Evaluation of intestinal atresia is usually initiated before birth. In our series the major diagnostic tool was antenatal ultrasound performed between 28-31 weeks of gestation, advanced imaging with antenatal MRI was also used in complex cases. Our accuracy of 70% in detecting small bowel atresia compares highly favorable with other similar series of 23-45% accuracy rate
7. Our hospital is a major antenatal diagnostic center with highly experienced perinatologists, this may contribute to our high diagnostic rates. Radiologic findings vary depending on the location of atresia. Diagnosis of duodenal atresia was mostly based on image of double bubble on ultrasound, in some cases polyhidramiosis was also noted. Jejunoileal atresia was diagnosed by dilated intestinal loops and polyhydramniosis, the number of dilated loops and echogenicity of the bowels increased as the atresia located more distally. In our series there was no correlation between polyhydramniosis and location of the atresia. Furthermore, our rates of antenatal detection did not differ between groups which is different than the current literature stating high antenatal diagnostic rates for more proximal atresia
9. As accepted in literature, duodenal atresia is more commonly associated with comorbidities whereas jejunoileal atresia is more often an isolated finding (10). interestingly, in our series, there was no significant difference in additional congenital anomalies between groups. In all groups cardiovascular comorbidities were the most common, followed by gastrointestinal and genitourinary anomalies. Although cardiovascular comorbidity was common in all groups, only 2 patients (1 duodenal atresia and 1 distal jejunoileal atresia) had complex cardiac anomalies and both died due to cardiac reasons. Further research is needed to explain our higher incidence of associated anomalies in both proximal and distal jejuno-ileal atresia groups (71% vs 75%).
The surgical approach in intestinal atresia depends on the location of the lesion, the anatomy, intraoperative condition, and the remaining bowel length4. In our center, surgical repairs were performed through a right upper quadrant transverse laparotomy in the post-natal period as soon as the patients were stable. Although surgeon’s individual preference plays an important role in the selection of operative approach, for duodenal atresia, treatment involved diamond shaped duodenoduodenostomy or duodenojejunostomy and in certain cases with extensive dilatation a prior tapering was also included. For proximal and distal jejunoileal atresia first choice of operation was resection of atretic loop and primary anastomosis but in cases with significant luminal disparity Santulli or Mikulicz type enterostomies were also performed. In our cohort around 30% of the neonates required re-laparotomy due to surgical complications after initial surgery. This number is higher than similar series in literature4,7. Both distal and proximal jejunoileal atresia has higher complication surgery rates than duodenal atresia but there was no statistical difference in between proximal and distal jejunoileal atresia. In all groups, bowel obstructions due to post-operative adhesions were the major reason of re-operation followed by anastomotic dehiscence. In our series there was no correlation between type of surgery and post-operative complications.
The mortality associated with neonatal intestinal obstruction ranges between 15% to 45% depending on the developmental status of a nation11. In this series, overall mortality was 15%. In a similar cohort, Tongsin et al. demonstrated greater intrauterine growth retardation in neonates with jejunal as compared to ileal atresia and linked this to increased mortality12. In our series, we have not found any significant correlation between mortality and prematurity or birth weight. The location of atresia did not directly correlate with mortality rates, cause of mortality was mostly associated with additional congenital anomalies in duodenal atresia and sepsis due to anastomotic complications in distal jejunoileal atresia.
Our data indicates an average of 2 weeks for infants with duodenal atresia and 3 to 4 weeks for proximal and distal jejunoileal atresia for full enteral nutrition. There was no statistically significant difference between proximal and distal jejunoileal atresia, both needed significantly longer time to full enteral nutrition compared to duodenal atresia and we believe this was the main reason for longer length of hospital stay (LOS) in both groups. International studies show a median LOS between 16 and 32 days in intestinal atresia13,14 our median of 24 days is consistent with literature. In regard to location of the atresia, duodenal atresia patients had significantly shorter LOS when compared to both proximal jejunoileal and distal jejunoileal atresia. In a recent national cohort study Schmedding at al. found that patients who needed a stoma had significantly longer LOS15 we also found that regardless of the place of atresia, presence of enterostomy significantly increases duration of hospitalization (median 28 vs 43 days. p=0.042).
Growth measurements are valued as the most important components of the nutritional assessment in children. A growth standard refers to a dataset and related growth charts that reflect a goal for the population. The WHO charts are considered a growth standard because they describe the growth of healthy children under optimal nutritional and environmental conditions16. Z-scores, that are units of standard deviation (SD) from the population mean, are used as reference lines in growth charts. The normal range is generally defined as between -2 SD and +2 SD (Z-scores between -2.0 and +2.0). There are only a few data documenting the longer-term outcomes of intestinal atresia. Shibuya et al.17 reports that in intestinal atresia, within two years of surgery accelerated growth to regain normal size occurs in children. In a similar cohort in 2018 Peng et al.18 reported that intestinal atresia patients who recover from the initial operation and survive the short-term postoperative period, have a good chance of having normal growth and development. Burjonrappa et al. revealed that, due to greater adaptability and increased surface area for absorption of the ileum, jejunal atresia offers better outcome when compared to ileal atresia19. While the effect of genetic make-up and environmental factors cannot be denied, we found out that despite similar gestational age, birth weight and additional comorbidities in the long-term there is significant developmental delay in distal jejunoileal atresia. Again, the numbers in this study are relatively small, but regardless the surgical approach; as the atresia located distally, although catch-up growth in weight is achieved in time, short stature remains constant.
The main limitation of this study is low sample size which could be explained by the rarity of the disease. Several factors, like specific operative details and other missing data points are subject to reporting and observer bias. Furthermore, it is a single-center study with retrospective data collection, some patient records were lost to follow up. Future multicentered studies with larger sample size investigating impact of location of intestinal atresia on long term results are advocated.
As a result, although initially there was no significant difference in prematurity, birth weight and addition congenital anomalies; in the long term distal jejunoileal atresia patients demonstrate signs of developmental delay. We believe distal atresia patients may benefit from more aggressive nutritional support in order to enhance their potential to achieve a normal growth. Multicentered studies with large numbers regarding long term effects of site of atresia are needed for providing reliable data for antenatal counselling.
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