Osteogenesis imperfecta presents with unique challenges to the anaesthetist. We report a case of osteogenesis imperfecta, posted for rush rodding femur managed caudal anaesthesia and sedation dexmedetomidine ketamine.

INTRODUCTION Osteogenesis imperfecta is a heritable disorder affecting bone and tooth development. Malocclusion is frequent in those affected by osteogenesis imperfecta, but this has not been studied in detail. The purpose of this study was to describe and quantify the severity of malocclusions in patients with osteogenesis imperfecta. METHODS Articulated dental casts were obtained from 49 pa...

osteogenesis imperfecta (oi) as an inherited connective tissue disorder can affect all tissues that contains type i collagen. well-known cardiac complications of this disease such as aortic root dilatation, aortic regurgitation and mitral valve prolapse have been rarely reported in the literature. coronary artery aneurysm is a rare cardiac complication in oi, as reported in a 19 year old female...

Osteogenesis imperfecta is a genetic disorder of type I collagen. Although multiple genotypes and phenotypes are associated with osteogenesis imperfecta, approximately 90% of the mutations are in the COL1A1 and COL1A2 genes. Osteogenesis imperfecta is characterized by bone fragility. Patients typically have multiple fractures or limb deformity; however, the spine can also be affected. Spinal ma...

Osteogenesis imperfecta is a disease characterized by increased fragility of the bones which are easily fractured by slight trauma. Patients suffering from this disorder usually have blue sclerae and flaccid ligaments; some of them become deaf later in life. Scattered reports of this disorder date back at least to 1678 (quoted by Seedorff in 1949). Vrolik (1849) described the disease in the new...

Osteogenesis imperfecta is a collagen related disorder characterized by increased bone fragility and low bone mass. The important oral finding in osteogenesis imperfect is the presence of dentinogenesis imperfecta. This article presents a case of osteogenesis imperfecta (type IV B) with dentinogenesis imperfecta where a 7-year-old girl had opalacent primary teeth associated with severe bone def...

Basilar impression is a well-recognised though rare complication of osteogenesis imperfecta. Three patients, all members of the same family, with advanced basilar impression complicating osteogenesis imperfecta tarda, are described. The clinical features in these cases illustrate the natural history of this condition: from asymptomatic ventricular dilatation, through the foramen magnum compress...

We report a unique case of unilateral cerebellar hypoplasia in a young Chinese girl with osteogenesis imperfecta type IV. Magnetic resonance imaging showed mild basilar invagination and impression. Although unilateral cerebellar hypoplasia and osteogenesis imperfecta may have been coincidental diagnoses, we propose possible mechanisms for unilateral cerebellar hypoplasia secondary to osteogenes...

osteogenesis imperfecta is a rare inherited connective tissue disorder with an expression that varies from mild to severe disease affecting bone, sclera and middle ear. fertility is preserved, especially in those patients with type 1. we present hereby a pregnant woman with osteogenesis imperfecta that had over 30 fractures in long bones and vertebrae. the object of this report was to determine...

OBJECTIVE To characterize the fracture pattern and the clinical history at the time of diagnosis of osteogenesis imperfecta. METHODS In this retrospective study, all patients with osteogenesis imperfecta, of both genders, aged 0-18 years, who were treated between 2002 and 2014 were included. Medical records were assessed to collect clinical data, including the presence of blue sclerae, dentin...