Prosthetic aspects in adult osteopetrosis Yoichiro Ogino, DDS, PhD,a Yasunori Ayukawa, DDS, PhD,b Yoko Tomita, DDS,c and Kiyoshi Koyano, DDS, PhDd Faculty of Dental Science, Kyushu University, Fukuoka, Japan Osteopetrosis (OP) is a rare condition characterized by skeletal sclerosis caused by dysfunctional osteoclasts. Though many reports have described severe infantile-malignant autosomal recessive OP, few have described the prosthetic management of adult OP. This report discusses the prosthetic treatment of adult OP. Although prosthodontists should try to reconstruct occlusal function as much as possible, a conservative prosthodontic approach may be a reasonable and recommended treatment option for minimizing the risk of further osteomyelitis and osteonecrosis. (J Prosthet Dent 2014;-:---) Osteopetrosis (OP), also known as Albers-Schönberg disease or marble bone disease, is a rare metabolic bone disease characterized by a marked increase in bone density due to defective osteoclastic function.1,2 The condition is quite rare, with an estimated prevalence ratio of 1:100 000 to 1:500 000.3 The diagnosis of OP depends largely on the radiographic appearance of the skeleton (generalized osteosclerosis) and is often the result of other disease processes such as bone fracture and osteomyelitis. Patients with OP exhibit a variety of clinical signs and symptoms, such as frequent fractures and bone pain. In addition, the human skeleton normally undergoes continuous resorption and deposition of bone, and because osteoclasts are nonfunctional in an individual with OP, the intertrabecular (marrow) spaces are filled in by the still-functioning osteoblasts. This results in displacement of the hematopoietic cells (as well as decreased vascularity), thereby causing myelophthisic anemia.4 Osteomyelitis and osteonecrosis are therefore often observed because of a compromised vascular supply, making jaw infection control one of the most important roles for dentists. Once infection and
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osteomyelitis are observed in patients with OP, they can be intractable because of poor-wound healing ability. This makes it more difficult for dentists and oral surgeons to decide the timing of the intervention and treatment options because of the risk of exacerbating symptoms. Hence, dentists should be aware of patients with OP. OP can be generally categorized into 3 types: malignant or infantile OP, intermediate OP, and benign or adult OP.4,5 Although most previous studies have described the management of OP from a surgical aspect,3-8 fewer reports are available regarding benign and adult
OP, especially from a prosthetic aspect. This clinical report presents the management of an OP patient with osteomyelitis from the prosthetic aspect.
CLINICAL REPORT A 54-year-old man complaining of pain and purulent drainage in the right maxilla sought care at the Department of Oral and Maxillofacial Surgery, Kyushu University Dental Hospital, in 2001. He had been diagnosed with OP after a right femoral fracture in the 1960s and had no family history of OP. The radiographic image demonstrated
1 Femoral bone radiograph showing dense sclerotic bone with dynamic hip screw.
Assistant Professor, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. Lecturer, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. c Graduate student, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. d Professor and chair, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. b
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2 A, B, Intraoral view of patient in 2006. Necrotic bone was evident in left maxilla and mandible. C, Panoramic radiograph showing osteosclerosis of maxilla and mandible and sequestrum.
3 Extracted teeth and isolated sequestrum of left mandible. osteosclerosis of the right femoral bone and dynamic hip screw as a result of a surgical intervention after the bone fracture (Fig. 1). He was admitted with purulent drainage in the maxilla in 1982 and 1987. In 2002, he underwent several operative procedures, including the extraction of the remaining teeth in the right maxilla and sequestrectomy of nonviable bone under local and general
anesthesia. In 2006, the first author started his prosthodontic treatment. At general examination, the patient did not show any systemic problems. However, an oral examination revealed osteomyelitis and a sequestrum in the left maxilla and mandible (Fig. 2A,B). A panoramic radiograph also showed osteosclerosis of the maxilla and mandible and a sequestrum (Fig. 2C). He
The Journal of Prosthetic Dentistry
showed continuous purulent drainage from the sequestrum and poor oral hygiene, with 6 periodontally involved teeth. Although the sequestrum prevented the fabrication of a removable dental prosthesis (RDP) of conventional design, he received partial RDPs. Antibiotics were provided to reduce the chances of the adjacent bone becoming infected and thus creating additional necrotic bone. Six months after the first examination, abscess formation associated with osteomyelitis was observed despite the antimicrobial treatment, and the abscess was drained. After necrotic bone was isolated from the host bone, the patient was scheduled for the extraction of multiple teeth and debridement of nonviable bone in the mandible. The operative procedures were performed under general anesthesia. In May 2008, after the surgical intervention in June 2007, RDPs were fabricated without coverage of the sequestrectomy site and before complete soft tissue closure to improve the stability
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4 A, Occlusal surface of wax tooth arrangement of mandibular denture. B, Intraoral photograph showing exposed bone (unhealed soft tissue) after surgery.
