REDEFINING THE GENITAL ABNORMALITY IN THE ROBINOW-SYNDROME

Purpose: We defined the anatomical structure of the male genital abnor mality in the Robinow syndrome. Features of this syndrome include meso melic brachymelia of the arms, bifid terminal phalanges of the hands a nd feet, characteristic facies, skeletal anomalies and hypoplastic ext ernal genitalia. Materials and Methods: Penile anatomy of 3 patients w ith the Robinow syndrome was assessed using computerized tomography an d magnetic resonance imaging. Results were compared to those of 4 cont rols who underwent imaging for pelvic malignancies. Results: Cross-sec tional imaging showed that normal penile crura were inserted onto the anteromedial aspect of the pubic bone. In contrast, in the Robinow syn drome they were inserted inferiorly and posteriorly onto the medial as pect of the ischial tuberosity. In addition, the crura in the Robinow syndrome extended posterior to a line intersecting both femoral shafts . Compared to controls, there was a significant gap between the symphy sis pubis and dorsal aspect of the penis. Conclusions: The penile anom aly in the Robinow syndrome is due to abnormal insertion of the penile crura, resulting in a penis that appears shorter and more inferiorly placed between the legs.