Congenital duodenal obstruction in the UK: a population-based study

Abstract

ObjectiveCongenital duodenal obstruction (CDO) comprising duodenal atresia or stenosis is a rare congenital anomaly requiring surgical correction in early life. Identification of variation in surgical and postoperative practice in previous studies has been limited by small sample sizes. This study aimed to prospectively estimate the incidence of CDO in the UK, and report current management strategies and short-term outcomes.DesignProspective population-based, observational study for 12 months from March 2016.SettingSpecialist neonatal surgical units in the UK.Main outcome measuresIncidence of CDO, associated anomalies and short-term outcomes.ResultsIn total, 110 cases were identified and data forms were returned for 103 infants giving an estimated incidence of 1.22 cases per 10 000 (95% CI 1.01 to 1.49) live births. Overall, 59% of cases were suspected antenatally and associated anomalies were seen in 69%. Operative repair was carried out mostly by duodenoduodenostomy (76%) followed by duodenojejunostomy (15%). Postoperative feeding practice varied with 42% having a trans-anastomotic tube placed and 88% receiving parenteral nutrition. Re-operation rate related to the initial procedure was 3% within 28 days. Two infants died within 28 days of operation from unrelated causes.ConclusionThis population-based study of CDO has shown that the majority of infants have associated anomalies. There is variation in postoperative feeding strategies which represent opportunities to explore the effects of these on outcome and potentially standardise approach. Short-term outcomes are generally good.

Outcomes and survival of infants with congenital duodenal obstruction following Kimura procedure with post-anastomosis jejunostomy feeding tube

“…Most of our CDO infants (64.7%) showed a polyhydramnios or double bubble during an antenatal sonography. This finding was higher than a previous report (23.3%) [ 16 ], but similar to the result (59%) in a study by Bethell et al [ 3 ]. However, only 32.7% of our patients had complete data on antenatal sonography.…”

“…Congenital duodenal obstruction (CDO) is the most common cause of intestinal obstruction in neonates [ 1 ]. Its incidence varies among studies, ranging from 1 in 5000 to 10,000 live births, and it is more common in male infants [ 2 , 3 ]. More than 50% of patients with CDO are associated with other congenital abnormalities, including Down syndrome, congenital heart disease (CHD), and VACTERL syndrome [ 2 , 4 , 5 ].…”

Microarray analysis in fetuses with duodenal obstruction: It is not just trisomy 21

“…Infants with DO have excellent short‐term outcomes after neonatal repair, 1 and long‐term survival is generally good at 86% to 90%, but infants with associated congenital anomalies have a higher mortality rate 40 . Regardless, pregnancy outcomes either depend on structural anomalies or on chromosomal aberrations 13 .…”

“…It requires immediate postnatal management. It is estimated to affect 1.2 per 10 000 live births, with males and females equally affected 1,2 . Typically, over half of these cases are detected prenatally on fetal ultrasonography in the second or third trimester, with the characteristic presence of a “double bubble” sign in the upper abdomen representing a dilated fluid‐filled stomach and proximal duodenum.…”

“…It is estimated to affect 1.2 per 10 000 live births, with males and females equally affected. 1,2 Typically, over half of these cases are detected prenatally on fetal ultrasonography in the second or third trimester, with the characteristic presence of a "double bubble" sign in the upper abdomen representing a dilated fluidfilled stomach and proximal duodenum. It is either an isolated finding or in association with other congenital anomalies, including cardiac defects, intestinal atresia, renal anomalies, and chromosomal aberrations.…”