Cases reported "Encephalocele"

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11/44. 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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12/44. Bacterial meningitis secondary to a transethmoidal encephalocele presenting to the emergency department.

    We present the case of a patient seen in the Emergency Department (ED) at the height of enteroviral meningitis season with the chief complaint of the worst headache of his life. He was subsequently found to have pneumococcal meningitis as the result of an encephalocele located within the left ethmoid sinus. The key features of the patient's past medical history, the steps to diagnosis, and a discussion of this exceedingly rare entity are detailed.
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13/44. Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases.

    OBJECTIVE AND IMPORTANCE: Gliomatosis cerebri (GC) is a rare type of primary brain tumor that diffusely infiltrates more than two lobes of the brain while the normal cerebral architecture is maintained. To the best of our knowledge, the association between an acquired tonsillar herniation and GC has never been reported. In this article, we describe three patients with progressive gliomatosis of the cerebellar hemispheres who subsequently showed signs and symptoms secondary to tonsillar herniation. Early recognition of this potentially life-threatening complication allowed us to recommend prompt surgical intervention. CLINICAL PRESENTATION: One patient with primary, or Type I, GC presented with suboccipital headaches, and two patients with secondary, or Type II, GC presented with the signs and symptoms of progressive myelopathy. Serial imaging studies demonstrated progressive involvement of the cerebellum, descent of the cerebellar tonsils through the foramen magnum, and cervicomedullary spinal cord compression. INTERVENTION: Once the tonsillar herniation was recognized, all three patients underwent posterior fossa decompression, a cervical laminectomy to the lowest level of the tonsillar herniation, and duraplasty. All three patients experienced immediate improvement in their conditions. CONCLUSION: Early recognition of tonsillar herniation, a possibly overlooked cause of death in patients with GC, allows for early surgical intervention as a potentially lifesaving procedure and significant improvement in the patient's condition.
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14/44. Apparent encephalocele in twin fetus papyraceus with twin-reversal arterial perfusion.

    An apparently iniencephalic or exencephalic monoamniotic monochorionic female twin fetus, delivered as a fetus papyraceus at 28 weeks of gestation, had severe anomalies of the central nervous system and spine, including occipital encephalocele with a defect of the occipital bone. The encephalocele contained a spherical mass of autolyzed brain tissue without identifiable cerebellum or vermis. The cervical canal was widely patent dorsally, there were severe bony anomalies, including agenesis and fusion of vertebrae in the cervicothoracic spine. The upper limbs were absent. All organs were hypoplastic and autolyzed. The hypoplastic heart had three chambers. The placenta was monochorionic and monoamniotic; barium injection showed a twin-reversal arterial perfusion and entanglement of the umbilical cords.
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15/44. Intrasphenoidal encephalocele associated with cerebrospinal fluid fistula and subdural hematomas: technical case report.

    OBJECTIVE AND IMPORTANCE: Intrasphenoidal encephalocele is a rare clinical entity that is often complicated by rhinorrhea, recurrent meningitis, and headache, but in no case has the association of rhinorrhea with subdural hematomas been described. A surgical procedure to stop persistent cerebrospinal fluid leakage is reported. CLINICAL PRESENTATION: A 59-year-old man sought care for intractable rhinoliquorrhea of 6 months' duration. Cranial computed tomographic and magnetic resonance imaging scans revealed a basal posterior frontal bony defect and an evocative image suggesting intrasphenoidal encephalocele. INTERVENTION: A transnasal transsphenoidal surgical procedure was performed; the encephalocele was removed, and the sphenoid sinus was filled with an inflatable pouch made of synthetic dura mater containing abdominal fat. Postoperative reduction of the rhinoliquorrhea, but not its total disappearance, was observed. Total disappearance was achieved only after endonasal, transmucosal inflation of the pouch with human fibrin glue. One of the subdural hematomas disappeared spontaneously, and the other was treated by a surgical procedure. CONCLUSION: The possible role of the presented technique in the treatment of cerebrospinal fluid leakage is discussed.
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16/44. Orbital trauma caused by bicycle hand brakes.

    PURPOSE: This report aims to increase awareness of an unusual mechanism of orbital injury sustained by bicycle riders. methods: In this retrospective small case series, we describe two cases of orbital injury caused by upper eyelid penetration. A 5-year-old boy (patient 1) and a 6-year-old boy (patient 2) presented to our service within a 2-week period. Both had been injured by similarly styled, handlebar-mounted bicycle hand brake levers. Patient 1 had an orbital roof fracture and penetrating brain injury and underwent repair of a left upper eyelid laceration, craniotomy for pseudoencephalocele, and ptosis repair. Patient 2 had orbital hemorrhage and underwent repair of left upper eyelid laceration. RESULTS: In both cases, a handlebar-mounted bicycle hand brake lever perforated the left eyelid when the rider fell onto it. Neither patient was wearing protective headwear or eyewear. Two months after surgery, patient 1 had 20/25 visual acuity OU and excellent cosmetic appearance. Patient 2 had baseline amblyopic vision 2 days after surgery but moved from town and was lost to follow-up. CONCLUSIONS: orbit injuries from bicycle brake levers are rare, and helmets or protective eyewear probably would not have prevented these injuries. However, a change in the design and/or mounting location of handlebar-mounted brake levers might help prevent further injuries of this type.
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17/44. naegleria fowleri hemorrhagic meningoencephalitis: report of two fatalities in children.