5 Frontal view with prostheses after surgery in 2008.
6 Frontal view with prostheses after complete epithelialization in 2009.
7 Sequestrum in left maxillary posterior region in 2010.
8 Intraoral view of patient’s maxilla after sequestrectomy in 2010.
and function of the mandibular denture (Figs. 3-5). Occlusal force was directed toward the buccal shelf to stabilize the denture. In 2009, after surgical resection and epithelialization, a conventional complete denture that provided occlusal
Ogino et al
contacts for only balanced occlusion (not functional occlusal force) on the left side was fabricated in the mandible (Fig. 6). In 2010, a more prominent sequestrum and its isolation were observed in the left maxilla (Fig. 7). Sequestrectomy
was limited to the isolated sequestrum area to avoid surgical intervention in the host bone, and the residual teeth were preserved to serve as abutment teeth for the retention of the maxillary prosthesis (Fig. 8). After the surgical
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Fortunately, in this patient, the postoperative healing and rehabilitation were satisfactory from surgical and prosthetic points of view. This clinical report suggests that patients with OP should be treated by an interdisciplinary rehabilitation team that includes oral surgeons and prosthodontists who are acquainted with the pathologic features of OP.
SUMMARY 9 Frontal view with definitive prostheses after sequestrectomy for left maxilla in 2010. procedures, a new maxillary RDP and a new mandibular complete denture were fabricated with a conventional technique (Fig. 9). Similarly, occlusal contacts for balanced occlusion were provided on the left side. The patient was satisfied with the appearance and function. At the time of preparation of this report, the patient had been followed for almost 3 years and was instructed to comply with an oral hygiene program on a 1-month recall schedule. The patient’s disease did not worsen, although incomplete wound closure was observed in the left maxilla.
DISCUSSION Adult OP (also called benign OP) is diagnosed in late adolescence or adulthood.3,4 The main clinical signs of the benign type of OP are fractures, osteomyelitis, and osteonecrosis of the jaw bone. These are generally caused by tooth extraction, pulpal necrosis, or periodontal disease. A lack of adequate bone vasculature causes poor wound healing and increases the incidence of infection.1-4 Recently, osteonecrosis of the jaws has been widely recognized as the secondary effects or results of radiotherapy for the treatment of head and neck cancer and bisphosphonate treatment.9,10 In many patients, these symptoms are reported after dental surgery. Most clinicians note osteonecrosis as a potential complication
induced by dental surgery after radiotherapy and bisphosphonate treatment. This clinical report serves to remind clinicians of OP as a risk factor of osteonecrosis. Once a sequestrum develops, surgical management is unavoidable.3-5 However, surgical interventions such as sequestrectomy and tooth extraction should be approached with caution because of poor healing capacity. When active osteonecrosis is identified in an patient with OP, prolonged and adequate antimicrobial treatment, not surgical intervention, is indicated.3,4 Furthermore, surgical management should be limited to necessary extractions and isolated bone to minimize the incidence of osteomyelitis and osteonecrosis. During active osteomyelitis or osteonecrosis and the wound healing process after surgical intervention (before soft tissue closure), a conservative prosthetic intervention may be recommended so as not to interfere with wound healing. After complete wound healing occurs, decisions regarding prosthetic interventions should be based on clinical judgment, depending on the presenting conditions and patient needs. In the present patient, some teeth were retained as abutment teeth. The presence of natural teeth after sequestrectomy is beneficial for prosthetic rehabilitation.10 In addition to limited surgical intervention, tooth preservation should be of paramount consideration.
