the mucosal defect just described.The condylar headswere relatively large and demonstrated a posterior inclination. The inferior mandib-
Volume Number
Osteomyelitis in dysosteosclerosis 145
7I 2
Fig.
3. Ankylosis of tooth root (lef) with bone (right).
Fig. 2. A, Photomicrograph of biopsy specimen. Nonvital sclerotic bone exhibiting mosaic pattern with reversal lines surrounded by bacterial colonies. B, Photomicrograph of biopsy specimen. Nonvital sclerotic bone surrounded by inflammatory infiltrate.
ular cortex was thickened. The mandible lacked normal trabecular bone structure. The maxillary sinuses were hypoplastic or absent. The glenoid fossaewere not well demonstrated. The zygomatic arches and temporal bones were abnormally radiopaque. With the patient under general anesthesia, a flap was raised from the mandibular right retromolar area along the crest of the alveolar ridge as far forward as the midline. Areas of friable hemorrhagic tissue were present on the lingual aspect of the residual ridge in the region of the symphysis and on the buccal aspectof the first molar area. Several pieces of bone were removed with a surgical bur, but attempts to surgically remove someof the hypoplastic teeth proved to be difficult becauseof apparent ankylosis. Chronic inflammatory tissue was removed by curettage. An epidural catheter was inserted throughout the area of pathosis and led out via a stab incision in the vestibule at the midline. The incision was closed by vertical mattress sutures. The surgical area was irrigated with a bacitracin solution four times a day for 5 days via the epidural catheter. All tissue removed was submitted for histologic examination. Microscopic examination of the bone fragments (Fig. 2) disclosed densesclerotic immature bundle bone exhibiting a mosaic pattern with numerous reversal lines;
Fig.
4. Posteroanterior cephalogram.
someareas of bone were nonvital and were surrounded by bacterial colonies and inflammatory cells. A diagnosis of bone sclerosisand osteomyelitis was given. The structure of the dentin, pulp, and cementum of the tooth fragments appeared to be normal, but there was lack of a normal periodontal ligament in some areas, and the tooth roots appeared to be fused to the alveolar bone by osteodentin (Fig. 3). The soft tissue specimenrevealed a cyst lined with a thin layer of inflamed epithelium associatedwith a moderate to heavy mixed inflammatory cell infiltrate in the cyst wall. Fragments of nonvital bone were also noted. Postoperative healing was uneventful. Some alveolar
146
Packota, Shiflman, and Hall
Fig.
5. Lateral cephalogram.
bone height was lost in the area of surgery close to the mandibular right canine region, and an alveolar mucosal defect remained on the lingual surface of this area, through which one of the impacted hypoplastic teeth was visible. The need for proper oral hygiene measures to prevent future infections was impressed on the patient. Despite the persistence of the mucosal defect through which a toothlike structure and bone were showing, the ankylosis of the impacted teeth and the possibility of trauma resulting in additional bone loss precluded further surgical treatment. The patient was referred for prosthodontic consultation in regard to fabrication of a new set of complete dentures. Posteroanterior and lateral cephalometric radiographs taken at the time of this referral revealed numerous abnormal findings (Figs. 4 and 5). The baseof the skull, the calvarium, the mandible, and the cervical vertebral bodieswere densely radiopaque. There was no evidence of pneumatization of the paranasal air sinuses or the mastoid processes. The pituitary fossaappearedto be small, but this area may have beenobscured by the overlying densebone of the skull. The bodies of the cervical vertebrae were unusually radiopaque, lacked evidence of normal cancellous bone, and appeared flattened and biconcave; several demonstrated anterior concavity. The jaws were small, and the mandibular angle was extremely obtuse. The posteroanterior view showed lateral flaring of the mandibular angles. DISCUSSION
