Kovačević Tanja, Polić Branka, Markić Joško, Ardalić Čatipović Tatjana, Bucat Marija, Mikulić Svjetlana, Žuvan Leona, Pogorelić Zenon, Despot Ranka, Žitko Vanda, Meštrović Julije, Lozić Bernarda, Jerončić Ana
Department of Pediatrics, University Hospital of Split, 21000 Split, Croatia.
School of Medicine, University of Split, 21000 Split, Croatia.
Healthcare (Basel). 2025 Feb 14;13(4):418. doi: 10.3390/healthcare13040418.
: 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. 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. 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 ( = 0.339), and most cases were complex Vogt 3B. The mortality rate dropped from 87 to 8% over a 30-year period ( < 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. 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.
被诊断患有食管闭锁(EA)这种罕见先天性畸形的新生儿的预后差异很大。由于全球数据有限且分散,主要区域中心为更好地了解EA的流行病学和治疗提供了关键契机。本研究旨在通过确定克罗地亚南部EA的总出生患病率和早期治疗结果来填补这些空白。从克罗地亚南部唯一的EA三级转诊中心的医疗记录中回顾性确定了所有EA病例(1991 - 2020年),并从整个背景人群中收集出生数据。我们从这个单一中心收集了新生儿的状况和诊断、手术发现、术后早期并发症及治疗结果的数据。共识别出53例病例,年平均总出生患病率为每10000例总出生数中有2.44例。未发现显著的性别差异(P = 0.339),且大多数病例为复杂的Vogt 3B型。在30年期间,死亡率从87%降至8%(P < 0.001)。活产儿的1年生存率为54%(95%置信区间40 - 68%),接受手术和重症监护的活产儿的1年生存率为64%(50 - 79%)。然而,16%的患者因血流动力学不稳定在手术前死亡,在接受手术的患者中,观察到高败血症、肺炎和肺不张发生率。克罗地亚南部的EA患病率与欧洲数据一致。2002年后生存率显著提高,这与专门的儿科重症监护病房和强化的儿科护理培训相吻合。需要在早期诊断和多学科方法上进一步取得进展以进一步降低死亡率。加强术后感染控制和优化术后呼吸支持对于将并发症降至最低也至关重要。