RESUMO
INTRODUCTION: Patients with multisutural or single craniosynostosis, often suffer from Chiari malformation and syringomyelia. The surgical management of syringomyelia in these patients is controversial. CASE REPORT: A 3-year-old girl was referred with complex craniosynostosis that had not been corrected surgically. She was asymptomatic despite the cranial MRI showed a Chiari malformation and one year later she developed a cervico-dorso-lumbar syringomyelia. She underwent a decompressive suboccipital craniectomy but subsequently suffered a worsening of syringomyelia. The intracranial pressure monitoring was pathological so it was decided to perform a decompressive bilateral fronto-parieto-temporal craniotomy and remodeling of the cranial vault, achieving a significant reduction of syringomyelia. CONCLUSIONS: After reviewing the literature, it is noted that there is currently no consensus on the treatment of syringomyelia in patients with craniosynostosis and Chiari malformation. Some authors recommend the simultaneous surgical suboccipital and cranial vault decompression, others only decompression of the cranial vault and other enlargement of the posterior fossa with distractors. In cases where the suboccipital decompression was performed first, the syringomyelia was not improved or stabilized. We conclude that the most effective treatment for patients with syringomyelia and craniosynostosis is decompressive remodeling of the cranial vault, as the main cause of syringomyelia is the raised intracranial pressure and lack of skull compliance.
TITLE: Tratamiento de la siringomielia en pacientes con malformacion de Chiari y craneosinostosis. Caso clinico y revision de la bibliografia.Introduccion. Los pacientes con craneosinostosis complejas o unisuturales presentan frecuentemente malformacion de Chiari y siringomielia. El tratamiento quirurgico de la siringomielia en estos pacientes es controvertido. Caso clinico. Niña de 3 años con craneosinostosis compleja no corregida quirurgicamente. Permanecio asintomatica a pesar de que en la resonancia magnetica craneal se evidencio una malformacion de Chiari y un año despues desarrollo una siringomielia cervicodorsolumbar. Se le realizo una craniectomia suboccipital descompresiva, pero posteriormente sufrio un empeoramiento de la siringomielia. El registro de presion intracraneal resulto patologico, por lo que se decidio realizar una craneotomia descompresiva frontoparietotemporal bilateral y remodelacion de la boveda craneal, con lo que se consiguio una disminucion significativa de la siringomielia. Conclusiones. Tras la revision de la bibliografia, se observa que actualmente no existe un consenso sobre el tratamiento de la siringomielia en los pacientes con craneosinostosis y malformacion de Chiari. Algunos autores recomiendan la simultanea descompresion quirurgica suboccipital y de la boveda craneal, otros solo la descompresion de la boveda craneal, y otros la ampliacion de la fosa posterior con distractores. En los casos en los que se realizo primero la descompresion suboccipital no se consiguio resolver ni estabilizar la siringomielia. Concluimos que el tratamiento mas eficaz para los pacientes con siringomielia y craneosinostosis es la remodelacion descompresiva de la boveda craneal, ya que el principal factor causante de la siringomielia es la hipertension intracraneal y la falta de distensibilidad del craneo.