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Panoramic radiograph showing a ground-glass appearance (circled areas) in the mandibular right molar region and maxillary left molar region. Distal surfaces of the mandibular right second molar and maxillary left first molar were affected by adjacent teeth showing radiolucency (arrows).
Source publication
Osteopetrosis is a generic term for generalized sclerotic conditions caused by rare genetic disorders. Decreased osteoclastic activities disturb bone remodeling, resulting in greater mineral density and greater compressive strength; therefore, bone fracture is a major physical symptom of osteopetrosis. Osteomyelitis of the maxilla or mandible is a...
Context in source publication
Context 1
... extraoral examination showed trismus, and the intraoral examination showed redness, swelling, and tenderness of the mandibular right second and third molar regions. The panoramic radiograph (Fig 1) showed horizontal impaction of the mandibular right third molar, a ground-glass appearance in the region, and radiolucency on the distal surface of the adjacent second molar. The distal surface of the maxillary left first molar also was affected by the adjacent second molar showing radiolucency. ...
Citations
... The abnormal bone density causes an expansion of skeletal tissue into marrow cavities at the expense of the bone marrow niche, leading to severe anemia, bleeding, frequent infections and hepatosplenomegaly due to increased extramedullary hematopoiesis. The increased susceptibility to infections leads to the development of dental caries and facial osteomyelitis, especially after dental surgery (Mikami et al., 2016). The limited bone marrow niches cause the circulation of high numbers of hematopoietic stem and progenitor cells (HSPCs; positive for CD34 marker) in the peripheral blood, especially in young patients with severe forms (Capo et al., 2021;Steward et al., 2005). ...
Autosomal recessive osteopetrosis (ARO) is a severe inherited bone disease characterized by defective osteoclast resorption or differentiation. Clinical manifestations include dense and brittle bones, anemia and progressive nerve compression, which hamper the quality of patients' lives and cause death in the first 10 years of age. This Review describes the pathogenesis of ARO and highlights the strengths and weaknesses of the current standard of care, namely hematopoietic stem cell transplantation (HSCT). Despite an improvement in the overall survival and outcomes of HSCT, transplant-related morbidity and the pre-existence of neurological symptoms significantly limit the success of HSCT, while the availability of human leukocyte antigen (HLA)-matched donors still remains an open issue. Novel therapeutic approaches are needed for ARO patients, especially for those that cannot benefit from HSCT. Here, we review preclinical and proof-of-concept studies, such as gene therapy, systematic administration of deficient protein, in utero HSCT and gene editing.
... Ao exame físico da cavidade oral foi encontrado um foco inflamatório e infeccioso em maxila direita, sem acometimento de mandíbula. Esse achado corroborou o diagnóstico de uma das principais complicações agudas da osteopetrose que é a osteomielite de mandíbula ou, menos frequentemente, de maxila, como evidencia uma revisão de 47 casos de osteomielite facial, em pacientes com osteopetrose, a qual encontrou que apenas 27,7% dos casos se restringiam à maxila 13 . ...
A osteopetrose infantil maligna (OIM) é a forma mais grave de osteopetrose (OP), que é um grupo de desordens raras e hereditárias que acometem o esqueleto humano, tornando-o mais denso. Uma das suas principais consequências é a invasão medular por esclerose óssea, levando a progressiva insuficiência medular e aplasia, o que predispõe a inúmeras infecções graves, incluindo a osteomielite. É uma doença que possui mortalidade de 99% até os dez anos de idade, com incidência de 1:500.000 nascidos vivos, cujo único tratamento disponível é o transplante de medula óssea precoce. O caso relatado é de um paciente do sexo masculino, 14 anos, com diagnóstico de OIM desde a infância, admitido na emergência do Hospital Infantil Albert Sabin com edema e dor facial à direita de início há um mês. Com base no exame físico e nos exames complementares foi diagnosticada osteomielite maxilar aguda. Além do quadro atual, o paciente possuía diversas sequelas da sua doença de base. Evoluiu com sepse durante a internação, fez uso de antibioticoterapia, e obteve alta hospitalar após um mês para cuidados paliativos domiciliares.
... They were then dehydrated using an ascending ethanol series. After carbon was evaporated from the samples, a qualitative analysis of the sample surface and analysis of the calcium, fluorine, and phosphorus surfaces were performed using EPMA (JXA-8200; JEOL Inc., Tokyo, Japan) 15) . The conditions were acceleration voltage of 10 kV and irradiation current of 2 × 10-8 A. ...
