Blog
About

7
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Commentary

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Syringomyelia refers to the presence of cavities within the spinal cord or dilatation of the central spinal cord canal. Although most cases are associated with a concomitant Chiari I malformation it can also be associated with cord tethering, intramedullary spinal lesions or traumatic injury.[1] Syringomyelia is occasionally an isolated or idiopathic finding. Many patients present with slowly progressive sensory symptoms (hypesthesia or dysthesia) primarily affecting the upper extremities. The classic presentation is numbness in a ‘cape-like distribution’ due to the disruption of decussating sensory fibres lying just anterior to the central canal.[2] Many patients, particularly children may present with non-sensory symptoms that can include muscle weakness and atrophy, scoliosis or brainstem dysfunction. The case reported in this issue[3] provides an excellent example of how clinicians must remain alert to syringomyelia as a diagnostic consideration. Distal weakness and atrophy has been documented in about one-third of children with syringomyelia.[2] Symptoms of lower extremity weakness may present abruptly and can mimic a compressive neuropathy.[4] Progressive hand weakness and atrophy due to syringomyelia has also been reported to clinically resemble an ulnar neuropathy.[5] Muscle weakness in such cases is likely the result of corticospinal tract disruption and/or anterior horn cells dysfunction. Chiari-associated syringomyelia has been linked with rapidly progressive flaccid paralysis[6] and brainstem dysfunction including apnea, dysphagia and vocal cord paralysis.[7 8] Scoliosis and back pain may be the presenting complaint in 20–40% of pediatric patients;[7 8] although, careful examination can identify motor or sensory deficits in these patients.[7] Neurosurgeons typically recommend patients with Chiari I associated syringomyelia to undergo suboccipital decompression with or without duroplasty as the initial treatment, reserving syrinx shunting for those cases in which the former modality of treatment fails. Spontaneous syrinx resolution is thought to be rare. The treatment of idiopathic syringomyelia represents a dilemma since syrinx shunting can carry the inherent risk of increasing neurological dysfunction. The majority of children with neurological symptoms attributable to syringomyelia will demonstrate complete symptom resolution within several months after successful posterior fossa decompression surgery.[9] Dysesthesia and motor symptoms (weakness) are more likely to show clinical improvement compared to scoliosis or hypesthesia.[9] Radiographic improvement is also commonly seen postoperatively although it tends to lag behind clinical recovery.[9] Even children with holocord syringomyelia on MR imaging of the spine and active denervation on electromyography may nevertheless demonstrate rapid and complete postoperative recovery.[4] Clinicians must therefore consider syringomyelia on their differential diagnosis of patients presenting not only with sensory loss and dysthesia but also those with motor weakness, progressive scoliosis and bulbar dysfunction.

          Related collections

          Most cited references 10

          • Record: found
          • Abstract: found
          • Article: not found

          Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years.

          The entity of hindbrain herniation without myelodysplasia in the very young child has been poorly described. A retrospective analysis of children diagnosed with Chiari I malformation (CM I) before their sixth birthday is presented. Since 1985, 31 children with CM I (0.3-5.8) years of age have been diagnosed at University of Iowa Hospitals and Clinics. Their records were reviewed for presenting symptoms, signs, radiographic findings, treatment, complications, and outcome. The average age at diagnosis was 3.3 years. Sixteen patients were under age 3. Chief presenting complaints included impaired oropharyngeal function (35%), scoliosis (23%), headache or neck pain (23%), sensory disturbance (6%), weakness (3%), and other (10%). Sixty-nine percent of children under age 3 had abnormal oropharyngeal function. Three patients under age 3 (19%) had undergone fundoplication and/or gastrostomy before diagnosis of CM I. Common physical findings included abnormal tendon reflexes (68%), scoliosis (26%), abnormal gag reflex (13%), and normal examination (13%). Vocal cord dysfunction (26%, all under age 3) and syringohydromyelia (52%) were also seen. Twenty-five patients were treated surgically at our institution with posterior fossa decompression, duraplasty, and cerebellar tonsillar shrinkage. Three patients were lost to follow-up. Ninety-one percent of patients reported improved symptomatology at last follow-up (mean: 3.9 years). Three patients required reoperation for recurrence of symptoms. Syringomyelia improved in all patients. Scoliosis resolved in 2 of 8 patients, improved in 5, and stabilized in 1. There was no permanent morbidity from surgery. We show that children with Chiari I abnormality are very likely to present with oropharyngeal dysfunction if under age 3, and either scoliosis or headache or neck pain worsened by valsalva if age 3 to 5. These symptoms are very likely to improve after Chiari decompression, which can be done with low morbidity. Very young children presenting with oropharyngeal dysfunction, pain worsened by valsalva, or scoliosis should prompt the clinician to consider CM I as a possible cause.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Outcome of Chiari-associated syringomyelia after hindbrain decompression in children: analysis of 49 consecutive cases.

