{"title":"Dandy-Walker syndrome: experience at the Hospital for Sick Children, Toronto.","authors":"A Asai, H J Hoffman, E B Hendrick, R P Humphreys","doi":"10.1159/000120445","DOIUrl":"https://doi.org/10.1159/000120445","url":null,"abstract":"<p><p>Thirty-five patients with the Dandy-Walker syndrome (DWS) treated over the years 1964-1987 at the Hospital for Sick Children, Toronto, were reviewed. Thirty-three patients (94%) had hydrocephalus at the time of diagnosis. The primary association of aqueduct stenosis was excluded by radiological investigation and clinical course in all patients. Associated central nervous system (CNS) anomalies were present in 10 patients. Seven had occipital encephalocele and in 1 of these there was an associated Klippel-Feil syndrome. Four patients had agenesis of the corpus callosum and 1 patient had polyporencephaly. The treatment of these patients has changed over the years in our institution so that during an earlier period, 3 patients were treated by resection of the cyst membranes. We then went through a period of shunting the lateral ventricle so that 21 patients were treated with either a lateral ventricle to peritoneal or lateral ventricle to atrial shunt. More recently, patients with the DWS have been treated with a cyst-peritoneal shunt and 10 patients were thus treated. Only 1 patient was treated with a simultaneous lateral ventricle and cyst-peritoneal shunt. Nine of the twenty-one patients (43%) with a primary lateral ventricle to peritoneum or lateral ventricle to atrial shunt developed a secondary aqueduct stenosis and an isolated fourth ventricle which required additional cyst-peritoneal shunting. Only 1 of the 10 patients treated with a primary cyst-peritoneal shunt required an additional lateral ventricle peritoneal shunt. Two patients initially treated with a lateral ventricle peritoneal shunt and with a primary cyst-peritoneal shunt subsequently had a percutaneous stereotactic third ventriculostomy.(ABSTRACT TRUNCATED AT 250 WORDS)</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 2","pages":"66-73"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120445","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13776665","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Dandy-Walker cyst upward herniation: the role of magnetic resonance imaging and double shunts.","authors":"P Pillay, G H Barnett, C Lanzeiri, R Cruse","doi":"10.1159/000120446","DOIUrl":"https://doi.org/10.1159/000120446","url":null,"abstract":"<p><p>Upward transtentorial herniation of a Dandy-Walker cyst is a potentially fatal complication, particularly after solitary shunting of the lateral ventricle for associated hydrocephalus. Two patients with upward transtentorial herniation of their Dandy-Walker cysts are presented to illustrate the diagnostic value of magnetic resonance (MR) imaging with the demonstration of unique 'keyhole' and 'snail' signs on the axial and sagittal MR images, respectively. In both patients, double and simultaneous shunting en-Y of a lateral ventricle and the posterior fossa cyst resulted in the resolution of the herniation syndrome.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 2","pages":"74-8; discussion 78-9"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120446","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13776667","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Ependymomas in two sisters and a maternal male cousin with mosaicism with monosomy 22 in tumour.","authors":"M L Savard, D M Gilchrist","doi":"10.1159/000120447","DOIUrl":"https://doi.org/10.1159/000120447","url":null,"abstract":"<p><p>A family with two sisters and their mother's male cousin had ependymomas. Of note is the fact that chromosome analysis on the tumour of the younger sister showed two cell lines, one a normal female line, the other with monosomy of chromosome 22.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 2","pages":"80-4"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120447","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13840284","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neuropathology of pediatric liver transplantation.","authors":"W A Hall, A J Martinez","doi":"10.1159/000120482","DOIUrl":"https://doi.org/10.1159/000120482","url":null,"abstract":"<p><p>We reviewed the clinical histories and autopsy records of 35 pediatric patients (ranging in age from 9 months to 18 years) who underwent orthotopic liver transplantation using ciclosporin and corticosteroids for immunosuppression. At the time of death, 19 children (54%) had encephalopathy, 16 (46%) were lethargic or in coma, 10 (29%) had seizures, and 10 were normal. Neuropathological lesions were found on postmortem examination in all 35 patients. Vascular lesions such as infarction, ischemia, thrombosis, and hemorrhage were the most common neuropathological findings (86%) followed by infectious processes (29%). Candida albicans (2 patients) and Aspergillus fumigatus (3 patients) were the only offending organisms identified, both causing meningoencephalitis. Alzheimer type II astrocytes, a characteristic feature of chronic liver disease, were the single most common autopsy finding (69%). Central pontine myelinolysis was seen in 3 children and basilar artery thrombosis affected 1 child. Neurological complications and their subsequent neuropathology are a significant cause of morbidity and mortality after pediatric liver transplantation. Vascular insults, electrolyte abnormalities, and infections that involve the central nervous system are directly related to liver function and the immunosuppression necessary to maintain graft viability. Only with continued observation after surgery combined with rapid medical and surgical treatment can we hope to improve the prognosis following liver transplantation in the pediatric population.