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.
Introduction:
Anorectal malformations (ARMs) and low-lying spinal cord (LLC) are commonly associated owing to their common embryonic origin. LLC may lead to tethered cord syndromes (TCS), requiring surgery. This study aimed to review the incidence of LLC in children with ARMs using ultrasonography (US) and magnetic resonance (MR) imaging, the incidence of TCS and the surgical outcomes of these patients after detethering.
Methods:
We conducted a retrospective study of children who underwent surgery for ARMs in 2002-2009 at KK Women's and Children's Hospital, Singapore.
Results:
Out of 101 (16.8%) ARM patients, 17 had LLC, of which 12 (70.6%) were high ARMs. 12 of the 17 (70.6%) patients had abnormal US and MR imaging findings. Five (29.4%) had normal US but abnormal MR imaging results; in these five patients, MR imaging was performed due to new symptoms and equivocal US findings. These 17 patients subsequently underwent surgical detethering. Three out of seven patients with TCS improved after surgery. None of the 17 patients had any complications.
Conclusion:
LLC appeared to be associated with high ARMs, although this was not statistically significant. LLC should be investigated for whenever ARM is diagnosed, regardless of its type. Lumbar US is useful for first-line screening for LLC. Abnormal US or onset of new symptoms should subsequently be investigated with MR imaging. Equivocal US findings are also likely to benefit from further MR imaging. Surgery to detether LLC can improve outcome in TCS, while prophylactic detethering for asymptomatic patients with lipoma of the filum terminale has very low surgical risk.
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