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1/17. Treatment of acute comitant esotropia in Chiari I malformation.

    PURPOSE: To explore the appropriate treatment of acute comitant esotropia in patients with Chiari I malformation. DESIGN: Interventional case reports and literature review. PARTICIPANTS: Two patients with Chiari I malformation presenting with acute comitant esotropia are described. INTERVENTION: strabismus surgery, then neurosurgical decompression of the Chiari I malformation was performed. MAIN OUTCOME MEASURE: Both patients were evaluated for resolution of esotropia and other ocular motility problems. RESULTS: After initially successful strabismus surgery, both patients developed recurrent esotropia with diplopia, which resolved on suboccipital decompression. CONCLUSION: Comitant esotropia may recur and other eye movement disorders may develop after initially successful strabismus surgery in patients with Chiari I malformation. The data suggest that the appropriate sequence of treatment should first be suboccipital decompression, then strabismus surgery if spontaneous realignment does not occur, but further studies are needed to confirm this impression.
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keywords = comitant, strabismus
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2/17. Isolated comitant esotropia and Chiari I malformation.

    PURPOSE: To report four patients with isolated comitant esotropia and Chiari I malformation and discuss the most appropriate management. methods: case reports and literature review. RESULTS: All four patients (5, 14, 16, and 37 years of age) presented with an isolated comitant esotropia that led to the diagnosis of Chiari I malformation. The first two patients underwent uncomplicated neurosurgical decompression of their malformation, followed by complete resolution of their esotropia. The third patient underwent strabismus surgery and experienced initial resolution of the esotropia, but eventual recurrence resulted in the strabismus surgery being repeated 5 years later. The fourth patient had strabismus surgery with resolution of the esotropia but only 2 months of follow-up. CONCLUSION: Although management of patients with Chiari I malformation and severe neurologic findings typically includes surgical decompression, management is less straightforward in cases with subtle findings or in which ocular findings are isolated. The decision to perform neurosurgical decompression or strabismus surgery should still be made on a case-by-case basis, with the understanding that strabismus surgery may provide only temporary ocular alignment.
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ranking = 1.0142246514233
keywords = comitant, strabismus
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3/17. Resolution of acute acquired comitant esotropia after suboccipital decompression for Chiari I malformation.

    PURPOSE: To report a case of acute comitant esotropia successfully treated with suboccipital decompression in a 9-year-old male patient with Chiari I malformation. DESIGN: Interventional case report. methods: A 9-year-old male with Chiari I malformation had acute onset of diplopia, headache, and comitant esotropia. RESULTS: About 9 months after suboccipital decompression, diplopia resolved and there was near orthophoria on examination 15 months after surgery. CONCLUSION: In view of our case and after a review of literature, we advocate primary suboccipital decompression to treat acute comitant esotropia in patients with Chiari I malformation. A follow-up period of at least 1 year rather than 6 months seems necessary to assess surgery effects.
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ranking = 1.1417735266759
keywords = comitant
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4/17. Regression of symptomatic syringomyelia after resection of posterior fossa tumour.

    syringomyelia associated with posterior fossa tumours is a very infrequent combination of pathological entities. The few cases which have been reported generally were asymptomatic in respect of the spinal cavitations. The authors report on a 36-year-old woman with a large extradural posterior fossa epidermoid tumour with a concomitant holocord symptomatic syringomyelia. Some of her symptoms were clearly attributed to the intraspinal cavitation. The lesions were both diagnosed by magnetic resonance imaging (MR). The patient did well after surgery of the brain lesion, with an objective improvement in her neurological status and a complete resolution of the syrinx documented by the MR 7 months after tumour removal.syringomyelia in this case could be explained by blockage of the cerebrospinal fluid (CSF) circulation at the foramen magnum which in turn resulted in cranio-spinal pressure dissociation. This led to an accumulation of extracellular fluid (ECF) in the central canal, starting cavitation. Consequently, the syrinx was slowly expanded by the long-standing "slosh" effect of the systolic pressure waves. However, also via a distortion mechanism within the posterior fossa a pathologically plugged obex could have contributed to syrinx formation by means of preventing drainage of fluid from the ventricular CSF system.
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ranking = 0.16311050381084
keywords = comitant
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5/17. Resolution of Chiari malformation after repair of a congenital thoracic meningocele: case report and literature review.

    OBJECTIVE AND IMPORTANCE: Many theories have been proposed regarding potential causative factors for Chiari malformations. An unusual case is described in which regression of a congenital Chiari malformation was observed after repair of a thoracic meningocele without direct surgical intervention to decompress the craniocervical junction. This supports the importance of an in utero craniospinal pressure gradient as a potential cause for congenital, but reversible, cerebellar herniation. CLINICAL PRESENTATION: A newborn baby was observed to have a thoracic meningocele. magnetic resonance imaging scan revealed a concomitant Chiari malformation. No neurological deficits were present at initial examination. INTERVENTION: The patient underwent surgical closure of the thoracic meningocele and untethering of the spinal cord at the site of the dural defect. A postoperative magnetic resonance imaging scan obtained 3 months after the operation revealed complete resolution of the cerebellar herniation. CONCLUSION: The resolution of the Chiari malformation in this child may have resulted from restoration of normal cerebrospinal fluid flow and elimination of the meningocele-related cerebrospinal fluid pressure gradient between the intracranial and intraspinal compartments.
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ranking = 0.16311050381084
keywords = comitant
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6/17. Acquired Chiari type I malformation managed by supratentorial cranial enlargement.

