Enfoque perinatal de la ventriculomegalia / Perinatal approach of ventriculomegaly

La Ventriculomegalia Fetal es relevante dado que tiene una alta prevalencia e importante asociación con otras anomalías en el Sistema Nervioso Central. El enfoque perinatal está íntimamente ligado al desarrollo de la Medicina Materno Fetal e implica una evaluación precoz de la anatomía del SNC fetal, utilizando como herramienta de apoyo la ultrasonografía, se debe tener un claro conocimiento de la anatomía cerebral fetal, de los criterios diagnósticos y protocolos estandarizados de manejo clínico actual. Considerar el uso de Resonancia Nuclear Magnética Fetal como técnica complementaria, y proporcionar Consejo Médico a los padres apoyado en Equipos Multidisciplinarios. La presente Revisión tiene el propósito de ser una puesta al día, que sea útil para los profesionales clínicos.

The Fetal ventriculomegaly is relevant since it has a high prevalence and association with other anomalies in the Central Nervous System. The perinatal approach is closely linked to the development of Maternal-Fetal Medicine and involves an early assessment of the anatomy of fetal CNS, using as a support tool ultrasonography, one must have a clear understanding of the fetal brain anatomy, diagnostic criteria and standardized protocols current clinical management. Consider using Nuclear Magnetic Resonance Fetal as a complementary technique, and provide parents Medical Council supported by multidisciplinary teams. This review is an update that is useful for clinicians.

Detección ecográfica y pronóstico de la ventriculomegalia fetal / Ultrasound detection and prognosis of fetal ventriculomegaly

Antecedentes: La ventriculomegalia fetal es un signo ecográfico muy sensible para las alteraciones del sistema nervioso central fetal. La morbilidad asociada a la misma depende principalmente de la etiología y de la presencia de otras malformaciones asociadas. Si es aislada y no progresiva se puede preveer un resultado posnatal favorable hasta en el 70 por ciento de los casos aunque hay una gran disparidad de cifras entre distintos centros y autores. Objetivo: Establecer la relación entre ventriculomegalia y pronóstico perinatal. Método: Se han revisado 60 casos diagnosticados mediante ecografía prenatal en el Hospital Universitario de Canarias siguiendo los criterios ecográficos de ventriculomegalia (astas posteriores mayores de 10 mm). Se realizaron cariotipos y serología materna y en líquido amniótico para completar el estudio. Resultados: Hubo un total de 23 interrupciones voluntarias de la gestación y 28 recién nacidos sin secuelas (47 por ciento). Un 70 por ciento de las ventriculomegalias aisladas correspondieron a recién nacidos sin secuelas. El 10 por ciento de las ventriculomegalias aparecieron en fetos con aneuploidías. Conclusión: El pronóstico fue favorable en los casos de ventriculomegalia no progresiva y aislada. La medida de la ventriculomegalia no predice el resultado. La variabilidad de resultados en la literatura obliga a cada centro a disponer de sus protocolos y datos para aconsejar a la pareja que ha de tomar una decisión sobre el futuro de su embarazo.

Background: Fetal ventriculomegaly is the most sensitive sonographic sign for central nervous system anomalies. Neonatal morbidity and mortality depends on the etiology of the ventriculomegaly and the presence of other malformations. Isolated non-progressive ventriculomegaly is associated with 70 percent favorable results, with a great disparity among studies. Aims: To establish the relationships between ventriculomegaly and perinatal outcome. Methods: The authors reviewed 60 cases of prenatally diagnosed fetal ventriculomegaly in the Canary Islands University Hospital. An ultrasonographic measurement of the lateral ventricles of 10 mm or more was considered as ventriculomegaly. Fetal and maternal serology was performed as well as fetal karyotyping. Results: 23 patients decided to terminate the pregnancy due to unfavorable prognosis. In 28 cases outcome was favorable. 70 percent of isolated ventriculomegaly corresponded to healthy newborns. 10 percent of the fetuses had aneuploidies. Conclusion: Prognosis is favorable if ventriculomegaly is isolated and non-progressive. Measurement of the lateral ventricle does not predict outcome. Disparity of results in literature makes it necessary for each Hospital to rely on their own data and guidelines to assess parents on the fate of the pregnancy.

Hidrocefalia unilateral: caso clínico y revisión de la literatura / Unilateral hydrocephalus: clinical case and literature review

La hidrocefalia congénita unilateral es una malformación extremadamente infrecuente (1 por 1.411 embarazos) y rara vez detectada en el período prenatal. Se considera una entidad distinta a la hidrocefalia bilateral, en cuanto a morbilidad, mortalidad y pronóstico perinatal. En la hidrocefalia unilateral se reporta una sobrevida elevada y un resultado neurológico favorable cuando el grado de dilatación es leve, estable en el tiempo y no asociada a otras malformaciones. El diagnóstico prenatal se realiza mediante ecografía y resonancia magnética y es confirmado al nacimiento con ecografía, tomografía axial computada o resonancia magnética. No existen actualmente pautas estandarizadas para su manejo y seguimiento a largo plazo. Se presenta a continuación un caso clínico de diagnóstico antenatal de hidrocefalia congénita unilateral y la revisión de la literatura.