            Chiari I malformation is complicated by syringomyelia in many cases. Hindbrain decompression remains first-line surgical treatment; however, the incidence, time course, and predictors of syrinx resolution remain unclear. We set out to determine predictors of syrinx improvement after hindbrain decompression for Chiari I- associated syringomyelia. Forty-nine consecutive pediatric patients undergoing posterior fossa decompression for Chiari I-associated syringomyelia were followed with serial magnetic resonance imaging evaluations postoperatively. Clinical, radiological, and operative variables were assessed as predictors of syrinx improvement as a function of time using Kaplan-Meier plots and log-rank analysis. Mean patient age was 11 +/- 5 years. Syringomyelia was symptomatic in 39 (80%) and asymptomatic in 10 (20%) cases. Twenty-one (54%) patients experienced symptom resolution (median, 4 mo postoperatively). Twenty-seven (55%) patients experienced radiographic improvement in syringomyelia (median, 14 mo postoperatively). After hindbrain decompression, motor symptoms were associated with a 2.35 increased hazard ratio for symptom improvement (P = 0.031) versus all other symptoms. Among patients with sensory deficits, dysesthesia was associated with a 3.12 increased hazard ratio for symptom improvement (P = 0.032) versus symptoms of paresthesia or anesthesia. In our experience, just more than one-half of patients with Chiari- associated syringomyelia demonstrated clinical and radiographic improvement after hindbrain decompression. Median time to radiographic improvement lagged behind clinical improvement by 10 months. Motor symptoms were more likely to improve with hindbrain decompression. Paresthesia or anesthesia symptoms were less likely to improve with hindbrain decompression. These findings may help guide surgical decision making and aid in patient education.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Chiari type I malformation in children.

              We reviewed the recent experience at Texas Children's Hospital by examining the records of 11 children who underwent suboccipital decompression for symptomatic Chiari type I malformation. Presenting complaints included neck pain (1 child), scoliosis (4 children), back pain (1 child), torticollis (1), motor dysfunction (1), and apnea (3 children). Neurologic findings were normal in 7 of the 11 children. The craniocervical junction and medulla were studied by magnetic resonance imaging, which revealed anatomy consistent with Chiari type I malformation in all cases. At surgery, all patients had tonsillar herniation to the first cervical vertebra or below. Three patients had syringomyelia. Postoperatively, either the patients were symptom free or, in the cases of scoliosis and torticollis, there was no progression. Our experience suggests that Chiari type I malformation may occur in childhood with varied and unusual clinical findings. Magnetic resonance imaging was essential to the diagnosis; the presence of tonsillar herniation was confirmed at surgery. The results of suboccipital decompression were favorable in this series.
                Bookmark

                Author and article information

                Journal
                J Neurosci Rural Pract
                JNRP
                Journal of Neurosciences in Rural Practice
                Medknow Publications (India )
                0976-3147
                0976-3155
                Jul-Dec 2011
                : 2
                : 2
                : 196-197
                JNRP-2-196
                3159365
                21897692
                Copyright: © Journal of Neurosciences in Rural Practice

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Commentary

                Neurosciences

                Comments

                Comment on this article