Siringomielia: revisión a propósito de un caso / Syringomyelia: review, case and report

La siringomielia supone un desafío diagnóstico, ya que es una entidad poco reconocida si no se tiene conciencia de su existencia. Al ser un cuadro progresivo, cuya clínica puede presentarse de forma larvada y ser coincidente con otras patologías neurológicas tales como la esclerosis múltiple, su detección suele realizarse en etapas tardías sobre todo en población adulta y más aún cuando se presenta de forma adquirida. Por lo que el estudio imagenológico con Resonancia Magnética adquiere especial relevancia, permitiendo identificar y clasificar la enfermedad, lo que brindará la base para decidir terapía.

Syringomyelia is a diagnostic challenge, since it is a poorly recognized disease, especially if its existence remains unknown. Being a progressive disease, whose clinic can present in a latent way and be coincident with other neurological pathologies such as multiple sclerosis, its detection is usually conducted in late stages, especially in the adult population and even more when it presents in an acquired way. Therefore, the imaging study with Magnetic Resonance acquires special relevance, allowing to be identified and classified, which will provide the basis for deciding on therapy

Management opinions from different centers (Rio de Janeiro).

INTRODUCTION: The surgical treatment of Chiari type 1 (CM1) malformation is controversial and depends largely on the preference of the surgeon. The evolution of neuroimaging resulted in an increased number of asymptomatic patients incidentally diagnosed. PURPOSE: To study retrospectively a population of 24 symptomatic patients with CM1 operated between 1999 and 2017 in which intraoperative ultrasonography (IOUS)-assisted posterior fossa-C1 decompression was used to decide whether the dura mater should be opened (CVD+) or not (CVD). RESULTS: Most of the patients complained of headache or neck pain, 15 had hydrosyringomyelia and 14 had some spinal cord involvement. Patients were categorized in improved, unchanged, or worse according the preoperative signs and symptoms. Overall, 19 patients improved, 3 deteriorated, and 2 remained unchanged. Among these, 4 out 5 had syringohydromyelia. CONCLUSIONS: IOUS-assisted posterior fossa-C1 decompression is our preferred option to treat CM1. Children submitted to intradural procedures, initially or subsequently, had increased postoperative complications. CSF fistula or pseudomeningocele was the major cause of complication. The final result seems to correlate with the preoperative neurological status.

Craniocervical decompression with duraplasty and cerebellar tonsillectomy as treatment for Chiari malformation-I complicated with syringomyelia.

This study aimed to investigate the therapeutic effects of craniocervical decompression with duraplasty and cerebellar tonsillectomy for the treatment of Chiari malformation-I with syringomyelia (CM I-SM). From January 2005 to December 2011, 127 patients with CM I-SM underwent craniocervical decompression with duraplasty and cerebellar tonsillectomy and the therapeutic effects of these surgeries were evaluated using Tator scores. No patient in this study died or showed disease deterioration after the surgery. Re-examination by magnetic resonance imaging (MRI) showed that the cisterna magna was obviously larger after the operation in all but one patient. Moreover, syringomyelia (SM) was reduced in 76 patients. CM I-SM symptoms disappeared or decreased in 112 patients after following discharge. Follow-up was conducted in 84 of the patients and 79 of these patients exhibited improved symptoms. A second MRI re-examination showed that the cisterna magna was successfully constructed in 44 patients; 42 of these patients showed further eliminated or obviously reduced SM. Craniocervical decompression with duraplasty and cerebellar tonsillectomy achieved favorable therapeutic effects. Thus, craniocervical decompression with duraplasty and cerebellar tonsillectomy is a rational surgical approach with beneficial clinical effects. The proposed approach may have useful applications in the treatment of CM I-SM.

["Idiopathic" syringomyelia: report of a case]. / Siringomielia "idiopática"; a propósito de un caso.

Syringomyelia is the condition in which cavities fulfilled with CSF are found within spinal cord. Syringes are caused by obstructions of CSF pathways of different causes. If we can not find a cause responsible for the blockage, we call it "idiopathic" syringomyelia. Drainage procedures have been widely used but results, especially long-term results, are not favourable and complication rate is high. Some authors prefer to restore CSF circulation instead to drain the syrinx. Results of this treatment modality are better in literature than drainage procedures. Adequate radiological studies help to identify problem location and to plan the approach. A case of "idiopathic" syringomyelia is presented. Treatment consisted on arachnoid webs dissection and dural plastia. Clinical and radiological response was good. Literature concerned about this kind of syringomyelia and treatment modalities are reviewed.

[Incidental syringohydromyelography: report of a case]. / Siringohidromielografia incidental: registro de um caso.

The radiological findings of syringomyelography are described in a five years old patient who underwent a percutaneous injection of opaque contrast medium (Lipiodol) into the intraspinal syringomyelic cavity. The clinical picture, the usual diagnostic methods and the place of syringomyelography for the diagnosis of syringomielia are discussed.