The differential diagnosis initially considered for this patient included osteopetrosis and craniometa-
physeal dysplasia, but clinical, laboratory, and radiographic findings did not support either of these. Ultimately, a diagnosis of dysosteosclerosiswas made.2 Dental findings in patients with dysosteosclerosis have not been extensively described. The dental findings in the patient reported here were first described
briefly by Houston and coworkers*; however, no mention was made of the abnormal structure of the impacted teeth. The reports of Spranger and associates’ and Leisti and others6 were similar to that of Houston and coworkers. Utz5 reported that in his case the development of the dentition was “arrested” with teeth being rudimentally formed and retained in the jaw. Fryns and coworkers” were the only authors to report normal development of the teeth. Utz5 speculated that the disturbance of resorptive processesprevents normal tooth eruption; this would apparently be the result of a paucity of osteoclasts.2,6 However, ankylosis of the teeth is probably at least partially responsible for the lack of eruption. No reports appear to exist in the English-language literature demonstrating ankylosis of unerupted teeth in dysosteosclerosis. The histologic findings of the bone specimen from our patient were nonspecific, but consistent with those described for dysosteosclerosis.6,8 However, virtually all bone submitted in the biopsy was necrotic as a result of osteomyelitis. There are no reports of infection of the jawbones of any patients with dysosteosclerosis.Osteomyelitis and necrosis of the mandible resulting from the poor vascular supply of the bone is an occasional complication of osteopetrosis.9slo Because of the histologic similarities of bone structure seen in dysosteosclerosisand osteopetrosis,6% 8 osteomyelitis of the mandible must also be considered to be a potential clinical problem in patients with dysosteosclerosis. Therefore efforts must be made to avoid the introduction of infection into the jawbones of these patients. In this case the close proximity of the impacted tooth to the base of the alveolar mucosa probably resulted in the ulceration seen clinically, and oral bacteria were likely introduced into the jawbone through this defect. Both the abnormal density of the bone and ankylosis of the impacted teeth made the surgical management of the patient reported here difficult. Removal of impacted teeth in patients with dysosteosclerosis should be avoided, if possible, to avoid unnecessary trauma and introduction of infection. Since permanent teeth do not appear to erupt in thesepatients, preventive measuresshould be directed to maintaining the health and function of any erupted deciduous teeth if they are functional, and extracting deciduous teeth if they are carious and unrestorable. For clinically edentulous patients, complete dentures appear to be the treatment of choice. The function and esthetics gained through complete denture fabrication, however, must be weighed against the risk of introducing infection into the jawbones through ulceration of the mucosa by denture trauma.
Volume 7 1 Number 2 REFERENCES 1. Spranger JW, Langer LO, Wiedemann H-R. Bone dysplasias. An atlas of constitutional disorders of skeletal development. Philadelphia: WB Saunders, 1974:295-7. 2. Houston CS, Gerrard JW, Ives EJ. Dysosteosclerosis.Am J Roentgen01 1978;130:988-91. 3. Fryns JP, Vinken L, ClaessensS, Marien J, Geutjens J, Vanden Berghe H. Dysosteosclerosisin a mentally retarded boy. Acta Paediatr Belg 1980;33:53-6. 4. Pascual-Castroviejo I, Casas-Fernandez C, Lopez-Martin V, Martinez-Bermejo A. X-linked dysosteosclerosis.Eur J Pediatr 1977;126:127-38. 5. Utz VW. Manifestation der Dysosteoskleroseim Kieferbereich. Dtsch Zahnaerztl Z 1970;25:48-50. 6. Leisti J, Kaitila I, Lachman RS, Asch MJ, Rimoin DL. Dysosteosclerosis.Birth Defects 1975;11:349-51.