The Journal of Prosthetic Dentistry
A patient with OP and active osteonecrosis was treated both surgically and prosthetically. During active osteonecrosis and surgical procedures, conservative prosthetic treatment with appropriate antibiotic therapy and transfusions to treat complications is recommended. Prosthodontists need to determine the necessity of further prosthetic intervention on the basis of the patient’s condition and functional evaluations.
REFERENCES 1. Dorantes LM, Mejia AM, Dorantes S. Juvenile osteopetrosis: effects on blood and bone of prednisone and a low calcium, high phosphate diet. Arch Dis Child 1986;61: 666-70. 2. Piatt AD, Erhard GA, Araj JS. Benign osteopetrosis: report of nine cases. Am J Roentgenol Radium Ther Nucl Med 1956; 76:1119-31. 3. Lam DK, Sandor GK, Holmes HI, Carmichael RP, Clokie CM. Marble bone disease: a review of osteopetrosis and its oral health implications for dentists. J Can Dent Assoc 2007;73:839-43. 4. Ogütcen-Toller M, Tek M, Sener I, Bereket C, Inal S, Ozden B. Intractable bimaxillary osteomyelitis in osteopetrosis: review of the literature and current therapy. J Oral Maxillofac Surg 2010; 68:167-75. 5. Yamada T, Mishima K, Imura H, Ueno T, Matsumura T, Moritani N, Sugahara T. Osteomyelitis of the mandible secondary to infantile osteopetrosis: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 107:e25-9. 6. Krithika C, Neelakandan RS, Sivapathasundaram B, Koteeswaran D, Rajaram PC, Shetkar GS. Osteopetrosisassociated osteomyelitis of the jaws: a report of 4 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 108:e56-65.
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7. Barry CP, Ryan CD, Stassen LF. Osteomyelitis of the maxilla secondary to osteopetrosis: a report of 2 cases in sisters. J Oral Maxillofac Surg 2007;65: 144-7. 8. Long RG, Ziccardi VB. Osteopetrosis of the maxilla. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001; 91:139-40.
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5 9. Marx RE, Sawatari Y, Fortin M, Broumand V. Bisphosphonate-induced exposed bone (osteonecrosis/osteopetrosis) of the jaws: risk factors, recognition, prevention, and treatment. J Oral Maxillofac Surg 2005;63:1567-75. 10. Marunick M, Gordon S. Prosthodontic treatment during active osteonecrosis related to radiation and bisphosphonate therapy: a clinical report. J Prosthet Dent 2006;96:7-12.
Corresponding author: Dr Yoichiro Ogino 3-1-1 Maidashi, Higashi-ku Fukuoka, 812-8582 JAPAN E-mail: [email protected] Copyright ª 2014 by the Editorial Council for The Journal of Prosthetic Dentistry.
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osteomyelitis are observed in patients with OP, they can be intractable because of poor-wound healing ability. This makes it more difficult for dentists and oral surgeons to decide the timing of the intervention and treatment options because of the risk of exacerbating symptoms. Hence, dentists should be aware of patients with OP. OP can be generally categorized into 3 types: malignant or infantile OP, intermediate OP, and benign or adult OP.4,5 Although most previous studies have described the management of OP from a surgical aspect,3-8 fewer reports are available regarding benign and adult
OP, especially from a prosthetic aspect. This clinical report presents the management of an OP patient with osteomyelitis from the prosthetic aspect.
CLINICAL REPORT A 54-year-old man complaining of pain and purulent drainage in the right maxilla sought care at the Department of Oral and Maxillofacial Surgery, Kyushu University Dental Hospital, in 2001. He had been diagnosed with OP after a right femoral fracture in the 1960s and had no family history of OP. The radiographic image demonstrated
1 Femoral bone radiograph showing dense sclerotic bone with dynamic hip screw.
Assistant Professor, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. Lecturer, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. c Graduate student, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. d Professor and chair, Section of Implant and Rehabilitative Dentistry, Division of Oral Rehabilitation. b
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2 A, B, Intraoral view of patient in 2006. Necrotic bone was evident in left maxilla and mandible. C, Panoramic radiograph showing osteosclerosis of maxilla and mandible and sequestrum.
