Low-lying spinal cord and tethered cord syndrome in children with anorectal malformations

Andrew Teck Kwee Teo1, Bin Kee Gan, Janice Su Zhen Tung

  • 1Department of Paediatric Neurosurgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore.

Insights

Low-lying spinal cord (LLC) is often associated with anorectal malformations (ARMs). Early investigation with ultrasound and MRI is recommended for children with ARMs to diagnose LLC and tethered cord syndrome (TCS).

Area of Science:

  • Pediatric Surgery
  • Neuroscience
  • Radiology

Background:

  • Anorectal malformations (ARMs) and low-lying spinal cord (LLC) share a common embryonic origin, frequently co-occurring in pediatric patients.
  • LLC can lead to tethered cord syndrome (TCS), a condition often necessitating surgical intervention.
  • Understanding the incidence and outcomes of LLC in ARM patients is crucial for effective management.

Purpose of the Study:

  • To determine the incidence of LLC in children diagnosed with ARM using ultrasonography (US) and magnetic resonance (MR) imaging.
  • To review the incidence of TCS in this cohort and evaluate surgical outcomes following detethering procedures.
  • To assess the diagnostic utility of US and MR imaging in identifying LLC in ARM patients.

Main Methods:

  • A retrospective study was conducted on pediatric patients who underwent surgery for ARM between 2002 and 2009.
  • Data collected included imaging findings (US and MR) and outcomes of surgical detethering for LLC and TCS.
  • Incidence rates of LLC and TCS were analyzed in relation to ARM type and imaging results.

Main Results:

  • Out of 101 ARM patients, 17 (16.8%) were diagnosed with LLC, predominantly in high ARM cases (12/17).
  • Abnormal findings on US and MR imaging were present in 12 of the 17 LLC patients; 5 had normal US but abnormal MR.
  • Seventeen patients underwent surgical detethering; 3 of 7 with TCS showed improvement, with no reported complications.

Conclusions:

  • LLC is associated with ARM, particularly high types, warranting investigation regardless of ARM classification.
  • Lumbar US serves as an effective first-line screening tool for LLC; MR imaging is recommended for equivocal US findings or new symptoms.
  • Surgical detethering can improve outcomes for TCS, and prophylactic detethering in asymptomatic lipoma of the filum terminale carries low surgical risk.
Abstract

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