Singh Sompal, Nigam Sonu, Khurana Nita, Agarwal Satish Kumar, Mandal Ashish Kumar
Department of Pathology, Maulana Azad Medical College, New Delhi.
Indian J Pathol Microbiol. 2004 Jan;47(1):29-32.
Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare anomaly of fetal development of terminal respiratory structures. We report three cases of congenital cystic adenomatoid malformation (CCAM) of lung Stocker's type I. All the cases presented with progressive neonatal respiratory distress. One case developed hyaline membrane because of ventilator therapy. No other congenital malformation was found in any of our cases. Two of our cases were suspected on antenatal ultrasonography. Routine prenatal ultrasonography has increased the frequency of prenatal diagnosis of congenital cystic lung malformation including CCAM. The pathogenesis, management and prognosis of CCAM are discussed along with a review of literature.
肺先天性囊性腺瘤样畸形(CCAM)是终末呼吸结构胎儿发育过程中的一种罕见异常。我们报告3例I型肺先天性囊性腺瘤样畸形(CCAM)。所有病例均表现为进行性新生儿呼吸窘迫。1例因机械通气治疗发生透明膜病。我们所有病例均未发现其他先天性畸形。我们的2例病例在产前超声检查时被怀疑。常规产前超声检查提高了包括CCAM在内的先天性肺囊性畸形的产前诊断率。本文结合文献复习讨论了CCAM的发病机制、治疗及预后。