In recent years, tooth erosion due to tooth root exposure has increased. This is associated with an increase in remaining teeth in the elderly and frequent ingestion of acidic foods. Fluoride application is a clinical method for preventing tooth erosion; however, dentin solubility after topical fluoride application, according to the type of erosive acid, has not been adequately investigated. We studied the characteristics of demineralization and acid resistance of dentin to four erosive acids and evaluated the efficacy of topical fluoride application in preventing tooth erosion. Small blocks with a smooth surface were prepared by mirror-finishing the labial side of a bovine tooth root. In the experimental group, acidulated phosphate fluoride (APF) was applied topically for 4 minutes, while the control group received no fluoride treatment. Both groups were immersed in a remineralization solution for 1 h at 37 °C. Five samples from each group were subjected to tooth erosion and demineralization by immersion in lactic acid, phosphoric acid, acetic acid, and citric acid for 6 h at 37 °C. Demineralization and dentin solubility were evaluated by calculating mineral loss (ΔZ) and lesion depth (Ld) from the average surface roughness (Sa), difference in height profile, and contact microradiogram (CMR). We also performed electron probe microanalysis (EPMA) and X-ray photoelectron spectroscopy (XPS) qualitative analysis to assess fluoride dynamics and properties of the compounds formed on the dentin surface after APF application. Samples with APF application showed greater resistance towards all four erosive acids. While a highly calcified layer was found with lactic and acetic acid, resistance was not adequate against citric acid, which has chelating properties. Fluoridated calcium formed on the tooth surface after topical fluoride application. Fluoride ions are taken up by peritubular dentin through the dentinal tubules, resulting in increased acid resistance.
... Sparse bone may hamper primary stability, 25 while compact bone with reduction of trabecular spaces may show impaired vascularization. 26 The ideal bone for implant placement lies somewhere in between these two types. This intermediate type is, however, more difficult to visually differentiate, 8,27,28 especially if trabecular bone structure is not homogeneous in a particular dental implant site. ...
Objectives:
To develop and validate an automated classification method that determines the trabecular bone pattern at implant site based on 3D bone morphometric parameters derived from CBCT images.
Methods:
Twenty-five human cadaver mandibles were scanned using CBCT clinical scanning protocol. Volumes-of-interest comprising only the trabecular bone of the posterior regions were selected and segmented for 3D morphometric parameters calculation. Three experts rated all bone regions into one of the three trabecular pattern classes (sparse, intermediate and dense) to generate a reference classification. Morphometric parameters were used to automatically classify the trabecular pattern with linear discriminant analysis statistical model. The discriminatory power of each morphometric parameter for automatic classification was indicated and the accuracy compared to the reference classification. Repeated-measures ANOVAs were used to statistically compare morphometric indices between the three classes. Finally, the outcome of the automatic classification was evaluated against a subjective classification performed independently by four different observers Results: 86% correct classification was achieved with only structure-related parameters. Cross-validation showed a 79% model prediction accuracy. Bone-volume-fraction had the most discriminatory power in the automatic classification. Trabecular bone patterns could be distinguished based on most morphometric parameters, except for trabecular-thickness and degree-of-anisotropy. The inter observer agreement between the subjective observers was fair (0.25), while the test-retest agreement was moderate (0.46). In comparison with the reference standard, the overall agreement was moderate (0.44).
Conclusion:
Automatic classification performed better than subjective classification with a prediction model comprising only structure-related parameters. Advances in knowledge: Computer-aided trabecular bone pattern assessment based on morphometric parameters could assist objectivity in clinical bone quality classification.
... The literature review found 18 cases of maxillary osteomyelitis associated with ADO type II patients (Table 1) (Barry and Ryan, 2003;Trivellato et al., 2009;Junquera et al., 2005;€ Oǧütcen-Toller et al., 2010;Barry et al., 2007;Infante-Cossio et al., 2014;Kulyapina et al., 2016;Mikami et al., 2016;Celakil et al., 2016;V azquez et al., 2009;Jayachandran and Kumar, 2016;Crockett et al., 1986;Adachi et al., 2013;Ambika et al., 2010;Sopperman et al., 1971;Khademi et al., 2011;Arunkumar et al., 2011). The mean follow-up was 26.2 months, and the majority of cases presented with previous or simultaneous mandibular osteomyelitis. ...
... ADO type II is a rare genetic disease that leads to inadequate function of osteoclasts, due to impairment of cellular mechanisms such as the dysfunction of chloride membrane channels (Sobacchi et al., 1993;Kulyapina et al., 2016;Mikami et al., 2016). The clinical outcomes of the disease are disturbing for the patient and challenging for the treating physician, e.g. ...
