[Intracranial arachnoid cysts. A study of a series of 35 cases]. / Quistes aracnoideos intracraneales. Estudio de una serie de 35 casos.

INTRODUCTION: Arachnoid cysts are cavities with a content similar to cerebrospinal fluid, frequently communicating with the subarachnoid space. They make up 1% of the intracranial space occupying lesions, and although typically seen in children (13%), they may be undiagnosed until the patient has become adult. PATIENTS AND METHODS: We review a series of 35 cases of congenital intracranial arachnoid cysts in children, which had been investigated in the Neuropaediatric Department during 1987 1999. The patients had had cranial CAT and MR, and transcranial echography studies. RESULTS: In 85.7% there was a single cyst. The Sylvan fissure and posterior fossa were the commonest sites. The age of diagnosis was under 1 year in 54.3% and at birth in 25.7%. There were other associated malformations of the central nervous system in 31.4%, with agenesis of the corpus callosum in 45.5%. The most frequent clinical features on presentation of the condition were macrocephaly (31.4%) and epileptic crises (25.7%). The surgical treatment most often used was cystoperitoneal shunt. CONCLUSIONS: Congenital arachnoid cysts are caused by alteration in the embryogenesis of the central nervous system, and hence their association with other malformations and prevalence in children. The most frequent clinical findings were macrocephaly, due to the size of the cyst or the associated hydrocephaly, and epileptic seizures secondary to cortical irritation caused by pressure. Management of symptomatic lesions is surgical. The cystoperitoneal shunt was generally used in our series, since this was followed by clinical improvement in most cases.

Quistes aracnoideos intracraneales.Estudio de una serie de 35 casos / Intracranial arachnoid cysts. A study of a series of 35 cases

Introducción. Los quistes aracnoideos son cavidades con un contenido similar al LCR, frecuentemente comunicados con el espacio subaracnoideo. Representan el 1 por ciento de las lesiones ocupantes de espacio intracraneales y, aunque son característicos de la edad pediátrica, pueden no diagnosticarse hasta la edad adulta. Pacientes y métodos. Se revisa una serie de 35 casos de quistes aracnoideos intracraneales congénitos en la edad pediátrica durante los años 1987-1999 estudiados en la sección de Neuropediatría del Hospital 12 de Octubre (Madrid, España). Los pacientes se han estudiado con TAC y RM craneal y ecografía transcraneal. Resultados. La presencia de un único quiste sucedió en un 85,7 por ciento: la cisura de Silvio y la fosa posterior fueron las localizaciones más frecuentes. La edad de diagnóstico ocurrió durante el primer año en un 54,3 por ciento y al nacer en un 25,7 por ciento. Se asociaron a otras malformaciones del SNC en un 31,4 por ciento, y destaca la agenesia del cuerpo calloso en un 45,5 por ciento. La clínica de presentación más frecuente fue la macrocefalia (31,4 por ciento) y las crisis epilépticas (25,7 por ciento). El tratamiento quirúrgico más empleado fue la derivación cistoperitoneal. Conclusiones. El quiste aracnoideo congénito ocurre por una alteración en la embriogénesis del SNC, de ahí su asociación con otras malformaciones y la prevalencia en la edad pediátrica. La clínica más frecuente fue la macrocefalia, por el tamaño del quiste o por hidrocefalia asociada, y las crisis epilépticas secundarias a irritación cortical por presión. El manejo de las lesiones sintomáticas es quirúrgico; la derivación cistoperitoneal es la más empleada en nuestra serie, y con ella se obtuvo una mejoría clínica en la mayoría de los casos (AU)

[Intradural spinal arachnoid cyst associated with Noonan's syndrome]. / Quiste aracnoideo espinal intradural relacionado con síndrome de Noonan.

INTRODUCTION: Intradural spinal arachnoid cysts are collections of liquid similar to CSF, caused by a disorder of the arachnoid trabeculae. Noonan s syndrome is a condition involving multiple malformations, with autosomal dominant inheritance, variable penetration and expression and a phenotype similar to that of Turner s syndrome. We describe the case of a girl with Noonan s syndrome who presented with progressive scoliosis and signs and symptoms of a disorder of the spine related to an intradural spinal arachnoid cyst. CLINICAL CASE: A four year old girl with no significant previous clinical history was seen for slow growth (in size and weight), progressive scoliosis and urinary sphincter dysfunction. On physical examination there were features of polymalformation compatible with Noonan s syndrome and thoracic scoliosis. In view of this a spinal MR study was done. This showed an intradural arachnoid cyst from segment T4 to T11, with displacement and spinal narrowing which required a cyst-peritoneal shunt. CONCLUSIONS: Intradural spinal arachnoid cysts are collections of liquid produced due to arachnoid trabeculae. Noonan s syndrome is a condition involving multiple malformations with a phenotype which is very similar to that of Turner s syndrome. It is linked to alterations of tissue elastin which favor the appearance of arachnoid cysts. Arachnoid cysts are an uncommon cause of spinal compression and/or progressive scoliosis. Although this association has not been described previously, in the case of a patient with Noonan s syndrome, with clinical features of a spinal disorder and/or progressive scoliosis, the presence of a spinal arachnoid cyst should be considered.