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Syringomyelias in paediatrics: a retrospective study of 25 cases

I. Martínez-Albaladejo, M. Procházková, E. Sebastián-Pérez, B. Bernardino-Cuesta, C. Martínez-Ferrández, O.M. Suárez-Traba, M. Budke, J.J. García-Peñas [REV NEUROL 2016;63:193-200] PMID: 27569564 DOI: https://doi.org/10.33588/rn.6305.2016162

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Volumen 63 | Number 05 | Nº of views of the article 8.556 | Nº of PDF downloads 597 | Article publication date 01/09/2016

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ABSTRACT

INTRODUCTION Syringomyelia is defined as a cavity containing cerebrospinal fluid inside the spinal cord.

AIM To describe the clinical characteristics of a series of patients with syringomyelia, as well as its diagnosis and treatment.

PATIENTS AND METHODS We conducted a retrospective descriptive study by reviewing the medical records at our centre.

RESULTS We reviewed 25 patients diagnosed with syringomyelia. In five cases, the diagnosis was reached casually, and eight of them presented a previous severe pathology (tumour, bone or vascular). Two patients began with hydrocephalus and clinical signs and symptoms of intracranial hypertension and just two of them reported headaches as the only symptom. Four presented progressive scoliosis, two of them as the initial complaint, and required surgery with arthrodesis and the use of a corset, respectively. A notable feature was the earliness of the diagnosis. Most of them only presented a slight loss of strength, with normal somatosensory potentials and electromyogram. Check-ups were carried out with magnetic resonance. Eight patients required a decompressive craniectomy with posterior C1-C2 laminectomy, with drainage of the syringomyelic cavity in four cases. Nine of them required a bypass valve and a ventriculostomy also had to be performed in two of them.

CONCLUSIONS The presence of syringomyelia is rare in paediatric patients, and is generally associated with malformations in the posterior fossa and a medical history of spinal dysrhaphism. Progressive scoliosis stands out as a possible isolated manifestation. A multidisciplinary approach with regular radiological check-ups and evaluation by paediatric neurology and neurosurgery services are mandatory for its follow-up. KeywordsArnold-Chiari Dysrhaphism Myelomeningocele Scoliosis Syringomyelia Syringomyelic cavity CategoriesNeuropediatría Técnicas exploratorias

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Original

Syringomyelias in paediatrics: a retrospective study of 25 cases