Primary Fetal Lung Hypoplasia (PFLH): Imaging and Clinical Characteristics

Abstract

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.

Establishing Reference Intervals for Normal Fetal Lung Biological Parameters at 21–40 Weeks of Gestation in the Chinese Population: A Cross-Sectional Study

“…Triebwasser et al [2] conducted a systematic review of the value of two-dimensional ultrasound parameters in the evaluation of pulmonary hypoplasia, reporting that the TC, TC/AC, and lung area have clinical value in the diagnosis of pulmonary hypoplasia and that the lung area has excellent predictive accuracy (sensitivity 78-81%, specificity 75-100%). The Aafreen S [24] and Achiron R [25] studies reported that the right lung area was significantly smaller in patients with pulmonary dysplasia. Therefore, it is of great clinical value to establish the reference values of the lung area in the Chinese population.…”