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Osteomyelitis of the Maxilla Secondary to Osteopetrosis: A Report of 2 Cases in Sisters
144
OSTEOMYELITIS OF THE MAXILLA
J Oral Maxillofac Surg 65:144-147, 2007
Osteomyelitis of the Maxilla Secondary to Osteopetrosis: A Report of 2 Cases in Sisters Conor P. Barry, MB, MFD(RCSI),* C. David Ryan, FFDRCS, FRCSI,† and Leo F.A. Stassen, FDSRCS, FRCS, MA‡ The incidence of osteomyelitis has dramatically reduced since the introduction of antibiotics. When it now occurs in Western society, the possibility of predisposing immunosuppressive conditions or underlying bony pathology should be considered. Osteomyelitis is a complication in 10% of cases of osteopetrosis.1 Osteopetrosis (marble bone disease, Albers-Schönberg disease, osteosclerosis fragilis generalisata) is a congenital sclerosing disease of bone caused by aberrant osteoclast mediated bone resorption.2 There is normal production of bone with lack of physiological resorption. The disease represents a spectrum of clinical variants because of the heterogeneity of genetic defects resulting in osteoclast dysfunction.3 Three clinically distinct forms are recognized: an infantile malignant autosomal recessive form, an intermediate autosomal recessive form, and an adult benign autosomal dominant form. Infantile malignant autosomal recessive osteopetrosis is diagnosed within the first year of life. Patients typically present with pathologic fracture or failure to thrive with frequent respiratory, head and neck, or bony infections. Physical examination shows lethargy, small stature, marked hepatosplenomegaly, and dysmorphic features including macrocephaly, frontal
Received from the Department of Oral and Maxillofacial Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Dublin Dental Hospital, Dublin, Ireland. *Registrar. †Senior Lecturer/Consultant. ‡Professor. Address correspondence and reprint requests to Dr Barry: Department of Oral and Maxillofacial Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Dublin Dental Hospital, Lincoln Place, Dublin 2, Ireland; e-mail: [email protected] © 2007 American Association of Oral and Maxillofacial Surgeons
0278-2391/07/6501-0028$32.00/0 doi:10.1016/j.joms.2005.07.019
bossing, and hypertelorism. Neural palsies may occur, particularly affecting the optic, oculomotor, and facial nerves because of progressive thickening of bone at the expense of the neural canals. Intermediate autosomal recessive osteopetrosis is usually diagnosed toward the end of the first decade of life. Patients often present with pathological fracture. Clinical characteristics include delayed motor and cognitive development, mild disproportionate short stature, mild hepatosplenomegaly, and dysmorphic features such as macrocephaly and frontal bossing. Cranial nerve compression and mandibular osteomyelitis are common features. This form of osteopetrosis is rare. Most patients survive into adulthood but with significant disability. Autosomal dominant osteopetrosis (adult benign osteopetrosis) is a much milder condition. Patients have a normal life expectancy and many are asymptomatic. Patients may present in adulthood with recurrent fractures, back pain, bone pain, or osteomyelitis, particularly of the mandible. Cranial nerve palsies and osteoarthritis of the hips and knees are other potential features. Autosomal dominant osteopetrosis is divided into 2 radiological subtypes. In type I, cranial sclerosis and thickening affects mainly the calvarium. The calvarium is almost normal in type II, but the cranial base is sclerotic. Although both types involve generalized skeletal sclerosis, patients with the type II subtype have a higher risk for fractures. DIAGNOSIS
The diagnosis remains radiographic4 and is characterized by diffuse, increased radiodensity. Other features include absence of medullary cavities, widened metaphyses, “bone within bone” appearance especially of the pelvis, “rugger jersey spine,” and cranial sclerosis with “hair on end” appearance. The paranasal sinuses may be poorly formed or obliterated. The radiographic appearance varies between the different
145
BARRY ET AL
Table 1. DIAGNOSTIC FEATURES OF OSTEOPETROSIS
Radiographic Features
Laboratory Features
Infantile malignant: (autosomal recessive)
Osteosclerosis; metaphyseal widening; sclerosis of cranial base; calvarial “hair on end” appearance; underdevelopment of paranasal sinuses
