Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “PLATYBASIA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 55 records · Page 3Linked to original sources

Principles of management of osteometabolic disorders affecting the aging spine.

Osteoporosis is the most common contributing factor of spinal fractures, which characteristically are not generally known to produce spinal cord compression symptoms. Recently, an increasing number of medical reports have implicated osteoporotic fractures as a cause of serious neurological deficit and painful disabling spinal deformities. This has been corroborated by the present authors as well. These complications are only amenable to surgical management, requiring instrumentation. Instrumenting an osteoporotic spine, although a challenging task, can be accomplished if certain guidelines for surgical techniques are respected. Neurological deficits respond equally well to an anterior or posterior decompression, provided this is coupled with multisegmental fixation of the construct. With the steady increase in the elderly population, it is anticipated that the spine surgeon will face serious complications of osteoporotic spines more frequently. With regard to surgery, however, excellent correction of deformities can be achieved, by combining anterior and posterior approaches. Paget's disease of bone (PD) is a non-hormonal osteometabolic disorder and the spine is the second most commonly affected site. About one-third of patients with spinal involvement exhibit symptoms of clinical stenosis. In only 12-24% of patients with PD of the spine is back pain attributed solely to PD, while in the majority of patients, back pain is either arthritic in nature or a combination of a pagetic process and coexisting arthritis. In this context, one must be certain before attributing low back pain to PD exclusively, and antipagetic medical treatment alone may be ineffective. Neural element dysfunction may be attributed to compressive myelopathy by pagetic bone overgrowth, pagetic intraspinal soft tissue overgrowth, ossification of epidural fat, platybasia, spontaneous bleeding, sarcomatous degeneration and vertebral fracture or subluxation. Neural dysfunction can also result from spinal ischemia when blood is diverted by the so-called "arterial steal syndrome". Because the effectiveness of pharmacologic treatment for pagetic spinal stenosis has been clearly demonstrated, surgical decompression should only be instituted after failure of antipagetic medical treatment. Surgery is indicated as a primary treatment when neural compression is secondary to pathologic fractures, dislocations, spontaneous epidural hematoma, syringomyelia, platybasia, or sarcomatous transformation. Five classes of drugs are available for the treatment of PD. Bisphosphonates are the most popular antipagetic drug and several forms have been investigated.

Aged↗

Paget's disease of the spine and its management.

A review of the literature was conducted to study the pathomechanics by which Paget's Disease of bone (PD) alters the spinal structures that result in distinct spinal pathologic entities such as pagetic spinal arthritis, spinal stenosis, and other pathologies, and to assess the best treatment options and available drugs. The spine is the second most commonly affected site with PD. About one-third of patients with spinal involvement exhibit symptoms of clinical stenosis. In only 12-24% of patients with PD of the spine is back pain attributed solely to PD, while in the majority of patients back pain is either arthritic in nature or a combination of a pagetic process and coexisting arthritis. Neural element dysfunction may be attributed to compressive myelopathy by pagetic bone overgrowth, pagetic intraspinal soft tissue overgrowth, ossification of epidural fat, platybasia, spontaneous bleeding, sarcomatous degeneration and vertebral fracture or subluxation. Neural dysfunction can also result from spinal ischemia, when blood is diverted by the so-called "arterial steal syndrome". Because the effectiveness of pharmacologic treatment for pagetic spinal stenosis has been clearly demonstrated, surgical decompression should only be instituted after failure of antipagetic medical treatment. Surgery is indicated as a primary treatment when neural compression is secondary to pathologic fractures, dislocations, spontaneous epidural hematoma, syringomyelia, platybasia, or sarcomatous transformation. Since, in the majority of cases with pagetic spinal involvement, there are also coexisting osteoarthritic changes, antipagetic medical treatment alone may be disappointing. Therefore, one must be careful before attributing low back pain to PD alone. Five classes of drugs are available for the treatment of PD: bisphosphonates, calcitonins, mithramycin (plicamycin), gallium nitrate, and ipriflavone. Bisphosphonates are the most popular, and several forms have been investigated, but only the following forms have been approved for clinical use: disodium etidronate, clodronate, aledronate, risedronate, neridronate, pamidronate, tiludronate, ibadronate, aminohydroxylbutylidene bisphosphonate, olpadronate, and zoledronate. Several of these forms are still under investigation.

