1/223. Anatomical and embryological considerations in the repair of a large vertex cephalocele. Case report.The case of a neonate with a large vertex cephalocele is presented. The anatomical features of this anomaly were evaluated by means of magnetic resonance imaging and magnetic resonance angiography. Fusion of the thalami, dysgenesis of the corpus callosum, and failure of adequate formation of the interhemispheric fissure were characteristics of the major cerebral anomalies associated with the cephalocele. The absence of a falx in the midline, a split configuration of the superior sagittal sinus, and a dysgenetic tentorium with a concomitant abnormal venous drainage pattern were found in association with a large dorsal cyst. Repair of the anomaly was undertaken on the 3rd postnatal day. A cerebrospinal fluid shunt was required to treat hydrocephalus on Day 30. The child is well at age 3 years, but with significant developmental delay. The pathogenesis of this vertex cephalocele relates to semilobar holoprosencephaly and dorsal cyst formation. In addition, a disturbance in the separation of the diencephalic portion of the neural tube from the surface ectoderm or skin during the final phases of neurulation had occurred to help create the large cephalocele. Detailed preoperative imaging studies and awareness of the embryology and anatomy of this lesion facilitated the repair of the cephalocele. The prognosis of the child is determined not only by the presence of hydrocephalus, but also by the number of associated major cerebral anomalies. Options for treatment are discussed.- - - - - - - - - - ranking = 1keywords = cerebral (Clic here for more details about this article) |
2/223. Cranio-cerebral erosion: delayed diagnosis and treatment.Cranio-cerebral erosion is a well-known complication of calvarian fracture with underlying dural tear and cerebral injury in infancy and early childhood. The anatomy, pathogenesis and natural evolution of these lesions remain obscure. The common clinical symptoms are seizures, focal neurological deficits, impairment of consciousness and a soft subgaleal mass. Three patients of cranio-cerebral erosion who underwent delayed surgery in their adult lives are presented to illustrate the common and uncommon features, and their long-term outcome is discussed.- - - - - - - - - - ranking = 3.5keywords = cerebral (Clic here for more details about this article) |
3/223. Ocular malformations, moyamoya disease, and midline cranial defects: a distinct syndrome.PURPOSE: To report a 10-year-old girl with developmental anomalies of both optic disks, a chorioretinal coloboma, sphenopharyngeal meningoencephalocele, and moyamoya disease. methods: A full ophthalmologic examination, cranial magnetic resonance imaging and magnetic resonance angiography, and cerebral angiography were performed. RESULTS: The patient had a morning glory disk anomaly and microphthalmos of the right eye and optic nerve hypoplasia and retinochoroidal coloboma in the left eye. She had a midfacial cleft and an episode of seizures and a stroke. magnetic resonance imaging showed a sphenopharyngeal meningoencephalocele. magnetic resonance angiography and cerebral angiography demonstrated a pattern consistent with moyamoya disease. CONCLUSIONS: This patient had a distinct syndrome of optic disk, retinochoroidal, and carotid circulation anomalies with midline cranial defects. The recognition and treatment of the vascular abnormalities and cranial defects may prevent complications such as strokes that may occur during or after general anesthesia.- - - - - - - - - - ranking = 1keywords = cerebral (Clic here for more details about this article) |
4/223. Idiopathic temporal encephalocele: report of two cases.BACKGROUND: Idiopathic brain herniation into the middle ear is a rare condition that represents diagnostic and therapeutic challenges. OBJECTIVE: The authors present here two new cases of idiopathic brain herniation with special clinical presentation and emphasis on radiographic studies, particularly computed tomographic scan and magnetic resonance imaging, which allowed the authors to detect the malformation. RESULTS: The two patients underwent surgical treatment with infratemporal approach and recovered perfectly.- - - - - - - - - - ranking = 0.067005597501821keywords = brain (Clic here for more details about this article) |
5/223. Spontaneous closure of bony defect in a frontoethmoidal encephalomeningocele patient.The frontoethmoidal encephalomeningocele (FEEM) is a congenital herniation of meninges and brain tissue through the skull bony defect at the foramen cecum. The size of the defect may vary from a few millimeters to many. Those patients with a small defect may not always require a risky operation during childhood. We report on an infant whose bony defect has closed spontaneously with definite clinical evidence. It is proved that the skull defect and brain herniation are able to heal naturally, and this affirms an existence of the abortive subtype of FEE. Conservative treatment may be considered in those with a small bony defect, and surgery can be considered later when it is required.- - - - - - - - - - ranking = 0.067005597501821keywords = brain (Clic here for more details about this article) |
6/223. Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: a neural crest syndrome?We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of the craniofacial arteries, the forebrain, and the face. Data suggest that the link between these rare malformations is abnormal neural crest development.- - - - - - - - - - ranking = 0.03350279875091keywords = brain (Clic here for more details about this article) |
7/223. Central brain herniation secondary to juvenile diabetic ketoacidosis.We present the CT, MR, and autopsy findings of central brain herniation in a 9-year-old boy undergoing treatment for diabetic ketoacidosis (DKA). Severe cerebral edema resulting in central brain herniation is an uncommon complication of the treatment of DKA but carries with it high morbidity and mortality. Radiologic imaging and autopsy findings in this case revealed striking infarctions of central brain structures.- - - - - - - - - - ranking = 0.73451959125637keywords = cerebral, brain (Clic here for more details about this article) |
8/223. 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.- - - - - - - - - - ranking = 0.03350279875091keywords = brain (Clic here for more details about this article) |
9/223. Congenital unilateral buphthalmos in walker-warburg syndrome: a clinicopathological study.BACKGROUND AND PURPOSE: walker-warburg syndrome is a congenital autosomal recessive oculocerebral disorder characterised by hydrocephalus, brain agyria, microphthalmos and retinal dysplasia with or without meningoencephalocele. We describe an unusual finding of congenital unilateral glaucoma and buphthalmos in one eye and microphthalmos in the fellow eye of two neonates with Walker-Warburg syndrome. patients: Two neonates with walker-warburg syndrome and unusual findings of buphthalmos in one eye and a microphthalmic fellow eye are presented. RESULTS: Histological examination of the buphthalmic eyes revealed the presence of mesenchymal tissue in the anterior angle covered by endothelium. No anterior chamber angle was identified in the microphthalmic fellow eye and the iris was adherent to the corneal periphery. CONCLUSIONS: Congenital buphthalmos may also appear in walker-warburg syndrome. The buphthalmos may result from later embryonal ocular developmental arrest than that of the microphthalmic eye.- - - - - - - - - - ranking = 0.53350279875091keywords = cerebral, brain (Clic here for more details about this article) |
10/223. 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.- - - - - - - - - - ranking = 0.5keywords = cerebral (Clic here for more details about this article) |
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