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Split Cord Malformation—Spectrum of Associated Anomalies

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1. To highlight the spectrum of associated pathological (AP) entities that can occur with (split cord malformations) SCMs, 2. the limitations of imaging techniques to diagnose these entities preoperatively, and… Click to show full abstract

1. To highlight the spectrum of associated pathological (AP) entities that can occur with (split cord malformations) SCMs, 2. the limitations of imaging techniques to diagnose these entities preoperatively, and 3. the failure of current theories of embryogenesis to explain these complex malformations. Patients with SCMs and associated pathologies (AP) operated between January 2012 and December 2017 were included. All had neurological evaluation, plain radiographs, CT, and MRI. Postoperative complications were recorded. Follow-up ranged from 6 mo to 3 yr. A total of 24 of the 30 patients treated during this period had APs. Age: 1 mo 17 yr. A total of 16 had Type II SCMs and 8 had Type I SCMs. A total of 20 of the 24 patients were neurologically intact. Of the remaining 4, 3 had urinary disturbances and 1 had weakness of 1 lower extremity. All patients had 1 or more APs in addition to SCMs excluding fatty filum. High resolution MRI and/or CT were unable to completely identify the full spectrum of the pathology in these patients in whom the diagnosis was often made intraoperatively.The associated anomalies included in the descending order of frequency included: LDMs (limited dorsal myeloschisis), lipomas; spondylocostal dysostosis, spinal arachnoid cyst, spinal dermal sinus, and neurenteric cyst. Fatty filum was present in 22 of the 24 patients and were sectioned. Intraoperative neurophysiological monitoring was used in the last 6 patients. Complications included cerebrospinal fluid (CSF) leak in 4 patients and wound break down in 2. Of the 4 with neurological deficits, 2 improved. No patient worsened neurologically during the follow-up. In total 80% of SCMs have APs. Even high resolution imaging cannot completely identify the full spectrum of the pathology in SCMs A high index of suspicion and meticulous search for AP is required while operating patients with SCMs

Keywords: associated anomalies; pathology; spectrum associated; scms; spectrum; split cord

Journal Title: Neurosurgery
Year Published: 2019

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