Humans↗

Craniofacial morphology in the velo-cardio-facial syndrome.

The velo -cardio-facial syndrome is a recently delineated congenital malformation syndrome, probably of autosomal dominant inheritance. Previous reports have concentrated on facial, oropharyngeal, cardiac, speech, language, and psychological features of this fairly common syndrome. To date, no radiographic data have been presented which might help to further delineate the syndrome, nor has there been an explanation of the characteristic facial appearance seen in this syndrome. This current study reports on cephalometric evidence of platybasia (obtuse angulation of the cranial base) in the velo -cardio-facial syndrome. The finding of platybasia adds one more phenotypic feature to the syndrome and also may help to explain the facial features of retrognathia, malar flatness, and prominence of the nasal root.

Abnormalities, Multiple↗

Craniofacial dysostosis with syringomyelia and associated anomalies.

A 16-year old boy had craniofacial dysostosis, hydrocephalus, and syringomyelia. Other anomalies included platybasia, a Klippel-Feil anomaly, webbed toes, and a cutaneous hemangioma. Evaluation included cerebral angiography, ventriculography, and myelography.

Abnormalities, Multiple↗

Velopharyngeal insufficiency and articulation impairment in velo-cardio-facial syndrome: the influence of adenoids on phonemic development.

Velo-cardio-facial syndrome is the most common contiguous gene disorder in humans and constitutes 8% of patients with clefts of the secondary palate. Speech disorders, including severe hypernasality and articulation impairment have been documented as among the most common clinical manifestations of the disorder. A series of 36 consecutive patients with VCFS ranging in age from 3 to 14 years, all confirmed to have a 22q11.2 deletion, were studied to determine specific risk factors associated with VPI and articulation impairment. Factors studied included palatal clefting, hypotonia, platybasia, and adenoid size. The factor that correlated most strongly with speech disorders was adenoid hypoplasia or absence, a common manifestation in the syndrome. It is hypothesized that early identification of the absence or hypoplasia of the adenoids can result in the implementation of appropriate therapy plans to avoid severe disorders of speech intelligibility.

Abnormalities, Multiple↗

A cranio-cervical malformation presenting as acute respiratory failure.

An 18-year-old, previously healthy male presented with bilateral pneumonia and acute respiratory failure with severe carbon dioxide retention. The presence of mild brainstem signs and hypoventilation led to the discovery of a platybasia, basilar invagination, and kinking of the medulla oblongata with early syrinx. He was operated upon but postoperatively was noted to have a mixed type of sleep apnea. This case illustrates the diagnostic challenge in acute respiratory failure in a previously healthy young person and the possible pathogenic mechanisms underlying it.

Adolescent↗

A new dwarfing syndrome with extreme shortening of humeri and severe coxa vara.

A previously undescribed form of short-limbed dwarfism is reported in an 86-year-old man. All the long bones were shorter than normal, with extreme shortening of the humeri. Other features included dysplastic humeral heads, coxa vara with dysplasia of the femoral heads and acetabula, platybasia, and small vertebral bodies. In all other respects, including intelligence, the patient was normal for his age.

Aged↗

Some unusual features of Paget's disease of bone.

The usual features of Paget's disease of bone, e.g. large head, deafness due to involvement of the 8th cranial nerve and bowing of the legs are all well described in text books of medicine. However, some features such as platybasia with neurological deficit, involvement of the 2nd, 5th and 7th cranial nerves, hydrocephalus, dementia, dysphasia, corrugation of the skull, bone pain (with or without associated osteo-arthrosis of hips and knees), heart failure, triradiate deformity of the pelvis have either had an inadequate or no description in books. These features are unusual, but it is important to recognise them, as the advances made since the introduction of the very promising new drugs in recent years have not only provided increasing insight into the disease, but also have improved management of patients with Paget's disease of bone.