Congenital unilateral hydrocephalus is an extremely rare malformation (1 per 1,411 pregnancies), rarely detected in the prenatal period. It is considered a different entity than bilateral hydrocephalus, in terms of morbidity, mortality and perinatal outcome. Unilateral hydrocephalus has a high survival rate and favorable neurological outcome when dilatation is mild, stable over time and when not associated with other malformations. Prenatal diagnosis is performed by ultrasound and magnetic resonance imaging and confirmed at birth with ultrasound, CT sean or MRI. Not currently standard guidelines are available for management and long-term monitoring. We present a case report of antenatal diagnosis of congenital unilateral hydrocephalus and literature review.

Ventriculomegalia: diagnóstico antenatal en un Centro de Salud Familiar / Ventriculomegaly: antenatal diagnosis in a Family Health Center

Se presenta un caso de ventriculomegalia diagnosticado en un establecimiento de atención primaria, su derivación, su posterior asociación con una agenesia del cuerpo calloso y seguimiento postnatal inicial.

[Prevalence and risk factors of intraventricular hemorrhage in premature newborns less than 35 weeks in neonatal intensive care units of Isfahan]

Intraventricular hemorrhage [IVH] is an important cause of mortality and morbidity in premature infants. IVH can cause motor deficit, cerebral palsy, behavioral and intellectual disability in preterm newborns. The aim of this study was to assess the prevalence and risk factors of IVH in premature infants with a gestational of less than 35 weeks. From 2003-2005 we prospectively studied 167 preterm newborns less than 35 weeks at Alzahra and Shahid beheshti hospitals- Isfahan- Iran. Skull sonography performed at 7th day of life and IVH graded from grade 1-4. The exclusion criterion was neonatal death before the 7[th] day of life. Brain sonography was conducted at day 7 after birth, and IVH was categorized to four degrees. The association of maternal and neonatal characteristics with IVH was evaluated. The prevalence of IVH was 19.5% in newborns < 35 weeks and 25% in newborns < 1500 grams. The prevalence of IVH significantly correlated with gestational age [p=0.005] and low birth weight [p=0.002]. From 167 infants, 9[5.3%] had grade 1 IVH, 14[8.3%] had grade 2 IVH, 9[5.3%] had grade 3 IVH and 1[0.5%] infant had grade 4 IVH. The other important risk factors for IVH were low 5-minute apgar score, duration of mechanical ventilation, vaginal delivery, duration of oxygen therapy, endotracheal suctioning, low platelet count and surfactant use. The protecting factors included maternal steroids therapy, higher birth weight and higher gestational age. This study emphasizes on the importance of recognizing the risk factors of IVH. With reduction of these risk factors, the incidence of this critical disease can be reduced in newborns

Fowler-like syndrome with extreme oligohydramnios, growth restriction and without muscular hypoplasia.

We report the seventh family of Fowler like syndrome (proliferative vasculopathy and hydrocephaly-hydrencephaly syndrome) and first case from Indian subcontinent. A 35 weeks extremely growth retarded male baby showed enlarged ventricles, thinned out cerebral cortex, diffuse intra-cerebral as well as peri-ventricular calcification, cerebral and corneal vasculopathy, unilateral micro-ophthalmia along with corneal opacity and depressed pulsatile anterior fontanel. This case was different from others concerning association with extreme oligohydramnios (in contrast to polyhydramnios), extreme growth restriction (in contrast to normal growth) and absence of gross muscle hypoplasia. No causative factors like TORCH infection, chromosomal abnormality or positive family history was noted in this case.

Apert syndrome variant with overlapping features of Crouzon syndrome.

A rare variant of Apert syndrome having overlapping features of Crouzon syndrome is described. The salient features of the two syndromes are briefly discussed and overlapping features are highlighted. A possible genetic explanation for the same is mentioned.

Lesiones intraventriculares

En el presente artículo se hace una revisión de la literatura mas recientemente publicada sobre la imagenología de las lesiones intraventriculares, destacando su orígen, incidencia, aspecto escanográfico, imágen por resonancia magnética y diagnóstico tisular con base en su distribución por edad y localización anatómica. Para su abordaje diagnóstico se han dividido en lesiones atriales, del cuerpo de los ventrículos laterales, cercanas al agujero de Monro, del tercer ventrículo y del cuarto ventrículo

Hidrocefalia multiloculada: relato de dois casos / Multiloculated hydrocephalus: report of 2 cases

Hidrocefalia multiloculada é conceituada como quadro clínico-patológico que consiste de ventrículos aumentados e loculados e de cavidades porencefálicas paraventriculares. Apresentamos dois casos de Hidrocefalia multiloculada, um provavelmente conseqüente a processo infeccioso do sistema nervoso central e outro em decorrência de malformaçäo congênita. Aspectos tomográficos compatíveis ao diagnóstico säo ressaltados. A etiopatogenia, o tratamento e o prognóstico säo discutidos em face da literatura compulsada

Atresia congênita do foramen de Monro: relato de caso / Congenital atresia of the foramen of Monro: report of case

Os autores relatam caso de hidrocefalia univentricular devido a atresia congênita do foramen de Monro. A patogenia, o diagnóstico diferencial, o tratamento e a evoluçäo desta rara patologia säo discutidos