Mendes J, Martins V, Gonçalves M
Unidade de Cirurgia Pediátrica, Hospital de Santa Maria, Lisboa.
Acta Med Port. 1998 Nov;11(11):947-52.
From September 1989 to December 1994, 12 children with congenital bronchopulmonary cystic disease were operated in the Department of Pediatric Surgery, Santa Maria Hospital. Four cases of bronchogenic cyst (BC), 2 of cystic adenomatoid malformation (CAM), 3 of pulmonary sequestration (PS), and 3 of congenital lobar emphysema (CLE) were found. Age ranged between 9 days and 10 years without predominance of gender. Seven were younger than one year of age, of which 5 were less than 6 months old at the time of surgery. The CAT scan was most useful in the diagnosis and follow-up of these children. The review of these cases helped evaluate our experience in the diagnosis and surgical approach for these congenital malformations. Successful treatment depends on early diagnosis and the planning of therapeutic priorities, based on the embryological and pathophysiological knowledge at these anomalies. The embryological mechanisms implicated in the development are discussed and the results of surgical treatment are presented.
1989年9月至1994年12月，圣玛丽亚医院小儿外科为12例先天性支气管肺囊性疾病患儿实施了手术。其中发现4例支气管囊肿（BC）、2例囊性腺瘤样畸形（CAM）、3例肺隔离症（PS）和3例先天性大叶性肺气肿（CLE）。年龄范围在9天至10岁之间，无性别优势。7例年龄小于1岁，其中5例在手术时年龄小于6个月。计算机断层扫描（CAT）对这些患儿的诊断和随访最为有用。对这些病例的回顾有助于评估我们在这些先天性畸形的诊断和手术方法方面的经验。成功的治疗取决于早期诊断以及基于对这些异常的胚胎学和病理生理学知识制定治疗优先级计划。本文讨论了与发育相关的胚胎学机制，并展示了手术治疗结果。
