Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Oct 14;12(10):e10949.
doi: 10.7759/cureus.10949.

Tethered Cord Syndrome After Myelomeningocele Repair: A Literature Update

Leopoldo Mandic Ferreira Furtado  1 , José Aloysio Da Costa Val Filho  1 , François Dantas  1 , Camila Moura de Sousa  1
Affiliations
Review

Tethered Cord Syndrome After Myelomeningocele Repair: A Literature Update

Leopoldo Mandic Ferreira Furtado et al. Cureus. .

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.

Keywords: fetal surgery; myelomeningocele; secondary tethered cord syndrome; tethered cord syndrome.

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Anatomic landmarks of myelomeningocele
Two different anatomic presentations of MMC are depicted: in a plane fashion (left) or a saccular fashion (right). In both presentations, the placode is perceived as a non-neurulated spinal cord, seen in the red ellipsoid structure (delimitated by elliptical dot-points). The zona epitheliosa is the abnormal and poor vascular tissue surrounding the placode (double-headed black arrows), and the junctional zone (*) is the delimitation point of the normal skin and the zona epitheliosa MMC: myelomeningocele
Figure 2
Figure 2. Normopositioning of conus medullaris and standard radiological findings of secondary tethered cord syndrome
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) MMC: myelomeningocele
Figure 3
Figure 3. Features of several radiological tethering levels in children presenting with tethered cord syndrome after MMC repair
Sagittal T2-weighted spinal cord MR image displaying severe syringomyelia with a large adherence of placode, classified as grade II (A). Patients in B and C presented as grade III. Low conus position was associated with a dermoid cyst in an MMC repaired postnatally (B) or exhibited two intramedullary epidermoid cysts in a child who underwent fetal repair of MMC (C). Lumbar kyphosis is associated with a low conus in grade IV (D) MMC: myelomeningocele
Figure 4
Figure 4. Microsurgical approach for tethered cord after MMC repair
The pictures depict two steps of untethering. At the beginning of the microsurgical approach, the placode (area limited by an ellipse) is exposed and thick scar tissue (double black arrows) is seen dorsally (left). After circumferential untethering is performed, the placodium (ellipse) is released on both sides and caudally (*). The upper lumbar right nerve is seen (single black arrow) MMC: myelomeningocele; L: left side of the patient; R: right side of the patient; Cr: cranial; CD: caudal
See this image and copyright information in PMC

References

    1. Comparison of prenatal and postnatal management of patients with myelomeningocele. Cavalheiro S, da Costa MDS, Moron AF, Leonard J. Neurosurg Clin N Am. 2017;28:439–448. - PubMed
    1. Congress of Neurological Surgeons systematic review and evidence-based guideline on the incidence of tethered cord syndrome in infants with myelomeningocele with prenatal versus postnatal repair. Mazzola CA, Tyagi R, Assassi N, et al. Neurosurgery. 2019;85:0. - PubMed
    1. Myelomeningocele: surgical trends and predictors of outcome in the United States, 1988-2010. Kshettry VR, Kelly ML, Rosenbaum BP, Seicean A, Hwang L, Weil RJ. J Neurosurg Pediatr. 2014;13:666–678. - PubMed
    1. Tethered cord syndrome. Agarwalla PK, Dunn IF, Scott RM, Smith ER. Neurosurg Clin N Am. 2007;18:531–547. - PubMed
    1. Operative nuances of myelomeningocele closure. Perry VL, Albright AL, Adelson PD. Neurosurgery. 2002;51:719–723. - PubMed

LinkOut - more resources