ABSTRACT
Jejuno-ileal atresia, defined as a congenital defect in continuity of the bowel, is a common cause of intestinal obstruction in the newborn, accounting for between 80% and 95% according to various reports.1-3 The incidence of jejuno-ileal atresia varies from 1 in 330 and 1 in 400 live births,4 to between 1 in 1500 and 1 in 3000 live births.5 Jejuno-ileal occlusions occur more frequently than duodenal or colonic ones do.1,6 With improved neonatal and perioperative care, safe anesthesia, refined surgical techniques and management of short bowel syndrome, a survival rate of 90% can be expected. At the Red Cross War Memorial Children’s Hospital in Cape Town during the 41 years 1959-2000, 273 jejuno-ileal atresias, 194 (71%) jejunum and 79 (29%) ileum were seen (Table 47.1) compared to 189 duodenal and eight colonic atresias. Down syndrome is most uncommon in babies with jejuno-ileal atresia (only one baby in the Red Cross Hospital series) compared with duodenal atresias. The first successful surgical repair of an intestinal atresia was in 1911.7 The mortality rate remained high over the next 4 decades and it was only in the mid 1950s that an improved understanding of the pathogenesis and pathology of the condition led to innovative surgical techniques which resulted in improved surgical outcome.5,6
In 1889, Bland Sutton postulated that atresia occurred at the site of ‘obliterative embryological events’ and he quoted atrophy of the vitelline duct.8 In 1900, Tandler9
supported by embryonal studies, suggested that intestinal atresia was related to a lack of recanalization of the solid stage of the intestine, while others have questioned these theories.10-12 In 1952, Louw published the results of an investigation of 79 patients treated at Great Ormond Street, London, and suggested that jejuno-ileal atresia was probably due to a vascular accident rather than the result of inadequate recanalization.5 At his instigation, Barnard perfected the experimental model in pregnant mongrel bitches. Mesenteric vascular insults, such as volvulus, intussusception and interference with the blood supply to a segment of bowel were created in the dog fetus.13 This not only confirmed the hypothesis but led to a change in the surgical procedure for correcting atresias and stenosis of the jejunum and ileum with a marked improvement in outcome.14-16 Subsequently these experimental findings were confirmed by others in several different animal models and in clinical practice.17-21 Evidence of bowel infarction was present in 42% of 449 cases of jejuno-ileal atresia in a collected series which further supported the vascular hypothesis.22 Furthermore the localized nature of the vascular accident occurring late in fetal life would explain the low incidence (less than 10%) of coexisting abnormalities of extra-abdominal organs.
