Sigmoid diverticulitis in patients with Williams-Beuren syndrome: relatively high prevalence and high complication rate in young adults with the syndrome.

In a retrospective survey of 128 adults with Williams-Beuren syndrome (age range 18-62 years) sigmoid diverticulitis was reported in 10 patients (2 f, 8 m). The diagnosis of diverticulitis had been made between the ages of 17.1 and 39.6 years. An additional four patients (age range 23.5-32.2 years) had presented with sigmoid diverticulosis. In eight patients the course of the disease was complicated, some of them having to undergo multiple surgery. Conservative therapy was successful in only one female and one male patient with diverticulitis. Thus, we conclude that there is an increased prevalence of sigmoid diverticulitis in adult patients with Williams-Beuren syndrome (8% vs. 2% in the normal population in the age group below 40 years).