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Secondary Neurulation Defects-1 : Retained Medullary Cord
Kyung Hyun Kim1, Ji Yeoun Lee1,2, Kyu-Chang Wang1
1Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Korea.
Abstract:
Retained medullary cord (RMC) is a relatively recent term. Pang et al. newly defined the RMC as a late arrest of secondary neurulation leaving a non-functional vestigial portion at the tip of the conus medullaris. RMC, which belongs to the category of closed spinal dysraphism, is a cord-like structure that is elongated from the conus toward the cul-de-sac. Because intraoperative electrophysiological confirmation of a non-functional conus is essential for the diagnosis of RMC, only a tentative or an assumptive diagnosis is possible before surgery or in cases of limited surgical exposure. We suggest the term 'possible RMC' for these cases. An RMC may cause tethered cord syndrome and thus requires surgery. This article reviews the literature to elucidate the pathoembryogenesis, clinical significance and treatment of RMCs.
Insights
Retained medullary cord (RMC) is a congenital condition resulting from arrested secondary neurulation. This review clarifies RMC
Area of Science:
- Neuroscience
- Developmental Biology
- Pediatric Surgery
Background:
- Retained medullary cord (RMC) is a recently defined entity within closed spinal dysraphism.
- It represents a late arrest of secondary neurulation, resulting in a non-functional cord-like structure at the conus medullaris tip.
Purpose of the Study:
- To review the existing literature on retained medullary cords.
- To elucidate the pathoembryogenesis, clinical significance, and treatment of RMCs.
Main Methods:
- Literature review of retained medullary cord (RMC) cases.
- Analysis of diagnostic challenges and proposed terminology ('possible RMC').
Main Results:
- RMC is characterized by a vestigial portion at the conus medullaris tip.
- Intraoperative electrophysiological confirmation is crucial for definitive diagnosis.
- Tentative diagnoses are often made preoperatively or with limited surgical exposure.
Conclusions:
- RMC is a form of closed spinal dysraphism with potential to cause tethered cord syndrome.
- The term 'possible RMC' is proposed for cases with tentative or assumptive diagnoses.
- Surgical intervention is indicated for RMCs causing tethered cord syndrome.
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