Hb 2 WCC 2 Platelet 2 Acid phosphatase 1 1,25-dihydroxy vitamin D 1
Intermediate: (autosomal recessive)
Generalized skeletal sclerosis with medullary obliteration
Hb 2 Acid phosphatase 1
Adult benign: (autosomal dominant)
Generalized skeletal sclerosis. Type I: Cranial sclerosis affects mainly calvarium Type II: Sclerotic cranial base with normal calvarium
Hb Normal or 2 Acid phosphatase: Type I Normal Type II 1
Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
forms (Table 1). The hematological profile also depends on the clinical variant of osteopetrosis. In the infantile malignant form, encroachment of sclerotic bone on the marrow spaces results in pancytopenia and extramedullary hemopoiesis. Mild to moderate anemia may be present in the intermediate form. In the autosomal dominant form, there may be no hematological abnormalities. However, anemia of chronic disease may complicate the hematological picture where conditions such as osteoarthritis or osteomyelitis are present. Serum biochemistry also varies depending on the clinical variant. Calcium, phosphorus, and alkaline phosphatase levels are usually normal. Acid phosphatase is elevated in the infantile malignant, intermediate, and autosomal dominant type II forms, but normal in the autosomal dominant type I form. Levels of 1,25-dihydroxy vitamin D are invariably elevated in the infantile malignant form. MANAGEMENT
Medical treatment is based on efforts to stimulate host osteoclasts or to provide an alternative source of osteoclasts.2 Dietary calcium restriction and highdose calcitriol (1,25-dihydroxy vitamin D) have been used to stimulate osteoclast formation and bone resorption. High-dose corticosteroids and parathyroid hormone can improve hematopoiesis and bone resorption. Long-term administration of human interferon-gamma has been shown to stimulate bone resorption, hematopoiesis, and superoxide production of leukocytes. Osteomyelitis complicates osteopetrosis in 10% of cases1 due to impaired white cell function and reduced vascular supply. The most common site of involvement is the mandible.2 Osteomyelitis of the maxilla is very rare, attributed to its thin cortical bone and rich collateral blood supply. This article reports 2
cases of aggressive osteomyelitis of the maxilla occurring secondary to osteopetrosis in 2 sisters and highlights the difficulties in managing these patients.
Report of Cases CASE 1 A 28-year-old marginally intellectually challenged woman was referred to the maxillofacial department by her general dentist for investigation of poorly healing extraction sockets. He reported that her caregivers were concerned about her severe halitosis and finding blood on her pillow every morning. She had a history of multiple fractures attributed to osteogenesis imperfecta. Examination showed a poorly healing extraction socket in the upper left quadrant with bony sequestrae. There was a large (1 cm ⫻ 2 cm) oro-antral fistula (OAF) in the upper left buccal sulcus. Mucopurulent discharges were noted from both middle meati. Conventional panoramic and computed tomography (CT) imaging was performed. The extraction socket and surrounding bone in the region of the maxillary left first molar had a moth-eaten appearance and there was evidence of sequestrum formation (Fig 1). There was extensive bony destruction of the left nasal cavity and maxillary sinus extending inferiorly to the hard palate and laterally into the infratemporal fossa (Fig 2). A differential diagnosis of Wegener’s granulomatosis, antral malignancy, or severe osteomyelitis was considered. The patient’s chest radiographs on admission showed diffuse osteosclerosis of the ribs and thoracic vertebrae (Fig 3). The radiologic findings were sufficiently characteristic for a diagnosis of osteopetrosis to be made. Her admission bloods showed normal white cell and normal red cell counts but with microcytic, hypochromic anemia (hemoglobin 7.8 g/dL, HCT 0.268, MCV 68.5, MCH 19.8 pg). Differential white cell count showed lowered lymphocytes (1.36 ⫻ 109). She had a raised erythrocyte sedimentation rate (32 mm first Hr) and a raised serum phosphate level. The previous diagnosis of osteogenesis imperfecta was reviewed and altered to generalized osteopetrosis. A biopsy of the lateral wall of the nose and antrum and antral polypectomy were performed. The histopathology was reported as consistent with osteomyelitis.
