Spinal anomalies in classic bladder exstrophy

Introduction: Spinal anomalies are known to be associated with cloacal exstrophy in about half of all cases. This study was performed to determine the frequency of this association and the clinical sequelae in children born with classic bladder exstrophy. Patients and methods: X-ray films and/or reports of the axial spine in patients with bladder exstrophy were retrieved and reviewed. Films were examined with a paediatric radiologist with a specific interest in determining any abnormalities of the bony spine. Clinical charts of those with spinal anomalies were reviewed to determine any clinical neurological disorders associated with the X-ray findings. Results: Of 423 children currently on the exstrophy database with classic bladder exstrophy, only 300 had X-ray films or reports available for adequate review. Of these, 21 (7%) were classified as normal variants with findings such as spina bifida occulta, exostoses and lumbarization or sacralization of vertebrae. Abnormalities of spinal curvature were identified in eight patients (2.7%). all with scoliosis. Spinal dysraphism was diagnosed in 11 patients (3.7%), and included myelomeningocele (one), lipomeningocele (one), posterior lamina! defects in two or more vertebrae (six), vertebral fusion (two), hemivertebrae (two) and a scythe-shaped sacrum (one). Of the 11 patients with spinal dysraphism, only the one with myelomeningocele had a clinical neurological deficit, for an overall incidence in the classic exstrophy group of 0.3%. Conclusions: Spinal anomalies occur in children born with classic bladder exstrophy at a rate of about 13.4%, consisting of 7% normal variants, 2.7% scoliosis and 3.7% spinal dysraphism. The incidence of this association is significantly less than that for cloacal exstrophy (∼50%). Even in the case of spinal dysraphism, neurological dysfunction is rare, occurring in < 1% of patients in this series.