Neuro-Orthopedic Manifestations of the Omphalocele Exstrophy Imperforate Anus Spinal Defects Complex
| Autor: | Kristina D. Suson, John P. Gearhart, Angela D. Gupta, Jane E. Benson, Paul D. Sponseller, Thomas E. Novak |
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| Rok vydání: | 2010 |
| Předmět: |
Adult
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Adolescent Urology Lipomeningocele Sacral Agenesis Anus Imperforate Young Adult Spina bifida occulta Humans Medicine Abnormalities Multiple Musculoskeletal Diseases Child Retrospective Studies Omphalocele business.industry Spina bifida Bladder Exstrophy Infant medicine.disease Hypoplasia nervous system diseases Surgery Spinal Cord Child Preschool Orthopedic surgery Nervous System Diseases business Hemivertebrae Hernia Umbilical |
| Zdroj: | Journal of Urology. 184:1651-1655 |
| ISSN: | 1527-3792 0022-5347 |
| Popis: | The omphalocele-exstrophy-imperforate anus-spinal defects complex is a severe multisystem congenital defect. To comprehensively care for these patients one must appreciate the neurological and orthopedic impact on the overall health of the child.We retrospectively reviewed the medical records of 73 children with omphalocele-exstrophy-imperforate anus-spinal defects who were treated at our institution, identifying neurological and orthopedic anomalies, ambulatory ability and voiding status.No neurological data were available on 5 patients. Of the remaining 68 patients 9 had no spinal anomaly, 57 had spina bifida, 1 had hemivertebrae and 1 had coccygeal hypoplasia. We further classified the 47 spina bifida cases as spina bifida occulta in 6, meningocele/lipomeningocele in 12, myelomeningocele/lipomyelomeningocele in 24 and sacral agenesis in 6. Of the patients with spina bifida 35 had cord tethering. Commonly identified orthopedic anomalies were vertebral malformation in 59 patients, scoliosis in 25, clubfoot in 14 and limb length discrepancy in 8. Ambulatory status in 62 patients of walking age revealed that 37 ambulated fully, 15 ambulated with devices, 2 ambulated minimally with devices and 8 were wheelchair bound. Continence data were available on 61 closed cases. Of these patients 26 were incontinent, including 3 with conduit diversion, 1 with ureterostomy and 1 with vesicostomy. A total of 35 patients were socially continent, of whom 30 catheterized via a continent abdominal stoma and 5 voided/catheterized via the urethra.Early evaluation for neurosurgical and orthopedic anomalies is vital in these children. Despite the high incidence of spinal pathology most patients ambulate without assistance. Few children with omphalocele-exstrophy-imperforate anus-spinal defects achieve continence via the urethra. Vigilant followup is necessary to identify potentially correctable conditions. |
| Databáze: | OpenAIRE |
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