ABSTRACT
RESUMEN 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).
ABSTRACT The existent literature on Type I Chiari Malformation in adults was reviewed, making emphasis in its imaging diagnosis and medico-surgical treatment. Chiari malformation is a few frequent disease. It represents between 1 % and 4 % of all the neurosurgery pathologies. The diagnosis is made up 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 % in 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).
Subject(s)
Humans , Adult , Arnold-Chiari Malformation/surgery , Arnold-Chiari Malformation/diagnosis , Arnold-Chiari Malformation/etiology , Arnold-Chiari Malformation/physiopathology , Arnold-Chiari Malformation/diagnostic imaging , Syringomyelia , Cranial Fossa, Posterior/abnormalities , Decompressive Craniectomy , Surgical Procedures, Operative , Magnetic Resonance Spectroscopy/methods , Intracranial Hypertension , HydrocephalusABSTRACT
Resumen: La acondroplasia es la condición asociada a talla baja desproporcionada más frecuente, caracterizada por un crecimiento óseo anormal, que resulta en talla baja con extremidades cortas e inteligencia normal. Una de las complicaciones más habituales es la compresión medular, que puede ocurrir a cualquier nivel, siendo más frecuente en la unión cráneo cervical, generando alta morbimortalidad en los primeros años de vida, principalmente por muerte súbita. Presentamos una paciente de 1 año 10 meses con diagnóstico precoz de acondroplasia, que presentó en su evolución estenosis acueductal con compresión medular, sintomática, pesquisada en control rutinario, que requirió cirugía descompresiva con buena evolución posterior. Palabras clave: Acondroplasia, estenosis acueductal, compresión medular sintomática, hidrocefalia, craniectomía suboccip
Achondroplasia is the most frequent cause of disproportionate short stature, it is characterized by abnormal growth of long bones, rendering a short-limbed individual of normal intelligence. A serious potential complication is spinal compression, which can happen at any level but is particularly common at the craniocervical junction. It can cause important morbidity during the first few years of life, including sudden death. We present a 22-monthold patient diagnosed with achondroplasia, who developed aqueductal stenosis with symptomatic spinal cord compression, diagnosed during a routine consultation, requiring decompressive surgery with excellent results. Key words: Achondroplasia, aqueductal stenosis, symptomatic spinal cord compression, hydrocephalus, suboccipital craniectomy.
ABSTRACT
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).
Subject(s)
Humans , Male , Female , Adult , Nervous System Malformations/surgery , Arnold-Chiari Malformation/surgery , Arnold-Chiari Malformation/complications , Arnold-Chiari Malformation/diagnosis , Syringomyelia/complications , Syringomyelia/diagnosis , Review Literature as Topic , Nervous System Malformations/complications , Nervous System Malformations/pathology , Nervous System Malformations/epidemiology , Nervous System Malformations/diagnostic imagingABSTRACT
Achondroplasia is the most frequent cause of disproportionate short stature. Characterized by abnormal growth of long bones, it renders a short-limbed individual of normal intelligence. A serious potential complication is spinal compression, which can happen at any level but is particularly common at the craniocervical junction. It can cause important morbility during the first few years of life, including sudden death. We present a 22-month-old patient diagnosed with achondroplasia, who developed aqueductal stenosis with symptomatic spinal cord compression, diagnosed during a routine consultation, requiring decompressive surgery with excellent results.
La acondroplasia es la condición asociada a talla baja desproporcionada más frecuente, caracterizada por un crecimiento óseo anormal, que resulta en talla baja con extremidades cortas e inteligencia normal. Una de las complicaciones más habituales es la compresión medular, que puede ocurrir a cualquier nivel, siendo más frecuente en la unión cráneo cervical, generando alta morbimortalidad en los primeros años de vida, principalmente por muerte súbita. Presentamos una paciente de 1 año 10 meses con diagnóstico precoz de acondroplasia, que presentó en su evolución estenosis acueductal con compresión medular, sintomática, pesquisada en control rutinario, que requirió cirugía descompresiva con buena evolución posterior.
Subject(s)
Humans , Female , Infant , Achondroplasia/complications , Cerebral Aqueduct/surgery , Spinal Cord Compression/surgery , Cerebral Aqueduct/pathology , Spinal Cord Compression/diagnosis , Spinal Cord Compression/etiology , Constriction, Pathologic/surgery , Constriction, Pathologic/diagnosis , Constriction, Pathologic/etiology , Decompressive Craniectomy , Hydrocephalus/diagnosis , Magnetic Resonance Imaging , Treatment OutcomeABSTRACT
The best surgical treatment for Chiari malformation is unclear, especially in patients with syringomyelia. We reviewed the records of 16 patients who underwent suboccipital craniectomy at our institution between 2005 and 2008. Of the six patients who did not undergo duraplasty, four showed improvement postoperatively. Two patients without syringomyelia showed improvement postoperatively. Of the four patients with syringomyelia, three showed improvement, including two with a decrease in the cavity size. One patient showed improvement in symptoms but the syringomyelia was unchanged. The cavity size increased in the one patient who did not show improvement. Among the 10 patients who underwent duraplasty, improvements were noted in four of the five patients without syringomyelia and in all of the five with syringomyelia. There is a suggestion that patients with syringomyelia may have a higher likelihood of improvement after undergoing duraplasty.
A melhor opção de tratamento cirúrgico na malformação de Chiari é desconhecido, especialmente em paciente com siringomielia. Nós revisamos casos de 16 pacientes submetidos à craniectomia suboccipital em nossa instituição de 2005 à 2008. Dos 6 pacientes que não foram submetidos à duroplastia, quatro tiveram melhora pós operatória. Dois pacientes sem siringomielia tiveram melhora pós operatória. Dos quatro pacientes com siringomielia, três tiveram melhora, incluindo dois com diminuição do tamanho da cavidade. Um paciente teve melhora da sintomatologia mas a siringomielia não se modificou. Dez pacientes foram submetidos à duroplastia. Melhora foi observada em 4 de 5 pacientes sem siringomielia e 5 de 5 casos com siringomielia. Há uma sugestão de que pacientes com siringomielia possam ter uma maior taxa de melhora após serem submetidos à duroplastia.