3 Extracted teeth and isolated sequestrum of left mandible. osteosclerosis of the right femoral bone and dynamic hip screw as a result of a surgical intervention after the bone fracture (Fig. 1). He was admitted with purulent drainage in the maxilla in 1982 and 1987. In 2002, he underwent several operative procedures, including the extraction of the remaining teeth in the right maxilla and sequestrectomy of nonviable bone under local and general
anesthesia. In 2006, the first author started his prosthodontic treatment. At general examination, the patient did not show any systemic problems. However, an oral examination revealed osteomyelitis and a sequestrum in the left maxilla and mandible (Fig. 2A,B). A panoramic radiograph also showed osteosclerosis of the maxilla and mandible and a sequestrum (Fig. 2C). He
The Journal of Prosthetic Dentistry
showed continuous purulent drainage from the sequestrum and poor oral hygiene, with 6 periodontally involved teeth. Although the sequestrum prevented the fabrication of a removable dental prosthesis (RDP) of conventional design, he received partial RDPs. Antibiotics were provided to reduce the chances of the adjacent bone becoming infected and thus creating additional necrotic bone. Six months after the first examination, abscess formation associated with osteomyelitis was observed despite the antimicrobial treatment, and the abscess was drained. After necrotic bone was isolated from the host bone, the patient was scheduled for the extraction of multiple teeth and debridement of nonviable bone in the mandible. The operative procedures were performed under general anesthesia. In May 2008, after the surgical intervention in June 2007, RDPs were fabricated without coverage of the sequestrectomy site and before complete soft tissue closure to improve the stability
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4 A, Occlusal surface of wax tooth arrangement of mandibular denture. B, Intraoral photograph showing exposed bone (unhealed soft tissue) after surgery.
5 Frontal view with prostheses after surgery in 2008.
6 Frontal view with prostheses after complete epithelialization in 2009.
7 Sequestrum in left maxillary posterior region in 2010.
8 Intraoral view of patient’s maxilla after sequestrectomy in 2010.
and function of the mandibular denture (Figs. 3-5). Occlusal force was directed toward the buccal shelf to stabilize the denture. In 2009, after surgical resection and epithelialization, a conventional complete denture that provided occlusal
Ogino et al
contacts for only balanced occlusion (not functional occlusal force) on the left side was fabricated in the mandible (Fig. 6). In 2010, a more prominent sequestrum and its isolation were observed in the left maxilla (Fig. 7). Sequestrectomy
was limited to the isolated sequestrum area to avoid surgical intervention in the host bone, and the residual teeth were preserved to serve as abutment teeth for the retention of the maxillary prosthesis (Fig. 8). After the surgical
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Fortunately, in this patient, the postoperative healing and rehabilitation were satisfactory from surgical and prosthetic points of view. This clinical report suggests that patients with OP should be treated by an interdisciplinary rehabilitation team that includes oral surgeons and prosthodontists who are acquainted with the pathologic features of OP.
SUMMARY 9 Frontal view with definitive prostheses after sequestrectomy for left maxilla in 2010. procedures, a new maxillary RDP and a new mandibular complete denture were fabricated with a conventional technique (Fig. 9). Similarly, occlusal contacts for balanced occlusion were provided on the left side. The patient was satisfied with the appearance and function. At the time of preparation of this report, the patient had been followed for almost 3 years and was instructed to comply with an oral hygiene program on a 1-month recall schedule. The patient’s disease did not worsen, although incomplete wound closure was observed in the left maxilla.
