Background: A retained medullary cord (RMC) is a rare closed spinal dysraphism with a robust elongated neural structure continuous from the conus and extending to the dural cul-de-sac. Four cases of RMC extending down to the base of an associated subcutaneous meningocele at the sacral level have been reported.
Clinical Presentation: We report an additional case of RMC, in whom serial MRI examination revealed an enlargement of the meningocele associated with RMC over a 3-month period between 8 and 11 months of age, when he began to stand. At the age of 12 months, untethering of the cord was performed. Histologically, the presence of ependyma-lined central canals in the dense neuroglial cores was noted in all cord-like structures in the intradural and intrameningocele sacs and at the attachment to the meningocele.
Conclusion: It is conceivable that the hydrodynamic pressure with standing position and the check valve phenomenon were involved in meningocele enlargement. We should be mindful of these potential morphological changes.
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http://dx.doi.org/10.1007/s00381-018-3812-z | DOI Listing |
Afr J Paediatr Surg
October 2024
Department of Pediatric Surgery, AIIMS, Jammu, Jammu and Kashmir, India.
NMC Case Rep J
March 2024
Department of Neurosurgery, Saitama Medical University Hospital, Saitama, Japan.
We report a rare case of adult-onset sacral meningocele where compression triggered a specific headache. A 46-year-old woman presented with a headache, which worsened when she was in a sitting or supine position. A subcutaneous mass was observed on her left buttock, the compression of which also induced headache.
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December 2023
Ionis Pharmaceuticals, Inc., Carlsbad, CA, USA.
Lateral Meningocele Syndrome (LMS) is a monogenic disorder associated with NOTCH3 pathogenic variants that result in the stabilization of NOTCH3 and a gain-of-function. A mouse model (Notch3) harboring a 6691-TAATGA mutation in the Notch3 locus that results in a functional outcome analogous to LMS exhibits cancellous and cortical bone osteopenia. We tested Notch3 antisense oligonucleotides (ASOs) specific to the Notch3 mutation for their effects on Notch3 downregulation and on the osteopenia of Notch3 mice.
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