... There is no consensus regarding treatment for osteomyelitis associated with osteopetrosis in the maxilla. In this literature review, just 44.4% of the cases presented with complete healing after treatment (Mikami et al., 2016;Adachi et al., 2013;Sopperman et al., 1971;Krithika et al., 2009). Different antibiotics protocols were used, mainly amoxicillin with clavulanic acid (n ¼ 6) or clindamycin (n ¼ 4). ...
Osteopetrosis is a rare condition which presents increased bone density and deficient bone remodeling. The consequential complications include cranial nerve impairment due compression, bone fractures, and osteomyelitis. Maxillary osteomyelitis is uncommon even in osteopetrosis patients. This is a systematic review of the literature regarding the management and outcomes of maxillary osteomyelitis in patients with autosomal dominant osteopetrosis (ADO) type II. A case of this specific pathology is reported. There are 18 cases of maxillary osteomyelitis associated with ADO type II reported in the literature. The mean age of the patients reported was 33.5 (SD 15.9) years, and the male:female ratio was 1:1. Antibiotic therapy was variable, and amoxicillin with clavulanic acid was the main choice (33.33%). Surgery or sequestrectomy was performed in 88.89% of the studies. Complete healing was achieved in only 44.4% of cases. The treatment protocols remain controversial and often do not lead to complete healing. In the case that we report, complete healing was achieved after prolonged antibiotic therapy, hyperbaric oxygen therapy, and partial resection. In conclusion, the management of maxillary osteomyelitis in ADO type II patients is challenging, and complete resolution of the process is dependent on multiple interventions.
... [11] Pathologic bone remodeling, which causes greater mineral density and compressive strength, lacks the needed elasticity and as a result pathologic bone fracture develops which is the hall mark of osteopetrosis. [15][16][17] Hematopoietic stem cell transplantation may reduce the difficult airway-related problems in subsequent anesthesia procedures, but this has not been confirmed yet. [9] Patients with severe pancytopenia may develop heart failure or sepsis. ...
Osteopetrosis is a rare genetic disorder of osteoclast dysfunction leading to anatomical and physiological disorders. We present the anesthesia management for the femur fracture of a 4-year-old girl with malignant infantile type of osteopetrosis. She had a ventriculoperitoneal shunt, impaired motion, visual disturbance, growth failure, facial deformity, heart murmur of moderate tricuspid regurgitation, and left ventricular heart failure, with splenomegaly and severe anemia.
Osteopetrosis (marble bone disease) is a family of rare genetic disorders characterized by impaired osteoclast function leading to hyperdense, hypovascular, brittle bone. Typical imaging shows increased bone mass and thickened cortical and trabecular bone. Bones are more prone to fracture and osteomyelitis may develop. When considering dental implant placement in a patient with osteopetrosis, the potential for bony fracture and/or osteomyelitis should be considered along with the decreased likelihood of successful osseointegration because of hypovascularity. This clinical report describes an unusual imaging presentation and successful osseointegration of multiple dental implants supporting an implant-supported fixed mandibular prosthesis with long-term survival.
Osteopetrosis (OP), also known as marble bone disease is an extremely rare inherited skeletal disorder, characterised by abnormal functioning of the osteoclasts that results in incremental bone deposition with lack of normal bone remodelling. This leads to the formation of hard and brittle bone can readily fracture. The compromised quality of marrow tissue with poor vascularity reduces bone healing and increases the risk of infections. The risk of jawbone osteomyelitis in patients with OP is high and invariably involves mandible. Involvement of maxilla is rare and has been sparingly reported in the literature. This paper highlights a case of extensive necrosis of maxilla and mid-face skeleton following tooth extraction in a patient with OP. Review of the English literature identifying 23 previously published reports of maxillary osteomyelitis in patients with OP is also presented. Demographic details, history of tooth extraction, extent of involvement, clinical presentation, imaging finding, treatment provided and the outcome have been discussed.
Osteopetrosis is a rare hereditary disorder of bone that results in an increase in bone density due to gene mutation and osteoclastic dysfunction. This may lead to cranial nerve compression, bone fractures, and osteomyelitis. Osteomyelitis of the maxilla is rare even in osteopetrosis patients. We report on a case of 25 year old male who presented with multiple episodes of osteomyelitis of the maxilla following dental extractions. The patient was initially managed with incision and drainage of acute infection and intravenous Amoxicillin/Clavulanic acid. This was followed by debridement of necrotic bony margins and packing of BIPP as well as long-term clindamycin. Once osteomyelitis was clear, primary closure was achieved by buccal advancement flap and supported by acrylic obturator. Challenges in the management are highlighted including optimizing patient for surgical intervention due to chronic low haemoglobin levels and lack of healthy bony margins in the maxilla. The literature is also reviewed for similar cases.
