Tethered cord syndrome in children with anorectal malformations

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Object. Anorectal malformations are known to be associated with neurological deficits, which may contribute to the disability suffered by patients with these malformations. This study was undertaken to determine the incidence and pattern of sacral abnormalities in children with anorectal malformations, the incidence and nature of the neurological deficits, and the incidence and nature of operable intraspinal abnormalities in patients with this condition.

Methods. Neurological evaluation was performed in 81 children with anorectal malformations. Plain x-ray films were obtained to identify the presence of sacral abnormalities. The patients with neurological deficits were evaluated for the presence of operable intraspinal anomalies, and when such anomalies were identified, correction of the same was undertaken. In 21% of these children radiographic evidence of sacral abnormalities was shown. Fifteen percent of patients harbored neurological deficits, and 10% harbored operable intraspinal anomalies. In addition, one patient had split notochord syndrome. Patients with operable intraspinal anomalies underwent surgical correction, with resultant neurological improvement.

Conclusions. Bone abnormalities of the sacrum, neurological deficits, and operable intraspinal lesions are not uncommon in children with anorectal malformations. Because the neurological deficits can contribute to the disability suffered by these individuals, we recommend routine screening of patients with anorectal malformations and neurological deficits and/or sacral abnormalities for the early identification and treatment of potentially correctable intraspinal lesions.

Article Information

Address reprint requests to: Natarajan Muthukumar, M.Ch., Muruganagam, 138, Anna Nagar, Madurai 625 020, India. email: natmuthu@vsnl.com.

© AANS, except where prohibited by US copyright law.

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Figures

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    Photograph showing a child with anorectal malformation limb length discrepancy, and wasting of the muscles of the lower extremities on one side.

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    Left: Sagittal MR image obtained in a child with a high anorectal malformation and a tethered spinal cord extending to the S-4 level. Right: Sagittal MR image obtained in a child with a low anorectal malformation and tethered spinal cord extending to the sacral level and ending in a lipoma.

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    Plain x-ray film, anteroposterior view, obtained in a child with split notochord syndrome, demonstrating the typical inverted Y split of the spine.

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