The Experts below are selected from a list of 270 Experts worldwide ranked by ideXlab platform
Manisha Goyal - One of the best experts on this subject based on the ideXlab platform.
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Congenital Hydrocephalus, corpus callosum agenesis, and prosencephalic cyst with supernumerary nostril: A neurocristopathy
Asian journal of neurosurgery, 2018Co-Authors: Rahul Gupta, Dinesh Kumar Barolia, Manisha GoyalAbstract:A 3-month-old-male infant presented with enlargement of head since birth. Clinical and radiological evaluation revealed Congenital Hydrocephalus, corpus callosum agenesis, prosencephalic cyst, and cranial vault deficiency with supernumerary nostril on the left side. Right ventriculoperitoneal shunt (Chhabra shunt) surgery was performed. The patient did well postoperatively. Parents of the patient have been counseled for repair of supernumerary nostril. Congenital Hydrocephalus with corpus callosum agenesis is rare. Furthermore, supernumerary nostril is a very rare anomaly with
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Congenital Hydrocephalus corpus callosum agenesis and prosencephalic cyst with supernumerary nostril a neurocristopathy
Asian journal of neurosurgery, 2018Co-Authors: Rahul Gupta, Dinesh Kumar Barolia, Manisha GoyalAbstract:A 3-month-old-male infant presented with enlargement of head since birth. Clinical and radiological evaluation revealed Congenital Hydrocephalus, corpus callosum agenesis, prosencephalic cyst, and cranial vault deficiency with supernumerary nostril on the left side. Right ventriculoperitoneal shunt (Chhabra shunt) surgery was performed. The patient did well postoperatively. Parents of the patient have been counseled for repair of supernumerary nostril. Congenital Hydrocephalus with corpus callosum agenesis is rare. Furthermore, supernumerary nostril is a very rare anomaly with <40 cases reported in the literature till date. To the best of our knowledge, Congenital Hydrocephalus, corpus callosum agenesis, prosencephalic cyst, and cranial vault deficiency associated with supernumerary nostril have not been reported till date. We herein briefly review the pertinent literature and describe the embryopathogenesis of this rare association. We propose that this association is a neurocristopathy.
Helen Dolk - One of the best experts on this subject based on the ideXlab platform.
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Congenital Hydrocephalus prevalence prenatal diagnosis and outcome of pregnancy in four european regions
European Journal of Paediatric Neurology, 2010Co-Authors: Ester Garne, Maria Loane, Marieclaude Addor, Patricia A Boyd, Ingeborg Barisic, Helen DolkAbstract:OBJECTIVE: To describe prevalence, prenatal diagnosis and outcome for fetuses and infants with Congenital Hydrocephalus. METHODS: Data were taken from four European registries of Congenital malformations (EUROCAT). The registries included are based on multiple sources of information and include information about livebirths, fetal deaths with GA > or = 20 weeks and terminations of pregnancy for fetal anomaly (TOPFA). All cases from the four registries diagnosed with Congenital Hydrocephalus and born in the period 1996-2003 were included in the study. Cases with Hydrocephalus associated with neural tube defects were not included in the study. RESULTS: Eighty-seven cases with Congenital Hydrocephalus were identified during the study period giving an overall prevalence of 4.65 per 10,000 births. There were 41 livebirths (47%), four fetal deaths (5%) and 42 TOPFA (48%). Nine percent of all cases were from a multiple pregnancy. Additional non-cerebral major malformations were diagnosed in 38 cases (44%) and karyotype anomalies in eight cases (9%). Median GA at TOPFA was 21 weeks. Among livebirths 61% were diagnosed prenatally at a median GA of 31 weeks (range 17-40 weeks) and median GA at birth was 37 weeks. Fourteen liveborn infants (34%) died within the first year of life with the majority of deaths during the first week after birth. CONCLUSION: Congenital Hydrocephalus is a severe Congenital malformation often associated with other Congenital anomalies. CH is often diagnosed prenatally, although sometimes late in pregnancy. A high proportion of affected pregnancies result in termination for severe fetal anomaly and there is a high mortality in livebirths.
