Hirschsprung's Disease in Oman

The incidence of Hirschsprung's disease (HD) was studied retrospectively in Oman using hospital-based data. In Oman there is a single pediatric surgery unit where a register has been kept from 1989 to 1994, and because all cases are referred to this unit, a national survey could be carried out. There were 85 children with HD born between 1989 and 1994, and during the period there were 261,000 livebirths among Omani nationals. The population frequency in Oman is 1 in 3,070 (0.3/1,000). Eighty percent of cases presented in the first 6 months. The incidence in different regions and within different tribes of Oman was also studied. The highest frequency (1 in 1,800) is in the North Sharqiya region. There was not a significant seasonal influence in spite of the very high temperatures seen in the desert summer. The ratio of male to female cases was 2.9:1 overall, but less for longer-segment involvement. The consanguinity rate (first and second cousins) was 75%, which is higher than the level of consanguinity in the Omani population. Down's syndrome was observed in nine cases (11%), and a variety of other malformations were seen, including piebaldism, deafness, and HD in two sibships.