Osteomyelitis in dysosteosclerosis 147 7. Roy C, Maroteaux P, Kremp L, Courtecuisse V, Alaguille D. Un nouveau syndrome osseux avec anomalies cutanies et troubles neurologiques. Arch Fr Pediatr 1968;25:893-905. 8. Kaitila I, Rimoin DL. Histologic heterogeneity in the hyperostotic bone dysplasias. Birth Defects 1976;12:71-9. 9. Aegerter E, Kirkpatrick JA. Orthopedic diseases. Philadelphia: WB Saunders Company, 1975:147-54. 10. Steiner M, Gould A, Means W. Osteomyelitis of the mandible associatedwith osteopetrosis.J Oral Maxillofac Surg 1983;41: 395-405. Reprint requests to: Garnet V. Packota, DMD, MSc Dental Clinic, University of Saskatchewan Saskatoon, Saskatchewan Canada S7N OWO
OF DIAGNOSTIC
AND SURGICAL
SCIENCES,
COLLEGE
OF DENTISTRY,
UNIVERSITY
OF SASKATCHEWAN Dysosteosclerosis is a rare bone dysplasia that has radiographic, histopathologic, and clinical similarities to osteopetrosis. This article reviews the dental findings reported in patients with dysosteosclerosis and presents the first reported case of osteomyelitis of the mandible in a patient with this disease. This is also the first report that demonstrates ankylosis of impacted teeth in dysosteosclerosis. (ORAL SURC ORAL MED ORAL PATHOL 1991;71:144-7)
D
ysosteosclerosisis a rare bone dysplasia characterized clinically by small stature, blindness, increasedbone fragility, and occasionally other neurologic and skin manifestations.’ Radiologic findings in the skeleton include thickening and sclerosis of the baseof the skull, flattening, dorsal wedging, and sclerosis of the vertebral bodies, sclerosis of numerous other bones, and Erlenmeyer flask-like metaphyseal flaring of the tubular bones.le3The progressive thickening and sclerosis of the base of the skull often impinges on the optic canals, leading to blindness.2The diseaseusually manifests itself in early childhood.’ Dysosteosclerosisis believed to be transmitted asan autosomal recessive trait, but four caseshave been shown to have sex-linked recessive inheritance.4 Dental anomalies reported include delay in or lack of eruption of deciduous and permanent teeth,‘, 2.4-6 rudimentary development of teeth,5 enamel hypoplasia,‘, 2,4 malalignment of teeth,4,6 caries,4,6 and premature loss of teeth.4,7 There was apparently normal development of the dentition in one case.3 This article presents the first case history of a patient with dysosteosclerosisin whom osteomyelitis of the mandible developed.His clinical and radiographic findings have been previously reported.2
aAssociate Professor, Oral Radiology. bClinicaI Associate Professor, Oral Surgery. ‘Assistant Professor, Oral Pathology. 7/12/15676 144
1.
Panoramic radiograph.
CASEREPORT
A blind 27-year-old white man, short in stature, had pain and swelling of the right mandibular alveolus of 3 weeks’ duration. The pain and swelling arose shortly after he noticed a small, hard object penetrating the mucosaof the alveolar ridge in the mandibular right premolar area. On examination, the jaws were clinically edentulous and atrophic. There was a fluctuant swelling of the right mandibular alveolar ridge extending lingually in the canine region and buccally in the molar region. A yellow-white “toothlike” structure was seen penetrating the mucosa in the canine region. Pus could be expressedthrough the mucosal defect by digital pressure. A panoramic radiograph (Fig. 1) showed numerous impacted malformed teeth with hypoplastic enamel caps in both jaws. There was an area of radiolucency in the alveolar bone of the mandible at the site of the mucosal defect just described.The condylar headswere relatively large and demonstrated a posterior inclination. The inferior mandib-
Volume Number
Osteomyelitis in dysosteosclerosis 145
7I 2
Fig.