Aged↗

Transpalatal excision of the odontoid process.

A patient with platybasia of the skull and temporomandibular joint ankylosis is presented. Relief of brain stem compression by the impingement of the odontoid through a soft, hypoplastic clivus was done by first doing a mandibular condylectomy to open the mouth. A transoral-transpharyngeal route was then employed to excise the odontoid process.

Adult↗

[Congenital malformation of the skeleton in Weiser-Maples guinea pigs].

Some abnormalities were observed in the occipital bone, cervical vertebrae and thoracic vertebrae of Weiser-Maples guinea pigs. In the occipital bone, the medial basilar impression was suggested to occur in 40 (32.8%) out of 122 animals. The basilar impression was classified into right, left and both side types and observed in 24, 11 and 5 animals, respectively. The basilar impression was known to be accompanied in human with some anomalies such as platybasia, Klippel-Feil syndrome, deformation of foramen magnum and so-on. These anomalies were also observed in guinea pigs. The fusion of the axis with the 3rd cervical vertebra was observed in 12 (10.5%) out of 114 animals. The deformation was sometimes observed in the temporal, interparietal, atlas and axis as well as the occipital bone. The fusion of the 7th cervical vertebra with the 1st thoracic vertebra was found in 46 (51.7%) out of 89 animals. This fusion was thought to have no relation with the basilar impression. Weiser-Maples guinea pigs are now in 19 generations of sibmating. Because these abnormalities as mentioned above are all thought to be inherited, the selective breeding will make Weiser-Maples guinea pigs suitable for the study of the basilar impression.

Animals↗

The spine in osteogenesis imperfecta: a review of treatment.

Progressive spinal deformity can be an anathma for indivudials with osteogenesis imperfecta. Scoliosis or khyphosis develop indolently, being less dramatic than long bone fractures, but once significant deformities evolve, they tend to remain progressive on into adulthood. State of the art spinal fixation systems are of little help in correcting such deformities due to poor bone stock. However, most curves can be arrested by posterior spinal fusion, performed either in situ, or by utilizing basic Harrington type instrumentation with methylmethacylate supplemtation for the hook sites, along with Drummond wires where feasible. Platybasia is yet another issue involving the spine which may be complicated by neurologic deterioration. It has been posulated as a cause of death, but can respond to shunting and brain stem decompression when recognized.

Braces↗

Spectrum of occipitoatlantoaxial injury in young children.

OBJECT: Injuries of the occipitoatlantoaxial (Oc-C2) region are the predominant form of cervical injury in children younger than 10 years of age. Magnetic resonance (MR) imaging can be used to visualize directly the traumatic ligamentous and soft-tissue abnormalities of the Oc-C2 region. A retrospective review was undertaken to examine the spectrum of pediatric Oc-C2 injuries seen on MR imaging, their correlation with plain x-ray film and computerized tomography findings, and their clinical course. METHODS: Seventy-one consecutive children younger than 10 years of age underwent cervical MR imaging for evaluation of traumatic injury. Magnetic resonance imaging was used to document abnormalities in 23 children; 20 of these injuries involved the Oc-C2 region. Abnormalities in the Oc-C2 region included disruptions of the musculature, apical ligament, atlantooccipital joint(s), tectorial membrane, and spinal cord. A spectrum of injury with progressive involvement of these structures was seen, ranging from isolated muscular injury to the multiple soft-tissue and ligamentous disruptions with craniocervical dislocation. Involvement of the tectorial membrane was the critical threshold in the transition from stable to unstable injury. Analysis of plain x-ray films revealed that a novel interspinous C1-2:C2-3 ratio criteria of greater than or equal to 2.5 was predictive of tectorial membrane abnormalities on MR imaging, with 87% sensitivity and 100% specificity. In patients with tectorial membrane abnormalities who underwent immobilization alone, interim platybasia was demonstrated on follow-up MR images. Conclusions. A progressive spectrum of distinct Oc-C2 injuries can occur in young children; the tectorial membrane is a critical stabilizing ligamentous structure in the Oc-C2 complex; and tectorial membrane abnormalities may be identified by a C1-2:C2-3 ratio of greater than or equal to 2.5.

