Further studies and data that allow clinicians to design adequate and safe treat. Sometimes the fractures happen for no known reason. Evaluation of oral problems in an osteogenesis imperfecta population. The placement of osseointegrated dental implants in a patient with type iv b osteogenesis imperfecta.
Systemic and dental manifestations of oi and its medical and dental treatments are discussed in this paper. Osteogenesis imperfecta is a rare condition caused by an abnormality of the extracellular matrix. Nov 07, 20 dentinogenesis imperfecta di associated with osteogenesis imperfecta oi is a genetic disorder that affects the connective tissues and results in dentine dysplasia. This segment of the emedtv library provides a detailed overview of the conditions, including its causes, symptoms, treatment options, and more. Osteogenesis imperfecta is classified as type i, ii, iii, iv, v, or vi.
Osteogenesis imperfecta oi refers to a heterogeneous group of congenital, nonsexlinked, genetic disorders of collagen type i production, involving connective tissues and bones. People with this condition have bones that break fracture easily, often from mild trauma or with no apparent cause. A 5yearold child with the diagnosis of oi was referred to the dental school of shaid beheshti university of medical sciences. Dentinogenesis imperfecta associated with osteogenesis imperfecta. Osteogenesis imperfecta overview nih osteoporosis and.
Osteogenesis imperfecta oi is a genetic disorder in which bones fracture break easily. Anesthesia recommendations for patients suffering from osteogenesis imperfecta disease name. Osteogenesis imperfecta type 1 is the mildest form of oi and is characterized by bone fractures during childhood and adolescence that often result from minor trauma. Osteogenesis imperfecta oi is an inherited genetic bone disorder that is present at birth. Dentinogenesis imperfecta type ii and type iii usually occur in people without other inherited disorders. Diagnosis is usually based on family history, a detailed clinical examination and pedigree construction. People with this condition have bones that break easily, often from little or no trauma, however, severity varies among affected people. A 5yearold child with the diagnosis of oi was referred.
Osteogenesis imperfecta brittle bone disease types niams. Dentinogenesis imperfecta di associated with osteogenesis imperfecta oi is a genetic disorder that affects the connective tissues and results in dentine dysplasia. Dentinogenesis imperfecta di associated with osteogenesis imperfecta oi is a genetic disorder that affects the connective tissues and results. The complications arising from dentinogenesis imperfecta are complex to treat and imply a great challenge to the dentist. Bisphosphonates, osteonecrosis, osteogenesis imperfecta and. Pi dental blog osteogenesis imperfecta and dental implants. Osteogenesis imperfecta an overview sciencedirect topics. Osteogenesis imperfecta oi is a generalized disorder of connective tissue manifested by bone fragility, blue sclerae, and other variable soft tissue manifestations. This case report discusses the systemic and dental manifestations of oi and di in a 4yearold child, with moderate presentation of both disorders, who was treated at king fahd military medical complex in dhahran. Dentinogenesis imperfecta genetics home reference nih. Osteogenesis imperfecta radiology reference article. Osteogenesis imperfecta, also known as brittle bone disease, is a genetic disorder that causes bones to break easily without cause. There are at least four clinical subtypes, most of which have an autosomal dominant inheritance, but.
Dentistry handling patient with osteogenesis imperfecta. Besides bone fragility, features like laxity of the ligaments, blue sclera, growth retardation, and scoliosis are also observed. The term osteogenesis imperfecta means imperfect bone formation. Osteogenesis imperfecta type i genetic and rare diseases.
The dental perspective on osteogenesis imperfecta in a danish. These teeth may be misshapen, may chip or break easily, and will require special care. Other symptoms may include a blue tinge to the whites of the eye, short height, loose joints, hearing loss, breathing problems and problems with the teeth. Ninetyfive patients 95 met the study criteria, and 15 coordinators of the specialized dental clinics.
It is characterized by an increased susceptibility to bone fractures and decreased bone density. Osteogenesis imperfecta type iv with dental findings in. This study aimed at determining the characteristic orodental manifestations odms in patients with osteogenesis imperfecta oi in. A classification system of different types of oi is commonly used to help describe how severely a person with oi is affected. Marini jc, bordenick s, heavner g, rose s, hintz rl, rosenfeld rg et al. This case report discusses the systemic and dental manifestations of oi and di in a 4yearold child, with moderate presentation of both disorders, who was treated at king fahd. Manejo estomatologico del paciente con osteogenesis. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. This condition is a type of dentin dysplasia that causes teeth to be discolored most often a bluegray or yellowbrown color and translucent giving teeth an opalescent sheen. Severity varies widely, ranging from intrauterine fractures and perinatal lethality to very mild forms without fractures. Cuidados dentales generales en personas con oi infancia y adolescencia.
Di results in structural defects in dentin formation in the deciduous or both the deciduous and permanent teeth. Osteogenesis imperfecta oi is always associated with bone fragility. Anesthesia recommendations for patients suffering from. The hallmark feature of osteogenesis imperfecta is osteoporosis and fragile bones that fracture easily, as well as, blue sclera, dental fragility and hearing loss. This paper presents a case with dentinogenesis imperfecta di associated with osteogenesis imperfecta. Clinical features, dental findings and dental care management in. The condition affects the bodys ability to produce collagen, a protein in the bodys connective tissue. The clinical and radiological features of oi manifest in different age groups, although the disease is congenital in nature. Clinical features, dental findings and dental care management in osteogenesis imperfecta. Osteogenesis imperfecta oi is a hereditary disorder characterized by increased tendency for bone fractures due to high fragility. A few older individuals with type ii have had progressive highfrequency hearing loss in addition to dental abnormalities, but it is not known whether this hearing loss is related to dentinogenesis imperfecta.
