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Tethered cord associated with anorectal malformation
Kazuyoshi Morimoto1, Osamu Takemoto, Akatsuki Wakayama
1Department of Neurosurgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Osaka, Japan. fr8kmrmt@mch.pre.osaka.jp
Insights
This study highlights the association between tethered spinal cords and anorectal malformations in children. Early spinal cord imaging is crucial for diagnosing these often-hidden conditions and guiding neurosurgical intervention.
Area of Science:
- Pediatric Neurosurgery
- Congenital Malformations
- Spinal Cord Anomalies
Background:
- Tethered cord syndrome can present with various congenital anomalies.
- Anorectal malformations (ARMs) are sometimes associated with spinal cord abnormalities.
- The link between ARMs and tethered cords, especially without skin stigma, requires further investigation.
Purpose of the Study:
- To report on a cohort of children with co-occurring tethered cord and anorectal malformations.
- To emphasize the importance of neurosurgical evaluation in children with ARMs.
- To explore potential hypotheses for the observed association.
Main Methods:
- Retrospective review of 10 pediatric patients with tethered cord and ARM.
- Analysis of surgical data for spinal cord untethering.
- Radiographic examination of the caudal spine for tethered cord diagnosis.
Main Results:
- Ten children (6 boys, 4 girls; mean age 1.7 years) with tethered cord and ARM were identified.
- The ARMs included vesicointestinal fissures, cloacal exstrophies, and rectovesical/rectobulbar fistulas.
- Eight patients lacked typical lumbosacral skin stigmata, underscoring the need for imaging.
Conclusions:
- A significant association exists between anorectal malformations and tethered spinal cords.
- Neurosurgical correction of tethered cords may be beneficial for associated neurological deficits.
- Spinal cord imaging is essential for accurate diagnosis in this patient population.
Abstract:
Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.
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