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Congenital Hydrocephalus prevalence prenatal diagnosis and outcome of pregnancy in four european regions
Archives of Disease in Childhood, 2008Co-Authors: Ester Garne, Maria Loane, Marieclaude Addor, Patricia A Boyd, Ingeborg Barisic, Helen DolkAbstract:Objective To describe prevalence, prenatal diagnosis and outcome for fetuses and infants with Congenital Hydrocephalus. Methods Data from four European registries of Congenital malformations (EUROCAT). The registries included are all based on multiple sources of information and include information about live births, fetal deaths with GA >20 weeks and terminations of pregnancy after prenatal diagnosis of malformations (TOPFA). All cases from the four registries diagnosed with Congenital Hydrocephalus and born in the period 1996–2003 were included in the study. Cases with Hydrocephalus associated with neural tube defects were not included in the study. Results Ninety cases with Congenital Hydrocephalus were identified during the study period giving an overall prevalence of 4.81 per 10,000 births. There were 44 live births (49%), 4 fetal deaths (4%) and 42 TOPFA (47%). Non-cerebral malformations were diagnosed in 32 cases (36%) and karyotype anomalies in eight cases (9%). Median GA at TOPFA was 21 weeks. Among live births 43% were diagnosed prenatally with a mean GA at 32 weeks (range 19–40 weeks) and a median GA at birth at 36 weeks. Thirteen live-born infants (30%) did not survive, nine of whom were diagnosed prenatally. The majority of deaths in live-born cases occurred within the first week of birth. Conclusion Prevalence of Congenital Hydrocephalus falls within the European definition of a rare disease which is 5 per 10,000 births. Associated malformations and karyotype anomalies occur frequently together with Congenital Hydrocephalus. Prenatal diagnosis is often followed by termination of pregnancy and mortality among live births is high.
Rahul Gupta - One of the best experts on this subject based on the ideXlab platform.
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Congenital Hydrocephalus, corpus callosum agenesis, and prosencephalic cyst with supernumerary nostril: A neurocristopathy
Asian journal of neurosurgery, 2018Co-Authors: Rahul Gupta, Dinesh Kumar Barolia, Manisha GoyalAbstract:A 3-month-old-male infant presented with enlargement of head since birth. Clinical and radiological evaluation revealed Congenital Hydrocephalus, corpus callosum agenesis, prosencephalic cyst, and cranial vault deficiency with supernumerary nostril on the left side. Right ventriculoperitoneal shunt (Chhabra shunt) surgery was performed. The patient did well postoperatively. Parents of the patient have been counseled for repair of supernumerary nostril. Congenital Hydrocephalus with corpus callosum agenesis is rare. Furthermore, supernumerary nostril is a very rare anomaly with
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Congenital Hydrocephalus corpus callosum agenesis and prosencephalic cyst with supernumerary nostril a neurocristopathy
Asian journal of neurosurgery, 2018Co-Authors: Rahul Gupta, Dinesh Kumar Barolia, Manisha GoyalAbstract:A 3-month-old-male infant presented with enlargement of head since birth. Clinical and radiological evaluation revealed Congenital Hydrocephalus, corpus callosum agenesis, prosencephalic cyst, and cranial vault deficiency with supernumerary nostril on the left side. Right ventriculoperitoneal shunt (Chhabra shunt) surgery was performed. The patient did well postoperatively. Parents of the patient have been counseled for repair of supernumerary nostril. Congenital Hydrocephalus with corpus callosum agenesis is rare. Furthermore, supernumerary nostril is a very rare anomaly with <40 cases reported in the literature till date. To the best of our knowledge, Congenital Hydrocephalus, corpus callosum agenesis, prosencephalic cyst, and cranial vault deficiency associated with supernumerary nostril have not been reported till date. We herein briefly review the pertinent literature and describe the embryopathogenesis of this rare association. We propose that this association is a neurocristopathy.
Ester Garne - One of the best experts on this subject based on the ideXlab platform.
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Congenital Hydrocephalus prevalence prenatal diagnosis and outcome of pregnancy in four european regions
European Journal of Paediatric Neurology, 2010Co-Authors: Ester Garne, Maria Loane, Marieclaude Addor, Patricia A Boyd, Ingeborg Barisic, Helen DolkAbstract:OBJECTIVE: To describe prevalence, prenatal diagnosis and outcome for fetuses and infants with Congenital Hydrocephalus. METHODS: Data were taken from four European registries of Congenital malformations (EUROCAT). The registries included are based on multiple sources of information and include information about livebirths, fetal deaths with GA > or = 20 weeks and terminations of pregnancy for fetal anomaly (TOPFA). All cases from the four registries diagnosed with Congenital Hydrocephalus and born in the period 1996-2003 were included in the study. Cases with Hydrocephalus associated with neural tube defects were not included in the study. RESULTS: Eighty-seven cases with Congenital Hydrocephalus were identified during the study period giving an overall prevalence of 4.65 per 10,000 births. There were 41 livebirths (47%), four fetal deaths (5%) and 42 TOPFA (48%). Nine percent of all cases were from a multiple pregnancy. Additional non-cerebral major malformations were diagnosed in 38 cases (44%) and karyotype anomalies in eight cases (9%). Median GA at TOPFA was 21 weeks. Among livebirths 61% were diagnosed prenatally at a median GA of 31 weeks (range 17-40 weeks) and median GA at birth was 37 weeks. Fourteen liveborn infants (34%) died within the first year of life with the majority of deaths during the first week after birth. CONCLUSION: Congenital Hydrocephalus is a severe Congenital malformation often associated with other Congenital anomalies. CH is often diagnosed prenatally, although sometimes late in pregnancy. A high proportion of affected pregnancies result in termination for severe fetal anomaly and there is a high mortality in livebirths.
