The Neuro-Orthopedic Manifestations of the Omphalocele Exstrophy Imperforate Anus Spinal Defects (OEIS) Complex

Abstract

Purpose OEIS complex is a severe multi-system congenital defect. As the pediatric urologist assumes a primary role in the comprehensive management of these patients, it is important to appreciate the impact of neurologic and orthopedic manifestations on the overall health of the child. Material and Methods We retrospectively reviewed the medical records of 75 children with OEIS treated at our institution. We identified neurologic and orthopedic anomalies and correlated them with the ambulatory ability and urologic status of the child. Results No neurologic data was available in 5 patients. Of the remaining 70 patients, 8 had no spinal anomalies, 61 had spina bifida (SB), and one had coccygeal hypoplasia. Thirty-eight of the SB patients were additionally classified: 5 as SB occulta, 13 as meningocele/lipomeningocele, 16 as myelomeningocele/lipomyelomeningocele, and 4 as sacral agenesis. Half of the patients with SB (30) had a tethered cord. The most common identified orthopedic anomalies requiring follow-up or surgery included vertebral malformation (62), scoliosis (25), club foot (6), and limb length discrepancy (8). The ambulatory status for 56 patients over the age of two revealed 40 to be fully ambulatory, 7 to ambulate with assistive devices, 2 to ambulate minimally with assistive devices, and 7 to be wheel-chair bound. Continence data was available in 63 closed cases. Twenty-three were incontinent, including 3 with conduit diversions. Forty patients were socially continent, with 35 catheterizing via continent abdominal stomas and 5 voiding/cathing through the urethra. Conclusions Early evaluation of neurosurgical and orthopedic anomalies is vital in caring for children with OEIS. Despite a high incidence of spinal pathology, most patients are able to ambulate without assistance. Very few children with OEIS achieve continence per urethra with volitional voiding. Vigilant follow up of spinal pathology in these children will help promptly identify correctable problems such as cord tethering which can significantly impact quality of life.