Osteomalacia, myopathy and basilar impression.
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A 21-year-old Japanese man with basilar impression with an anomalous configuration of the odontoid process and many other vertebral anomalies is reported. We thought that posterior decompression alone would be hazardous; therefore, in one session, the odontoid tip and anterior arch of the atlas were removed transorally, and posterior fixation between the occipital squama and the lamina of C-3 using acrylic plastic was performed. This treatment resulted in marked clinical improvement and required only a short hospital stay.
A case of adult type I Arnold-Chiari malformation associated with basilar impression, syringomyelia, atlantoaxial dislocation, and occipitalization of the atlas is reported. Preoperative magnetic resonance imaging clearly revealed evidence of severe anterior compression of the cervicomedullary junction due to basilar impression and a sharp clivoaxial angle. Therefore, transoral anterior decompression and fusion were performed, resulting in an improvement of the patient's neurologic signs and symptoms. Postoperative magnetic resonance imaging showed an obvious reduction of the tonsillar herniation and syringomyelia, as well as an improvement of the cervicomedullary compression.
A very rare case of traumatic basilar impression is reported. The patient, a 57-year-old man, was hit on the head vertically in the parietal region. X ray of the cervical spine and computed tomography (CT) scans showed intracranial indentation of the atlas and the odontoid process with a depressed fracture around the foramen magnum. There are no previous reports about this type of fracture.
A 4-year-old boy with hypochondroplasia presented with delay in gross motor development. Magnetic resonance imaging demonstrated basilar impression with compression at the craniovertebral junction and mild degree of hydrocephalus. Posterior fossa decompression resulted in improvement in neurologic function and relief of hydrocephalus.
Osteogenesis imperfecta is a disease of bone formation subdivided into two types, congenita and tarda. It is associated with bony fragility, blue sclerae and abnormality of tooth dentin. Rarely the tarda form is associated with basilar invagination or infolding of the foramen magnum and upper cervical segments into the posterior fossa. This results in hydrocephalus and a spectrum of neurologic dysfunction known as the foramen magnum compression syndrome. Many radiologic methods have been used to evaluate basilar invagination including plain film and CT. We describe a patient with osteogenesis imperfecta tarda examined with CT, with a unique finding of diffuse cerebral atrophy associated with basilar invagination.
OBJECTIVE: A surgical variation of the technique of facial translocation procedure is proposed, which has been called midfacial translocation for approach to the entire medial and lateral region of the middle third of the face, including the rhinopharynx, sphenoid sinus, pterygomaxillary fossa, odontoid process, and clivus. PATIENTS AND METHODS: The medical records of five treated patients accordingly were reviewed for an analysis of the surgical technique, the disease, the topography of the lesion, and the complications. RESULTS: The approach permitted ventral decompression of the bulbomedullary junction with resection of the C1 arch and the odontoid process in four patients and resection of a chordoma of the clivus located along the midline and extending intradurally in the fifth patient. Only one patient presented with dehiscence of the posterior half of the soft palate, this being the only complication observed following surgery in these patients. Three months postoperatively, no patient presented any aesthetic alteration of the face. Functionally, there was only infraorbital hypoaesthesia on the side of flap rotation. CONCLUSION: The technique of midfacial translocation provides both good surgical approach and access to the rhinopharynx, pterygomaxillary fossa, high odontoid process and clivus, with few adverse sequelae for the patient.
BACKGROUND: In the management of basilar invagination, traction therapy may help by pulling down the odontoid process away from the brain stem that may result in clinical and radiological improvement. We aimed to discuss the role of the halo vest apparatus traction on the reduction of severe anterior compression pathologies in basilar invagination. CASE DESCRIPTION: We describe a simple and safe cervical traction method by the halo vest apparatus that is followed by rigid posterior occipitocervical fixation and foramen magnum decompression in a patient who presented with basilar invagination and symptoms of severe brain stem compression. An MR-suitable halo vest apparatus was used for reduction of the deformity. The reduction of the basilar invagination was achieved gradually by distracting the halo crown in stages. CONCLUSION: The halo vest apparatus can be safely used in complex craniocervical junction anomalies. An effective cervical traction can be performed in basilar invagination, and reduction of the deformity may be achieved without the risk of overdistraction. In some cases, even partial reduction of the deformity may facilitate brain stem and spinal cord relief without any need of posterior decompression. Patients may benefit from ambulatory functions because bed rest is eliminated in this procedure. Neurovascular structures and the degree of the reduction can be observed on MRIs when an MR-suitable device is used.
