OCCIPITAL ENCEPHALOCELE AND MURCS ASSOCIATION - CASE-REPORT AND REVIEW OF CENTRAL-NERVOUS-SYSTEM ANOMALIES IN MURCS PATIENTS

The combination of MURCS association (Mullerian duct and renal agenesi s, upper limb and rib anomalies) and occipital encephalocele occurred in a stillborn girl of 41 weeks gestation, The malformations are compa tible with a defect in the organization of the paraxial mesoderm that gives rise to occipital, cervical, and thoracic somites and adjoining intermediate mesoderm. These structures contribute to the occipital bo ne, cervical spine, upper limbs, and urogenital system, Brain imaging may be useful in assessing MURCS patients, if cranial malformations pr ove to be clinically important in these individuals. (C) 1996 Wiley-Li ss, Inc.