DISCUSSION Adult OP (also called benign OP) is diagnosed in late adolescence or adulthood.3,4 The main clinical signs of the benign type of OP are fractures, osteomyelitis, and osteonecrosis of the jaw bone. These are generally caused by tooth extraction, pulpal necrosis, or periodontal disease. A lack of adequate bone vasculature causes poor wound healing and increases the incidence of infection.1-4 Recently, osteonecrosis of the jaws has been widely recognized as the secondary effects or results of radiotherapy for the treatment of head and neck cancer and bisphosphonate treatment.9,10 In many patients, these symptoms are reported after dental surgery. Most clinicians note osteonecrosis as a potential complication
induced by dental surgery after radiotherapy and bisphosphonate treatment. This clinical report serves to remind clinicians of OP as a risk factor of osteonecrosis. Once a sequestrum develops, surgical management is unavoidable.3-5 However, surgical interventions such as sequestrectomy and tooth extraction should be approached with caution because of poor healing capacity. When active osteonecrosis is identified in an patient with OP, prolonged and adequate antimicrobial treatment, not surgical intervention, is indicated.3,4 Furthermore, surgical management should be limited to necessary extractions and isolated bone to minimize the incidence of osteomyelitis and osteonecrosis. During active osteomyelitis or osteonecrosis and the wound healing process after surgical intervention (before soft tissue closure), a conservative prosthetic intervention may be recommended so as not to interfere with wound healing. After complete wound healing occurs, decisions regarding prosthetic interventions should be based on clinical judgment, depending on the presenting conditions and patient needs. In the present patient, some teeth were retained as abutment teeth. The presence of natural teeth after sequestrectomy is beneficial for prosthetic rehabilitation.10 In addition to limited surgical intervention, tooth preservation should be of paramount consideration.
The Journal of Prosthetic Dentistry
A patient with OP and active osteonecrosis was treated both surgically and prosthetically. During active osteonecrosis and surgical procedures, conservative prosthetic treatment with appropriate antibiotic therapy and transfusions to treat complications is recommended. Prosthodontists need to determine the necessity of further prosthetic intervention on the basis of the patient’s condition and functional evaluations.
REFERENCES 1. Dorantes LM, Mejia AM, Dorantes S. Juvenile osteopetrosis: effects on blood and bone of prednisone and a low calcium, high phosphate diet. Arch Dis Child 1986;61: 666-70. 2. Piatt AD, Erhard GA, Araj JS. Benign osteopetrosis: report of nine cases. Am J Roentgenol Radium Ther Nucl Med 1956; 76:1119-31. 3. Lam DK, Sandor GK, Holmes HI, Carmichael RP, Clokie CM. Marble bone disease: a review of osteopetrosis and its oral health implications for dentists. J Can Dent Assoc 2007;73:839-43. 4. Ogütcen-Toller M, Tek M, Sener I, Bereket C, Inal S, Ozden B. Intractable bimaxillary osteomyelitis in osteopetrosis: review of the literature and current therapy. J Oral Maxillofac Surg 2010; 68:167-75. 5. Yamada T, Mishima K, Imura H, Ueno T, Matsumura T, Moritani N, Sugahara T. Osteomyelitis of the mandible secondary to infantile osteopetrosis: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 107:e25-9. 6. Krithika C, Neelakandan RS, Sivapathasundaram B, Koteeswaran D, Rajaram PC, Shetkar GS. Osteopetrosisassociated osteomyelitis of the jaws: a report of 4 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 108:e56-65.
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7. Barry CP, Ryan CD, Stassen LF. Osteomyelitis of the maxilla secondary to osteopetrosis: a report of 2 cases in sisters. J Oral Maxillofac Surg 2007;65: 144-7. 8. Long RG, Ziccardi VB. Osteopetrosis of the maxilla. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001; 91:139-40.
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5 9. Marx RE, Sawatari Y, Fortin M, Broumand V. Bisphosphonate-induced exposed bone (osteonecrosis/osteopetrosis) of the jaws: risk factors, recognition, prevention, and treatment. J Oral Maxillofac Surg 2005;63:1567-75. 10. Marunick M, Gordon S. Prosthodontic treatment during active osteonecrosis related to radiation and bisphosphonate therapy: a clinical report. J Prosthet Dent 2006;96:7-12.
Corresponding author: Dr Yoichiro Ogino 3-1-1 Maidashi, Higashi-ku Fukuoka, 812-8582 JAPAN E-mail: [email protected] Copyright ª 2014 by the Editorial Council for The Journal of Prosthetic Dentistry.