146
OSTEOMYELITIS OF THE MAXILLA
FIGURE 1. Orthopantomograph showing bony resorption of the left maxilla. Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
The patient was treated empirically with metronidazole 500 mg and cefuroxime 750 mg intravenously 3 times daily along with bethamethasone sodium phosphate 0.1% nasal drops. She improved after a few days and was transferred to outpatient care. Her condition regressed within a month. Conservative management was favored due to her general
medical condition and social circumstances. The condition remains unresolved for the past 5 years and acute episodes are managed with antibiotic therapy.
FIGURE 2. Chest radiograph showing osteosclerosis of the ribs and thoracic vertebrae.
FIGURE 3. Computed tomography of the skull showing extensive bony destruction of the left midface.
Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
CASE 2 The younger sister (27 years old at the time of referral) of patient 1 was referred to the maxillofacial department 3 years after her sister for investigation of severe halitosis and a chronic nasal discharge for over 1 year. She was severely intellectually impaired and had previously been diagnosed with osteopetrosis based on a clinical history of multiple fractures and characteristic radiological features. Admission blood tests were all normal (hemoglobin 13.3 g/dL; white cell count 7.34 ⫻ 109). Radiographs showed the characteristic features of osteopetrosis. Examination under general anesthetic showed an OAF (1 cm ⫻ 1 cm in size) with a purulent discharge in the region of the upper right
147
BARRY ET AL second premolar and first molar. An antral wash with debridement of the OAF was performed. Histopathology was reported as osteomyelitis of osteopetrotic bone. The OAF was closed with a buccal advancement mucoperiosteal flap. Co-amoxiclav 1.2 g intravenously 3 times daily was prescribed. The condition has maintained a chronic course.
Discussion Osteomyelitis is a well-recognized complication of osteopetrosis.1,3-11 Some patients with osteopetrosis may be asymptomatic and many cases are initially diagnosed when the patient presents with osteomyelitis.7 The diagnosis of osteopetrosis is based on a history of numerous fractures and radiological findings of osteosclerosis, although the radiological features are sufficiently characteristic for a diagnosis to be made with certainty.4,8 Osteomyelitis secondary to osteopetrosis tends to be refractory due to the reduced blood supply and accompanying anemia and neutropenia. There are few reports of successful treatment and in many cases the osteomyelitis remains unresolved.5,6 Treatment of osteomyelitis of the jaws includes incision and drainage, antibiotics, sequestrectomy, extraction of teeth, saucerization, decortication, resection of the jaw, and hyperbaric oxygen. Resection and hyperbaric oxygen therapy are the only methods of treatment reported as being successful where the osteomyelitis is secondary to osteopetrosis.6 Obturators are the favored method of filling the maxillary defect.7,8 Free bone grafting is not recommended as the blood supply to the graft bed is compromised. Use of a free vascularized, myo-osseous flap may be more favorable but may be precluded due to absence of a suitable donor site in these patients.9 The 2 patients reported were sisters living in different long-term care facilities and were unknown to each other. The management of their osteomyelitis was compromised not only by their underlying medical condition but also by their social circumstances and intellectual impairment. Patients presenting with osteomyelitis of the jaws should be questioned about a history of recurrent
fractures. Skull and chest radiographs should be obtained as well as full blood count and renal and bone profiles to screen for abnormalities consistent with osteopetrosis (Table 1). If a diagnosis of osteopetrosis is made, medical consultation should be sought, as management of the underlying osteopetrosis may improve outcome. In conclusion, osteomyelitis is now such a rare entity that when presented with osteomyelitis of the maxilla or intractable osteomyelitis of the mandible, the maxillofacial surgeon should consider the possibility of osteopetrosis as an underlying pathology and investigate appropriately. Family members of patients with osteopetrosis should be screened for osteopetrosis and if present, conservative dental plans recommended to avoid potentially intractable osteomyelitis.
