Abnormalities of intestinal rotation and congenital intrinsic duodenal obstruction

Abstract

Background Congenital duodenal obstruction (CDO) is a unique anomaly with variable presentation depending on the site, type of obstruction, as well as the presence of
associated anomalies. This is a review of our experience with 51 infants and children with CDO, stressing on the importance of associated rotational abnormalities of the gut.
Methods Over a period of 18 years (January 1993– December 2011), 51 infants and children with CDO were treated at our hospital. Their medical records were reviewed retrospectively for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment, and outcome.
Results Fifty-one (27 boys and 24 girls) infants and children with intrinsic CDO were treated. Their birth weight ranged from 1.38 to 3.8 kg (mean 2.3 kg). Sixteen (31.4%)
were premature. The mean maternal age was 25 years (range 18–40 years). Seven presented at 5 months, 18 days, 3.5 years, 1.8 years, 18 days, 1.5 years, and 2 months of age, respectively. Associated anomalies were observed in 34 (66.7%) of them. Sixteen (31.4%) had Down’s syndrome and 11 (21.6%) had congenital heart disease. Nine (17.6%) had rotational abnormalities of the gut, including two with situs inversus. The site of duodenal obstruction was located in the second part of duodenum in 48 (94%) of them. In two, the site of obstruction was in the fourth part of duodenum, whereas in the third it was located in the third part of duodenum. The causes of obstruction were duodenal atresia in 20 (39.2%), without a gap in eight (15.7%) and with a gap in 12 (23.5%), duodenal stenosis in 11 (21.6%), and duodenal diaphragm in 20 (39.2%). In 14 (27.5%) patients, an annular pancreas was observed. The different operative procedures were as follows: duodenoduodenostomy in 26; excision of the duodenal diaphragm and duodenoplasty in 12; Ladd’s procedure, excision of the duodenal diaphragm, duodenoplasty, and appendectomy in four; Ladd’s procedure, appendectomy, and duodenoduodenostomy in two; and duodenojejunostomy in seven. Two underwent reduction duodenoplasty to decrease the size of the duodenum. Three required reoperations, two because of an anastomotic leak and another because of duodenal dysfunction. Sixteen (31.4%) required total parenteral nutrition. Four died, resulting in an overall survival of 92.2%.
Conclusion CDO is classified as intrinsic and extrinsic depending on the cause. Rotational abnormalities including situs inversus are among the rare but interesting anomalies associated with intrinsic CDO. The presence of malrotation or nonrotation with congenital bands does not exclude the presence of an associated intrinsic cause, which should be looked for intraoperatively. This is to obviate the possibility of further subsequent operative treatment. 

Keywords: congenital duodenal obstruction, duodenal diaphragm, malrotation, situs inversus