1/9. cochlear implantation in a child with osteogenesis imperfecta.osteogenesis imperfecta (OI) is a hereditary disease of connective tissue and affects bone, dentine, sclera, joint, tendon, blood vessels, heart valves, and skin. Approximately 50% of the adult patients with OI have associated hearing impairment. To date, only three cases of cochlear implantation in adults with OI have been reported, but none in children. We present a case of cochlear implantation in a congenitally deaf 6-year-old boy with OI. The Nucleus 24 Contour device was successfully implanted using the suprameatal approach (SMA). At 6 months post-initial stimulation there was no evidence of non-acoustic nerve excitation (i.e. facial twitching) or discomfort, and significant progress in auditory abilities was manifested by open set word identification.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Ruptured cerebral artery aneurysm and bacterial meningitis in a man with osteogenesis imperfecta.This report describes a 38-year-old man with osteogenesis imperfecta who died of a ruptured cerebral artery aneurysm and bacterial meningitis. He had multiple long bone fractures in the past, and approximately 4 months before death, he had surgery to relieve symptoms of basilar impression. The surgery was complicated by a postoperative wound infection. For the next 4 months, he had intermittent headaches and vomiting. He was found dead in his bed at home. At autopsy, he had a ruptured anterior communicating artery aneurysm and bacterial meningitis. cerebrospinal fluid and blood cultures had growth of staphylococcus aureus. osteogenesis imperfecta is a disorder of type I collagen. Type I collagen is present in many tissues, including blood vessels. The etiology of cerebral artery aneurysm formation is multifactorial. Some patients with cerebral artery aneurysms have been shown to have abnormalities in type III collagen. There has not been a reported relationship made between abnormalities in type I collagen and aneurysms. meningitis can also result in cerebral artery aneurysms, but they are usually due to aspergillus or mycobacterium species. The case we report is unique; cerebral artery aneurysm formation may have been due to osteogenesis imperfecta and/or bacterial meningitis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/9. Failed tissue expansion in a patient with osteogenesis imperfecta.This is a report of a failure of tissue expansion in a 43-year-old patient suffering from a mild form of osteogenesis imperfecta tarda. Filling of the expander was not possible. skin histology showed thrombosis of the blood vessels and vessel wall necrosis. This finding supports the theory that tissue expansion does not depend as much on mechanical skin laxity as on the proliferative potential of the skin.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/9. Cardiovascular pathology in osteogenesis imperfecta type IIA with a review of the literature.Lethal perinatal osteogenesis imperfecta (OI Type II) is a biochemically diverse collagen disorder characterized by short, crumpled long bones, beaded ribs, blue sclerae and thin, fragile skin. cardiovascular abnormalities are rarely described. Using morphometry and light and electron (SEM and TEM) microscopy, we analyzed the hearts and great vessels from 2 fetuses with OI Type IIA and compared the findings with age-matched controls. The heart weights and atrioventricular valve (AVV) circumferences were reduced in OI. The chordae tendineae were short and fragile; both the AVVs and the chordae tendineae were hypercellular. TEM showed relatively little organized collagen in the chordae tendineae of OI fetuses. Furthermore, quantitative evaluation of collagen fibril size revealed a decrease in the cross-sectional diameter. There was also a marked decrease in the adventitial and intramural collagen of the intramyocardial arteries and great vessels in OI. Our study reports, for the first time, specific lesions in the cardiovascular systems of patients with OI Type II and reviews the cardiovascular pathology in other forms of OI.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