References 1. Johnston C, Lavy N, Lord T, et al: Osteopetrosis: A clinical, genetic, metabolic, and morphologic study of the dominantly inherited, benign form. Medicine 47:149, 1968 2. Kocher M, Kasser J: Osteopetrosis. Am J Orthop 32:222, 2003 3. Popoff S, Marks S: The heterogeneity of the osteopetrosis reflects the diversity of cellular influences during skeletal development. Bone 17:437, 1995 4. Stoker D: Osteopetrosis. Semin Musculoskelet Radiol 6:299, 2002 5. Barry CP, Ryan CD: Osteomyelitis of the maxilla secondary to osteopetrosis: Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 95:12, 2003 6. Steiner M, Gould A, Means W: Osteomyelitis of the mandible associated with osteopetrosis. J Oral Maxillofac Surg 41:395, 1983 7. Hanada T, Furuta S, Moriyama I, et al: Maxillary osteomyelitis secondary to osteopetrosis. Rhinology 34:242, 1996 8. Crockett D, Stanley R, Lubka R: Osteomyelitis of the maxilla in a patient with osteopetrosis (Albers-Schonberg disease). Otolaryngol Head Neck Surg 95:117, 1986 9. Bakeman R, Abdelsayad R, Sutley S, et al: Osteopetrosis: A review of the literature and report of a case complicated by osteomyelitis of the mandible. J Oral Maxillofac Surg 56:1209, 1988 10. Lawoyin D, Daramola J, Ajagbe H, et al: Osteomyelitis of the mandible associated with osteopetrosis: Report of a case. Br J Oral Maxillofac Surg 26:330, 1988 11. Fernandez J, Frias M, Hernandez S: Infantile osteopetrosis: A case report with osteomyelitis of the maxilla. J Clin Pediatr Dent 27:77, 2002
OSTEOMYELITIS OF THE MAXILLA
J Oral Maxillofac Surg 65:144-147, 2007
Osteomyelitis of the Maxilla Secondary to Osteopetrosis: A Report of 2 Cases in Sisters Conor P. Barry, MB, MFD(RCSI),* C. David Ryan, FFDRCS, FRCSI,† and Leo F.A. Stassen, FDSRCS, FRCS, MA‡ The incidence of osteomyelitis has dramatically reduced since the introduction of antibiotics. When it now occurs in Western society, the possibility of predisposing immunosuppressive conditions or underlying bony pathology should be considered. Osteomyelitis is a complication in 10% of cases of osteopetrosis.1 Osteopetrosis (marble bone disease, Albers-Schönberg disease, osteosclerosis fragilis generalisata) is a congenital sclerosing disease of bone caused by aberrant osteoclast mediated bone resorption.2 There is normal production of bone with lack of physiological resorption. The disease represents a spectrum of clinical variants because of the heterogeneity of genetic defects resulting in osteoclast dysfunction.3 Three clinically distinct forms are recognized: an infantile malignant autosomal recessive form, an intermediate autosomal recessive form, and an adult benign autosomal dominant form. Infantile malignant autosomal recessive osteopetrosis is diagnosed within the first year of life. Patients typically present with pathologic fracture or failure to thrive with frequent respiratory, head and neck, or bony infections. Physical examination shows lethargy, small stature, marked hepatosplenomegaly, and dysmorphic features including macrocephaly, frontal
Received from the Department of Oral and Maxillofacial Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Dublin Dental Hospital, Dublin, Ireland. *Registrar. †Senior Lecturer/Consultant. ‡Professor. Address correspondence and reprint requests to Dr Barry: Department of Oral and Maxillofacial Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Dublin Dental Hospital, Lincoln Place, Dublin 2, Ireland; e-mail: [email protected] © 2007 American Association of Oral and Maxillofacial Surgeons
0278-2391/07/6501-0028$32.00/0 doi:10.1016/j.joms.2005.07.019
