Background and objectives: Outcomes of neonates diagnosed with esophageal atresia (EA), a rare congenital malformation, vary widely. Due to limited and fragmented data globally, major regional centers offer a crucial opportunity to better understand EA’s epidemiology and the management. This study aimed to address these gaps by determining total birth prevalence and early treatment outcomes of EA in southern Croatia. Methods: All EA cases (1991–2020) were retrospectively ascertained from medical documentation at the only tertiary referral center for EA in southern Croatia, with birth data collected from the entire background population. We collected data on neonates’ status and diagnosis, operative findings, early postoperative complications, and treatment outcome from this single center. Results: A total of 53 cases were identified, with an average total birth prevalence of 2.44 per 10,000 total births/year. No significant sex differences were found (p = 0.339), and most cases were complex Vogt 3B. The mortality rate dropped from 87 to 8% over a 30-year period (p < 0.001). The 1-year survival rate was 54% (95% CI 40–68%) for liveborns and 64% (50–79%) for liveborns who underwent surgery and intensive care. However, 16% of patients died before surgery due to hemodynamic instability, and among those operated on, high sepsis, pneumonia, and atelectasis rates were observed. Conclusions: EA prevalence in southern Croatia aligns with European data. Survival improved significantly after 2002, coinciding with a dedicated pediatric ICU and enhanced pediatric care training. Further advancements in early diagnosis and a multidisciplinary approach are needed to further reduce mortality. Strengthened postoperative infection control and optimized postoperative respiratory support are also crucial to minimizing complications.

We assessed the methodological quality and transparency of all the national clinical practice guidelines that were published in Croatia up until 2017 and explored the factors associated with their quality rating. An in-depth quantitative and qualitative analysis was performed using rigorous methodology. We evaluated the guidelines using a validated AGREE II instrument with four raters; we used multiple linear regressions to identify the predictors of quality; and two focus groups, including guideline developers, to further explore the guideline development process. The majority of the guidelines (N = 74) were developed by medical societies. The guidelines’ quality was rated low: the median standardized AGREE II score was low, 36% (IQR 28–42), and so were the overall-assessments. The aspects of the guidelines that were rated best were the “clarity of presentation” and the “scope and purpose” (median ≥ 59%); however, the other four domains received very low scores (15–33%). Overall, the guideline quality did not improve over time. The guidelines that were developed by medical societies scored significantly worse than those developed by governmental, or unofficial working groups (12–43% per domain). In focus group discussions, inadequate methodology, a lack of implementation systems in place, a lack of awareness about editorial independence, and broader expertise/perspectives in working groups were identified as factors behind the low scores. The factors identified as affecting the quality of the national guidelines may help stakeholders who are developing interventions and education programs aimed at improving guideline quality worldwide.