HIRSCHSPRUNGS-DISEASE IN OMAN

The incidence of Hirschsprung's disease (HD) was studied retrospective ly in Oman using hospital-based data. In Oman there is a single pediat ric 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 1 994, and during this 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 incidenc e in different regions and within different tribes of Oman was also st udied. The highest frequency (1 in 1,800) is in the North Sharqiya reg ion. There was not a significant seasonal influence in spite of the ve ry high temperatures seen in the desert summer. The ratio of male to f emale 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 mal formations were seen, including piebaldism, deafness, and HD in two si bships. Copyright (C) 1997 by W.B. Saunders Company.