Canals-Riazuelo J, Boix-Ochoa J, Peiro J L, Ezzedine M, Cobos Barroso N, Liñan Cortés S, Torán Fuentes N
Departamento de Cirugía Pediátrica, Hospital Universitario Materno-Infantil Vall d'Hebrón, Barcelona.
Cir Pediatr. 1994 Apr;7(2):92-6.
We present our experience of 26 cases of cystic adenomatoid malformations of the lung (CAM), treated in the Department of Pediatric Surgery in our hospital between 1967-1991. There were two clinical pictures: one neonatal severe respiratory distress and successive repeated pulmonary infection appearing after the patient's first year of life and requiring both urgent diagnosis and treatment. Embryological development determines the pathologic classification of this entity in 3 types. Basic examinations by image are analyzed, bearing in mind their diagnostic value and the patterns they show. After analysing all the conditioning factors, no explanation has been found to the different course that this affectation (< 1 month and > 1 year of age respectively). Normally, neonatal mortality is closely related to other malformations, particularly to cardiovascular ones. Differential diagnosis is very important in the neonatal period, especially with regard to diaphragmatic hernia, lobar emphysema and pulmonary cysts. All these cases have been verified and classified by means of a pathologic study, which has shown the need for surgical operation. In the follow up of the patients no alteration has been noticed in the pulmonary function.
我们介绍了1967年至1991年间在我院小儿外科治疗的26例肺囊性腺瘤样畸形（CAM）的经验。有两种临床表现：一种是新生儿严重呼吸窘迫，另一种是患者1岁后出现反复肺部感染，需要紧急诊断和治疗。胚胎发育决定了该疾病的病理分类为3种类型。分析了影像学的基本检查，同时考虑其诊断价值及所显示的模式。在分析所有影响因素后，尚未找到对该疾病（分别为<1个月和>1岁）不同病程的解释。通常，新生儿死亡率与其他畸形密切相关，尤其是心血管畸形。在新生儿期鉴别诊断非常重要，特别是与膈疝、大叶性肺气肿和肺囊肿的鉴别。所有这些病例均通过病理研究得到证实和分类，结果表明需要进行手术治疗。在对患者的随访中，未发现肺功能有改变。
