Severe arterial occlusive disorder and brachysyndactyly in a boy: A further case of Grange syndrome?

We report on a 15-year-old boy with stenosis and occlusion of multiple cran ial, renal, and celiac arteries, aneurysm of the basilar artery, bilateral cutaneous syndactyly between fingers IV-V, partial cutaneous syndactyly bet ween fingers III-IV on the right hand, brachydactyly, and borderline mental retardation. The clinical course was characterized by recurrent abdominal pain, gastritis, and high blood pressure. The pattern of the clinical and r adiological findings is different from fibromuscular dysplasia (FMD) and Mo yamoya disease, and highly suggestive of a syndrome described by Grange in four siblings (MIM#602531) Grange et ai, [1998: Am J Med Genet 75: 469-480] , (C) 2001 Wiley-Liss, Inc.