Kantor Navot, Wayne Carolyn, Nasr Ahmed
Department of Surgery, Children's Hospital of Eastern Ontario, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada.
Faculty of Medicine, University of Ottawa, Ottawa, Canada.
Pediatr Surg Int. 2018 Jun;34(6):613-620. doi: 10.1007/s00383-018-4264-y. Epub 2018 Apr 9.
For the approximately three quarters of patients with a prenatal diagnosis of congenital pulmonary airway malformation (CPAM) who are asymptomatic at birth, the chance of eventually developing symptoms is unknown. We sought to explore the natural history of asymptomatic CPAM.
We searched EMBASE, MEDLINE, and the first 50 results from Google Scholar. Studies describing the natural history of prenatally diagnosed, initially asymptomatic CPAM were included. For asymptomatic patients initially managed non-surgically, we tabulated the proportion who went on to develop symptoms as well as the median age at symptom development.
We included data from 19 retrospective studies on 353 patients. Of the 128 patients managed expectantly, 31 (24.2%) developed symptoms requiring surgical intervention. The median age at symptom development was 7.5 months (range 15 days-5 years).
The risk for developing respiratory symptoms exists with originally asymptomatic CPAM patients, but the exact risk is difficult to predict. Parents may be given the value of approximately 1 in 4 as an estimate of the proportion of asymptomatic CPAM patients who go on to develop symptoms, which will help them make an informed decision regarding the option of elective surgery.
对于出生时无症状的先天性肺气道畸形（CPAM）产前诊断患者中约四分之三的人而言，最终出现症状的几率尚不清楚。我们试图探究无症状CPAM的自然病史。
我们检索了EMBASE、MEDLINE以及谷歌学术搜索的前50个结果。纳入描述产前诊断、最初无症状的CPAM自然病史的研究。对于最初采用非手术治疗的无症状患者，我们列出了出现症状的患者比例以及症状出现的中位年龄。
我们纳入了来自19项关于353例患者的回顾性研究的数据。在128例接受观察等待的患者中，31例（24.2%）出现了需要手术干预的症状。症状出现的中位年龄为7.5个月（范围为15天至5年）。
最初无症状的CPAM患者存在出现呼吸道症状的风险，但确切风险难以预测。可为家长提供约四分之一的数值，作为对无症状CPAM患者中出现症状者比例的估计，这将有助于他们就是否选择择期手术做出明智决定。
