Isolated unilateral pulmonary agenesis and other fetal thoracic anomalies

摘要

IMPORTANCE: Although the diagnostic workup and management regimens for many of the common fetal thoracic lesions have been well described, the understanding of pulmonary agenesis is more limited. A better understanding of the published reports of this condition is essential to provide proper care for these complicated pregnancies. OBJECTIVE: The aims of this study were to provide a better understanding of the difficulties in the diagnosis and management of fetal thoracic lesions and to consolidate what is known about unilateral pulmonary agenesis. EVIDENCE ACQUISITION: We performed a review of the English medical literature covering the last 20 years (1993-2013) in PubMed, MEDLINE, and MD Consult using search terms pulmonary agenesis, pulmonary aplasia, unilateral pulmonary agenesis, unilateral pulmonary aplasia, lung agenesis, lung aplasia, unilateral lung agenesis, and unilateral lung aplasia. RESULTS: Prenatal diagnosis of pulmonary agenesis and other fetal thoracic lesions can be particularly challenging given that many anomalies have similar appearance on ultrasound. Fetal magnetic resonance imaging has been used in several of the reported cases to clarify the diagnosis. Once confirmed, there are several important prognostic factors to consider in the management of unilateral pulmonary agenesis. Poor prognostic factors include right-sided agenesis, the presence of genetic abnormalities, and other associated congenital anomalies. CONCLUSION AND RELEVANCE: Fetal magnetic resonance imaging can be a useful imaging modality when the diagnosis is unclear after ultrasound imaging. The management of cases with a poor prognosis should be guided by the nature of the associated anomalies. Cases of isolated pulmonary agenesis have an overall good prognosis and can be managed conservatively during pregnancy, with consideration of delivery at a tertiary care facility in case postnatal respiratory issues arise. TARGET AUDIENCE: Obstetricians and gynecologists, family physicians LEARNING OBJECTIVES: After completing this CME activity, physicians should be better able to distinguish unilateral pulmonary agenesis from other thoracic congenital anomalies, identify the main predictors of prognosis for unilateral pulmonary agenesis, and identify the congenital anomalies that can be associated with unilateral pulmonary agenesis.