Idiopathic osteosclerosis mimicry of a tooth: case report

This report presents two cases of idiopathic osteosclerosis involving the maxilla and mandible which were identified as a buccally impacted canine and a retained root, respectively, on clinical and plain radiographical examinations. Both patients were females who presented with hypodontia. Radiographic evaluation revealed solitary well-defined radiopaque masses with thickened cortical border. Both patients were undergoing orthodontic treatment and one was planned for a surgical traction of unerupted tooth prior to cone-beam CT assessment. In this report, we reviewed the clinical findings and explained the radiographic appearance of idiopathic osteosclerosis through plain radiographs and cone-beam CT to facilitate its identification among general dentists and oral and maxillofacial radiologists.     

Unusual presentation of idiopathic osteosclerosis: a case report

Idiopathic osteosclerosis (IO) typically manifests as an incidentally discovered, well-defined homogenous radiopacity that blends in with the surrounding bone. Once the condition is diagnosed, treatment is neither indicated nor necessary; however, atypical variants of osteosclerosis have been described and may pose a diagnostic and management dilemma for the clinician who encounters them. This case report discusses the differential conditions that should be considered when an atypical variant of IO is encountered. While the diagnosis in this case was fortuitously confirmed by biopsy, routine follow-up is the recommended course of action.     

Autosomal recessive Robinow syndrome: a case report

Recessive Robinow syndrome (RRS) is an extremely rare short stature genetic condition with significant and characteristic oral, dental, and facial attributes that generally requires coordinated multidisciplinary dental and medical interventions. This paper describes the clinical findings and dental management over the course of 4 years of a US-born Caucasian female with recessive Robinow syndrome who presented to our dental clinic at age 11 years, 0 months and who is now age 15 years, 3 months. Of special interest was the extent to which the patient's hyperplastic gingival tissues impeded both normal tooth eruption and orthodontic tooth movement.     

Rieger综合征-附1例家系病例报告

目的：分析1例Rieger综合征的典型家系病例，对临床医师认识和诊断这一与口腔发育异常密切相关的罕见遗传病提供线索和信息。方法：对1例Rieger综合征患进行家系调查，对家系成员进行临床检查，修复治疗和表型分析。结果：临床诊断1例Rieger综合征病例家系，显示常染色体显性的遗传方式，该综合征可导致严重的上颌骨发育不足和多数恒牙先天缺失。结论：Rieger综合征是导致先天性牙齿缺失的重要遗传性疾病之一，由于修复治疗的需要，可能对口腔医师在这一疾病的发现和诊断中起到重要作用。     

Differential diagnosis and treatment of autosomal dominant osteosclerosis of the mandible

A 20-year-old patient who presented with concerns about her large mandible was found to have a generalized mild cortical sclerosis. She was treated successfully with staged orthognathic surgery, despite the dense sclerosis of the jaws.     

Autosomal dominant inherence of multiple supernumerary teeth

The case is presented of 16 supernumerary teeth in an 11-year-old Chinese female patient with congenital cardiopathy. It was found that the patient's mother had the same clinical manifestation. A detailed investigation into the family history was carried out. Though the etiology of this dental anomaly remains unclear, genetics as a key factor in the development of supernumerary teeth is highlighted.     

Autosomal recessive osteopetrosis: case report of two siblings

Malignant osteopetrosis is a rare congenital disorder of bone resorption, occurring in less than 1 in 200 000 births. It is caused by the failure of osteoclasts to resorb immature bone. We report on two siblings with autosomal recessive osteopetrosis. The prominent clinical features were short stature, progressive blindness, defective development of primary and permanent teeth, and anemia.     

Osteomyelitis of the mandible associated with autosomal dominant osteopetrosis: a case report

Osteopetrosis is a rare hereditary bone disorder presenting with variable clinical features and is characterized by an increase in bone density. The main clinical findings of the benign-type of osteopetrosis (autosomal dominant) are fractures and osteomyelitis of the mandible, leading to the detection of the disease. We report a case of autosomal dominant osteopetrosis with secondary mandibular osteomyelitis. Clinical and radiological patterns and treatment evaluation were assessed. The patient was referred for evaluation and treatment of an acute abscess in the left side of the face and osteomyelitis of the mandible. Several imaging studies were performed to assess the lesion and the adjacent bone and soft tissue structures. Treatment consisted of intravenous antibiotic therapy, debridement of the necrotic bone and sequestrum, and extra-oral drainage of the abscess, with submandibular access. Healing was uneventful.     

A rare complication of idiopathic osteosclerosis

Idiopathic osteosclerosis (IO) is described as a localized no expansible radiopacity with unknown etiology. The IO is generally asymptomatic and could appear as round, elliptical or irregular in shape. The internal aspect is usually uniformly radiopaque. IO should be distinguished from condensing osteitis of dental origin, or other alveolar bone related radiopacities such as periapical cemental dysplasia. This condition may cause changes in tooth position or problems during orthodontic treatment. The purpose of the present study is to report a case of tooth resorption caused by ectopic eruption rote caused by IO. This condition represents a rare complication of IO.     

Analysis of a kindred with amelogenesis imperfecta

Amelugenesis imperfecta (A1) is a group of hereditary disorders whose manifestations are generally considered as being confined to the teeth. Pedigree analysis of a kindred revealed 2 siblings affected with AI which was consistent with an autosomal recessive mode of inheritance. Cephalometric evaluation showed both children to have an obtuse gonial angle and steep mandibutar plane. These skeletal changes were manifested as a severe anterior open bite. Histological evaluation of the teeth demonstrated numerous enamel changes including altered prism morphology, prism coalescence and disruption, globular inclusions, and irregular crystallite orientation. The clinical and histological data are consistent with autosonial recessive pigmented hypomaturation amelogenesis imperfecta . The enamel defects appear to be caused by the combination of decreased mineral deposition with abnormal crystallite and prism formation. Some areas of enamel hypoplasia seem to exist as well.     

Analysis of a kindred with amelogenesis imperfecta

Amelogenesis imperfecta (AI) is a group of hereditary disorders whose manifestations are generally considered as being confined to the teeth. Pedigree analysis of a kindred revealed 2 siblings affected with AI which was consistent with an autosomal recessive mode of inheritance. Cephalometric evaluation showed both children to have an obtuse gonial angle and steep mandibular plane. These skeletal changes were manifested as a severe anterior open bite. Histological evaluation of the teeth demonstrated numerous enamel changes including altered prism morphology, prism coalescence and disruption, globular inclusions, and irregular crystallite orientation. The clinical and histological data are consistent with autosomal recessive pigmented hypomaturation amelogenesis imperfecta. The enamel defects appear to be caused by the combination of decreased mineral deposition with abnormal crystallite and prism formation. Some areas of enamel hypoplasia seem to exist as well.