Paria Pradipprava, Das Saptarshi, Gayen Sibnath, Ghosh Sibarjun, Basu Rajarshi, Bandyopadhyay Goutam
Indian J Chest Dis Allied Sci. 2016 Jan-Mar;58(1):53-7.
Congenital cystic adenomatoid malformation (CCAM) occurs secondary to the cystic adenomatous over-growth of terminal bronchioles, which results in the secondary inhibition of alveolar growth. In most of the cases, respiratory distress is the presenting feature during the neonatal period. In about 90% of patients, recurrent respiratory infections necessitating chest imaging reveal CCAM before the age of two years. We describe here the occurrence of congenital cystic adenomatoid malformation of right lung in a 12-year-old girl presenting with haemoptysis and hypovolaemic shock. She underwent right middle lobectomy; and histopathological examination confirmed the diagnosis. She has been doing well on follow-up.
先天性囊性腺瘤样畸形(CCAM)继发于终末细支气管的囊性腺瘤样过度生长,这会导致肺泡生长的继发性抑制。在大多数情况下,呼吸窘迫是新生儿期的主要表现特征。在约90%的患者中,因反复呼吸道感染而进行胸部影像学检查时,在两岁前发现CCAM。我们在此描述一名12岁女童出现咯血和低血容量性休克,诊断为右肺先天性囊性腺瘤样畸形。她接受了右肺中叶切除术;组织病理学检查确诊。随访期间她情况良好。