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 6","pages":"269-75"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120482","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13634379","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Spinal extradural arachnoid cyst.","authors":"M T Stechison, E B Hendrick, E Cohen","doi":"10.1159/000120438","DOIUrl":"https://doi.org/10.1159/000120438","url":null,"abstract":"<p><p>A relatively unusual cause of progressive paraparesis is a spinal arachnoid cyst. The following is a case report of this lesion in an adolescent. The CT-myelographic and MR features, as well as the management of this case are discussed, followed by a review of the pertinent English literature on this topic.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 1","pages":"36-8"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120438","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13777056","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Eight-year-old girl with psychomotor retardation and seizures.","authors":"V Jay, G Ronen, S H Tucker","doi":"10.1159/000120469","DOIUrl":"https://doi.org/10.1159/000120469","url":null,"abstract":"","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 4","pages":"207-14"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120469","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13629472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Spinal cord injury without radiographic abnormality in children.","authors":"R K Osenbach, A H Menezes","doi":"10.1159/000120464","DOIUrl":"https://doi.org/10.1159/000120464","url":null,"abstract":"<p><p>Between 1970 and 1988, 35% of children with traumatic myelopathy demonstrated spinal cord injury without radiographic abnormality (SCIWORA). Young children, especially children under 3 years were particularly vulnerable. Mechanisms of injury were age-specific and included flexion, hyperextension and longitudinal traction. Over 80% of injuries involved the cervical cord. Ninety-five percent of all severe injuries occurred in younger children. Neurologic recovery was directly dependent on the degree of initial neurologic injury. Management included dynamic films to exclude obvious fracture or instability, CT or polytomography to exclude occult fracture, and MRI or CT-myelography to exclude surgical lesions. Cervical immobilization is the cornerstone of therapy for prevention of recurrent episodes of SCIWORA.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 4","pages":"168-74; discussion 175"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120464","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13630788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Stereotactic surgery in children.","authors":"J V Pattisapu, M L Walker, M P Heilbrun","doi":"10.1159/000120444","DOIUrl":"https://doi.org/10.1159/000120444","url":null,"abstract":"<p><p>Stereotactic biopsy has been popularized over the last decade since the advent of newer, more sophisticated instrumentation and technology. However, less than 300 cases of pediatric stereotaxy are recorded in the literature and few reports emphasize the necessary modifications required in children. Sixty-six stereotaxic procedures were performed on 62 children using the Brown-Roberts-Well (BRW) apparatus at our institutions. The mean age was 9.0 years (range 5 months to 18 years). The indication for the technique was diagnostic in 36 cases and therapeutic in 9 cases; location was a factor in 21 children with the lesion involving a 'deficit-prone' area of the brain. The BRW technique was applicable in 61 procedures (93%). Inability to enter cysts or biopsy of necrotic tissue and tumor capsule occurred in 5 cases. One patient had a transient increase in cerebral edema, for an overall complication rate of 2%. It appears that the BRW stereotactic technique is equally effective in children and adults and will no doubt play an ever increasing role in the future of pediatric neurosurgery.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 2","pages":"62-5"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120444","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13840282","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The cause of Chiari II malformation: a unified theory.","authors":"D G McLone, P A Knepper","doi":"10.1159/000120432","DOIUrl":"https://doi.org/10.1159/000120432","url":null,"abstract":"<p><p>The cause of the Chiari II hindbrain deformity in children born with a myelomeningocele can be explained by the lack of distention of the embryonic ventricular system. Defective occlusion and an open neural tube precludes the accumulation of fluid and pressure within the cranial vesicles. This distention is critical to normal brain development. The small posterior fossa, cerebral disorganization, and lückenschädel are the result.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"15 1","pages":"1-12"},"PeriodicalIF":0.0,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120432","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13840301","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}