Department of Surgery, University of Ottawa, Ottawa, Ontario, Canada.
Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
JAMA Netw Open. 2020 Feb 5;3(2):e1921036. doi: 10.1001/jamanetworkopen.2019.21036.
Orofacial cleft (OFC) is one of the most common congenital malformations, with a wide variation in incidence worldwide. However, population-based studies on the incidence of OFC in North America are lacking.
To examine the incidence of OFC in Ontario, Canada, and to compare risk factors and mortality associated with children with OFC vs children without OFC.
DESIGN, SETTING, AND PARTICIPANTS: This population-based retrospective cohort study used health administrative data from the province of Ontario, Canada. Children with OFC who were born from April 1, 1994, to March 31, 2017, in Ontario were each matched to 5 children without OFC based on sex, date of birth (±30 days), and mother's age (±5 years). Analyses were conducted from September 2018 to January 2019.
Children born with OFC.
Incidence of OFC over time and regional variation. Risk factors for OFC were assessed using 1-way analysis of variance for means, Kruskal-Wallis for medians, and χ2 tests for categorical variables. Adjusted Cox regression models were used to assess mortality.
From 1994 to 2017, 3262 children were born with OFC in Ontario, Canada, and they were matched to 15 222 children born without OFC. Incidence of OFC in Ontario was 1.12 cases per 1000 live births, with wide geographic variation and a lower incidence from 2004 to 2017 compared with 1994 to 2003 (1.02 vs 1.13 cases per 1000 live births; P = .002), especially for the subgroup with cleft palate (0.52 vs 0.44 cases per 1000 live births; P = .006). Children with OFC, compared with children without OFC, were more likely to be born prematurely (406 children [13.3%] vs 1086 children [7.1%]; P < .001; standardized difference, 0.21) and had lower mean (SD) birth weight (3215.3 [687.6] g vs 3382.6 [580.0] g; P < .001; standardized difference, 0.26). The mortality rate among children with OFC was higher than among matched children without OFC (hazard ratio, 10.60; 95% CI, 7.79-14.44; P < .001). When mortality was adjusted for the presence of congenital or chromosomal anomalies, the risk of death was not significantly different between children with OFC and those without OFC (hazard ratio, 1.35; 95% CI, 0.73-2.72).
These findings suggest that incidence of OFC In Ontario, Canada, decreased from 1994 to 2017. Mortality in children with OFC was high, especially in the first 2 years of life, and was predominantly associated with the presence of other congenital or chromosomal anomalies. Further research is required to better understand the causes of wide geographical variations of OFC incidence and improve the survival of these patients.
唇腭裂 (OFC) 是最常见的先天性畸形之一，其发病率在全球范围内差异很大。然而，北美的基于人群的 OFC 发病率研究却很缺乏。
研究加拿大安大略省 OFC 的发病率，并比较 OFC 患儿与无 OFC 患儿相关的风险因素和死亡率。
设计、地点和参与者：这项基于人群的回顾性队列研究使用了来自加拿大安大略省的健康管理数据。1994 年 4 月 1 日至 2017 年 3 月 31 日期间在安大略省出生的患有 OFC 的儿童，按照性别、出生日期（±30 天）和母亲年龄（±5 岁）与 5 名无 OFC 的儿童进行匹配。分析于 2018 年 9 月至 2019 年 1 月进行。
出生时患有 OFC 的儿童。
随时间的 OFC 发病率和区域变化。使用单因素方差分析均值、Kruskal-Wallis 检验中位数和卡方检验分类变量来评估 OFC 的风险因素。使用调整后的 Cox 回归模型来评估死亡率。
1994 年至 2017 年期间，加拿大安大略省有 3262 名儿童出生时患有 OFC，与 15222 名未患有 OFC 的儿童相匹配。安大略省 OFC 的发病率为每 1000 例活产 1.12 例，具有广泛的地域差异，与 1994 年至 2003 年相比，2004 年至 2017 年的发病率较低（1.02 比 1.13 例每 1000 例活产；P=0.002），尤其是对于伴有腭裂的亚组（0.52 比 0.44 例每 1000 例活产；P=0.006）。与无 OFC 的儿童相比，患有 OFC 的儿童更有可能早产（406 名儿童[13.3%]比 1086 名儿童[7.1%]；P<0.001；标准化差异，0.21），出生体重的平均值（标准差）较低（3215.3[687.6]g 比 3382.6[580.0]g；P<0.001；标准化差异，0.26）。患有 OFC 的儿童死亡率高于无 OFC 的匹配儿童（风险比，10.60；95%CI，7.79-14.44；P<0.001）。当死亡率根据先天性或染色体异常的存在进行调整时，患有 OFC 的儿童与无 OFC 的儿童的死亡风险没有显著差异（风险比，1.35；95%CI，0.73-2.72）。
这些发现表明，加拿大安大略省的 OFC 发病率从 1994 年至 2017 年下降。患有 OFC 的儿童死亡率很高，尤其是在生命的头 2 年，主要与其他先天性或染色体异常有关。需要进一步研究以更好地了解 OFC 发病率的广泛地域差异的原因，并改善这些患者的生存率。
