Cases reported "Meningocele"

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1/25. paraparesis after excision of intrathoracic meningoceles in a patient with neurofibromatosis.

    Intrathoracic meningocele associated with neurofibromatosis is a rarity. We treated a 16-year-old boy with neurofibromatosis, marked kyphoscoliosis, and two right-sided intrathoracic meningoceles. Because his chief complaints of cough and chest pain were thought to be caused by the meningoceles, resection of these lesions was performed prior to correction of the spinal deformity. On the day after the resection, complete paraplegia developed, followed by recovery to paraparesis. Decompressive lumbar puncture was performed, but intraspinal pressure was normal. Postoperative spinal cord damage and consequent paresis may have resulted from a loss of pressure buffering by the meningocele, which rendered the cord vulnerable to injury. The possibility of a similar unusual complication should be borne in mind when treating patients with intrathoracic meningocele associated with neurofibromatosis.
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2/25. 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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3/25. Resolution of Chiari malformation after repair of a congenital thoracic meningocele: case report and literature review.

    OBJECTIVE AND IMPORTANCE: Many theories have been proposed regarding potential causative factors for Chiari malformations. An unusual case is described in which regression of a congenital Chiari malformation was observed after repair of a thoracic meningocele without direct surgical intervention to decompress the craniocervical junction. This supports the importance of an in utero craniospinal pressure gradient as a potential cause for congenital, but reversible, cerebellar herniation. CLINICAL PRESENTATION: A newborn baby was observed to have a thoracic meningocele. magnetic resonance imaging scan revealed a concomitant Chiari malformation. No neurological deficits were present at initial examination. INTERVENTION: The patient underwent surgical closure of the thoracic meningocele and untethering of the spinal cord at the site of the dural defect. A postoperative magnetic resonance imaging scan obtained 3 months after the operation revealed complete resolution of the cerebellar herniation. CONCLUSION: The resolution of the Chiari malformation in this child may have resulted from restoration of normal cerebrospinal fluid flow and elimination of the meningocele-related cerebrospinal fluid pressure gradient between the intracranial and intraspinal compartments.
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4/25. meningocele-induced positional syncope and retinal hemorrhage.

    meningocele is recognized as a rare, usually asymptomatic condition not associated with acute neurologic symptoms. We herein describe the case of a patient with a longstanding history of a lower back "mass" and recurrent syncope who became acutely unresponsive and developed bilateral retinal hemorrhages when she was placed in the supine position to undergo carotid sonography. MR imaging revealed a large, dorsal lumbar meningocele. The episode likely was caused by acutely increased intracranial pressure caused by displacement of CSF from the meningocele intracranially.
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5/25. High and low pressure states associated with posterior sacral meningocele.

    We describe the rare cases of a 44-year-old woman and a 28-year-old woman each presenting with a large posterior sacrococcygeal mass and alternating symptoms of high and low intracranial pressure. The first patient underwent excision of her large sacral meningocele and simple ligation of the neck, resulting in resolution of all her associated symptoms. The second patient suffered traumatic rupture of the meningocele; she underwent excision of the redundant sac and repair of the dural defect using a musculofascial flap, also resulting in resolution of her symptoms.
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6/25. diagnosis and management of spontaneous cerebrospinal fluid-middle ear effusion and otorrhea.

    OBJECTIVES/HYPOTHESIS: Spontaneous leak of cerebrospinal fluid (CSF) into the middle ear can occur in adults without a history of temporal bone trauma or fracture, meningitis, or any obvious cause. Therefore, clues may be lacking that would alert the otolaryngologist that fluid medial to an intact eardrum, or fluid emanating from an eardrum perforation, is likely to be CSF fluid. A review of relevant medical literature reveals that herniation of the arachnoid membrane through a tegmen defect may be congenital, or CSF leak may occur when dynamic factors (i.e., brain pulsations or increases in intracranial pressure) produce a rent in the arachnoid membrane. Because tegmen defects may be multiple rather than single, identifying only one defect may not be sufficient for achieving definitive repair. Data on nine cases of spontaneous CSF leak to the ear in adult patients from four medical centers are presented and analyzed to provide collective information about a disorder that can be difficult to diagnose and manage. STUDY DESIGN: Retrospective review of nine cases of spontaneous CSF middle ear effusion/otorrhea. RESULTS: The majority of patients presented with symptoms of aural fullness and middle ear effusion. Many developed suspicious clear otorrhea only after insertion of a tympanostomy tube. Two patients had multiple defects in the tegmen and dura, and five patients had meningoencephaloceles confirmed intraoperatively. Five patients underwent combined middle cranial fossa/transmastoid repair. Materials used in repair included temporalis fascia, free muscle graft, Oxycel cotton, calvarial bone, pericranium, bone wax, and fibrin glue. CONCLUSIONS: CSF middle ear effusion/otorrhea can develop in adults without a prior history of meningitis or head trauma or any apparent proximate cause. Although presenting symptoms can be subtle, early suspicion and confirmatory imaging aid in establishing the diagnosis. Because surgical repair by way of a mastoid approach alone can be inadequate if there are multiple tegmen defects, a middle fossa approach alone, or in combination with a transmastoid approach, should be considered in most cases.
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7/25. Orbital varices, cranial defects, and encephaloceles: an unrecognized association.

