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Primary Fetal Lung Hypoplasia (PFLH): Imaging and Clinical Characteristics
Reuven Achiron1,2, Yinon Gilboa1,2, Yishay Salem3,2
1Obstetrics and Gynecology, Sheba Medical Center Tel-Hashomer, Ramat Gan, Israel.
Insights
Primary fetal lung hypoplasia (PFLH) is a rare, often lethal condition. Bilateral PFLH is typically fatal, while unilateral PFLH has a variable prognosis depending on associated anomalies.
Area of Science:
- Medical Imaging
- Pediatric Pathology
- Fetal Medicine
Background:
- Primary fetal lung hypoplasia (PFLH) is a rare congenital anomaly.
- Accurate diagnosis and prognostic assessment are crucial for affected neonates.
Purpose of the Study:
- To characterize the in utero and postnatal imaging and clinical features of PFLH.
- To identify key factors influencing the prognosis of PFLH.
Main Methods:
- Retrospective review of 12 fetuses and neonates diagnosed with PFLH over an 11-year period.
- Analysis of prenatal sonography, postnatal MRI, CTA, and histological findings.
Main Results:
- 12 cases of PFLH identified: 4 bilateral and 8 unilateral (7 right, 1 left).
- Bilateral PFLH cases had poor outcomes (termination or early death).
- Unilateral right PFLH cases frequently associated with Scimitar syndrome (5/7) and gastrointestinal tract anomalies (2/7).
Conclusions:
- Bilateral PFLH is a lethal condition.
- Unilateral PFLH prognosis varies, with associated anomalies like Scimitar syndrome and GIT issues being significant factors.
- Evaluation of lung vascularity and associated anomalies is critical for prognosis in PFLH.
Abstract:
Purpose To describe in utero and postnatal imaging and clinical characteristics of primary fetal lung hypoplasia (PFLH). Methods A retrospective review of fetuses and neonates diagnosed in one academic tertiary center during an eleven-year period. Results 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. Conclusion 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.
