S Syndrome, Duodenal Atresia with Paradoxical Presence of Distal Bowel Gas

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Journal of Perinatology (2011) 31, 223–224 r 2011 Nature America, Inc. All rights reserved. 0743-8346/11 www.nature.com/jp IMAGING CASE REPORT Down’s syndrome, duodenal atresia with paradoxical presence of distal bowel gas

TE Herman and MJ Siegel Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO, USA

Journal of Perinatology (2011) 31, 223–224; doi:10.1038/jp.2010.151 a slight male predominance (1.4:1).2 Associated anomalies are Down’s syndrome, seen in 46% of patients with duodenal atresia. Case presentation Other anomalies include the VACTERL association (vertebral, anus, A 3645-g male infant was born to a 34-year-old gravid 3, para 3 cardiac, tracheal esophageal and limb anomalies), occurring in mother after a pregnancy complicated by gestational diabetes and 5 to 15% of cases, Feingold’s syndrome (various combinations abnormal fetal sonograms, which demonstrated an atrioventricular of microcephaly, limb malformations, esophageal and duodenal (AV) canal defect and polyhydramnios. Amniocentesis was atresia) and alveolar capillary dysplasia.3,4 performed, which showed trisomy 21. The baby was brought to the In the normal pancreas, the main pancreatic duct or duct neonatal intensive care unit, where an initial plain radiograph of of Wirsung extends transversely from left to right through the the abdomen (Figure 1) was performed. Because of persistent pancreas and drains into the major duodenal papilla below vomiting with oral feeds, an upper gastrointestinal (GI) contrast the pylorus. Frequently there is an additional duct, which arises in study was performed on the third day of life using water-soluble the neck of the pancreas and opens into the minor papilla of the iodine-preparation contrast (Figure 2). duodenum above the major papilla. It is known as the accessory pancreatic duct or duct of Santorini. Wirsung duct is the more ventral duct and Santorini duct is the more dorsal duct. Pancreatic 5 Denouement and discussion duct anatomy can be categorized into four types. In types A and B, Wirsung duct is the main pancreatic duct and drains into the The upper GI demonstrates duodenal atresia with so-called major papilla of the duodenum. In type A, Santorini duct is paradoxical presence of distal bowel gas. The gas is able to pass obliterated or absent. In type B anomaly, which is the most through the major and minor duodenal papillae, which straddle common, Santorini duct is an accessory or smaller pancreatic duct the area of atresia. The patient underwent duodenoduodenostomy for duodenal atresia on the fourth day of life and subsequent repair of AV canal was done as well. Paradoxical presence of gas in the distal bowel with duodenal atresia appears to have been ﬁrst recognized in 1972.1 It must be differentiated from cases of partial duodenal obstruction with the presence of distal gas, such as duodenal stenosis, incomplete duodenal diaphragm, Ladd’s bands, annular pancreas or pre- duodenal portal vein. Duodenal atresia is not an uncommon congenital anomaly and occurs in 1 in 10 000 births.2 In fetal life, this anomaly is associated with polyhydramnios. Duodenal atresia, unlike jejunal and ileal atresia, is a developmental anomaly due to failure to recanalize the duodenum after its solid phase. The solid phase of the duodenum occurs because of proliferation of the endodermal epithelium. Subsequent recanalization is by apoptosis. Duodenal atresia is often associated with other anomalies and has

Correspondence: Dr TE Herman, Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 South Kingshighway Boulevard, St Louis, MO 63110, USA. Figure 1 Abdomen supine radiograph. The bowel gas pattern at birth is normal, E-mail: [email protected] with air in distal bowel loops. A double bubble is not present; a nasogastric Received 26 August 2010; accepted 16 September 2010 tube is in place. Down’s syndrome, duodenal atresia TE Herman and MJ Siegel 224

minor papilla of the duodenum, and Wirsung duct forms an accessory pancreatic duct that is obliterated or drains into the major papilla of the duodenum. Pancreatic ductal systems other than types A, B and C are defined as type D. In our patient, the presence of two ducts and two duodenal papillae indicates a type B or C pancreatic duct system anomaly.5 In patients who have duodenal atresia and simultaneously have type B or type C pancreatic ductal system, duodenal atresia develops in the area between the two papillae. If the papillae are incompetent, they will allow gas to flow from the proximal duodenum into the duct of Santorini above the area of atresia, and from there gas can enter the duct of Wirsung below the atresia and flow into the distal duodenum. This accounts for the presence of distal gas in the small bowel in the presence of complete duodenal atresia. In this patient, the pancreatic duct anomaly was shown well by the upper GI series preoperatively, which demonstrated contrast passing through the ducts into the distal duodenum.

Conﬂict of interest The authors declare no conﬂict of interest.

References 1 Kassner EG, Sutton AL, deGroot TJ. Bile duct anomalies associated with duodenal atresia; paradoxical presence of small bowel gas. Am J Roentgenol 1972; 116: 577–583. Figure 2 Series of sequential ﬁlms from the upper gastrointestinal series (UGI). 2 Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: associated anomalies, (a) Contrast ﬁlls the stomach and dilated proximal duodenum, with a small prenatal diagnosis and outcome. Pediatr Surg Int 2009; 25: 727–730. amount of contrast present in a tiny tubular structure medially (straight arrow), 3 Chopra M, James C, Adams S, Mowat D. Duodenal atresia: consider Feingold syndrome. representing the pancreatic duct of Santorini. Contrast passes into the duct because of an incompetent accessory pancreatic ampulla. (b) Contrast in the tiny J Pediatr Child Health 2010; 46: 136–137. duct accessory pancreatic duct passes through the major pancreatic duct (curved 4 Gutierrez C, Rodriguez A, Palenzuela S, Forteza C, Diaz-Rossello JL. Congenital arrow) into the nondilated distal duodenum (DD). The dilated proximal misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent duodenum (PROX) is also seen. neonatal pulmonary hypertension: report of 2 affected siblings. Pediatr Dev Pathol 2000; 3: 271–276. 5 Bang S, Suh JH, Park BR, Park SW, Song SY. The relationship of anatomic and enters the minor papilla in the duodenum. In type C anomaly, variation of pancreatic duct and pancreaticobiliary diseases. Yonsei Med J 2006; 2: Santorini duct is the main pancreatic duct and drains into the 243–248.

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