Vascular abnormalities in Adams-Oliver syndrome: cause or effect?

  title={Vascular abnormalities in Adams-Oliver syndrome: cause or effect?},
  author={Ed Swartz and Shubhayan Sanatani and George Gabor Stephen Sandor and Richard A Schreiber},
  journal={American journal of medical genetics},
  volume={82 1},
We describe a young girl diagnosed with the Adams-Oliver syndrome (AOS) associated with double outlet right ventricle, portal hypertension, and pulmonary hypertension. We hypothesize that a congenital vascular abnormality is the underlying pathogenesis and that the cutaneous defects characteristically seen in AOS represent the most common manifestation of this. We suggest that AOS should not merely be considered a syndrome consisting of aplasia cutis congenita and terminal transverse limb… CONTINUE READING