They have a frequency of 0 1/10000 live births.
0 They are often associated with other anomalies, especially trisomy 21 (Down syndrome).
0 They occur almost entirely in the second part of the duodenum.
0 Various pancreatobiliary anomalies such as bifid common bile duct are also accompanied.
0 They are examined in two groups as intrinsic (stenosis, atresia, mucosal diaphragm, wind sock deformity) and extrinsic (annular pancreas, rotation anomalies, Ladd bands, preduodenal portal vein).
0 The most common causes of duodenal obstructions are atresia, Ladd bands and annular pancreas.
0 50% of cases are accompanied by polyhydramnios.
0 The most common anomaly accompanying babies with duodenal atresia and annular pancreas is trisomy 21 (Down syndrome).
0 The second most common rotational anomalies are followed by esophageal atresia and TEF, followed by cardiac, genitourinary and anorectal anomalies, respectively.
Duodenal Atresia
• It is divided into three main types (Type 1,2,3)
• In Type 1, the continuity of the duodenum is not impaired when viewed from the outside, but there is a difference in diameter between the proximal and distal segments. There is a wind sock deformity.
• In type 2, the distal and proximal segments are separate, there is no mesentery defect.
• In type 3, the distal and proximal segments are separate, but there is a mesentery defect.
• The majority of atresia is in the periampullary region.
• Proximal segment and stomach are dilated.
Annular Pancreas
• 26% accompanied by atresia or intrinsic obstructions.
• The second part of the duodenum is partially or completely surrounded by the pancreas.
• 85% is located in the 2nd part of the duodenum.
Clinic and Diagnosis
• The most common symptom is biliary vomiting. The onset of symptoms depends on the degree of obstruction, but most occur within the first week.
• Dehydration findings occur within 24-48 hours due to vomiting, epigastric distension is present.
• Peristaltic waves are seen due to the movements of the stomach (from left to right).
• There is hyperbilirubinemia due to dehydration and prematurity.
• Diagnosis can be made with antenatal ultrasound and MRI.
• ADBG has a double-bubble sign.
Treatment
• Nasogastric decompression.
• Antibiotherapy and correction of fluid-electrolyte disorders.
• Duodenojejunostomy or duodenoduodenostomy can be performed surgically.