Arterial tortuosity syndrome

We describe a patient with arterial tortuosity syndrome (ATS), a rare disor der comprising generalized tortuosity and elongation of all major arteries, soft skin, joint laxity, severe keratoconus, and diffuse tortuosity of the carotids and of intracranial arteries. The patient's probably affected bro ther and sister died at an early age. Cytochemical studies excluded Ehlers- Danlos type IV and type VII syndromes. We review 11 previously described pa tients. (C) 2000 Wiley-Liss, Inc.