1/16. 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.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/16. 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.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
3/16. Circumferential decompression of the foramen magnum for the treatment of syringomyelia associated with basilar invagination.Posterior fossa decompression utilizing suboccipital craniectomy and duraplasty remains the standard surgical treatment for Chiari-associated syringomyelia. In the presence of basilar invagination, anterior decompression, typically transoral odontoidectomy, or posterior decompression may be performed. We report two cases in which anterior and posterior (circumferential) decompression of the foramen magnum was used to treat cervical syringomyelia successfully. These cases demonstrate that circumferential decompression of the foramen magnum may be necessary in some cases of cervical syringomyelia associated with basilar invagination and Chiari malformation.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
4/16. Cervical arachnoidal cyst with basilar impression and arnold-chiari malformation: a case report.A case of cervical spinal arachnoidal cyst is presented. The association with basilar impression and arnold-chiari malformation is a peculiarity seldom reported. The clinical aspects with remission and exacerbation are discussed. The importance of the neuroradiological findings and the usefulness of the neurophysiological examination (evoked potentials) are suggested.- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
5/16. Spontaneous regression of syringomyelia in hajdu-cheney syndrome with severe platybasia. Case report.Hadju-Cheney syndrome (HCS) is a rare autosomal-dominant disorder with variable expressivity. It is characterized by facial dysmorphism, premature tooth loss, osteolysis of distal phalanges, and skull abnormalities. In some cases, progressive platybasia can occur and can lead to Chiari malformation with an obstruction of cerebrospinal fluid flow. To the best of the authors' knowledge, only five cases of HCS-associated syringomyelia have been reported in the literature. Because of the rarity of this association, little is known about its natural history. The authors present the case of a 16-year-old boy affected by HCS. On initial magnetic resonance (MR) imaging, a severe basilar invagination with Chiari malformation and cervicothoracic syringomyelia was documented. The syringomyelia had no clinical manifestations. A repeated MR image demonstrated a spontaneous resolution of the syrinx with no changes in the tonsil or the platybasia. The regression of the syringomyelia was confirmed by a control MR imaging examination performed after a 2-year period. No changes in the patient's clinical conditions were found during the follow-up period. This is the first case of spontaneous regression of the syringomyelia despite a severe platybasia in HCS. It did not appear correlated to a modification of the tonsil's structure or position. This observation illustrates one possible evolution of syringomyelia in the natural history of HCS and raises the question of the potential mechanisms involved in the spontaneous drainage of the syringomyelic cavity.- - - - - - - - - - ranking = 2keywords = malformation (Clic here for more details about this article) |
6/16. Transoral decompression evaluated by cine-mode magnetic resonance imaging: a case of basilar impression accompanied by Chiari malformation.Cine-mode magnetic resonance imaging provides simultaneous images of cerebrospinal fluid flow dynamics. A patient with a basilar impression accompanied by a Chiari malformation and von Recklinghausen's disease who underwent transoral decompression is reported. Preoperative cine-mode magnetic resonance imaging visualized an associated obstruction of cerebrospinal fluid pulsatile flow at the level of the foramen magnum. Tonsilar herniation (Chiari I malformation) and hydrocephalus were also present. Postoperatively, the obstruction of cerebrospinal fluid flow was resolved concomitant with the correction of the cervicomedullary angulation. On the basis of observations made by magnetic resonance imaging, the surgical treatment of basilar impression accompanied by Chiari malformation is briefly discussed.- - - - - - - - - - ranking = 7keywords = malformation (Clic here for more details about this article) |
7/16. Successful treatment of adult arnold-chiari malformation associated with basilar impression and syringomyelia by the transoral anterior approach.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.- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
8/16. exophthalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy.We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II arnold-chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar symptoms, proximally pronounced muscular atrophy as well as a left hemihypesthesia was shown neurologically. Although the orbit CT was normal, sonography revealed thickened ocular muscles. There was euthyroidism in diffuse goiter with negative thyroid autoantibody findings. Because of lack of definitive detection of muscular swelling, tumor, or vascular anomaly in the various images, orbital dysplasia in the context of a malformation syndrome affecting several organs is suggested as the cause of the exophthalmos.- - - - - - - - - - ranking = 2keywords = malformation (Clic here for more details about this article) |
9/16. Total alleviation of downbeat nystagmus in basilar impression by transoral removal of the odontoid process.The presence of primary position downbeat nystagmus indicates disease at the cervicomedullary junction where craniovertebral anomalies are commonly encountered. This paper reports a patient whose only symptom was disabling oscillopsia and downbeat nystagmus secondary to basilar impression and arnold-chiari malformation. He experienced total resolution of his symptoms following transoral removal of the odontoid process. In the past it was felt that ocular signs and symptoms did not improve with surgery for craniovertebral anomalies. In light of this report, the ocular symptoms of craniovertebral anomalies may be considered indications in themselves for surgical intervention.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
10/16. MR imaging of familial basilar impression.Basilar impression was found in three members of one family. The mother showed an asymptomatic deformity, her eldest son complained of headache, drop-attacks, nystagmus, unilateral ophthalmoplegia, and ataxia; the middle son presented with headache, nystagmus, and hemiparesis. Magnetic resonance (MR) imaging demonstrated convexobasia of various degrees with elevation of the upper spine and malformation of the occipital bone. The medulla oblongata and the pons were flattened and dislocated backward in two cases. Chiari malformation was present in one case and mild hydrocephalus in another. A comparison of MR with CT imaging demonstrates some advantages of the former method in the assessment of the neural structures directly involved in basilar impression.- - - - - - - - - - ranking = 2keywords = malformation (Clic here for more details about this article) |
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