Prenatal findings and differential diagnosis of scimitar syndrome and pulmonary sequestration

Abstract

Objectives: Scimitar syndrome and pulmonary sequestration (PS) have overlapping features. This accounts for postnatal descriptions of a 'sequestrated lung' in scimitar-syndrome patients. We review the similarities and differences in these two conditions, and the antenatal ultrasound findings that allow correct prenatal identification and differential diagnosis.

Methods: This was a retrospective analysis of prenatally diagnosed cases of scimitar syndrome or PS that underwent fetal echocardiography between January 1995 and November 2004.

Results: There were five cases of PS and six of scimitar syndrome. Right-sided mediastinal shift (ipsilateral relative to the affected lung) was the commonest indication for referral in scimitar syndrome whereas in sequestration, referral was because of hyperechogenic lung and contralateral mediastinal shift. Lung echogenicity was normal in scimitar syndrome but focally increased in PS. Abnormal, unobstructed pulmonary venous drainage was identified prenatally in four cases of scimitar syndrome. It was normal in three fetuses with sequestration but abnormal and obstructed in the other two cases complicated by hydrothorax. Abnormal systemic arterial supply to the affected lung was easily demonstrated in all fetuses with PS, but could not be shown prenatally in scimitar-syndrome fetuses. Postnatal embolization was warranted in one case with PS and four with scimitar syndrome, one each in the neonatal period.

Conclusion: Prenatally, scimitar syndrome and PS are clearly distinct entities based on lung echogenicity and laterality of mediastinal shift. Hyperechogenic lung and dextrocardia indicate the possibility of PS and scimitar syndrome, respectively. Complete delineation of the vascular pattern should be attempted in all, but is more challenging in scimitar syndrome. Obstructed venous return in PS may identify fetuses at risk of developing hydrothorax.