bossing, and hypertelorism. Neural palsies may occur, particularly affecting the optic, oculomotor, and facial nerves because of progressive thickening of bone at the expense of the neural canals. Intermediate autosomal recessive osteopetrosis is usually diagnosed toward the end of the first decade of life. Patients often present with pathological fracture. Clinical characteristics include delayed motor and cognitive development, mild disproportionate short stature, mild hepatosplenomegaly, and dysmorphic features such as macrocephaly and frontal bossing. Cranial nerve compression and mandibular osteomyelitis are common features. This form of osteopetrosis is rare. Most patients survive into adulthood but with significant disability. Autosomal dominant osteopetrosis (adult benign osteopetrosis) is a much milder condition. Patients have a normal life expectancy and many are asymptomatic. Patients may present in adulthood with recurrent fractures, back pain, bone pain, or osteomyelitis, particularly of the mandible. Cranial nerve palsies and osteoarthritis of the hips and knees are other potential features. Autosomal dominant osteopetrosis is divided into 2 radiological subtypes. In type I, cranial sclerosis and thickening affects mainly the calvarium. The calvarium is almost normal in type II, but the cranial base is sclerotic. Although both types involve generalized skeletal sclerosis, patients with the type II subtype have a higher risk for fractures. DIAGNOSIS
The diagnosis remains radiographic4 and is characterized by diffuse, increased radiodensity. Other features include absence of medullary cavities, widened metaphyses, “bone within bone” appearance especially of the pelvis, “rugger jersey spine,” and cranial sclerosis with “hair on end” appearance. The paranasal sinuses may be poorly formed or obliterated. The radiographic appearance varies between the different
145
BARRY ET AL
Table 1. DIAGNOSTIC FEATURES OF OSTEOPETROSIS
Radiographic Features
Laboratory Features
Infantile malignant: (autosomal recessive)
Osteosclerosis; metaphyseal widening; sclerosis of cranial base; calvarial “hair on end” appearance; underdevelopment of paranasal sinuses
Hb 2 WCC 2 Platelet 2 Acid phosphatase 1 1,25-dihydroxy vitamin D 1
Intermediate: (autosomal recessive)
Generalized skeletal sclerosis with medullary obliteration
Hb 2 Acid phosphatase 1
Adult benign: (autosomal dominant)
Generalized skeletal sclerosis. Type I: Cranial sclerosis affects mainly calvarium Type II: Sclerotic cranial base with normal calvarium
Hb Normal or 2 Acid phosphatase: Type I Normal Type II 1
Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
forms (Table 1). The hematological profile also depends on the clinical variant of osteopetrosis. In the infantile malignant form, encroachment of sclerotic bone on the marrow spaces results in pancytopenia and extramedullary hemopoiesis. Mild to moderate anemia may be present in the intermediate form. In the autosomal dominant form, there may be no hematological abnormalities. However, anemia of chronic disease may complicate the hematological picture where conditions such as osteoarthritis or osteomyelitis are present. Serum biochemistry also varies depending on the clinical variant. Calcium, phosphorus, and alkaline phosphatase levels are usually normal. Acid phosphatase is elevated in the infantile malignant, intermediate, and autosomal dominant type II forms, but normal in the autosomal dominant type I form. Levels of 1,25-dihydroxy vitamin D are invariably elevated in the infantile malignant form. MANAGEMENT
Medical treatment is based on efforts to stimulate host osteoclasts or to provide an alternative source of osteoclasts.2 Dietary calcium restriction and highdose calcitriol (1,25-dihydroxy vitamin D) have been used to stimulate osteoclast formation and bone resorption. High-dose corticosteroids and parathyroid hormone can improve hematopoiesis and bone resorption. Long-term administration of human interferon-gamma has been shown to stimulate bone resorption, hematopoiesis, and superoxide production of leukocytes. Osteomyelitis complicates osteopetrosis in 10% of cases1 due to impaired white cell function and reduced vascular supply. The most common site of involvement is the mandible.2 Osteomyelitis of the maxilla is very rare, attributed to its thin cortical bone and rich collateral blood supply. This article reports 2
cases of aggressive osteomyelitis of the maxilla occurring secondary to osteopetrosis in 2 sisters and highlights the difficulties in managing these patients.