    PURPOSE: To highlight an apparently unreported association between orbital varices and malformations of the cranial skeleton with or without abnormalities of the underlying cerebral parenchyma. DESIGN: Retrospective, noncomparative review of case notes and radiologic imaging. PARTICIPANTS: Three hundred ten patients attending the Orbital Clinic at Moorfields eye Hospital, london, with a diagnosis of low-pressure, low-flow orbital venous anomalies. methods: All available orbital imaging for patients with orbital venous anomalies was examined. For those with defects of the cranial base, the radiologic characteristics were noted and the clinical details were reviewed. MAIN OUTCOME MEASURES: The presence and type of orbital roof or medial wall defects and associated nasal or other cranial anomalies. RESULTS: Imaging was adequate for review in 222 of 310 patients (72%), and anomalies of the neighboring cranium or cerebral structure were found in 10 of 222 patients (4.5%). In the group with cranial anomalies, the proportion of men (7/10; 70%) did not significantly differ from that in the group with varices but without cranial anomalies (93/212; 44%; P = 0.19, Fisher exact test). Orbital varices were associated with 3 types of cranial anomaly: major midline encephaloceles (type I anomaly; 4 cases), large superomedial defects of the orbital wall (type II; 3 cases), or defects of the greater wing of the sphenoid (type III; 3 cases). CONCLUSIONS: Clinicians should be aware of the possibility of significant cranio-orbital or cranionasal anomalies in patients with orbital venous anomalies; these anomalies can vary from minor defects in the cranial base to large encephalomeningoceles. This rare association should not be overlooked when orbital varices become markedly inflamed, because intranasal encephaloceles are a known predisposition to recurrent meningitis.
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8/25. Intradural spinal vein enlargement in craniospinal hypotension.

    We present a case of craniospinal hypotension in a 45-year-old woman with an associated epidural pseudomeningocele extending the entire length of the spine. The epidural pseudomeningocele was caused by a CSF leak at the T8 level. In addition to typical low-pressure symptoms, the epidural pseudomeningocele caused atypical symptoms characterized by positional thoracic radiculopathy. Craniospinal hypotension was associated with massive cervical epidural venous engorgement, as well as enlargement of the posterior spinal cord vein, which was reminiscent of a dural arteriovenous fistula at CT myelography. Enlargement of the posterior spinal vein is explained by the Monro-Kellie hypothesis, and the spinal analog to enlarged cerebral veins known to be associated with intracranial hypotension.
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9/25. Lumbo-peritoneal shunting improved spinal cord compression due to a large anterior sacral meningocele.

    OBJECTIVE: Lumbo-peritoneal shunt was used as an alternative treatment in a large anterior sacral meningocele, a rare congenital malformation, is illustrated. CLINICAL PRESENTATION: A 48-year-old female patient presented with a two-month history of progressive paraparesis, leg pain and numbness of both legs. The investigation demonstrated spina bifida with a huge sac of meningocele, protruding anteriorly into the pelvic cavity. On two previous surgical operations for closure, the fistula through the posterior transsacral approach failed 20 years ago at another hospital, but her symptoms had subsided spontaneously without explanation. She came to Thammmasat Hospital with an episode of spinal cord compression for 2 months. MANAGEMENT: Because of the old surgical scar and high pressure of the meningocele, a lumbo-peritoneal shunt was selected to drain the cerebrospinal fluid from the meningocele to the peritoneal cavity. CONCLUSION: Lumbo-peritoneal shunting is an optional treatment for a large sacral meningocele, especially in a large fistula with unsuccessful surgical closure.
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10/25. optic nerve sheath meningoceles. Clinical and radiographic features in 13 cases with a review of the literature.

    Thirteen patients with dilated intraorbital optic nerve sheaths with an expanded, patulous cerebrospinal fluid (CSF) space were studied with high-resolution computed tomography (CT) or magnetic resonance imaging (MRI). Eleven patients had bilateral findings. headache or visual complaints, or both, were present in all patients. Signs of optic nerve dysfunction were present in eight patients. Three patients had visual acuity worse than 20/200. cerebrospinal fluid pressure was mildly elevated in two patients. Three patients underwent a surgical procedure; visual acuity improved in one. The authors propose the term meningocele for this condition and suggest MRI with fat-suppression techniques and off-axis sagittal views as the radiographic procedure of choice.
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