Abstract
Esophageal atresia (EA), although a rare congenital anomaly, represents one of the most common gastrointestinal birth defects. There is a gap in our knowledge regarding the impact of perioperative critical care in infants born with EA. This study addresses EA types, disease severity stratification, and mortality in a retrospective cohort at a single institution. Institutional Review Board approved our retrospective cross-sectional study of term-born (n = 53) and premature infants (28–37 weeks of gestation; n = 31) that underwent primary surgical repair of EA at a single institution from 2009–2020. Demographic and clinical data were obtained from the electronic medical record, Powerchart (Cerner, London, UK). Patients were categorized by (i) sex, (ii) gestational age at birth, (iii) types of EA (in relation to respiratory tract anomalies), (iv) co-occurring congenital anomalies, (v) severity of disease (viz. American Society of Anesthesiologists (ASA) and Pediatric Risk Assessment (PRAm) scores), (vi) type of surgical repair for EA (primary anastomosis vs. Foker process), and (vii) survival rate classification using Spitz and Waterston scores. Data were presented as numerical sums and percentages. The frequency of anatomical types of EA in our cohort parallels that of the literature: 9.5% (8/84) type A, 9.5% (8/84) type B, 80% (67/84) type C, and 1% (1/84) type D. Long-gap EA accounts for 88% (7/8) type A, 75% (6/8) type B, and 13% (9/67) type C in the cohort studied. Our novel results show a nearly equal distribution of sex per each EA type, and gestational age (term-born vs. premature) by anatomical EA type. PRAm scoring showed a wider range of disease severity (3–9) than ASA scores (III and IV). The survival rate in our EA cohort dramatically increased in comparison to the literature in previous decades. This retrospective analysis at a single institution shows incidence of EA per sex and gestational status for anatomical types (EA type A-D) and by surgical approach (primary anastomosis vs. Foker process for short-gap vs. long-gap EA, respectively). Despite its wider range, PRAm score was not more useful in predicting disease severity in comparison to ASA score. Increased survival rates over the last decade suggest a potential need to assess unique operative and perioperative risks in this unique population of patients. Presented findings also represent a foundation for future clinical studies of outcomes in infants born with EA.
Highlights
Esophageal atresia (EA), a rare congenital anomaly with a stable world-wide prevalence (1) represents one of the most common gastrointestinal birth defects with reported incidence of 1 in 3,000 to 1 in 4,500 live births (2)
Mortality Risk Assessment Considering our retrospective data collection spans period of last 11 years (2009-2020), it was used for comparison to previously published survival rates in infants born with esophageal atresia (EA) as per two different scoring systems: (i) Waterston et al (21), and (ii) Spitz et al (20)
Anatomical Types of Esophageal Atresia We report similar distribution of EA patients according to the anatomical classification (Figure 1A) compared to the literature (4, 31, 32) one should keep in mind that definition of long-gap EA might differ
Summary
Esophageal atresia (EA), a rare congenital anomaly with a stable world-wide prevalence (1) represents one of the most common gastrointestinal birth defects with reported incidence of 1 in 3,000 to 1 in 4,500 live births (2). If the gap of the esophagus is too large to be repaired by direct anastomosis (>3 cm or >2 vertebral bodies in length), EA is defined as long-gap EA, which is more commonly found in anatomical types A and B of EA (9). At our institution, the latter cases undergo a unique type of EA repair, the Foker process (5– 8). Short-gap EA is more commonly found with VACTERL anomalies (vertebral, anorectal, cardiac, tracheo-esophageal fistula and/or esophageal atresia, renal, and limb defects/ malformations) relative to long-gap EA (9). CHARGE syndrome (coloboma, heart defects, choanal atresia, growth retardation, genital abnormalities, and ear abnormalities) (10) can further complicate the care of infants born with EA
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