Its primary feature is fractures usually caused by minimal impact. Literature searches were performed with dental and osteogenesis imperfecta, as in dental manifestations of oi. Definition osteogenesis imperfecta oi is a genetic disorder characterized by bones that break easily, often from little or no apparent cause. This case report discusses the systemic and dental manifestations of oi and di in a 4yearold child, with moderate presentation of. All patients but one had had or were having bisphosphonate treatment at the time of the extractions. Dentinogenesis imperfecta is an autosomal dominant genetic disorder with abnormal dentin structure affecting both primary and permanent dentitions leading to discolouration and attrition of teeth. Pdf orodental manifestations in different types of osteogenesis. Osteogenesis imperfecta oi, or brittle bone dis ease, is a congenital disease. Osteogenesis imperfecta oi is a group of genetic disorders that mainly affect the bones. Evaluation of growth hormone axis and responsiveness to growth stimulation of short children with osteogenesis imperfecta. The growth hormone and somatomedin axis in short children with osteogenesis imperfecta.
Manejo estomatologico del paciente con osteogenesis imperfecta. Types i and iv are subdivided by whether or not opalescent dentin is present. Brittle bone disease rare genetic disorder in which bone are fragile and fracture easily resulting in bone deformity an autosomal dominant disease a person with oi has a 50% chance of passing on the gene and the disease to their children. Pdf dentinogenesis imperfecta di associated with osteogenesis imperfecta oi is a genetic disorder that affects the connective tissues and. Osteogenesis imperfecta is a genetic disorder of increased bone fragility and low bone mass. Osteogenesis imperfecta oi is a group of hereditary genetic conditions of the connective tissue characterized by brittle bones and fractures. Today, most of the babies born with oi have a good chance of leading independent, successful, and satisfying lives. Dec 29, 2011 osteogenesis imperfecta oi is a hereditary connective tissue disorder, clinically characterized by a variable degree of bone fragility with recurrent fractures, scoliosis, bone deformities, and short stature as well as nonskeletal abnormalities including blue sclerae, abnormal dentition, and joint hyperlaxity. Clinical manifestations and dental management of dentinogenesis. Osteogenesis imperfecta is a bone disease characterized by bones that break easily. This case report discusses the systemic and dental manifestations of oi and di in a. There are at least four clinical subtypes, most of which have an autosomal dominant inheritance, but new mutations occur, especially in the lethal forms. Dentinogenesis imperfecta di is a genetic disorder of tooth development.
Osteogenesis imperfecta genetics home reference nih. To report on dental characteristics and treatment load in danish adult patients with osteogenesis imperfecta oi. People with this condition have bones that break easily, often from mild trauma or with no apparent cause. Osteogenesis imperfecta great ormond street hospital. Osteogenesis imperfecta oi, or brittle bone disease, is a congenital disease due to a mutation in a gene responsible for creating the collagen protein. Osteogenesis imperfecta oi is a genetic disorder of connective tissues caused by an abnormality in the synthesis or processing of type i collagen. Osteogenesis imperfecta due to the work of many dedicated scientists and physicians, a great deal has been learned about osteogenesis imperfecta in the last 10 years.
Osteogenesis imperfecta was classified several years ago into four types based on clinical, radiological and genetic features sillence, 1988. Find out about the symptoms of brittle bone disease. After examining the available sound medicalscientific evidence the repatriation medical authority is of the view that there is sound medical. It causes bone fragility leading to fractures that may be frequent, and a variable articular hyperlaxity. Oral cavity problems related to osteogenesis imperfecta may include the following. Osteogenesis imperfecta oi is a genetic condition present from birth. Osteogenesis imperfecta osteogenesis imperfecta laura arias millan. Dentinogenesis imperfecta is an autosomal dominant dental development anomaly that affects both the primary and permanent dentition. This information sheet from great ormond street hospital gosh describes osteogenesis imperfecta oi, what causes it and how it can be managed. Osteogenesis imperfecta oi, also known as brittlebone disease, is a genetic and inherited disorder characterized by fragile bones that break easily without a specific cause. Shields type i occurs with osteogenesis imperfecta oi due to a defect in type i collagen. Osteogenesis imperfecta also known as brittle bone disease is a heterogeneous group of inherited bone dysplasias characterized by skeletal deformity and bone fragility. Pdf clinical manifestations and dental management of. The cause is the mutations in the genes that codify of precollagen.
Dental care for people with osteogenesis imperfecta. Osteogenesis imperfecta is a genetic disease, also called brittle bone disease, that causes bones to be weak and break easily. For example, a person may have just a few or as many as several hundred fractures in a lifetime. The teeth do not correctly match up making biting difficult. Pi dental center is a prosthodontic specialty practice that provides advanced dental implant treatment, restorative and cosmetic dental. Osteogenesis imperfecta oi is a group of genetic disorders that mainly. Osteogenesis imperfecta oi is an inherited connective tissue disorder with many phenotypic presentations.