The mermaid malformation: cloacal exstrophy and occult spinal dysraphism

Five infants with cloacal exstrophy underwent neurological evaluation and radiographic examination of the caudal spine shortly after birth. Each was found to have occult spinal dysraphism. Four had terminal myelocystoceles, and one had a lipomyelomeningocele. Pathological anatomy was confirmed during surgery for the release of the tethered spinal cords. The striking association between cloacal exstrophy and occult spinal dysraphism suggests a common developmental defect in the caudal pole of the embryo. A hypothesis is offered to explain this association. Terminal myelocystocele and lipomyelomeningocele appear to be part of a continuum of lesions associated with skin-covered spina bifida.