5/9. osteogenesis imperfecta of the temporal bone and its relation to otosclerosis.osteogenesis imperfecta (OI) designates a heterogeneous group of heritable disorders of connective tissue that in addition to bone may affect tendons, ligaments, fascia, skin, sclerae, blood vessels, teeth, and hearing. The current classification identifies at least four major syndrome groups or types. It also recognizes a considerable number of additional syndromes that may represent supplementary types or subgroups. Loss of hearing is the least constant of the prominent features of OI. Its incidence varies between 26% and 60%. In OI, formation and remodelling of bone are variously affected. In the temporal bone the development of the inner ear capsule may be involved severely. In the stapes the disturbance in lamellar bone formation can lead to extreme thinness, dehiscence, and nonunion of the stapedial superstructure with the footplate. osteogenesis imperfecta can be associated with otosclerosis, another bone dysplasia with a different morphology. otosclerosis, in turn, may interfere with sound conduction and perception. Thus, the hearing loss encountered in OI may be the result of OI, otosclerosis, or a combination of both.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/9. Subhyaloid hemorrhage in osteogenesis imperfecta tarda.The clinical characteristics of osteogenesis imperfecta are the result of a defect in the collagen fibrils. This paper presents a case of osteogenesis imperfecta tarda in which a large subhyaloid hemorrhage occurred. This complication, which has never before been reported in the literature, could be the result of a deficiency of collagen in and around the blood vessels of the eye.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/9. Spontaneous carotid-cavernous fistula in osteogenesis imperfecta.A case of spontaneous carotid-cavernous fistula is presented in a patient suffering from osteogenesis imperfecta tarda type I. The patient was operated on under local anesthesia by intraluminal occlusion of the cavernous carotid artery with a Fogarty catheter, in accordance with Prolo's technique, with total remission except for homolateral loss of vision due to postglaucoma atrophy. the pathogenetic phenomena described in the literature are reviewed, with the suggestion that osteogenesis imperfecta be included with Ehlers-Danlos disease, Marfan's syndrome, and pseudoxanthoma elasticum, as a connective tissue disease with increased weakness of vessel walls that can produce a spontaneous carotid-cavernous fistula. Reports of cases operated on by intraluminal occlusion with various types of balloon-tipped catheters are also reviewed. The good results obtained place this technique among the elective ones for the treatment of carotid-cavernous fistula.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/9. osteogenesis imperfecta type II: microvascular changes in the CNS.osteogenesis imperfecta, type II is a rare hereditary disease of connective tissue with abnormalities of type I collagen. It is invariably fatal in the neonatal period. We report 2 cases with abnormal cerebral cortical white matter consisting of abundant, perivenous microcalcifications, proliferated vascular endothelium and focal aggregates of germ cells. Histochemically, microcalcifications lie within nodules composed of a PAS-positive, carbohydrate-rich matrix. This matrix also stains with alcian blue suggestive of a proteoglycan component. Immunoperoxidase staining reveals that some of these nodules are surrounded by type I collagen. Adjacent vessels show endothelial proliferation associated with markedly redundant basement membranes as confirmed by reactivity with anti-type IV collagen. Although the cerebral cortex has a normal neuronal cytoarchitecture, the areas that appear externally dysplastic overlie nests of germ cells within the underlying white matter suggestive of impaired migration. These changes most likely occurred during the third trimester and may involve abnormal interactions of proteoglycans and the extracellular matrix with collagen.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Microvascular and cellular defects of the periosteum of osteogenesis imperfecta.periosteum stripped from fragments of diaphyseal long bones from osteogenesis imperfecta (OI) patients was examined both in vivo and in vitro. The OI periosteum was thickened and characteristic finding was a defective microvascular system. The vascular walls of arterioles and capillaries were thick and the lumina of many of these vessels were narrowed or occluded by hypertrophic endothelial cells and multiplication of smooth muscle cell layers. Circulatory deficiency in the OI periosteum might be regarded as one of the causes of the slender, osteopenic diaphyseal bone characteristic of the disorder. A higher proliferative activity was observed in cultured OI periosteal cells, but the cytoplasmic structure showed increased myelinoid bodies and smooth endoplasmic reticulum and decreased lysosomes and rough endoplasmic reticulum. These findings may be related to the abnormal metabolic activity of OI periosteal cells.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |