Retained medullary cord and terminal myelocystocele as a spectrum: case report

Childs Nerv Syst. 2022 Jun;38(6):1223-1228. doi: 10.1007/s00381-021-05351-0. Epub 2021 Sep 17.

Abstract

The caudal portion of the spinal cord, the medullary cord, is formed by secondary neurulation. One of the distinctive features of secondary neurulation compared to primary neurulation is that the medullary cord normally degenerates into a filum in humans. Various anomalies have been known to originate from degenerating process errors. One anomaly is terminal myelocystocele (TMCC), which is a closed spinal dysraphism with an elongated caudal spinal cord. The terminal part is filled with cerebrospinal fluid (CSF) and protrudes into the dorsal extradural space. Another anomaly is the retained medullary cord (RMC), which is a nonfunctioning cord-like structure extending to the cul-de-sac. In a 1-month-old boy, we identified an RMC with cystic dilatation of the caudal end extending to the epidural space at the very bottom of the cul-de-sac, resembling a degenerating terminal balloon, which is an essential feature of TMCC. Hence, this case may be considered an intermediate form between TMCC and RMC. This case provides clinical evidence that TMCC and RMC share the same pathoembryogenic origin, namely, failure of the regression phase of secondary neurulation.

Keywords: Retained medullary cord; Secondary neurulation; Terminal balloon; Terminal myelocystocele.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Infant
  • Male
  • Meningomyelocele* / complications
  • Meningomyelocele* / diagnostic imaging
  • Meningomyelocele* / surgery
  • Neurulation
  • Spina Bifida Occulta* / complications
  • Spinal Cord / surgery
  • Spinal Dysraphism* / surgery