[Hydrocephalus due to hyperplasia of the choroid plexuses in a patient with trisomy 9 mosaicism. A real diagnostic and therapeutic challenge]. / Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomia 9. Un verdadero reto diagnostico y terapeutico.

INTRODUCTION: Trisomy 9 is an unusual chromosome abnormality in live-born patients, which is frequently accompanied by functional and structural anomalies of the central nervous system. Among many other alterations, several studies have been published in the English-speaking literature that show an association between chromosome 9 abnormality and pathologies affecting the choroid plexuses. CASE REPORT: We report the case of a 4-month-old male with trisomy 9 mosaicism associated to hydrocephalus secondary to choroid plexus hyperplasia, who was referred due to a clinical picture of intracranial hypertension. The cerebrospinal fluid (CSF) drainage procedure that was initially chosen caused massive ascites due to an excessive production of CSF, and led to a cascade of multiple surgical interventions, which included endoscopic and drainage procedures. CONCLUSIONS: This is another example of an association between choroid plexus pathologies and chromosome 9 abnormality. Due to its scarce incidence, diagnosis of hydrocephalus secondary to plexus hyperplasia is difficult, as is selecting its most suitable treatment. In this type of hydrocephalus there is a double pathophysiological mechanism, which involves an increase in CSF production and a decrease in its reabsorption. Despite taking these considerations into account, the treatment of hydrocephalus secondary to plexus hyperplasia is a real challenge that usually leads to multiple surgical interventions ranging from plexectomy or coagulation of the choroid plexuses to the implantation of CSF drainage devices.

TITLE: Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomia 9. Un verdadero reto diagnostico y terapeutico.Introduccion. La trisomia 9 es una cromosomopatia inusual en pacientes nacidos vivos, que frecuentemente se acompaña de anomalias funcionales y estructurales del sistema nervioso central. Entre otras muchas alteraciones, varios trabajos en la bibliografia anglosajona demuestran una asociacion entre cromosomopatia 9 y patologia de los plexos coroideos. Caso clinico. Varon de 4 meses de vida con mosaicismo de trisomia 9 asociado a hidrocefalia secundaria a hiperplasia de los plexos coroideos, que fue remitido por clinica de hipertension intracraneal. El procedimiento derivativo de liquido cefalorraquideo por el que optamos inicialmente provoco una ascitis masiva debida a la produccion desmesurada de liquido cefalorraquideo, y desemboco en una cascada de multiples intervenciones quirurgicas, entre las que se incluyeron procedimientos endoscopicos y derivativos. Conclusiones. Se trata de un ejemplo mas de asociacion entre patologia de los plexos coroideos y cromosomopatia 9. Debido a su escasa incidencia, es dificil establecer el diagnostico de hidrocefalia secundaria a hiperplasia de los plexos y, por tanto, el tratamiento mas adecuado. En este tipo de hidrocefalia existe un doble mecanismo fisiopatologico, que implica un aumento de produccion de liquido cefalorraquideo y una disminucion de su reabsorcion. A pesar de tener en cuenta dicha consideracion, el tratamiento de la hidrocefalia secundaria a hiperplasia de los plexos supone un verdadero reto que habitualmente pasa por multiples procedimientos quirurgicos, desde la plexectomia o coagulacion de los plexos coroideos hasta la implantacion de dispositivos de derivacion de liquido cefalorraquideo.

[The effectiveness of gravitational valves in the prevention of over-drainage in adult patients with hydrocephalus]. / Efectividad de las válvulas gravitacionales en la prevención del hiperdrenaje en pacientes adultos con hidrocefalia.

