Obstetrics and Gynecology, Sheba Medical Center Tel-Hashomer, Ramat Gan, Israel.
Tel-Aviv University, Sackler Faculty of Medicine, Tel-Aviv, Israel.
Ultraschall Med. 2017 Jun;38(3):301-309. doi: 10.1055/s-0042-124361. Epub 2017 Jun 14.
To describe in utero and postnatal imaging and clinical characteristics of primary fetal lung hypoplasia (PFLH). A retrospective review of fetuses and neonates diagnosed in one academic tertiary center during an eleven-year period. 12 cases of PFLH were identified. 4 were bilateral and 8 had unilateral involvement. Prenatal sonographic characteristics, postnatal magnetic resonance imaging (MRI), computerized tomographic angiography (CTA), and histologic findings are described. 3 of the 4 bilateral cases were evaluated during fetal live. 2 were terminated and 2 died shortly after delivery. Among the 8 cases with unilateral PFLH, 7 involved the right lung and 1 the left lung. In fetuses with right hypoplasia, 5 showed characteristic features of Scimitar syndrome, while associated gastrointestinal tract (GIT) anomalies were presented in 2 cases. In this group 3 were born alive and the other 5 were terminated. Primary PFLH is a rare anomaly that lethal in its bilateral form and with variable prognosis in its unilateral variant. Targeted evaluation of lung vascularity and exclusion of associated anomalies, especially of the GIT, are important prognostic factors.
描述原发性胎儿肺发育不全（PFLH）的宫内和产后影像学及临床特征。对一个学术性三级中心在十一年期间诊断的胎儿和新生儿进行回顾性研究。发现 12 例 PFLH。其中 4 例为双侧，8 例为单侧受累。描述了产前超声特征、产后磁共振成像（MRI）、计算机断层血管造影（CTA）和组织学发现。4 例双侧病例中有 3 例在胎儿期进行了评估。其中 2 例终止妊娠，2 例分娩后不久死亡。在 8 例单侧 PFLH 中，有 7 例累及右肺，1 例累及左肺。在右肺发育不全的胎儿中，有 5 例表现出弯刀综合征的特征性表现，而在 2 例中存在相关的胃肠道（GIT）异常。在这一组中，有 3 例存活分娩，5 例终止妊娠。原发性 PFLH 是一种罕见的异常，双侧形式是致命的，单侧变异的预后不同。对肺血管性的靶向评估和排除相关异常，特别是胃肠道异常，是重要的预后因素。
