1/393. 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) |
2/393. Development of a middle fossa arachnoid cyst. A theory on its pathogenesis.The progression of congenital arachnoid cysts has seldom been documented. We report the case of a child who was diagnosed with arrested hydrocephalus at the age of 13 months. neuroimaging studies performed when the girl was 22 months old showed the appearance of an arachnoid cyst in the right middle fossa, while the previously enlarged ventricles seemed to have decreased in size. To the best of our knowledge, the paradoxical expansion of an arachnoid pouch following a reduction in the size of the ventricular system has not previously been documented. We advance the hypothesis that the development of some cases of arachnoid cyst might be pathogenically related to impaired CSF dynamics associated with pre-existing hydrocephalus. We also briefly review the pertinent literature on the formation and evolution of congenital cerebral arachnoid cysts.- - - - - - - - - - ranking = 0.5keywords = cerebral (Clic here for more details about this article) |
3/393. Fetal cataract in congenital toxoplasmosis.We report a case of the prenatal diagnosis of fetal cataract due to congenital toxoplasmosis. To the best of our knowledge, this is the first report of such a case. We discuss the long-term ocular sequelae of the condition and how they should affect prenatal counselling.- - - - - - - - - - ranking = 39.063361285626keywords = toxoplasmosis (Clic here for more details about this article) |
4/393. Pneumoencephalus and convulsions after ventriculoscopy: a potentially catastrophic complication.A nine-year-old boy with hydrocephalus underwent ventriculoscopy under general anesthesia. After introduction of ventriculoscope the patient had sudden bradycardia, hypotension, and shrinkage of ipsilateral cerebral hemisphere. The ventriculostomy was abandoned. At the end of anesthesia and endotracheal extubation, the patient developed generalized convulsions. Reexploration of wound did not reveal anything significant; however, postoperative CT scan of head showed massive pneumoencephalus. The patients received elective ventilation of lungs for 24 hours and made complete recovery. The authors describe the reasons for these complications and further management.- - - - - - - - - - ranking = 0.5keywords = cerebral (Clic here for more details about this article) |
5/393. Three children with congenital toxoplasmosis: early report from a Swedish prospective screening study.The aim of this prospective study was to define the incidence of congenital toxoplasmosis in sweden. blood eluates collected on filter papers, Guthrie cards, from 40978 newborn babies were analysed for specific immunoglobulin m (IgM) and IgG antitoxoplasma antibodies. This is a preliminary report of three children with congenital toxoplasmosis, defined by the occurrence of antitoxoplasma-specific IgM antibodies. Two children were asymptomatic at birth. They were both normally developed at the age of 12 and 15 months, respectively. The third child had unidentified but uncomplicated symptoms of infection in the neonatal period. As a result of the screening congenital toxoplasmosis was confirmed and treatment instituted. Microphthalmus and peripheral chorioretinitis were detected in one eye. In spite of the chemotherapeutic treatment he developed hydrocephalus needing neurosurgical intervention at the age of 3 months. His development at 14 months was normal. The incidence in sweden of congenital toxoplasmosis detected by specific IgM antitoxoplasma antibodies in blood from filter papers is less than 1:10000.- - - - - - - - - - ranking = 62.501378057002keywords = toxoplasmosis (Clic here for more details about this article) |
6/393. cleidocranial dysplasia with neonatal death due to central nervous system injury in utero: case report and literature review.cleidocranial dysplasia (CCD), an uncommon disorder involving membranous bones, is rarely lethal in early life. The calvaria is defective and wormian bones are present. Abnormalities of the clavicles vary in severity from a minor unilateral defect to bilateral absence. This report concerns pre- and postmortem anatomical and radiological findings in a 15-day-old female neonate with CCD. Her postnatal course was characterized by seizures and recognition of hydrocephalus during the first day of life. The calvaria was hypoplastic with numerous wormian bones. A pseudofracture of the right clavicle was present. hydrocephalus was present in the brachycephalic brain which had a severely thinned cerebral cortex. hemosiderin in the ventricular lining and marked subependymal gliosis were interpreted as evidence of old intraventricular hemorrhage that had occurred in utero. A CCD-related condition, Yunis-Varon syndrome (YVS), is noted for early lethality and for developmental and secondary abnormalities of the central nervous system. The present case only partially matches the phenotype of YVS and might represent a part of a spectrum of phenotypic variants ranging from viable CCD to lethal YVS.- - - - - - - - - - ranking = 2.038742269175keywords = cerebral, central nervous system, nervous system (Clic here for more details about this article) |
7/393. hydrocephalus associated with glycogen storage disease type ii (Pompe's disease).The authors describe a case of hydrocephalus in an 8-month, 2-week-old infant who had been previously diagnosed with glycogen storage disease type ii. Cranial imaging revealed no evidence of obstruction within the ventricular system. This case adds to the central nervous system complications associated with this disorder. Several possible mechanisms for the hydrocephalus observed in this infant are discussed.- - - - - - - - - - ranking = 0.30774845383499keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/393. recurrence of the severe form of microgastria-limb reduction defect in a consanguineous family.This report concerns two sibs from a consanguineous Sudanese family with microgastria-limb reduction defect associated with hydrocephalus and agenesis of corpus callosum. We suggest that these cases together with other previously reported cases of central nervous system (CNS) anomalies associated with microgastria-limb reduction defect could represent an autosomal recessive syndrome differing from the classical microgastria-limb reduction defect by its severity, presence of CNS anomalies and its pattern of inheritance.- - - - - - - - - - ranking = 0.30774845383499keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/393. Variants of congenital ocular motor apraxia: associations with hydrocephalus, pontocerebellar tumor, and a deficit of vertical saccades.BACKGROUND: Congenital ocular motor apraxia (coma) is characterized by the inability to generate volitional horizontal saccadic eye movements in the absence of other focal neurologic abnormalities. SUBJECTS: We report on two children (ages 5 months and 3 years) whose coma did not adhere to these classic criteria. The children were followed up clinically with serial ocular motor examinations and neuroimaging over a period of 3 years. RESULTS: In the first child horizontal coma was associated initially with neonatal communicating hydrocephalus. Two and one half years after the first signs of coma, a fourth ventricle medulloblastoma appeared. The second child, who recovered from a periventricular hemorrhage caused by perinatal asphyxia, manifested vertical coma and compensatory vertical head thrusts. CONCLUSIONS: coma may be associated with hydrocephalus, pontocerebellar tumor, and periventricular hemorrhage. These rare variants of coma emphasize that the eye movement deficits may arise from several locations, cerebral as well as pontocerebellar, in the neuronal pathways generating saccades.- - - - - - - - - - ranking = 0.5keywords = cerebral (Clic here for more details about this article) |
10/393. Intracranial dislocation of a lumbo-peritoneal shunt-catheter: case report and review of the literature.We report on the dislocation of the tip of a lumbo-peritoneal shunting catheter into the cerebral parenchyma 10 months after insertion. The progressive migration towards the deep structures of the brain, once the catheter had left the peritoneal cavity, might have been caused by CSF-flow. Such hypothesis is supported by modern MRI technology visualizing CSF-flow in a spino-cerebral direction.- - - - - - - - - - ranking = 1keywords = cerebral (Clic here for more details about this article) |
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