    INTRODUCTION: Acquired Chiari type-I malformation in hydrocephalic patients who have undergone surgical treatment was initially thought to depend on a craniocephalic disproportion induced by the cerebrospinal (CSF) shunt. However, most of the reports in the literature deal with children with lumbo-peritoneal shunts and emphasize the pathogenic role of the cranio-spinal pressure differential across the foramen magnum brought about by this type of shunt. METHOD: In the present report, the authors describe two further cases of symptomatic acquired Chiari type-I malformation observed in two adolescents operated on for correction of pseudotumor cerebri in one (lumbo-peritoneal shunt) and of a suprasellar arachnoid cyst (cysto-ventriculo-peritoneal shunt) in the other. RESULTS: In both subjects, both the clinical manifestations and the cerebellar tonsillar herniation regressed after supratentorial cranial expansion, without the need for any manipulation of the shunt devices implanted earlier. DISCUSSION: These results, together with the observation of the concomitant upward and downward herniation of the cerebellum in both patients, indicate that secondary craniocephalic disproportion plays a relevant role in the genesis of acquired Chiari type-I malformation in children bearing extrathecal CSF shunts.
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ranking = 0.16311050381084
keywords = comitant
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7/17. Progressive cerebellar tonsillar herniation with recurrent divergence insufficiency esotropia.

    The Chiari malformations are characterized by herniation of posterior fossa contents through the foramen magnum. Chiari I malformation is currently defined as ectopia of the cerebellar tonsils more than 5 mm below the foramen magnum. Extension of the cerebellar tonsils up to 3 mm may be found in the normal population. Although Chiari malformations are congenital, symptoms often do not manifest until the third and fourth decades of life, or even later. patients usually present with headache, lower cranial nerve palsies, downbeat nystagmus, ataxia, or dissociated anesthesia of the trunk and extremities. Definitive diagnosis is made by magnetic resonance imaging (MRI), which shows the compressed tonsils extending through the foramen magnum into the cervical subarachnoid space. One of the rare presenting signs of Chiari I malformations is acquired esotropia with a divergence insufficiency pattern. We report such a case in which the initial neuroimaging showed tonsillar herniation, but of insufficient magnitude to meet diagnostic criteria for Chiari I malformation. When the strabismus recurred after initially successful eye muscle surgery, follow-up scan showed progressive tonsillar herniation.
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ranking = 0.0071123257116459
keywords = strabismus
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8/17. Chiari I malformation and acute acquired comitant esotropia: case report and review of the literature.

    The authors describe a rare case of a Chiari I malformation presenting with acute acquired comitant esotropia (AACE) in a 5-year-old boy. A posterior fossa decompression with duraplasty and a C1-2 laminectomy were performed. There was an immediate postoperative improvement in the esotropia, which completely resolved by 7 months following surgery. The pertinent literature is discussed and reasons are presented for recommending posterior fossa decompression in certain patients, rather than strabismus surgery, as the initial treatment for esotropia. The authors suggest that in patients with AACE, even subtle symptoms and signs of Chiari I malformation should prompt imaging of the posterior fossa. Strong consideration should be given to performing posterior fossa decompression in patients with Chiari I malformation and AACE before strabismus surgery because the esotropia may completely resolve with decompression.
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ranking = 0.82977717047751
keywords = comitant, strabismus
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9/17. Resolution of neurogenic arterial hypertension after suboccipital decompression for Chiari malformation. Case report.

    A Chiari malformation Type I may remain asymptomatic until the patient has reached adulthood and acute presentation of symptoms occurs. In several clinical and experimental studies it has been shown that essential hypertension is associated with vascular compression of the brainstem, particularly of the rostral ventrolateral medulla oblongata. Nevertheless, two cases of Chiari malformation and neurogenic arterial hypertension have been reported. In this article the authors describe a patient with Chiari malformation Type I and neurogenic arterial hypertension. A simple suboccipital decompression not only provided neurological improvement, but also led to resolution of the hypertension. In cases of Chiari malformation and concomitant neurogenic arterial hypertension, careful preoperative clinical and neuroimaging assessments may reveal the cause of the arterial hypertension. Resolution of neurogenic arterial hypertension may be expected even in a case of simple suboccipital decompression.
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ranking = 0.16311050381084
keywords = comitant
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10/17. Syringobulbia in a pediatric population.

    OBJECTIVE: To better understand the presentation, management, and outcome of syringobulbia in the pediatric age group. methods: The University of iowa pediatric neurosurgery database was searched for patients under the age of 18 with a diagnosis of syringobulbia. The patients' records were retrospectively reviewed for demographic data, chief complaint and presenting symptoms, neurological and radiographic findings, treatment, outcome, and complications. Children with open neural tube defects and Chiari II malformations were excluded. RESULTS: Six pediatric patients were identified as meeting inclusion criteria. The average age at time of surgery was 14.8 years. The chief complaints were vision impairment in three children and numbness, gait instability, and headache worsened with Valsalva in one patient each. Other prominent symptoms included sleep apnea and weakness. All patients showed at least one cranial nerve dysfunction. Radiographs revealed hindbrain herniation and associated syringomyelia in all cases. Two patients had scoliosis. Treatment was posterior fossa decompression with cerebellar tonsillar shrinkage, opening of foramen of Magendie, and duraplasty. Two patients also required concomitant ventral decompression. The cavity of syringobulbia communicated with syringomyelia and the fourth ventricle in most children but was distinct from the fourth ventricle. Two patients received fourth ventricle to subarachnoid shunts. Follow-up averaged 3.2 years, and all patients clinically improved after surgery. magnetic resonance imaging documented resolution of syringobulbia in all cases, with syringomyelia improving in all cases. There was no permanent morbidity or mortality in the series. CONCLUSION: Syringobulbia is strongly associated with Chiari malformation and syringomyelia, and patients often present because of cranial nerve palsies. Posterior fossa decompression is a safe and effective treatment.
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ranking = 0.16311050381084
keywords = comitant
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