AIM: To evaluate the effect of gravitational valves on over-drainage in hydrocephalus in adults. PATIENTS AND METHODS: We performed a retrospective study of the shunt systems placed in patients over the age of 18 years between 1998 and 2006. Patients were divided into two groups: non-GV group (without gravitational valve) and GV group (with a gravitational valve, Aesculap-Miethke 5/35). The complications that occurred during the first year following the placement of the shunt system were recorded. RESULTS: Of a total of 137 patients, 91 were from the non-GV group and 46 belonged to the GV group. Mean age: non-GV group, 62.1 years; and GV group, 64.2 years, without any significant differences. In 80 patients the aetiology was chronic adult hydrocephalus, 19 were due to expansive processes, 15 due to vascular causes, eight pseudo tumours, six post-traumatic injuries and nine were due to other causes. In the non-GV group, 9.89% presented over-drainage, whereas there were no cases in the GV group; the difference was statistically significant (p = 0.029). In the rest of the complications there were no significant differences between the two groups. The total complications in the non-GV group were 25.27% and in the GV group, 6.52%, and there were significant differences (p = 0.01), although, above all, at the expense of over-drainage, because if this complication was excluded, then the differences were no longer significant (p = 0.175). CONCLUSIONS: In our series, the use of gravitational valves in the prevention of over-drainage in adult hydrocephalus proved to be more effective than employing valves without the gravitational device.

Efectividad de las válvulas gravitacionales en la prevención del hiperdrenaje en pacientes adultos con hidrocefalia / The effectiveness of gravitational valves in the prevention of over drainage in adult patients with hydrocephalus

Resumen. Objetivo. Evaluar el efecto de las válvulas gravitacionales sobre el hiperdrenaje en la hidrocefalia en el adulto. Pacientes y métodos. Estudio retrospectivo de los sistemas de derivación colocados en pacientes mayores de 18 años entre 1998 y 2006. Se dividió a los pacientes en dos grupos: grupo no VG (sin válvula gravitacional) y grupo VG (con válvula gravitacional, Aesculap-Miethke 5/35). Se contabilizaron las complicaciones producidas dentro del primer año tras la colocación del sistema de derivación. Resultados. Sobre un total de 137 pacientes, 91 fueron del grupo no VG y 46 del grupo VG. Edad media: grupo no VG, 62,1 años, y grupo VG, 64,2 años, sin que las diferencias fueran significativas. La etiología en 80 pacientes fue hidrocefalia crónica del adulto, 19 por procesos expansivos, 15 por causas vasculares, ocho pseudotumores, seis postraumáticos y nueve por otras causas. En el grupo no VG, un 9,89% presentó hiperdrenaje, mientras que en el grupo VG no hubo ningún caso; la diferencia fue estadísticamente significativa (p = 0,029). En el resto de las complicaciones no se obtuvieron diferencias significativas entre ambos grupos. Las complicaciones totales en el grupo no VG fueron del 25,27% y en el grupo VG del 6,52%, y hubo diferencias significativas (p = 0,01), aunque, sobre todo, a expensas del hiperdrenaje, ya que si se excluía esta complicación las diferencias no eran significativas (p = 0,175). Conclusión. En nuestra serie queda probada la eficacia de las válvulas gravitacionales en la prevención del hiperdrenaje en la hidrocefalia en el adulto comparado con las válvulas sin dispositivo gravitacional (AU)

Summary. Aim. To evaluate the effect of gravitational valves on over-drainage in hydrocephalus in adults. Patients and methods. We performed a retrospective study of the shunt systems placed in patients over the age of 18 years between 1998 and 2006. Patients were divided into two groups: non-GV group (without gravitational valve) and GV group (with a gravitational valve, Aesculap-Miethke 5/35). The complications that occurred during the first year following the placement of the shunt system were recorded. Results. Of a total of 137 patients, 91 were from the non-GV group and 46 belonged to the GV group. Mean age: non- GV group, 62.1 years; and GV group, 64.2 years, without any significant differences. In 80 patients the aetiology was chronic adult hydrocephalus, 19 were due to expansive processes, 15 due to vascular causes, eight pseudo tumours, six post-traumatic injuries and nine were due to other causes. In the non-GV group, 9.89% presented over-drainage, whereas there were no cases in the GV group; the difference was statistically significant (p = 0.029). In the rest of the complications there were no significant differences between the two groups. The total complications in the non-GV group were 25.27% and in the GV group, 6.52%, and there were significant differences (p = 0.01), although, above all, at the expense of over-drainage, because if this complication was excluded, then the differences were no longer significant (p = 0.175). Conclusions. In our series, the use of gravitational valves in the prevention of over-drainage in adult hydrocephalus proved to be more effective than employing valves without the gravitational device (AU)

Neumoencéfalo a tensión tras un registro de presión intracraneal / Tension pneumocephalus following intracranial pressure recording

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