3. Ankylosis of tooth root (lef) with bone (right).
Fig. 2. A, Photomicrograph of biopsy specimen. Nonvital sclerotic bone exhibiting mosaic pattern with reversal lines surrounded by bacterial colonies. B, Photomicrograph of biopsy specimen. Nonvital sclerotic bone surrounded by inflammatory infiltrate.
ular cortex was thickened. The mandible lacked normal trabecular bone structure. The maxillary sinuses were hypoplastic or absent. The glenoid fossaewere not well demonstrated. The zygomatic arches and temporal bones were abnormally radiopaque. With the patient under general anesthesia, a flap was raised from the mandibular right retromolar area along the crest of the alveolar ridge as far forward as the midline. Areas of friable hemorrhagic tissue were present on the lingual aspect of the residual ridge in the region of the symphysis and on the buccal aspectof the first molar area. Several pieces of bone were removed with a surgical bur, but attempts to surgically remove someof the hypoplastic teeth proved to be difficult becauseof apparent ankylosis. Chronic inflammatory tissue was removed by curettage. An epidural catheter was inserted throughout the area of pathosis and led out via a stab incision in the vestibule at the midline. The incision was closed by vertical mattress sutures. The surgical area was irrigated with a bacitracin solution four times a day for 5 days via the epidural catheter. All tissue removed was submitted for histologic examination. Microscopic examination of the bone fragments (Fig. 2) disclosed densesclerotic immature bundle bone exhibiting a mosaic pattern with numerous reversal lines;
Fig.
4. Posteroanterior cephalogram.
someareas of bone were nonvital and were surrounded by bacterial colonies and inflammatory cells. A diagnosis of bone sclerosisand osteomyelitis was given. The structure of the dentin, pulp, and cementum of the tooth fragments appeared to be normal, but there was lack of a normal periodontal ligament in some areas, and the tooth roots appeared to be fused to the alveolar bone by osteodentin (Fig. 3). The soft tissue specimenrevealed a cyst lined with a thin layer of inflamed epithelium associatedwith a moderate to heavy mixed inflammatory cell infiltrate in the cyst wall. Fragments of nonvital bone were also noted. Postoperative healing was uneventful. Some alveolar
146
Packota, Shiflman, and Hall
Fig.
5. Lateral cephalogram.
bone height was lost in the area of surgery close to the mandibular right canine region, and an alveolar mucosal defect remained on the lingual surface of this area, through which one of the impacted hypoplastic teeth was visible. The need for proper oral hygiene measures to prevent future infections was impressed on the patient. Despite the persistence of the mucosal defect through which a toothlike structure and bone were showing, the ankylosis of the impacted teeth and the possibility of trauma resulting in additional bone loss precluded further surgical treatment. The patient was referred for prosthodontic consultation in regard to fabrication of a new set of complete dentures. Posteroanterior and lateral cephalometric radiographs taken at the time of this referral revealed numerous abnormal findings (Figs. 4 and 5). The baseof the skull, the calvarium, the mandible, and the cervical vertebral bodieswere densely radiopaque. There was no evidence of pneumatization of the paranasal air sinuses or the mastoid processes. The pituitary fossaappearedto be small, but this area may have beenobscured by the overlying densebone of the skull. The bodies of the cervical vertebrae were unusually radiopaque, lacked evidence of normal cancellous bone, and appeared flattened and biconcave; several demonstrated anterior concavity. The jaws were small, and the mandibular angle was extremely obtuse. The posteroanterior view showed lateral flaring of the mandibular angles. DISCUSSION
The differential diagnosis initially considered for this patient included osteopetrosis and craniometa-
physeal dysplasia, but clinical, laboratory, and radiographic findings did not support either of these. Ultimately, a diagnosis of dysosteosclerosiswas made.2 Dental findings in patients with dysosteosclerosis have not been extensively described. The dental findings in the patient reported here were first described
