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1/15. A craniocervical injury-induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. Case report.

    The authors report on a 19-year-old man with an acquired tonsillar herniation caused by a craniocervical junction injury in which serial magnetic resonance (MR) images demonstrated patent and isolated segments of the central canal participating in the dilation and then formation of a cervical syrinx. The patient was involved in a motor vehicle accident; he developed tonsillar herniation as a complication of subarachnoid and epidural hemorrhage, predominantly observed around the cisterna magna and upper cervical canal. Repeated MR images obtained over an 11-month period indicated the for mation and acute enlargement of the syrinx. Ten months after the accident, the patient presented with sensory disturbance in both upper extremities and spasticity due to syringomyelia. He underwent craniocervical decompressive surgery and doraplasty, which reduced the size of syringomyelia. The authors postulate that the patent central canal may play a role in determining the location of a syrinx remote from a focus of cerebrospinal fluid obstruction.
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2/15. Chiari Type I malformation and upper airway obstruction in adolescents.

    Chiari Type I malformation is characterized by cerebellar tonsil herniation through the foramen magnum. Manifestations may include new-onset upper airway obstruction. We describe two patients who had bilateral vocal cord paralysis and Chiari Type I malformation. These cases highlight the importance of suspecting a neurologic abnormality such as Chiari malformation in new-onset upper airway obstruction and/or bilateral vocal cord paralysis in adolescents in the absence of an obstructive lesion, though the development of cranial neuropathies or radiographic evidence may be delayed. Establishing a proper diagnosis is critical as Chiari I malformation can be treated with neurosurgical decompression, with possible improvement.
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3/15. Anesthetic management in a child with arnold-chiari malformation and bilateral vocal cord paralysis.

    We report a case of a child who was scheduled for an emergency ventriculoperitoneal shunt procedure. The patient had a type II arnold-chiari malformation (ACM) and associated hydrocephalus and presented with near complete respiratory obstruction from bilateral abductor vocal cord palsy. early diagnosis and management of airway abnormalities associated with ACM may be lifesaving. Chiari malformations and anesthesia management are discussed.
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4/15. 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.
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5/15. 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.
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6/15. Decrease in size of intrathoracic meningocele following insertion of a ventriculo-venous shunt.

    A case of spontaneous disappearance of an intrathoracic meningocele following insertion of a ventriculo-venous shunt is reported. The shunt was performed for treatment of hydrocephalus secondary to arnold-chiari malformation. The intrathoracic meningocele reappeared when the shunt obstructed and decreased in size when the obstruction was relieved.
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7/15. sleep apnea and vocal cord paralysis secondary to type I Chiari malformation.

    A previously healthy 13-year-old boy without myelodysplasia who had mild scoliosis was seen with complaints of nasal congestion, noisy nighttime breathing, and difficulty sleeping. Flattening of the inspiratory loop on the flow-volume curve was found on pulmonary function testing, suggesting a variable extrathoracic obstruction due to a laryngeal lesion. Bilateral abductor vocal cord paralysis and sleep apnea developed precipitously following general anesthesia. Further workup demonstrated a type-I Chiari malformation with syringomyelia. Brainstem abnormalities such as Chiari malformation with secondary tenth cranial nerve deficits should be considered in previously healthy children and adolescents with signs and symptoms of upper airway obstruction and apnea.
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keywords = obstruction
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8/15. An unusual form of laryngeal paralysis associated with arnold-chiari malformation.

    This paper alerts the otolaryngologist to a previously unreported type of laryngeal paralysis (adductor type) in association with the arnold-chiari malformation. A survey of the literature fails to reveal any similar documented cases of absent laryngeal sensory function in association with this neurological defect. All prior reports have dealt with abductor laryngeal paralysis associated with the arnold-chiari malformation in which airway obstruction was the predominant laryngeal symptom. In the patient reported here, aspiration was the predominant symptom and was due to total loss of laryngeal sensation. This report describes this patient's clinical findings in detail, reviews the literature, and discusses management.
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keywords = obstruction
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9/15. Obstructive sleep apnoea with arnold-chiari malformation.

    A case of obstructive sleep apnoea associated with the arnold-chiari malformation is described, in which the loss of pharyngeal sensation seems to have played an important part in the aetiology of the obstruction of the upper airway.
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keywords = obstruction
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10/15. Diagnosis and treatment of obstructive sleep apnea of the larynx.

    To determine the mechanism for obstructive sleep apnea in two patients with clinical abnormalities of laryngeal function, airflow dynamics during sleep were analyzed. The site of airway obstruction was assessed by examining pressure gradients across specific airway segments. The relation between maximal inspiratory airflow and nasal pressure was analyzed to determine (1) the critical pressure, a measure of the collapsibility of the laryngeal airway, and (2) the effect of nasal continuous positive airway pressure on airflow during sleep. Large inspiratory pressure gradients developed during sleep between the supraglottic and pleural spaces, indicating that collapse had occurred in the larynx. Elevated critical pressures of -6.4 and 1.2 cm H2O, respectively, occurred in the two patients. When the nasal pressure was raised to 10 cm H2O, normal levels of tidal airflow occurred, and obstructive apneas were eliminated. These findings indicate that sleep apnea was caused by laryngeal airflow obstruction that resulted from elevations in the collapsibility of the larynx. The response to nasal continuous positive airway pressure suggested that laryngeal sleep apnea was similar to pharyngeal sleep apnea in pathophysiologic characteristic and response to treatment.
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ranking = 2
keywords = obstruction
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