SUPERStudy: Duodenal atresia
Introduction: Duodenal atresia is a congenital condition in which the lumen of the duodenum is completely obstructed, preventing the passage of stomach contents into the intestines. It is one of the most common causes of intestinal obstruction in neonates.
Etiology: Often seen in conjunction with other congenital anomalies, such as cardiac defects, malrotation, or annular pancreas. Duodenal stenosis or atresia is the most common GI tract malformation in children with Down syndrome, occurring in 3.9%.
Epidemiology: Occurs in approximately 1 in 5,000 to 10,000 live births.
Pathophysiology: Duodenal atresia results from failure of the duodenum to recanalize during embryonic development (around 3-7 weeks gestation). The obstruction typically occurs distal to the ampulla of Vater, leading to bilious vomiting, although it can occasionally occur proximally, resulting in non-bilious vomiting.
Clinical Manifestations
Vomiting: Begins within the first 24 to 48 hours of life and is typically bilious (green) if the obstruction is distal to the ampulla of Vater.
Abdominal distension: Minimal or localized in the upper abdomen.
Failure to pass meconium in severe cases.
Dehydration and electrolyte imbalances may develop if the condition is not promptly treated.
Diagnosis
Radiological Findings: Double-Bubble Sign on X-ray – Two air-filled structures representing the stomach and proximal duodenum, with no gas distally in the bowel.
Treatment
Management: Duodenal atresia is a critical diagnosis in neonates presenting with early vomiting, and rapid identification and management are essential to prevent complications.
Initial Stabilization: Nasogastric or orogastric tube placement to decompress the stomach. Intravenous fluid resuscitation to address dehydration and electrolyte imbalances. Correction of acid-base disturbances.
Surgical Treatment: Duodenoduodenostomy – The creation of a connection between the proximal and distal duodenum to bypass the atretic segment.
Prognosis: With timely surgical intervention, the prognosis is excellent. Neonates with isolated duodenal atresia typically recover fully, but the overall prognosis depends on the presence of associated anomalies, particularly in cases with Down syndrome or severe cardiac defects.
SUPERPoint: Duodenal atresia presents with the classic “double bubble” sign on X-ray, a hallmark diagnostic feature caused by air trapped in the stomach and proximal duodenum, making it one of the most recognizable congenital causes of neonatal intestinal obstruction.
SUPERformula: Congenital obstruction of the duodenum + Failure of embryonic recanalization + Polyhydramnios during pregnancy + Bilious vomiting shortly after birth + Double bubble sign on X-ray + Association with Down syndrome + Requires surgical correction (duodenoduodenostomy) = Duodenal Atresia
Reference:
Current Diagnosis & Treatment: Pediatrics, 26e
Maya Bunik, William W. Hay, Myron J. Levin, Mark J. Abzug
Schwartz’s Principles of Surgery, 11eCharles Brunicardi, Dana K. Andersen, Timothy R. Billiar, David L. Dunn, Lillian S. Kao, John G. Hunter, Jeffrey B. Matthews, Raphael E. Pollock
Pathology: The Big Picture
Walter L. Kemp, Dennis K. Burns, Travis G. Brown
Williams Obstetrics, 26eGary Cunningham, Kenneth J. Leveno, Jodi S. Dashe, Barbara L. Hoffman, Catherine Y. Spong, Brian M. Casey