Cases reported "Encephalocele"

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1/44. An unusual presentation of an encephalocele to the otolaryngologist.

    An unusual case of encephalocele causing obstruction of the upper airway in a neonate is described. The patient presented with a large mass in the neck, which extended from the base of the skull to the level of the larynx. Although there was no evidence of a defect of the skull base, subsequent excision and histological examination confirmed an encephalocele.
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2/44. A rare case of upper airway obstruction in an infant caused by basal encephalocele complicating facial midline deformity.

    A four-month-old male infant with basal encephalocele of the transsphenoidal type presented with upper airway obstruction and facial midline deformity, including cleft lip, cleft palate, hypertelorism and exophthalmos. Basal encephalocele is a rare disease, and usually not detectable from the outside. In this case, initially the cause of an upper airway obstruction was considered to be posterior rhinostenosis, and posterior rhinoplasty with inferior nasal conchectomy was scheduled. However, in preoperative examination, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a bony defect in the sphenoidal bone and a cystic mass in communication with cerebrospinal fluid, herniating into the nasal cavity through the bony defect. The mass was diagnosed as a transsphenoidal encephalocele, the scheduled operation cancelled, and tracheostomy performed for airway management. The possibility of basal encephalocele should be considered in the case of upper airway obstruction with facial midline deformity.
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3/44. Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients.

    Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks are usually accompanied by episodes of recurrent meningitis, hearing loss, or chronic headache. In this article, we report seven new cases of spontaneous CSF leakage. Six of these patients had conductive hearing loss and serous otitis media, and three had recurrent meningitis. Prior to a definitive diagnosis, six patients had received myringotomy tubes, which produced profuse clear otorrhea. Three patients had positive beta-2 transferrin assays. Computed tomography and magnetic resonance imaging confirmed a defect in the temporal bone tegmen. A combined transmastoid and middle fossa surgical approach with a three-layer closure was used to repair the tegmen defect. All patients had a lumbar drain placed prior to surgery. In addition to describing the seven new cases, we review the history of CSF leakage and discuss diagnostic methods, surgical findings, and our recommendations for management.
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ranking = 38.121766587877
keywords = headache
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4/44. Therapeutic effectiveness of acetazolamide in hindbrain hernia headache.

    A 43-year-old man had been suffering from exertional headache for 10 years. Sagittal sections on magnetic resonance imaging showed a Chiari type 1 malformation and a cerebellar arachnoid cyst. This syndrome, named hindbrain hernia headache, disappeared with oral acetazolamide. This treatment should be tried in patients with hindbrain hernia headache prior to considering surgical decompression of the foramen magnum.
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ranking = 266.85236611514
keywords = headache
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5/44. Flaccid quadriplegia from tonsillar herniation in pneumococcal meningitis.

    A young woman with fulminant pyogenic meningitis became quadriplegic, areflexic and flaccid due to herniation of the cerebellar tonsils and compression of the upper cervical cord. This state of spinal shock was associated with absent F-waves. intracranial pressure was greatly elevated and there was an uncertain relationship of tonsillar descent to a preceding lumbar puncture. Partial recovery occurred over 2 years. Tonsillar herniation can cause flaccid quadriplegia that may be mistaken for critical illness polyneuropathy. This case demonstrates cervicomedullary infarction from compression, a mechanism that is more likely than the sometimes proposed infectious vasculitis of the upper cord.
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6/44. Chiari malformation and sleep-disordered breathing: a review of diagnostic and management issues.

    Chiari Malformation (CM) encompasses several patterns of congenital or acquired cerebellar herniation through the foramen magnum. This may result in brain-stem compression that impacts control of breathing and is associated with obstructive and central apneas. A high clinical suspicion for sleep-disordered breathing is needed in the care of such patients after as well as before corrective surgery. To introduce a review of CM with a focus on the relevance to sleep medicine, we present a case of a 13-year-old female who was diagnosed with CM Type 1 in the course of an evaluation of symptomatic central sleep apnea. After initial improvement following surgery there was recurrence of brain-stem compression. The only clinical expression of which was polysomnographically evident recurrence of sleep apnea.
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ranking = 1130.9145718542
keywords = breathing
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7/44. Bilateral defects of the tegmen tympani associated with brain and dural prolapse in a patient with pulsatile tinnitus.

    A pulsating ear drum is a rare otological finding and usually indicates a vascular pathology. We report the case of a woman who presented with pulsatile tinnitus following an upper respiratory tract infection. She was found to have bilateral pulsating tympanic membranes on otological examination, in association with bilateral dehiscence of the tegmen tympani and a spontaneous encephalocele on the right side and a meningocele on the left side, demonstrated on computerized tomography (CT) scan of her temporal bones.
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8/44. 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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9/44. Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: a case report and review of the literature on de novo growth of cavernomas.

    BACKGROUND: Cerebral cavernomas are rare vascular anomalies and their expansile growth has been considered to be mainly due to recurrent hemorrhages. They are not generally reported to show aggressive behavior.CASE DESCRIPTION: A 27-year-old male presented with headache, visual disturbances, and a 17-year history of seizures. He was known to have a temporal lobe lesion on CT scan, consistent with a diagnosis of cavernous malformation but with no relevant family history. Serial clinical and radiological follow-up revealed a progressive increase in the size of the lesion with formation of a growing cyst of 7 cm in diameter, which produced mass effect, resulting in the clinical picture of cerebral herniation. The patient underwent emergency surgical resection with symptomatic relief. There was no evidence of significant hemorrhage at surgery.CONCLUSION: Cavernous malformations can show expansile growth without any evidence of a presaging hemorrhagic event and, in the manner of a neoplastic lesion, present with raised intracranial pressure and cerebral herniation.
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ranking = 38.121766587877
keywords = headache
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10/44. Cerebral heterotopia appearing as an extranasal polyp.

    Cerebral heterotopia represents a rare tumorlike lesion related in its development to the encephaloceles but differing from these by non-connection to the cranial cavity. We describe an unusual case of cerebral heterotopia appearing as a pedunculated polyp on the upper lip of a newborn. As the distinction between encephaloceles and cerebral heterotopias is based on the correlation of radiologic and clinical findings, the surgical treatment of tumorlike lesions of the nasal and pharyngeal region in children requires a careful preoperative evaluation of their possible connection to the cranial cavity.
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