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Vol. 13. Issue 6.
Pages 455-462 (January 2002)
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Vol. 13. Issue 6.
Pages 455-462 (January 2002)
Meningocele sacro anterior en pacientes con síndrome de Currarino: aportación de dos casos
Anterior sacral meningocele with Currarino's syndrome: Report of two cases
J. Dios Seoane
, S. Amaro, M.G. Fantini, A. Blanco, L. López, A. Rodiño, E. Rossi, A. Vázquez
Servicio de Neurocirugía. Complejo Hospitalario Juan Canalejo. La Coruña
J. Díaz*, V. Goyanes**, V. Dios García***
* Servicio de Neurorradiología. Complejo Hospitalario Juan Canalejo. La Coruña
** Servicio de Genética. Complejo Hospitalario Juan Canalejo. La Coruña
*** Servicio de Medicina Legal. Complejo Hospitalario Juan Canalejo. La Coruña
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Resumen

El meningocele sacro anterior es la hernia congénita del saco tecal en la pelvis a través de un defecto óseo en la pared anterior del sacro.

La asociación de anomalía anorectal, defecto sacro anterior y masa presacra, conocida como triada de Currarino, puede ser diagnosticada durante la primera década de la vida, en el ochenta por cien de los casos, sin embargo, en el del síndrome incompleto, los síntomas aparecen en el adulto.

Para evitar complicaciones tardías y debido a que esta rara patología tiene un claro origen genético, la investigación de la familia es de vital importancia en el diagnóstico precoz y tratamiento.

Revisamos la literatura y aportamos dos casos de meningocele sacro anterior con fístula rectal y síndrome incompleto de Currarino, uno de ellos complicado con meningitis y con esta anomalía en un miembro de su familia. Se discute la importancia del diagnóstico precoz y tratamiento de estas malformaciones y la necesidad de una investigación genética familiar.

Palabras clave:
Meningocele sacro anterior
Síndrome de Currarino
Summary

The anterior sacral meningocele is the congenital hernia of the thecal sac into the pelvis through a bone defect of the anterior wall of the sacrum.

The association of anorectal anomaly, anterior sacral defect and presacral mass, known as Currarino's triad, can be diagnosed during the first decade of Ufe in the eighty per cent of the cases However, in the incomplete syndrome, the symptoms appear in adults. In order to avoid late complications and due to the clear genetic origin of this uncommon pathology, the investigation of the family is of paramount importance for the early diagnosis and treatment.

We review the literature and add two cases of anterior sacral meningocele with rectal fístula and incomplete Currarino's syndrome. In one of the cases the pathology is olso present in another member of the same family and is complicated with meningitis.

The importance of early diagnosis and treatment of these malformations and the need for fammily genetic investigation are discussed.

Key words:
Anterior sacral meningocele
Currarino's Syndrome

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