Spontaneous intracranial hypotension: a cause of severe acute headache.
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Severe acute headache is a common presenting symptom to an accident and emergency department. Spontaneous intracranial hypotension (SIH) is an increasingly recognised cause of these symptoms and has characteristic clinical and imaging findings. SIH is characterised by headache worse on standing, low opening cerebrospinal fluid pressures at lumbar puncture and uniform pachymeningeal enhancement with gadolinium enhanced magnetic resonance imaging of the brain, all in the absence of dural trauma. Atypical presentations occur and severe neurological decline can rarely be associated with this condition. A review of five patients presenting recently to our institution with classical imaging findings together with a review of the literature is presented. (+info)
Transsphenoidal encephaloceles are rare and the transsellar variety is the least common. We present a 1-year-old male patient with transsellar transsphenoidal encephalocele which herniated into the oral cavity through the congenital split palate. The patient was operated on using a combined transcranial and transpalatal approach without mortality or permanent morbidity. Clinical findings, imaging reviews, surgical repair techniques and postoperative morbidity are discussed with the relevant literature. We conclude that repair of a transsphenoidal encephalocele should be coordinated between a team of neurosurgeons and otorhinolaryngologists. Our surgical outcome supports a transcranial approach for the treatment of these difficult lesions, with transpalatal dissection and exposure. (+info)
Nontraumatic skull base defects with spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients.
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BACKGROUND AND PURPOSE: Defects at the skull base leading to spontaneous CSF rhinorrhea are rare lesions. The purpose of our study was to correlate CT and MR findings regarding the location and content of CSF leaks in 27 patients with endoscopic sinus surgery observations. MATERIALS AND METHODS: Imaging studies in 27 patients with intermittent CSF rhinorrhea (CT in every patient including 10 examinations with intrathecal contrast, plain CT in 2 patients, and MR in 15 patients) were analyzed and were retrospectively blinded to intraoperative findings. RESULTS: CT depicted a small endoscopy-confirmed osseous defect in 3 different locations: 1) within the ethmoid in 15 instances (53.6% of defects) most commonly at the level of the anterior ethmoid artery (8/15); 2) adjacent to the inferolateral recess of the sphenoid sinus in 7 patients including one patient with bilateral lesions (8/28 defects, 28.6%); 3) within the midline sphenoid sinus in 5 of 28 instances (17.9%). Lateral sphenoid defects (3.5 +/- 0.80 mm) were larger than those in ethmoid (2.7 +/- 0.77 mm, P < or = 0.029) or midsphenoid location (2.4 +/- 0.65 mm, P < or = 0.026). With endoscopy proven arachnoid herniation in 24 instances as reference, MR was correct in 14 of 15 instances (93.3%), CT cisternography in 5 of 8 instances (62.5%). Plain CT in 1 patient was negative. CONCLUSION: In patients with a history of spontaneous CSF rhinorrhea, CT was required to detect osseous defects at specific sites of predilection. MR enabled differentiating the contents of herniated tissue and allowed identification of arachnoid tissue as a previously hardly recognized imaging finding. (+info)
Basal encephalocele associated with morning glory syndrome: case report.
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The basal encephaloceles refer to rare entities and they correspond to herniation of brain tissue through defects of skull along the cribiform plate or the sphenoid bone. A rare morning glory syndrome, with characteristic retinal defect has been reported in association with basal encephaloceles. Hypophysis hormonal deficiencies may occur. We accounted for a pituitary dwarfism with delayed diagnosed transsphenoidal encephalocele associated with morning glory syndrome, showing the alterations found in retinography, computed tomography and magnetic resonance imaging. (+info)
Basal encephalocele associated with hypoplasia of the internal carotid artery.
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A 22-year-old woman presented with a basal encephalocele associated with hypoplasia of the internal carotid artery (ICA) manifesting as a 6-year history of decreased vision in the right eye. She underwent encephalo-arterio-synangiosis under a diagnosis of hypoplasia of the ICA at age 6 years. Magnetic resonance imaging showed the encephalocele medial to the right temporal lobe. Frontotemporal craniotomy was performed for resection of the encephalocele and repair of the defect of the skull base. The pathogeneses of these developmental anomalies were probably related to developmental failure of the embryonic primordium during the 4th and 10th weeks. (+info)
Linkage to chromosome 2q36.1 in autosomal dominant Dandy-Walker malformation with occipital cephalocele and evidence for genetic heterogeneity.
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A median defect in the region of the root of the nose, in between the two orbits, was discovered in the dried skull of a 44-year-old female cadaver, during routine undergraduate teaching. The two small nasal bones articulated with each other and the cribriform plate of the ethmoid. The lacrimal bones and frontal processes of the maxillary bones were also deformed. We propose that the median defect was due to abnormality at the fonticulus frontalis, the prenasal space and the interorbitonasal part of the nasal capsule, as well as defects in the ossification of the maxilla, lacrimal and frontal bones. (+info)