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We reviewed 26 patients with anorectal or urogenital malformations managed by the neurosurgical service at The Children’s Hospital for tethered spinal cord as diagnosed by spinal MRI. There were 13 patients with cloacal exstrophy, 5 with the Vater association, 3 with imperforate anus, and 5 with other complex anomalies. Tethering spinal cord lesions included myelocystocele, lipomyelomeningocele, and filum lipoma. We review the clinical course, management, correlation of MRI and intraoperative findings, and outcome of the patients, as well as the embryology which underlies the association of these malformations.

Lateral meningoceles were first described by Lehman et al. [(1977); J Pediatr 90: 49-54] in a patient with other skeletal findings and distinctive craniofacial features. Subsequently, six more patients with the so-called lateral meningocele syndrome (LMS) have been reported. We describe the findings in three new cases and expand the phenotype. The existence of an affected mother and daughter supports the hypothesis that LMS is a dominant disorder affecting primarily the connective tissue.

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