Neuro-orthopedic manifestations of the omphalocele exstrophy imperforate anus spinal defects complex.
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Suson
Kristina D
KD
Division of Urology, University of Maryland Medical Center, USA.
Novak
Thomas E
TE
Gupta
Angela D
AD
Benson
Jane
J
Sponseller
Paul
P
Gearhart
John P
JP
D000015:Abnormalities, Multiple; D000293:Adolescent; D000328:Adult; D001006:Anus, Imperforate / Q000150:complications*; D001746:Bladder Exstrophy / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D006554:Hernia, Umbilical / Q000150:complications*; D006801:Humans; D007223:Infant; D009140:Musculoskeletal Diseases / Q000209:etiology*; D009422:Nervous System Diseases / Q000209:etiology*; D012189:Retrospective Studies; D013116:Spinal Cord / Q000002:abnormalities*; D055815:Young Adult