Report of Cases CASE 1 A 28-year-old marginally intellectually challenged woman was referred to the maxillofacial department by her general dentist for investigation of poorly healing extraction sockets. He reported that her caregivers were concerned about her severe halitosis and finding blood on her pillow every morning. She had a history of multiple fractures attributed to osteogenesis imperfecta. Examination showed a poorly healing extraction socket in the upper left quadrant with bony sequestrae. There was a large (1 cm ⫻ 2 cm) oro-antral fistula (OAF) in the upper left buccal sulcus. Mucopurulent discharges were noted from both middle meati. Conventional panoramic and computed tomography (CT) imaging was performed. The extraction socket and surrounding bone in the region of the maxillary left first molar had a moth-eaten appearance and there was evidence of sequestrum formation (Fig 1). There was extensive bony destruction of the left nasal cavity and maxillary sinus extending inferiorly to the hard palate and laterally into the infratemporal fossa (Fig 2). A differential diagnosis of Wegener’s granulomatosis, antral malignancy, or severe osteomyelitis was considered. The patient’s chest radiographs on admission showed diffuse osteosclerosis of the ribs and thoracic vertebrae (Fig 3). The radiologic findings were sufficiently characteristic for a diagnosis of osteopetrosis to be made. Her admission bloods showed normal white cell and normal red cell counts but with microcytic, hypochromic anemia (hemoglobin 7.8 g/dL, HCT 0.268, MCV 68.5, MCH 19.8 pg). Differential white cell count showed lowered lymphocytes (1.36 ⫻ 109). She had a raised erythrocyte sedimentation rate (32 mm first Hr) and a raised serum phosphate level. The previous diagnosis of osteogenesis imperfecta was reviewed and altered to generalized osteopetrosis. A biopsy of the lateral wall of the nose and antrum and antral polypectomy were performed. The histopathology was reported as consistent with osteomyelitis.
146
OSTEOMYELITIS OF THE MAXILLA
FIGURE 1. Orthopantomograph showing bony resorption of the left maxilla. Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
The patient was treated empirically with metronidazole 500 mg and cefuroxime 750 mg intravenously 3 times daily along with bethamethasone sodium phosphate 0.1% nasal drops. She improved after a few days and was transferred to outpatient care. Her condition regressed within a month. Conservative management was favored due to her general
medical condition and social circumstances. The condition remains unresolved for the past 5 years and acute episodes are managed with antibiotic therapy.
FIGURE 2. Chest radiograph showing osteosclerosis of the ribs and thoracic vertebrae.
FIGURE 3. Computed tomography of the skull showing extensive bony destruction of the left midface.
Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
Barry et al. Osteomyelitis of the Maxilla. J Oral Maxillofac Surg 2007.
CASE 2 The younger sister (27 years old at the time of referral) of patient 1 was referred to the maxillofacial department 3 years after her sister for investigation of severe halitosis and a chronic nasal discharge for over 1 year. She was severely intellectually impaired and had previously been diagnosed with osteopetrosis based on a clinical history of multiple fractures and characteristic radiological features. Admission blood tests were all normal (hemoglobin 13.3 g/dL; white cell count 7.34 ⫻ 109). Radiographs showed the characteristic features of osteopetrosis. Examination under general anesthetic showed an OAF (1 cm ⫻ 1 cm in size) with a purulent discharge in the region of the upper right
147
BARRY ET AL second premolar and first molar. An antral wash with debridement of the OAF was performed. Histopathology was reported as osteomyelitis of osteopetrotic bone. The OAF was closed with a buccal advancement mucoperiosteal flap. Co-amoxiclav 1.2 g intravenously 3 times daily was prescribed. The condition has maintained a chronic course.
