Cases reported "Encephalocele"

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1/9. Intrasphenoidal encephalocele and spontaneous CSF rhinorrhoea.

    Intrasphenoidal encephalocele is a rare clinical entity. In the international literature only 16 cases have been reported up today, with female predominance. Clinically they manifest at middle and advanced ages (40-67 years), when spontaneous CSF rhinorrhoea or recurrent meningitis occurs. We present our case, a 46 years old female, who had CSF rhinorrhoea from the right vestibule for 10 months. The diagnosis was based on the history and the high-resolution brain and skull base CT-scanning in conjunction with opaque fluid injection in the subarachnoidal space through a lumbar puncture. She was successfully treated with an operation, through an endonasal trans-ethmoid microendoscopic approach, using the Draf and Stammberger technique. We discuss the pathogenesis of the intrasphenoidal encephalocele, the existence of small occult defects in the skull base, which cause, at the middle and advanced ages, CSF fistula with spontaneous CSF rhinorrhoea and/or recurrent meningitis. Finally we emphasize the advantages of the endonasal surgical approach for the treatment of this condition.
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2/9. 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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3/9. 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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4/9. life-threatening intracranial hypotension after diagnostic lumbar puncture.

    intracranial hypotension syndrome as a complication of diagnostic lumbar puncture is a rarely observed entity. intracranial hypotension syndrome is characterized by postural headache, neck pain/stiffness, blurred vision, nausea, vomiting, clouding of consciousness, dizziness and vertigo. The majority of cases resolve spontaneously with conservative treatment. Rarely, epidural blood patch is required. We report a 41-year-old man with multiple sclerosis, who developed intracranial hypotension syndrome after diagnostic lumbar puncture and who did not respond to conservative treatment. A subdural hematoma was subsequently found, when the patient showed considerable worsening of clinical conditions with life-threatening symptoms. Surgical evacuation of the subdural hematoma was not sufficient to improve significantly the patient's conditions, while complete symptoms remission was achieved 12 hours after epidural blood patch. We stress the need for epidural blood patch in any case of post-diagnostic lumbar puncture postural headache which does not resolve with conservative therapy.
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ranking = 7
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5/9. Management of multiple spontaneous nasal meningoencephaloceles.

    OBJECTIVES/HYPOTHESIS: Multiple spontaneous nasal meningoencephaloceles in the same patient are rare lesions. Although many skull base defects occur after prior trauma or surgery, otolaryngologists must be aware of the potential for spontaneous encephaloceles. We present our experience with this unusual condition and discuss its pathophysiology and unique management issues. STUDY DESIGN: Retrospective. methods: review of medical records, radiographic images, and cerebrospinal fluid pressures. RESULTS: We identified 5 patients with multiple, simultaneous, spontaneous encephaloceles: 4 patients with 2 encephaloceles and 1 patient with 3 encephaloceles (11 in all). Locations of the 11 encephaloceles were sphenoid lateral recess (6), frontal sinus with supraorbital ethmoid extension (2), ethmoid roof (1), frontal sinus (1), and central sphenoid (1). Three patients had bilateral sphenoid lateral recess encephaloceles, accounting for all six in that location. All four patients with available radiographic studies demonstrated empty sella turcica. Surgical approaches included endoscopic transpterygoid approach to the lateral sphenoid recess (3), endoscopic approach to ethmoid and central sphenoid (3), and osteoplastic flap with frontal sinus obliteration (2). We had 100% success at latest endoscopic follow-up (mean period, 17 mo). Three patients had postoperative lumbar punctures with mean cerebrospinal fluid pressure of 28.3 cm water (range, 19-34 cm; normal range, 0-15 cm). Conclusions: Multiple spontaneous encephaloceles can be managed safely and successfully using endoscopic and extracranial approaches. A high index of suspicion for this diagnosis must be maintained, especially in patients with radiographic evidence of laterally pneumatized sphenoid sinuses or empty sella. Spontaneous encephaloceles and cerebrospinal fluid leaks represent a form of intracranial hypertension.
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6/9. empty sella syndrome, diagnosed as allergic rhinitis.

    Unilateral cerebrospinal fluid (CSF) rhinorrhea as the only manifestation of the primary empty sella syndrome is a rare event. A case of a middle-aged male patient complaining for intermittent unilateral rhinorrhea, which started 5 months earlier, is reported. The persistence of this state was attributed to an allergic rhinitis. The initial work-up excluded the above diagnosis and an erroneous radiological diagnosis led to a puncture of the left maxillary sinus. A lateral X-ray of the skull and CT scan led to the diagnosis of empty sella syndrome, possibly due to an adenoma or a meningocele.
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7/9. Contraindications to lumbar puncture as defined by computed cranial tomography.

    Papilloedema is not always an adequate predictor of potential complications from lumbar puncture, and many clinicians are using computed tomography (CT) before lumbar puncture in an effort to identify more accurately the "at risk" patient. This paper identifies the following anatomical criteria defined by CT scanning that correlate with unequal pressures between intracranial compartments and predispose a patient to herniation following decompression of the spinal compartment: lateral shift of midline structures, loss of the suprachiasmatic and basilar cisterns, obliteration of the fourth ventricle, or obliteration of the superior cerebellar and quadrigeminal plate cisterns with sparing of the ambient cisterns. These criteria should be considered to be contraindications to lumbar puncture.
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8/9. Respiratory arrest and cervical spinal cord infarction following lumbar puncture in meningitis.

    A 6-year-old child with meningitis had a respiratory arrest 20 minutes after a lumbar puncture. Thereafter she required maintenance on a ventilator, had a flaccid quadriplegia, and died 12 days later. Necropsy showed infarction of the central portion of the cord at the level of the decussation of the pyramids. The suggested mechanism of damage is compression of the arterial supply to the cord at the level of the foramen magnum by herniated cerebellar tonsils; concomitant hypotension may have contributed to production of the damage. Four similar cases, who survived with residual deficit, have also been reported. Other separate mechanisms by which the cord can be damaged in meningitis are vasculitis, thrombosis and arachnoiditis.
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9/9. Fatal brain lesion following spinal anaesthesia. Report of a case.

    Leakage of cerebrospinal fluid following dural puncture and subsequent downward sagging of the brain have been suggested as a cause of the rare subdural haematomas occurring after spinal or attempted epidural anaesthesia which have been reported in the literature. We report a case where this mechanism probably produced a fatal brain lesion, due to the herniation of the uncus against the tentorium cerebelli 15 days after spinal anaesthesia. The patients complained of severe headache and nausea after the procedure. The reported case shows that postdural-puncture headache may be a sign of more serious sequelae and should be treated by the means available.
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