Royal Hospital for Sick Children, Edinburgh, United Kingdom.
Regional Maternity Survey Office, Public Health England, Newcastle upon Tyne, United Kingdom.
Birth Defects Res. 2017 Jul 3;109(11):857-859. doi: 10.1002/bdr2.1011. Epub 2017 Apr 12.
Unilateral lung agenesis is an uncommon congenital abnormality, with a lack of reported accurate incidence estimates. Prognosis is also uncertain, with older literature reporting poor outcomes.
The North of England register of congenital anomalies (Northern Congenital Abnormality Survey) records cases of congenital anomalies to mothers' resident in the region. We used the register to identify all patients with congenital lung agenesis born between 2004 and 2013 to calculate an accurate incidence estimate and report clinical outcomes with contemporary management.
Four patients with congenital lung agenesis were born during the study period, giving an estimated incidence in the North of England of 1.22 per 100,000 live births (95% confidence interval, 0.33-3.11). Two patients had associated congenital heart disease requiring corrective surgery, and one had musculoskeletal anomalies. All four patients are alive and well without a regular oxygen requirement.
Contrary to previous reports, the medium term outcomes in our patients have been good, even when lung agenesis is associated with other congenital anomalies. Long-term prognosis with modern management remains unknown, and the potential for the development of pulmonary hypertension remains a concern. Birth Defects Research 109:857-859, 2017. © 2017 Wiley Periodicals, Inc.
单侧肺发育不全是一种罕见的先天性异常，缺乏准确的发病率估计。预后也不确定，旧文献报告预后不良。
英格兰北部畸形登记处（北方先天性异常调查）记录了居住在该地区的母亲所生的先天性畸形病例。我们使用该登记处识别出所有在 2004 年至 2013 年间出生的先天性肺发育不全患者，以计算出准确的发病率估计，并报告与当代管理相关的临床结果。
在研究期间，有 4 名先天性肺发育不全患者出生，这使得英格兰北部的发病率估计为每 100,000 例活产儿中有 1.22 例（95%置信区间，0.33-3.11）。两名患者患有需要矫正手术的先天性心脏病，一名患者患有肌肉骨骼异常。所有 4 名患者均存活且无需定期吸氧，情况良好。
与以往的报告相反，我们患者的中期结果良好，即使肺发育不全与其他先天性异常有关。现代管理的长期预后仍不清楚，发展为肺动脉高压的潜在风险仍然存在。出生缺陷研究 109：857-859，2017。© 2017 Wiley Periodicals, Inc.
