Prevalence and survival of patients with anorectal malformations: A population-based study

被引:24
|
作者
Cassina, Matteo [1 ]
Leon, Francesco Fascetti [2 ]
Ruol, Michele [2 ]
Chiarenza, Salvatore Fabio [3 ]
Scire, Gabriella [4 ]
Midrio, Paola [5 ]
Clementi, Maurizio [1 ]
Gamba, Piergiorgio [2 ]
机构
[1] Univ Padua, Dept Womens & Childrens Hlth, Clin Genet Unit, Padua, Italy
[2] Univ Padua, Dept Womens & Childrens Hlth, Pediat Surg Unit, Padua, Italy
[3] San Bortolo Hosp, Pediat Surg Unit, Vicenza, Italy
[4] Univ Verona, Pediat Surg Unit, Verona, Italy
[5] CaFoncello Hosp, Pediat Surg Unit, Treviso, Italy
关键词
Anorectal malformation; Epidemiology; Survival; Population-based study; DIAGNOSIS; ANOMALIES; ETIOLOGY; REGISTRY;
D O I
10.1016/j.jpedsurg.2019.03.004
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Background: Anorectal malformations (ARMs) arc the most frequent congenital intestinal anomalies. The aim of this study was to describe the epidemiology of anorectal defects between 1981 and 2014 and to evaluate patients' survival. Methods: A population-based study using data collected by an Italian, regional registry of birth defects and by the local Pediatric Surgery Units. Results: A total of 428 individuals with ARM were identified, with an overall prevalence of 3.09 per 10,000 births. Characteristics associated with decreased survival were low birth weight (<2500 g) (HR 6.4; 95% CI, 2.3-17.9), the presence of two or more additional major defects (HR 7.9; 95% Cl, 2.2-27.8), and birth before year 2000 (HR 4.7; 95% CI, 1.8-11.8). The 10-year survival probability was 100% for individuals with isolated ARM, regardless of their birth weight. Survival of patients with non-isolated ARM varied according to their year of birth and birth weight: 73.3% (>= 2500 g) and 23.8% (<2500 g) in children born before 2000; 97.9% (>= 2500 g) and 68.8% (<2500 g) in children born after year 2000. Conclusions: This study found a significant improvement in the survival of individuals with anorectal malformations over the past decades and identified the strongest predictors of mortality. (C) 2019 Elsevier Inc. All rights reserved.
引用
收藏
页码:1998 / 2003
页数:6
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