Atlanto-Axial Joint↗

[Pathogenesis of syringomyelia in the light of our observations].

The author treated surgically 10 patients with syringomyelia. In 6 cases Arnold--Chiari syndrome was found, in 2 cases platybasia with basilar impression, besides that 2 patients had complete or partial occlusion of Maganide's foramen and in 4 cases a persisting central canal communicated widely with the 4th ventricle. In 3 cases these findings were associated with postinflammatory changes. In only one case the changes in the atlanto-occipital area could not have been defined clearly. The author compares his observation with those reported by others reaching the conclusion that they confirm the hydrodynanic mechanism of syringomyelia. In most cases syringomyelial is a result of congenital or congenital and acquired malformations in the medullo-bulbar area leading to distrubances in the circulation of cerebrospinal fluid.

Arnold-Chiari Malformation↗

Congenital displacement of temporal cortex into the central spinal canal.

This is a report of the first recorded observation of displacement of temporal cortex into the central spinal canal in an infant with Arnold Chiari malformation, platybasia, aqueductal atresia, hydrocephalus and meningomyelocele. The combination of an absent right cerebellar hemisphere and malformed fourth ventricular roof provided the anatomical background for this unique event.

Arnold-Chiari Malformation↗

Craniocervical CT and MR imaging of Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome is a rare, inherited disorder characterized by myotonia, skeletal deformities, facial dysmorphism, and growth retardation. In this report of an adolescent male patient with Schwartz-Jampel syndrome, CT and MR imaging revealed basilar invagination, platybasia, Chiari I malformation, hyperpneumatized mastoids with intramastoid dural sinuses, platyspondyly, bulbous zygoma, and blunted pterygoid processes.

Adolescent↗

Contribution to the etiology of spina bifida cystica.

Authors report of bone anomalies found out by plain X-ray examination in 61 mothers and 40 fathers of children with spina bifida cystica. There was ascertained hyper-pneumatisation of skull bones 18 times, platybasia or basilar impression 15-times, spina bifida occulta 11-times and deformation of lumbosacral vertebrae 8-times. Only in 49 parents of handicapped children the roentgenological picture of their skeleton was physiological. In comparison with the control group of 100 persons chosen by random access the incidence of skeleton anomalies of parents of children with congenital defects of the neural tube was significantly higher.

Adolescent↗

[A case of up-beat nystagmus associated with Chiari malformation type I (author's transl)].

A case of Chiari malformation type 1 with sign of upbeat nystagmus was reported. The patient was a 9-year-old girl who was admitted to the Department of Neurosurgery with the chief complaints of snoring and headaches. The neurological examination on admission revealed a lower cranial nerve disturbance bilaterally and upbeat nystagmus in the primary ocular position that increased in amplitude upon gazing upward, and horizontal nystagmus on gazing laterally. The skull tomogram showed platybasia. The vertebral arteriogram and the pneumoencephalotomogram showed downward displacement of cerebellar tonsils to the level of C2. The patient received suboccipital craniectomy and C1, C2 laminectomy for decompression and the foramen of Majendie was opened. From the operative findings the diagnosis was confirmed as Chiari malformation type 1. In the first two postoperative weeks, the upbeat nystagmus as well as other symptoms was gradually improved. It was therefore considered that the responsible lesion of the upbeat nystagmus in this case might be in the lower brain stem or the inferior vermis due to mechanical compression or circulatory disturbance.

Arnold-Chiari Malformation↗