Total intestinal atresia: Revisiting the pathogenesis of congenital atresias

Abstract

Despite various theories to explain the pathogenesis of atresias, the exact mechanism is still controversial. Currently, atresias are believed to result from vascular accidents and less likely due to the failure of recanalization. We report a case which challenges this belief. A 1-day-old neonate was explored for suspected jejunal atresia. Apart from Type III jejunal atresia, 15 cm from DJ junction, there was surprisingly no distal lumen in the intestine from jejunum till rectum. Multiple enterotomies revealed the whole of the remaining jejunum, ileum, and large colon to be a solid cord-like structure. No distal luminal contents or histopathological evidence of ischemic damage was seen, thus suggesting the probable etiology to be a failure of recanalization of the gut cord rather than a late vascular accident. Such rare cases provide insights into possible embryogenetic mechanisms which can then aid in formulating preventive measures.

Keywords

• Congenital gut anomalies
• embryological defects
• failure of recanalization
• total intestinal atresia
• vascular accidents