Objective: The purpose of this study is to describe the imaging findings in a group of fetuses with suspected agenesis of the septum pellucidum (ASP) and to evaluate their clinical outcome.Methods: This is a retrospective multicenter study on a cohort of fetuses diagnosed with suspected ASP, between 2008 and 2017. The records of each patient, including ultrasound (US) and magnetic resonance studies, were reviewed and compared with the postnatal findings.Results: Forty-seven patients were included in the study at a mean gestational age of 26.6 weeks. In 17 patients, the ASP was considered isolated. Fourteen patients delivered live-born, and all 14 are developing normally. Three were lost to follow-up.Twenty-four patients had associated malformations involving the central nervous system (CNS); 13 were delivered (normal development [5], abnormal [6] and no follow-up [2]). Nine patients opted for termination, and two pregnancies were lost to follow-up.Six patients had non-CNS associated findings, two were delivered with normal neurological development and four had a termination.Conclusions: Isolated ASP is usually associated with a favorable outcome; but in the presence of associated malformations, there is at least a 50% risk of abnormal development. Current imaging techniques can provide an accurate prognosis in cases when ASP appears isolated.

HighlightsThe prevalence of rudimentary uterine horn ectopic pregnancy is very low (1 in 76 000–150 000 pregnancies).Early diagnosis of a rudimentary horn pregnancy is the key to successful management.The medical treatment may be a successful adjuvant therapy to surgical removal in asymptomatic women.

The accuracy of ultrasound in the prediction of BWD is limited, particularly in pregnancies without fetal growth restriction. Clinical decisions should not rely on BWD alone.

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