    Two cases of hemorrhagic meningoencephalitis secondary to naegleria fowleri infection confirmed by postmortem analysis are described. The first patient is a 5-year-old boy who presented with a severe headache, neck stiffness, and lethargy. His neurologic examination was significant for somnolence and nuchal rigidity. cerebrospinal fluid studies and structural neuroimaging were consistent with hemorrhagic meningoencephalitis. Another 5-year-old boy presented to a different institution 2 miles away in the same week with similar complaints. Both patients declined rapidly and expired within 48 hours of admission secondary to transtentorial herniation caused by the mass effect of inflammation, edema, and hemorrhage with displacement of the brain stem. Histopathologic and immunochemistry analysis of brain tissue revealed the presence of Naegleria trophozoites in both cases.
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18/44. Is there an upper limit of intracranial pressure in patients with severe head injury if cerebral perfusion pressure is maintained?

    Authors of recent studies have championed the importance of maintaining cerebral perfusion pressure (CPP) to prevent secondary brain injury following traumatic head injury. Data from these studies have provided little information regarding outcome following severe head injury in patients with an intracranial pressure (ICP) greater than 40 mm Hg, however, in July 1997 the authors instituted a protocol for the management of severe head injury in patients with a glasgow coma scale score lower than 9. The protocol was focused on resuscitation from acidosis, maintenance of a CPP greater than 60 mm Hg through whatever means necessary as well as elevation of the head of the bed, mannitol infusion, and ventriculostomy with cerebrospinal fluid drainage for control of ICP. Since the institution of this protocol, nine patients had a sustained ICP greater than 40 mm Hg for 2 or more hours, and five of these had an ICP greater than 75 mm Hg on insertion of the ICP monitor and later experienced herniation and expired within 24 hours. Because of the severe nature of the injuries demonstrated on computerized tomography scans and their physical examinations, these patients were not aggressively treated under this protocol. The authors vigorously attempted to maintain a CPP greater than 60 mm Hg with intensive fluid resuscitation and the administration of pressor agents in the four remaining patients who had developed an ICP higher than 40 mm Hg after placement of the ICP monitor. Two patients had an episodic ICP greater than 40 mm Hg for more than 36 hours, the third patient had an episodic ICP greater than of 50 mm Hg for more than 36 hours, and the fourth patient had an episodic ICP greater than 50 mm Hg for more than 48 hours. On discharge, all four patients were able to perform normal activities of daily living with minimal assistance and experience ongoing improvement. Data from this preliminary study indicate that intense, aggressive management of CPP can lead to good neurological outcomes despite extremely high ICP. Aggressive CPP therapy should be performed and maintained even though apparently lethal ICP levels may be present. Further study is needed to support these encouraging results.
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19/44. Heminasal proboscis with associated microphthalmos and encephalocele.

    Heminasal proboscis is a rare congenital malformation that presents complex management issues when associated with other craniofacial abnormalities. A newborn male, known to have a facial mass on prenatal ultrasonography, was delivered by planned induction at 37 weeks' gestation. He was intubated immediately because of anticipated respiratory difficulty. He had a right-sided proboscis, pedicled on the right medial canthal region. Additional airway evaluation identified a piriform aperture and choanal stenosis. magnetic resonance imaging confirmed a hypoplastic orbit with aplastic optic nerve and a naso-orbito-ethmoidal encephalocele. Right upper and lower eyelid colobomas were present. The airway was initially stabilized with a tracheostomy. Beginning at the age of 10 weeks, he underwent several staged procedures to excise the encephalocele and to reconstruct the nose, orbit, eyelid, and lacrimal drainage system. The encephalocele was exposed via a bicoronal approach and frontal craniotomy. The dural defect was repaired with a pericranial flap. Nasal reconstruction provided an adequate nasal passage and tip after three staged procedures.Creation of a conjunctival flap prepared the right eye for later insertion of a prosthetic shell. The patient remained successfully decannulated 3 years after his last reconstructive procedure. Identification of all associated anomalies is important before initiating surgical repair. The presence of coexisting craniofacial abnormalities requires a multispecialty approach and coordination of surgical procedures.
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20/44. Spontaneous motor cortex encephalocele presenting with simple partial seizures and progressive hemiparesis. Case report and review of the literature.

    Several cases of congenital or acquired temporal encephaloceles have been reported in the literature as the causative mechanism of simple and/or complex partial seizures. In this report the authors describe a rare case of spontaneous parietal encephalocele presenting with simple partial seizures and progressively increasing contralateral upper-extremity motor deficit. The unusual anatomical location of an encephalocele associated with seizures and the delayed seizure onset represent distinctive characteristics in this case. Preoperative imaging included surface electroencephalography, computerized tomography, and brain magnetic resonance imaging. Frameless neuronavigation and intraoperative cortical mapping were used to aid resection of the encephalocele, and the dural and bone defects were reconstructed. The surgical outcome in this case was excellent, and the patient has remained seizure free. The pertinent literature is reviewed in this report.
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