Malformación de Chiari Tipo I en adultos. Un enfoque médico-quirúrgico. Parte I / Type I Chiari malformation in adults. A medico-surgical approach. Part I

Se revisó la literatura existente sobre la Malformación de Chiari Tipo I en el adulto, haciendo énfasis en su diagnóstico imagenológico y tratamiento médico-quirúrgico. La malformación de Chiari es una enfermedad poco frecuente. Representa entre el 1 y el 4% de todas las patologías neuroquirúrgicas. El diagnóstico se realiza meses y hasta años después de comenzada la sintomatología y se confirma en el 100% de los casos con Resonancia magnética de cráneo. El tratamiento es quirúrgico en los pacientes sintomáticos, siendo controversial en aquellos oligosintomáticos o con diagnóstico casual. La cirugía siempre debe realizarse con el apoyo de monitoreo neurofisiológico, que puede ser determinante en la técnica quirúrgica a emplear. La mortalidad asociada a la cirugía es muy baja, oscilando entre el 0 y 0,5% según la mayoría de las series. Lo más importante es la selec­ción de los pacientes para el tratamiento quirúrgico. No debe asumirse una actitud expectante en espera de un deterioro neurológico que justifique la cirugía, cuando ésta se hace a tiempo los resultados son mejores y con un mínimo de complicaciones (AU).

The aim of this article is reviewing the existent literature on Type I Chiari malformation in adults and making emphasis in its imaging diagnosis and medico-surgical treatment. Chiari malformation is a few frequent diseases. It represents 1-4 % of all the neurosurgical diseases. The diagnosis is made months and even years after the beginning of the symptoms and it is confirmed by cranial magnetic resonance in the 100 % of the cases. The treatment is surgical in symptomatic patients, and it is controversial in the oligosymptomatic ones and in those with casual diagnosis. The surgery should be performed with the support of neurophysiological monitoring that might be determinant in the surgical technique to use. The mortality associated to the surgery is low, ranging from 0 and 0.5 % according to most of the series. The most important thing is the choice of the patients for the surgical treatment. The neurological deterioration should not be expected to justify the surgery; when the surgery is carried out on time the results are better and with the minimum of complications (AU).

Central nervous system anomalies diagnosed antenatally and post-delivery management

To find out the prevalence of the central nervous system anomalies [CNS] and the outcome after surgical correction of operable defects. This is a retrospective review of all cases diagnosed antenatally with CNS anomalies in the Department of Obstetrics and Gynecology at King Abdulaziz University Hospital [KAUH], Jeddah, Kingdom of Saudi Arabia from January 1997 to March 2005, and their outcome including perinatal deaths and postoperative outcome following surgical correction carried out as treatment or palliative. Ninety CNS anomalies were diagnosed antenatally, and 86 were confirmed postnatally. Forty-one [47.7%] were neural tube defects [NTDs] [24 were spina bifida associated with hydrocephalus, 3 encephalocele, 14 anencephaly], 36 [41.9%] were hydrocephalus, 4 [4.65%] holoprosencephaly, 3 [3.4%] microcephaly, and 2 [2.32%] with brain cysts. Of the 41 cases that were operated, 6 [14.6%] died, 26 [63.4%] survived with severe neurological sequelae, 6 [14.6%] lost follow-up, and 3 [7.3%] did well for the period of follow-up. The prevalence of CNS anomalies is comparable to worldwide prevalence. The NTDs are important component of these anomalies, and implementation of strategies to decrease the rate of these anomalies would be beneficial to prevent them as they carry a high rate of handicap and suffering, even after surgical correction

Laparotomía de giordano-cherney en la operacion cesarea: una alternativa en las malformaciones del sistema nervioso central fetal

Las malformaciones del sistema nervioso central son la segunda malformación más frecuente en recién nacidos. La elección de la vía de parto es controversial. Nuestro centro ha adoptado el parto cesárea como de elección para resolver tales casos mediante laparotomía tipo Pfannenstiel y longitudinal media infraumbilical. La observación de algunas dificultades que ambas técnicas presentan nos llevó a adaptar, un abordaje extraído de la oncoginecología, a la práctica obstétrica. Presentamos 7 casos de malformaciones del sistema nervioso central resueltos satisfactoriamente mediante laparotomía de Giordano-Cherney. La cirugía se realizó en forma electiva con un tiempo promedio de 72 minutos. No se registraron complicaciones en el intra o post operatorio. El manejo del dolor se realizó en forma rutinaria. Todas las pacientes evolucionaron favorablemente. No se presentaron complicaciones en los recien nacidos atribuibles a la cirugía

The central nervous system malformations are the second more frequent malformation in new born. The election of the way of delivery is controversial. Our center has adopted the cesarean section to resolve such cases, using either a Pfannenstiel laparotomy or a longitudinal infraumbilical section. The observation of some difficulties in both techniques carried us to adapt, a oncoginecological technique, to the obstetric practice. We present 7 cases with central nervous malformations resolved satisfactorily using a Giordano-Cherney laparotomy. The surgery was carried out in elective form, with an average time of 72 minutes. No complications were registered intra or post surgery. The pain management was carried out in routine form. All patients evolved satisfactorily. No complications in new born where attributed to surgery