Influence of Down's syndrome on management and outcome of patients with congenital intrinsic duodenal obstruction

Abstract

Purpose Children with Down's syndrome (DS) have been reported to have a high incidence of congenital heart disease (CHD) and respiratory tract infection. These anomalies and complications lead to poor outcomes, especially after treatment of congenital intrinsic duodenal obstruction (CIDO). The aim of this study was to review and compare the outcome of management of CIDO at a single tertiary institute for children in Thailand. Methods We reviewed the medical records of patients with CIDO, who were treated at Queen Sirikit National Institute of Child Health (Bangkok, Thailand) from 1997 to 2006. The patients were classified into 2 groups: A = CIDO with DS and B = CIDO with non-DS (NDS). Each group was then divided into 2 subgroups: A 1 = DS without CHD, A 2 = DS with CHD, B 1 = NDS without CHD, and B 2 = NDS with CHD. Comparisons of management and outcome between group A and group B, subgroup A 1 and subgroup B 1, and subgroup A 2 and subgroup B 2 were performed. Statistical differences were analyzed by the χ 2 test at a P < .05. Results A total of 227 patients (male-female = 108:119) underwent surgical correction of CIDO. Of 227 patients, 86 (37.9%) were shown to have DS (group A), and 141 (62.1%) were NDS patients (group B). Demographic information did not differ between the groups, except for the mothers' age at pregnancy and the operative procedure used for duodenoduodenostomy. Group A had a higher incidence of CHD than group B (66.3% vs 26.2%; P < .05). Early outcome regarding survival rate (SR) was not statistically different between groups (87.2% vs 92.9%; P = .15), but the rate of early postoperative complications in group A was higher than that in group B (45.3% vs 31.1%; P = .03). The overall SR of group A at long-term follow-up was worse than that in group B (68.6% vs 86.7%; P < .05). Late complications and sequelae in group A were also more frequent than in group B (34.6% vs 9.2%; P < .05). When comparing early outcome between DS and NDS patients without CHD, the SR of subgroup A 1 was lower than that of subgroup B 1 (86.5% vs 96.5%; P = .04), but the rate of early postoperative complications did not differ (41.5% vs 24.1%; P = .05). At long-term follow-up, the overall SR of subgroup A 1 was worse than that of subgroup B 1 (73% vs 95.6%; P < .05) because of a higher incidence of late death because of recurrent respiratory tract infection in the A 1 subgroup. In addition, late complications and sequelae were more common in subgroup A 1 than in subgroup B 1 (37.5% vs 8.9%; P < .05). In patients with CHD, there was no statistical difference between early and long-term results of SR or complications between subgroup A 2 and subgroup B 2. The overall SR at long-term follow-up was 63% in subgroup A 2 and 56% in subgroup B 2 ( P = .5). Conclusions Early outcome of CIDO management revealed minimal differences between DS and NDS patients. However, these differences were clearer in long-term outcomes. The DS patients had lower long-term SR and higher rates of complications and sequelae than NDS patients. It is concluded that DS negatively impacts the management and outcome of patients with CIDO.