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We report the presence of basilar invagination, an unexpected and previously undescribed abnormality of the skull base, in 7 of 38 long-term survivors of multisystem Langerhans' cell histiocytosis. The abnormality is acquired, but its pathogenesis is uncertain.
OBJECTIVE: To tell the rare association of Arnold-Chiari II malformation and basilar impression and to describe an exceptional clinical state with a particular surgical approach. MATERIALS AND METHODS: To analyse a case of a patient suffering from this congenital malformation, her progressive clinical state, the surgical approach. RESULTS: N.G. a 42-year-old woman, suffers from a cerebello-spino-medullar disturbance responsible for a major dependence and respiratory insufficiency with a risk of death. The results of the surgical treatment meant to decompress the craniovertebral junction are exceptional. DISCUSSION: The analysis of the literature show the special feature of this case as much for the rarity of the diagnosis as for the progressive clinical state and the remarkable post operative result. CONCLUSION: The association of these two malformations is seldom alluded to in the literature and such a post operative result has apparently never been described. In addition, this case tends to prove that a second operation's worth considering if the first has shown its short-fall whichever the patient's clinical state.
The authors describe a case of a megadolichobasilar anomaly associated with basilar impression, bilateral megadolichocarotid arteries and an occipito-vertebral anastomosis. The concurrence of these anomalies lends support to the hypothesis that congenital factors play a part in the origin of the megadolichobasilar anomaly.
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OBJECTIVE: To determine on radiographs the presence of Basilar Impression (BI) in children with Osteogenesis Imperfecta (OI). To confirm this sign and altered geometrical relationships of the craniocervical junction in course of time with magnetic resonance imaging (MRI). METHODS AND PATIENTS: In a cohort study of 130 patients with OI (OI type I: 85; OI type III: 21; OI type IV: 24) lateral radiographs of the skull and cervical spine were made in a standardised way. MRI scans were performed when BI was suspected based upon protrusion of the odontoid above Chamberlain's line. Intracranial abnormalities as well as the basal angle were described. Neurological examination was performed in patients with conclusive BI at MRI-scan. RESULTS AND DISCUSSION: In eight patients BI could be confirmed by MRI-scan. None of the children had or developed in time neurological symptoms or signs. Follow up of BI by MRI scans was done in seven patients (mean: 5 years; range: 2-6 years). No alteration of intracranial findings were seen at subsequent investigation, although in one child Chamberlain's line increased from 8 (first MRI) to 15 mm (last MRI). BI can be diagnosed by radiographs but in the extreme osteoporotic bone and altered anatomy of the craniocervical junction of children with OI MRI is preferable. As intracranial pathology can be demonstrated by MRI, also a relation can be laid to possible neurological symptoms and signs at clinical examination. CONCLUSION: In our cohort study no alteration of the intracranial contents was seen at subsequent MRI scans. Although anatomic deformations exist in BI, no neurological symptoms or signs were present in our study and no operative reconstruction had to be performed. Periodical MRI-scan has not been of influence on the clinical decision making process. At the moment we perform a MRI-scan if BI is suspected at lateral skull radiographs. The MRI images serve as reference findings to anticipate on possible future symptoms and signs of neurological deficit.
The expansive lesions, whether tumoral or not, originated at the level of the anatomical region of the skull base (SB), show a great histologic variety and clinicaly they cause a variable chronic neurological disfunction. Surgical treatment appears to be the best therapeutic option. An exhaustive knowledge of the topographic anatomy of this area is the mandatory in order to design an appropriate surgical strategy. In many cases, a narrow cooperation with specialists is necesary. As in any other surgical activity, a complete excision of the lesion and an optimal functional and aesthetic rehabilitation, without complications, is the challenge of the surgical team. The approach to the anatomical area of the SB is not single, but is based on a number of procedures, although none of them could be considered the best, or without technical difficulty or any complications. Within the group of transoral approaches, the Le Fort I-Palatal split (LFPS) technique has been considered by different authors an excellent way to approach the clivus and the occipito-vertebral joint. We report the case of a patient, treated in cooperation with the Department of Neurosurgery of our hospital. He was clinical and radiologically diagnosed of basilar impresion with bulbar compression, and the MRI revealed the presence of a located extradural fibrous injury above the odontoid apophysis. Therefore we chose the use of a LFPS to approach this lesion. With an optimal surgical field, a complete excision of the lesion was obtained. The postoperatory result in the subsequent follow-up was highly satisfactory.