Department of Pediatrics, University of California, San Francisco, CA, USA.
Ultrasound Obstet Gynecol. 2010 Jan;35(1):113-6. doi: 10.1002/uog.7520.
Primary pulmonary hypoplasia is a rare, usually lethal, condition presenting only after birth without other congenital abnormalities. We describe the first case of fraternal twins diagnosed prenatally with primary pulmonary hypoplasia. Both had diffuse hypoplasia of the pulmonary arteries initially identified by fetal echocardiography and confirmed at autopsy following termination. These cases permit examination of the histopathology of this disease in the fetal stage of development.
原发性肺发育不全是一种罕见的、通常致命的疾病,仅在出生后出现,无其他先天性异常。我们描述了首例经产前诊断的原发性肺发育不全的同卵双胞胎。这对双胞胎最初通过胎儿超声心动图发现肺动脉弥漫性发育不全,并在终止妊娠后尸检证实。这些病例允许在胎儿发育阶段检查这种疾病的组织病理学。