Tethered cord syndrome in children with anorectal malformations

Object. Anorectal malformations are known to be associated with neurologica l deficits, which may contribute to the disability suffered by patients wit h these malformations. This study was undertaken to determine the incidence and pattern of sacral abnormalities in children with anorectal malformatio ns, the incidence and nature of the neurological deficits, and the incidenc e and nature of operable intraspinal abnormalities: in patients with this c ondition. Methods. Neurological evaluation was performed in 81 children with anorecta l malformations. Plain x-ray films were obtained to identify the presence o f sacral abnormalities. The patients with neurological deficits were evalua ted for the presence of operable intraspinal anomalies, and when such anoma lies were identified, correction of the same was undertaken. In 21% of thes e children radiographic evidence of sacral abnormalities was shown. Fifteen percent of patients harbored neurological deficits, and 10% harbored opera ble intraspinal anomalies. In addition, one patient had split notochord syn drome. Patients with operable intraspinal anomalies underwent surgical corr ection, with resultant neurological improvement. Conclusions. Bone abnormalities of the sacrum, neurological deficits, and o perable intraspinal lesions are not uncommon in children with anorectal mal formations. Because the neurological deficits can contribute to the disabil ity suffered by these individuals, we recommend routine screening of patien ts with anorectal malformations and neurological deficits and/or sacral abn ormalities for the early identification and treatment of potentially correc table intraspinal lesions.