Pinar Aydin Ozturk, Harun Emre Sen, Abdurrahman Arpa, Muharrem Karatas, Baris Aslanoglu, Kamuran Aydin, Sezer Onur Gunara, Volkan Etus
{"title":"Complex Type Split Cord Malformation: A Two-Center Study.","authors":"Pinar Aydin Ozturk, Harun Emre Sen, Abdurrahman Arpa, Muharrem Karatas, Baris Aslanoglu, Kamuran Aydin, Sezer Onur Gunara, Volkan Etus","doi":"10.1159/000551495","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Complex spinal dysraphism is the condition where multiple dysraphic pathologies are observed in the same patient. Complex type SCM is a compound dysraphic phenotype involving disruptions at multiple developmental stages, characterized by the co-occurrence of SCM and spina bifida aperta. This study aimed to understand the differences between complex SCMs from classical SCM cases in terms of embryological aspects, perioperative management, and follow-up.</p><p><strong>Results: </strong>The study included 44 patients who underwent surgery for complex SCM. Of the patients, 81.8% were operated on during the neonatal period in the same session with spina bifida aperta surgery. Surgery was planned for 11.4% of patients for follow-up purposes, and for 6.8% following detection of SCM on MRI performed due to gait disturbances. It was observed that 68.2% of patients were plegic/severely paretic, 22.7% had moderate paresis, and 9.1% had mild paresis/normally. Regarding the type of spina bifida aperta, 72.7% of patients had myelomeningocele, and 27.3% had myeloschisis. Type 1 SCM was detected in 79.5% of patients, Type 2 SCM in 13.6%, and Type 1.5 SCM in 6.8%. Additionally, 93.2% had tethered cord/thickened-fatty filum terminale, 90.9% had posterior fusion defects, 77.3% had hydrocephalus, 68.2% had kyphoscoliosis, 65.9% had Chiari malformation, 54.5% had syringomyelia, 45.5% had intracranial pathologies other than hydrocephalus, and 4.6% had lipoma.</p><p><strong>Conclusion: </strong>Complex SCMs should not be viewed solely as a spinal pathology; they should be evaluated multidisciplinarily in the perioperative period in terms of anomalies accompanying spina bifida aperta. In these cases are diagnosed at a younger age, the incidence of Type 1 SCM increases, while the frequency of occurrence in the lumbar region decreases and increases in the thoracic region. Furthermore, it has been determined that additional anomalies accompanying spina bifida aperta occur more frequently with complex SCMs.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"1-12"},"PeriodicalIF":1.3000,"publicationDate":"2026-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Neurosurgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1159/000551495","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction: Complex spinal dysraphism is the condition where multiple dysraphic pathologies are observed in the same patient. Complex type SCM is a compound dysraphic phenotype involving disruptions at multiple developmental stages, characterized by the co-occurrence of SCM and spina bifida aperta. This study aimed to understand the differences between complex SCMs from classical SCM cases in terms of embryological aspects, perioperative management, and follow-up.
Results: The study included 44 patients who underwent surgery for complex SCM. Of the patients, 81.8% were operated on during the neonatal period in the same session with spina bifida aperta surgery. Surgery was planned for 11.4% of patients for follow-up purposes, and for 6.8% following detection of SCM on MRI performed due to gait disturbances. It was observed that 68.2% of patients were plegic/severely paretic, 22.7% had moderate paresis, and 9.1% had mild paresis/normally. Regarding the type of spina bifida aperta, 72.7% of patients had myelomeningocele, and 27.3% had myeloschisis. Type 1 SCM was detected in 79.5% of patients, Type 2 SCM in 13.6%, and Type 1.5 SCM in 6.8%. Additionally, 93.2% had tethered cord/thickened-fatty filum terminale, 90.9% had posterior fusion defects, 77.3% had hydrocephalus, 68.2% had kyphoscoliosis, 65.9% had Chiari malformation, 54.5% had syringomyelia, 45.5% had intracranial pathologies other than hydrocephalus, and 4.6% had lipoma.
Conclusion: Complex SCMs should not be viewed solely as a spinal pathology; they should be evaluated multidisciplinarily in the perioperative period in terms of anomalies accompanying spina bifida aperta. In these cases are diagnosed at a younger age, the incidence of Type 1 SCM increases, while the frequency of occurrence in the lumbar region decreases and increases in the thoracic region. Furthermore, it has been determined that additional anomalies accompanying spina bifida aperta occur more frequently with complex SCMs.
期刊介绍：
Articles in ''Pediatric Neurosurgery'' strives to publish new information and observations in pediatric neurosurgery and the allied fields of neurology, neuroradiology and neuropathology as they relate to the etiology of neurologic diseases and the operative care of affected patients. In addition to experimental and clinical studies, the journal presents critical reviews which provide the reader with an update on selected topics as well as case histories and reports on advances in methodology and technique. This thought-provoking focus encourages dissemination of information from neurosurgeons and neuroscientists around the world that will be of interest to clinicians and researchers concerned with pediatric, congenital, and developmental diseases of the nervous system.
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