briefly by Houston and coworkers*; however, no mention was made of the abnormal structure of the impacted teeth. The reports of Spranger and associates’ and Leisti and others6 were similar to that of Houston and coworkers. Utz5 reported that in his case the development of the dentition was “arrested” with teeth being rudimentally formed and retained in the jaw. Fryns and coworkers” were the only authors to report normal development of the teeth. Utz5 speculated that the disturbance of resorptive processesprevents normal tooth eruption; this would apparently be the result of a paucity of osteoclasts.2,6 However, ankylosis of the teeth is probably at least partially responsible for the lack of eruption. No reports appear to exist in the English-language literature demonstrating ankylosis of unerupted teeth in dysosteosclerosis. The histologic findings of the bone specimen from our patient were nonspecific, but consistent with those described for dysosteosclerosis.6,8 However, virtually all bone submitted in the biopsy was necrotic as a result of osteomyelitis. There are no reports of infection of the jawbones of any patients with dysosteosclerosis.Osteomyelitis and necrosis of the mandible resulting from the poor vascular supply of the bone is an occasional complication of osteopetrosis.9slo Because of the histologic similarities of bone structure seen in dysosteosclerosisand osteopetrosis,6% 8 osteomyelitis of the mandible must also be considered to be a potential clinical problem in patients with dysosteosclerosis. Therefore efforts must be made to avoid the introduction of infection into the jawbones of these patients. In this case the close proximity of the impacted tooth to the base of the alveolar mucosa probably resulted in the ulceration seen clinically, and oral bacteria were likely introduced into the jawbone through this defect. Both the abnormal density of the bone and ankylosis of the impacted teeth made the surgical management of the patient reported here difficult. Removal of impacted teeth in patients with dysosteosclerosis should be avoided, if possible, to avoid unnecessary trauma and introduction of infection. Since permanent teeth do not appear to erupt in thesepatients, preventive measuresshould be directed to maintaining the health and function of any erupted deciduous teeth if they are functional, and extracting deciduous teeth if they are carious and unrestorable. For clinically edentulous patients, complete dentures appear to be the treatment of choice. The function and esthetics gained through complete denture fabrication, however, must be weighed against the risk of introducing infection into the jawbones through ulceration of the mucosa by denture trauma.
Volume 7 1 Number 2 REFERENCES 1. Spranger JW, Langer LO, Wiedemann H-R. Bone dysplasias. An atlas of constitutional disorders of skeletal development. Philadelphia: WB Saunders, 1974:295-7. 2. Houston CS, Gerrard JW, Ives EJ. Dysosteosclerosis.Am J Roentgen01 1978;130:988-91. 3. Fryns JP, Vinken L, ClaessensS, Marien J, Geutjens J, Vanden Berghe H. Dysosteosclerosisin a mentally retarded boy. Acta Paediatr Belg 1980;33:53-6. 4. Pascual-Castroviejo I, Casas-Fernandez C, Lopez-Martin V, Martinez-Bermejo A. X-linked dysosteosclerosis.Eur J Pediatr 1977;126:127-38. 5. Utz VW. Manifestation der Dysosteoskleroseim Kieferbereich. Dtsch Zahnaerztl Z 1970;25:48-50. 6. Leisti J, Kaitila I, Lachman RS, Asch MJ, Rimoin DL. Dysosteosclerosis.Birth Defects 1975;11:349-51.
Osteomyelitis in dysosteosclerosis 147 7. Roy C, Maroteaux P, Kremp L, Courtecuisse V, Alaguille D. Un nouveau syndrome osseux avec anomalies cutanies et troubles neurologiques. Arch Fr Pediatr 1968;25:893-905. 8. Kaitila I, Rimoin DL. Histologic heterogeneity in the hyperostotic bone dysplasias. Birth Defects 1976;12:71-9. 9. Aegerter E, Kirkpatrick JA. Orthopedic diseases. Philadelphia: WB Saunders Company, 1975:147-54. 10. Steiner M, Gould A, Means W. Osteomyelitis of the mandible associatedwith osteopetrosis.J Oral Maxillofac Surg 1983;41: 395-405. Reprint requests to: Garnet V. Packota, DMD, MSc Dental Clinic, University of Saskatchewan Saskatoon, Saskatchewan Canada S7N OWO