Abstract
Tethered cord syndrome (TCS) after myelomeningocele (MMC) repair (or secondary TCS) is a challenging condition characterized by neurological, orthopedic, and urological symptoms, which are combined with a low-lying position of the conus medullaris and damage to the stretched spinal cord owing to metabolic and vascular derangements. It has been reported that this syndrome affects, on average, 30% of children with MMC. In this review, we revisit the historical aspects of secondary TCS and highlight the most important concepts of diagnosis, treatment, and outcomes for secondary TCS as well as the current research regarding the impact of fetal MMC repair in the incidence and management of TCS. In the future, the development of synthetic models of TCS could shorten the learning curve of pediatric neurosurgeons, and research into the cellular proapoptotic features and increased inflammation biomarkers associated with TCS will also improve the treatment of this condition and minimize retethering of the spinal cord.
Highlights
Myelomeningocele (MMC) is an open neural tube defect that affects an average of three out of 10,000 live births, a statistic that is probably underestimated in developing countries; it is associated with high healthcare costs throughout a patient’s lifespan [1,2,3,4]
MMC is morphologically characterized by a placode, zona epitheliosa, and junctional zone and is most commonly localized in the lumbar region
Tethered cord syndrome (TCS) is a clinical syndrome and its recognition must be prompt and early in order to increase the chances of better results
Summary
Myelomeningocele (MMC) is an open neural tube defect that affects an average of three out of 10,000 live births, a statistic that is probably underestimated in developing countries; it is associated with high healthcare costs throughout a patient’s lifespan [1,2,3,4]. Normopositioning of the conus medullaris at the level of T12 on a sagittal T2-weighted spinal MR image (left) and low-lying position of the conus medullaris at S3 in a four-month-old child who underwent post-natal MMC repair and had no clinical symptoms of tethered cord syndrome (right). Caution must be exercised to avoid damage to the placode attached to the scar tissue, and electrophysiological intraoperative monitoring should be conducted to determine the patient’s level of functionality and to minimize additional neurological injuries during surgery [16] This procedure should release the placode in all directions, according to the grading system proposed by Kirollos and Van Hille, who considered three levels of untethering [43]. They attributed an important role to urology following urodynamic tests in the early diagnosis of bladder compromise to achieve better results for detethering
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