Cases reported "Platybasia"

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1/59. Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases.

    OBJECTIVES: To describe the clinical and neuroradiological features of basilar impression in patients with osteogenesis imperfecta type IV. methods: Four patients with basilar impression were ascertained in a population study of osteogenesis imperfecta. All four had detailed clinical and neuroradiological examination with both CT and MRI of the craniocervical junction and posterior fossa structures. RESULTS: All four showed significant compression of the posterior fossa structures and surgical decompression was performed with relief of symptoms. CONCLUSION: Symptoms of cough headache and trigeminal neuralgia occurring in patients with osteogenesis imperfecta are indications for detailed clinical and neuroradiological investigation to document basilar impression. ( info)

2/59. Neurofibromatosis, stroke and basilar impression. Case report.

    Neurofibromatosis type 1 (NF1) can virtually affect any organ, presenting most frequently with "cafe au lait" spots and neurofibromas. Vasculopathy is a known complication of NF1, but cerebrovascular disease is rare. We report the case of a 51-year-old man admitted to the hospital with a history of stroke four months before admission. On physical examination, he presented various "cafe au lait" spots and cutaneous neurofibromas. neurologic examination demonstrated right-sided facial paralysis, right-sided hemiplegia, and aphasia. Computed tomography scan of head showed hypodense areas in the basal ganglia and centrum semiovale. Radiographs of cranium and cervical spine showed basilar impression. Angiography revealed complete occlusion of both vertebral and left internal carotid arteries, and partial stenosis of the right internal carotid artery. A large network of collateral vessels was present (moyamoya syndrome). It is an uncommon case of occlusive cerebrovascular disease associated with NF1, since most cases described in the literature are in young people, and tend to spare the posterior cerebral circulation. Basilar impression associated with this case may be considered a pure coincidence, but rare cases of basilar impression and NF1 have been described. ( info)

3/59. Precise cannulation of the foramen ovale in trigeminal neuralgia complicating osteogenesis imperfecta with basilar invagination: technical case report.

    OBJECTIVE AND IMPORTANCE: trigeminal neuralgia is a rare feature of basilar invagination, which is itself a complication of osteochondrodysplastic disorders. Microvascular decompression is an unattractive option in medically refractory cases. The conventional percutaneous approach to the trigeminal ganglion is anatomically impossible because the foramen ovale points inferiorly and posteromedially. We report a new technique for image-guided trigeminal injection in a patient with basilar invagination complicating osteogenesis imperfecta. CLINICAL PRESENTATION: A 26-year-old woman with osteogenesis imperfecta presented with a 3-year history of typical left maxillary division trigeminal neuralgia, which was poorly controlled by carbamazepine at the maximum tolerated dose. She had obvious cranial deformities, left optic atrophy, delayed left eye closure, tongue atrophy, but normal facial sensation and corneal reflexes. A computed tomographic scan and magnetic resonance imaging confirmed severe basilar invagination. TECHNIQUE: Frameless stereotactic glycerol injection of the left trigeminal ganglion was performed under general anesthesia using the infrared-based EasyGuide Neuro system (Philips Medical Systems, Best, The netherlands) with magnetic resonance imaging and computed tomographic registration. The displaced and distorted left foramen ovale was cannulated via a true frameless stereotactic method with the trajectory determined by virtual pointer elongation. The needle placement was confirmed with injection of contrast medium into the trigeminal cistern. The path needed to enter the foramen traversed the right cheek, soft palate, and left tonsil. The patient went home pain-free with a preserved corneal reflex and no complications. CONCLUSION: Frameless stereotaxy allows customization to individual patient anatomy and may be adapted to a variety of percutaneous procedures used in areas where the anatomy is complex. ( info)

4/59. stroke following appendectomy under general anesthesia in a patient with basilar impression.

    We report a boy who developed a vertebral stroke immediately after an appendectomy. Basilar impression was diagnosed eight years after this event when skull roentgenograms revealed basilar impression with high standing tip of the odontoid. We speculate that muscle relaxation and cervical hyperextension during intubation in the presence of basilar impression resulted in vertebral artery dissection and stroke. We suggest that patients with vertebral stroke and no obvious risk factors should be evaluated for the presence of malformations of the craniovertebral junction to be able to take precautions against excessive neck movement during intubation. ( info)

5/59. 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. ( info)

6/59. trigeminal neuralgia associated with achondroplasia. Case report with literature review.

    A 59-year-old male with a history of 2 years of typical trigeminal neuralgia manifested the characteristics of achondroplasia. X-ray and magnetic resonance imaging demonstrated basilar impression, deformity of the posterior fossa with marked asymmetry of petrous bone and compression of pons and trigeminal nerve by the left vertebral artery and anterior inferior cerebellar artery. Microvascular decompression was performed through a suboccipital craniectomy. The neuralgia disappeared soon after surgery and remains completely resolved until today. This is the first reported case of trigeminal neuralgia in a patient with achondroplasia. The deformity of the skull base was considered to influence the development of the trigeminal neuralgia. ( info)

7/59. Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report.

    STUDY DESIGN: A case report of craniocervical spine lesions including basilar impression, atlantoaxial dislocation, and syringomyelia, with osteogenesis imperfecta is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the surgical management of craniocervical spine lesion with osteogenesis imperfecta. SUMMARY OF BACKGROUND DATA: osteogenesis imperfecta is known to have various spine lesions as complications. However, few reports have described craniocervical lesions associated with osteogenesis imperfecta. methods: A 14-year-old girl with osteogenesis imperfecta, Silence classification IVB, experienced difficulty walking, with marked motor disturbance and muscle weakness in the extremities. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities, and positive Babinski reflex and ankle clonus were observed bilaterally. Basilar impression, atlantoaxial dislocation, and syringomyelia were shown by plain radiography, tomography, three-dimensional computed tomography, and magnetic resonance imaging. RESULTS: In the reported patient, posterior fossa decompression and atlantoaxial posterior fusion could not be performed because the foramen magnum and upper cervical spine invaginated to the base of the skull. Therefore, occipitocervical spine fusion using titanium loop and wires was performed at the reduced position of the atlantoaxial dislocation, resulting in improvement of neurologic deficits. CONCLUSIONS: For patients with atlantoaxial dislocation, syringomyelia, and basilar impression without clinical symptoms or signs of brain stem compression, occipitocervical spine fusion alone at the reduction of the atlantoaxial dislocation may be indicated because these procedures improve neurologic deficits and prevent postoperative development of basilar impression and enlargement of syringomyelia. ( info)

8/59. Megadolichobasilar anomaly, basilar impression and occipito-vertebral anastomosis.

    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. ( info)

9/59. 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. ( info)

10/59. 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. ( info)
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