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Secondary Neurulation Defects: Retained Medullary Cord
Kyung Hyun Kim1,2, Ji Yeoun Lee1,3, Kyu-Chang Wang4,5
1Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Republic of Korea.
Abstract:
Retained medullary cord (RMC) is a defect resulting from impaired secondary neurulation. Intraoperatively, RMC is recognizable as an elongated cord-like structure caudal to the conus, that contains histologically confirmed neuroglial components and a lumen with an ependymal lining. It characteristically does not possess neurological function. This chapter aims to summarize (1) the mechanisms that lead to the occurrence of RMC; (2) the various forms of RMC, such as cystic RMC and 'possible RMC', and (3) the treatment strategies, especially untethering through limited exposure.
Insights
Retained medullary cord (RMC) is a neurodevelopmental defect. This review covers RMC
Area of Science:
- Neuroscience
- Developmental Biology
- Spinal Cord Anatomy
Background:
- Retained medullary cord (RMC) is a rare congenital anomaly.
- It arises from incomplete secondary neurulation during embryonic development.
- RMC is characterized by a distinct cord-like structure caudal to the conus medullaris.
Purpose of the Study:
- To elucidate the embryological mechanisms underlying RMC formation.
- To describe the diverse morphological presentations of RMC, including cystic variants.
- To outline current surgical strategies for RMC management, focusing on untethering.
Main Methods:
- Literature review of RMC pathogenesis and classification.
- Analysis of intraoperative findings and histological confirmation of RMC.
- Review of surgical techniques and outcomes for RMC treatment.
Main Results:
- RMC results from failed regression of the caudal neural tube.
- Histological analysis confirms neuroglial elements and ependymal lining.
- Surgical untethering via limited exposure is an effective treatment.
Conclusions:
- Understanding RMC mechanisms aids in diagnosis and classification.
- Varied RMC presentations necessitate tailored surgical approaches.
- Limited exposure untethering offers a viable treatment option for RMC.
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