Cases reported "Encephalocele"

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1/17. 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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2/17. Cerebral herniation after lumbar puncture in sarcoid meningitis.

    A patient with chronic meningitis due to neurosarcoidosis became comatose within minutes of a lumbar puncture and died 24 h later. The diagnosis of neurosarcoidosis was made post mortem. Development of cerebral herniation may have been exacerbated by lumbar puncture. It was proposed that arachnoid villi dysfunction may have contributed to very high intracranial pressures in this patient, since post mortem examination revealed communication between the ventricles and outlet foramina of the fourth ventricle, and that herniation was in part due to an acute pressure differential caused by lumbar puncture.
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3/17. Neuroradiology of basal anterior fossa (transethmoidal) encephaloceles.

    Encephaloceles of the basal anterior fossa, though primarily congenital in origin, may be insidious in presentation and remain undetected until adulthood. Conventional plain films and hypocycloidal tomograms show a basal osseous defect with its margins bevelled toward the nasopharynx. Magnification angiography is the diagnostic study of choice and reveals herniation of the anterior inferior frontal artery through the osseous defect. pneumoencephalography, the classical technique of investigation, was found to be unrewarding in demonstrating the herniated sac or in determining the presence or absence of brain tissue within the sac; however, it is of value in demonstrating a ventricular communication with the encephalocele and evaluating ventricular dilatation.
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4/17. Aetiology of nasopharyngeal glioma.

    A case of nasopharyngeal glioma is presented in which the postnatal scans clearly show intracranial communication, whilst subsequent scans and surgical exploration could not demonstrate this finding. This case seems to confirm that the aetiology of these lesions is due to an encephalocele that subsequently loses its connection with the brain.
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5/17. Nasal cerebral heterotopia: nasal atretic cephalocele.

    We report the case of a 4-year-old boy who presented with a congenital mass on the bridge of his nose. A magnetic resonance study failed to rule out a communication of the tumor with the cranial cavity. The lesion was totally removed. Histopathological study of the excised mass showed a peripheral zone of fibrous-connective tissue with a core of glial and neuronal elements. We discuss the origin of these masses and their relation to nasal cephaloceles. We suggest that this type of lesions should be included within the broader spectrum of atretic cephaloceles.
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6/17. The utility of magnetic resonance imaging in the diagnosis of intranasal meningoencephaloceles.

    We present three patients in whom the diagnosis of intranasal meningoencephalocele was made by magnetic resonance imaging. The initial clinical evaluation and computed tomographic examinations of these patients failed to distinguish between chronic inflammation and intranasal meningoencephalocele. Although both computed tomography and magnetic resonance imaging are used to distinguish between normal, inflammatory, and neoplastic tissue in the nasal cavity and paranasal sinuses, limitations do exist and these are the focus of our communication. A clear understanding of the efficacy of these radiographic modalities will enhance surgical planning and can preclude severe complications.
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7/17. Intraoperative management of an infant with a nasofrontal encephalocele.

    1. An encephalocele is a congenital malformation in which the structures of the central nervous system, in communication with cerebrospinal fluid pathways, herniate through a cranial defect. 2. classification of encephaloceles depends on their anatomical location within the cranium, and the prognosis and treatment largely depend on the site of such defects. 3. Although the operation is delayed until the infant gains strength, early correction (within the first month) minimizes facial deformity, prevents further damage to brain tissue herniating through the defect, and increases the chance of normal binocular vision.
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8/17. A case of rudimentary lateral parietal cephalocele: extracranial meningothelial and glial tissue without intracranial communication or bony defect.

    The authors report a case of a 7-month-old infant with a right lateral parietal scalp lesion intermittently leaking fluid similar to cerebrospinal fluid that histologically demonstrated meningothelial and glial cells. At surgical removal, however, no fibrous stalk or bony defect could be identified connecting the lesion with the intracranial compartment. While the embryologic mechanism of this lesion is unclear, the prognosis for normal neurological development appears excellent.
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9/17. Lateral frontal encephalocele associated with dysplasia of orbit, eyeball, and eyelid.

    A case is reported of a right lateral frontal encephalocele without communication with the midline structures and in concurrence with dysplasia of the orbit, eyeball, and eyelid.
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10/17. Tecto-cerebellar dysraphia with occipital encephalocele.

    We report four cases with the rare syndrome of tecto-cerebellar dysraphia with occipital encephalocele. The clinical features seen in these patients included episodic tachypnea and irregular breathing, opsoclonus, ataxia, marked hypotonia of the limbs, coloboma, and polydactyly. All four patients had midline occipital encephalocele. The cranial computed tomography scan showed partial to total agenesis of the vermis with a large communication between cisterna magna and the fourth ventricle. The computed tomography scan also showed partial deficiency of the midbrain tectum. We discuss the clinical and radiological findings and review the literature.
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