Discussion Osteomyelitis is a well-recognized complication of osteopetrosis.1,3-11 Some patients with osteopetrosis may be asymptomatic and many cases are initially diagnosed when the patient presents with osteomyelitis.7 The diagnosis of osteopetrosis is based on a history of numerous fractures and radiological findings of osteosclerosis, although the radiological features are sufficiently characteristic for a diagnosis to be made with certainty.4,8 Osteomyelitis secondary to osteopetrosis tends to be refractory due to the reduced blood supply and accompanying anemia and neutropenia. There are few reports of successful treatment and in many cases the osteomyelitis remains unresolved.5,6 Treatment of osteomyelitis of the jaws includes incision and drainage, antibiotics, sequestrectomy, extraction of teeth, saucerization, decortication, resection of the jaw, and hyperbaric oxygen. Resection and hyperbaric oxygen therapy are the only methods of treatment reported as being successful where the osteomyelitis is secondary to osteopetrosis.6 Obturators are the favored method of filling the maxillary defect.7,8 Free bone grafting is not recommended as the blood supply to the graft bed is compromised. Use of a free vascularized, myo-osseous flap may be more favorable but may be precluded due to absence of a suitable donor site in these patients.9 The 2 patients reported were sisters living in different long-term care facilities and were unknown to each other. The management of their osteomyelitis was compromised not only by their underlying medical condition but also by their social circumstances and intellectual impairment. Patients presenting with osteomyelitis of the jaws should be questioned about a history of recurrent
fractures. Skull and chest radiographs should be obtained as well as full blood count and renal and bone profiles to screen for abnormalities consistent with osteopetrosis (Table 1). If a diagnosis of osteopetrosis is made, medical consultation should be sought, as management of the underlying osteopetrosis may improve outcome. In conclusion, osteomyelitis is now such a rare entity that when presented with osteomyelitis of the maxilla or intractable osteomyelitis of the mandible, the maxillofacial surgeon should consider the possibility of osteopetrosis as an underlying pathology and investigate appropriately. Family members of patients with osteopetrosis should be screened for osteopetrosis and if present, conservative dental plans recommended to avoid potentially intractable osteomyelitis.
References 1. Johnston C, Lavy N, Lord T, et al: Osteopetrosis: A clinical, genetic, metabolic, and morphologic study of the dominantly inherited, benign form. Medicine 47:149, 1968 2. Kocher M, Kasser J: Osteopetrosis. Am J Orthop 32:222, 2003 3. Popoff S, Marks S: The heterogeneity of the osteopetrosis reflects the diversity of cellular influences during skeletal development. Bone 17:437, 1995 4. Stoker D: Osteopetrosis. Semin Musculoskelet Radiol 6:299, 2002 5. Barry CP, Ryan CD: Osteomyelitis of the maxilla secondary to osteopetrosis: Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 95:12, 2003 6. Steiner M, Gould A, Means W: Osteomyelitis of the mandible associated with osteopetrosis. J Oral Maxillofac Surg 41:395, 1983 7. Hanada T, Furuta S, Moriyama I, et al: Maxillary osteomyelitis secondary to osteopetrosis. Rhinology 34:242, 1996 8. Crockett D, Stanley R, Lubka R: Osteomyelitis of the maxilla in a patient with osteopetrosis (Albers-Schonberg disease). Otolaryngol Head Neck Surg 95:117, 1986 9. Bakeman R, Abdelsayad R, Sutley S, et al: Osteopetrosis: A review of the literature and report of a case complicated by osteomyelitis of the mandible. J Oral Maxillofac Surg 56:1209, 1988 10. Lawoyin D, Daramola J, Ajagbe H, et al: Osteomyelitis of the mandible associated with osteopetrosis: Report of a case. Br J Oral Maxillofac Surg 26:330, 1988 11. Fernandez J, Frias M, Hernandez S: Infantile osteopetrosis: A case report with osteomyelitis of the maxilla. J Clin Pediatr Dent 27:77, 2002