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At least 19 recordsLinked to original sources
PubMed1982

Basilar impression and platybasia in osteogenesis imperfecta tarda.

Osteogenesis imperfecta, a rare, genetically transmitted disorder of bone, is known to be associated with the development of basilar impression and platybasia. These deformities of the base of the skull may cause neurosurgical abnormalities secondary to compression of the brainstem and hydrocephalus. The case is presented of a young boy with a family history of osteogenesis imperfecta tarda who suffered respiratory arrest during hospitalization. Cranial nerves and pyramidal tract signs were demonstrated. Roentgenograms showed severe basilar impression and hydrocephalus. Decompression of the brainstem and shunting were performed with improvement in the patient's neurological status. This case represents a rare by significant central nervous system complication of osteogenesis imperfecta. Early recognition and implementation of aggressive treatment are important if irreversible neurological deficits are to be avoided.

Adolescent↗
PubMed1996

[Platybasia, basilar groove, odontoid process and kinking of the brainstem: a common etiology with idiopathic syringomyelia, scoliosis and Chiari malformations].

INTRODUCTION: A common aetiopathogenic theory for basilar groove (IMB), platybasia (PTB), odontoid retrocession (RTO), kinking of the brainstem (KTC) applied to idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID) and Arnold-Chiari malformation (ARCH) is presented. Confirmation is based on an abnormally low position of the conus medullaris (CMB) in the patients with SMI. MATERIALS AND METHOD: 292 patients with syringomyelia (SM), 231 with SMI were selected. Of these, 55 were chosen who had SMI and in whom the level of the conus medullaris (NCM) could be determined, together with the figures for SMI, IMB, PTB, RTO, KTC, ESCID and ARCH on cervical and lumbar MR. The position of the conus medullaris in 50 patients who did not have SM, ESCID nor ARCH on cervical and lumbar RM was determined. RESULTS: 32 patients had an increased basal angle (58.18%). There was an IMB in a quarter of the patients (25.45%). RTO was observed in half of the patients (47.27%). Just over one third presented a KTC. 6% of the control group had CM at the level of the body of L1, whilst 84.21% of the patients with SMI presented a partial or complete CM image at this level. CONCLUSIONS: CMB in SMIU and its close relationship with IMB, PTB, RTO AND KTC and also with ESCID and ARCH make it likely that they share the same aetiopathogenic mechanism: an abnormal lack of synchronization of the growth of the neuro-axis and the neural canal (AACNN), causing a specific disorder which is seen as different syndromes.

Adult↗
PubMed1997

[A new surgical treatment for syringomyelia, scoliosis, Arnold-Chiari malformation, kinking of the brainstem, odontoid recess, idiopathic basilar impression and platybasia].

INTRODUCTION: Based on medullary traction as responsible for idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID), Arnold Chiari malformation (ARCH), platybasia (PTB), basilar impression (IMB), odontoid recess (RTO) kinking of the brain stem (KTC) and considering the medullary traction to be transmitted by the filum terminale (FT), a surgical technique for the section of FT (SFT) is described in three cases of SMI, one of ESCID, and one of ARCH with no lumbar dysraphia. MATERIAL AND METHODS: A 34-year-old woman with cervico-brachialgias, paresthesias, bilateral babinski and a centro-medullary cavity C3-C7. A 26-year-old male with cervico-brachialgias, hypoestesia in left hemybody, and cervicobulbar cavity. A 19-year-old female with ESCID since the age of 14th, with episodes of reacuting, and 38o of dorsolumbar curvature. A 67-year-old woman with intense headache, hypoesthesia of the hands, paraparesia and ARCH. A 23-year-old man with marked tetraparesia, bilateral babinski, anesthesia of both legs, SMI, ESCID, ARCH and hydrocephaly. RESULTS: After SFT: in the SMIs the thermo-algesic, disesthetic and algic dissociation disappeared. In ESCID there was a reduction to 31o in the curvature in nine months. On ARCH the headaches ceased and there was recovery of touch and paraparesia. CONCLUSIONS: SFT is a useful etiological treatment for SMI, ESCID, ARCH. Also, in ESCID it is possible to avoid stress on the medulla due to its surgical reduction.

Adult↗
PubMed2000

Management of craniocervical junction dislocation.

The discovery of a craniocervical junction malformation requires management in three steps: (1) The patterns must be recognized using tomographic measurements (Chamberlain's line, Wackenheim's line). Dynamic flexion-extension studies are necessary to assess stability or instability. Stable patterns range from platybasia to basilar invagination, with gradual deformation, and are frequently associated with Chiari malformation. Unstable patterns characterized by odontoid instability are the equivalent of an odontoid fracture. The origin is malformative (hypoplasia, aplasia of the dens, os odontoidum), but the last may be difficult to distinguish from an old odontoid fracture. They are found in many syndromes (Down, Morquio, etc.). Unstable atlantoaxial patterns with atlas assimilation are hardly reducible; they evolve toward progressive instability. (2) The neurological consequences must be defined from the clinical features of the spinal cord and the cranial nerves. Both static and dynamic MRI scans must be performed; in this way identification of the neural abnormalities (hydromyelia, Chiari, etc.) and of the osseous compression is possible. (3) The most appropriate operative procedure must be selected: stable platybasia with a nervous compression by Chiari is cured only by posterior decompression; odontoid instability is cured by reduction and posterior fixation, using hooks and autologous bone grafts on the posterior arches of C-1 and C-2. Sometimes a transarticular screw fixation of C1-2 is necessary if there is a defect on the C-1 posterior arch. Craniocervical dislocations with assimilation of the atlas require posterior occipito-vertebral bony fixation with grafts and external halo immobilization or internal fixation with hooks or screws, with anterior transoral decompression in a second step.

Arnold-Chiari Malformation↗
PubMed2001

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↗
PubMed1984

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↗
PubMed1976

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↗
PubMed2000

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↗
PubMedDate not supplied

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↗
PubMed1975

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↗
PubMed1978

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↗
PubMedDate not supplied

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↗