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Congenital Hydrocephalus prevalence prenatal diagnosis and outcome of pregnancy in four european regions
Archives of Disease in Childhood, 2008Co-Authors: Ester Garne, Maria Loane, Marieclaude Addor, Patricia A Boyd, Ingeborg Barisic, Helen DolkAbstract:Objective To describe prevalence, prenatal diagnosis and outcome for fetuses and infants with Congenital Hydrocephalus. Methods Data from four European registries of Congenital malformations (EUROCAT). The registries included are all based on multiple sources of information and include information about live births, fetal deaths with GA >20 weeks and terminations of pregnancy after prenatal diagnosis of malformations (TOPFA). All cases from the four registries diagnosed with Congenital Hydrocephalus and born in the period 1996–2003 were included in the study. Cases with Hydrocephalus associated with neural tube defects were not included in the study. Results Ninety cases with Congenital Hydrocephalus were identified during the study period giving an overall prevalence of 4.81 per 10,000 births. There were 44 live births (49%), 4 fetal deaths (4%) and 42 TOPFA (47%). Non-cerebral malformations were diagnosed in 32 cases (36%) and karyotype anomalies in eight cases (9%). Median GA at TOPFA was 21 weeks. Among live births 43% were diagnosed prenatally with a mean GA at 32 weeks (range 19–40 weeks) and a median GA at birth at 36 weeks. Thirteen live-born infants (30%) did not survive, nine of whom were diagnosed prenatally. The majority of deaths in live-born cases occurred within the first week of birth. Conclusion Prevalence of Congenital Hydrocephalus falls within the European definition of a rare disease which is 5 per 10,000 births. Associated malformations and karyotype anomalies occur frequently together with Congenital Hydrocephalus. Prenatal diagnosis is often followed by termination of pregnancy and mortality among live births is high.
Jack M. Fletcher - One of the best experts on this subject based on the ideXlab platform.
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Are Shunt Revisions Associated with IQ in Congenital Hydrocephalus? A Meta -Analysis
Neuropsychology Review, 2016Co-Authors: C. Nikki Arrington, Ashley L. Ware, Yusra Ahmed, Paulina A. Kulesz, Maureen Dennis, Jack M. FletcherAbstract:Although it is generally acknowledged that shunt revisions are associated with reductions in cognitive functions in individuals with Congenital Hydrocephalus, the literature yields mixed results and is inconclusive. The current study used meta-analytic methods to empirically synthesize studies addressing the association of shunt revisions and IQ in individuals with Congenital Hydrocephalus. Six studies and three in-house datasets yielded 11 independent samples for meta-analysis. Groups representing lower and higher numbers of shunt revisions were coded to generate effect sizes for differences in IQ scores. Mean effect size across studies was statistically significant, but small (Hedges’ g = 0.25, p
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are shunt revisions associated with iq in Congenital Hydrocephalus a meta analysis
Neuropsychology Review, 2016Co-Authors: Nikki C Arrington, Ashley L. Ware, Yusra Ahmed, Paulina A. Kulesz, Maureen Dennis, Jack M. FletcherAbstract:Although it is generally acknowledged that shunt revisions are associated with reductions in cognitive functions in individuals with Congenital Hydrocephalus, the literature yields mixed results and is inconclusive. The current study used meta-analytic methods to empirically synthesize studies addressing the association of shunt revisions and IQ in individuals with Congenital Hydrocephalus. Six studies and three in-house datasets yielded 11 independent samples for meta-analysis. Groups representing lower and higher numbers of shunt revisions were coded to generate effect sizes for differences in IQ scores. Mean effect size across studies was statistically significant, but small (Hedges' g = 0.25, p < 0.001, 95 % CI [0.08, 0.43]) with more shunt revisions associated with lower IQ scores. Results show an association of lower IQ and more shunt revisions of about 3 IQ points, a small effect, but within the error of measurement associated with IQ tests. Although clinical significance of this effect is not clear, results suggest that repeated shunt revisions because of shunt failure is associated with a reduction in cognitive functions.