A review of pregnancies complicated by congenital sacrococcygeal teratoma in the West Midlands region over an 18-year period: population-based, cohort study

Objectives To describe the epidemiology and outcomes of sacrococcygeal teratoma (SCT) and identify the factors affecting prognosis in a population‐based cohort. Methods Analyses of fetal SCTs from a population‐based congenital anomaly register between 1995 and 2012, linked to regional datasets. A systematic literature review was performed for published studies on perinatal SCT (1995 to 2012). Results Thirty‐six confirmed SCT cases were identified, giving a total prevalence of 0.30 per 10 000 births (95%CI 0.20–0.39). Twenty‐three cases (63.9%) were diagnosed prenatally. There were six false positive prenatal diagnoses, and the positive predictive value of ultrasound for SCT was 79.3%. Secondary complications in prenatally diagnosed cases were polyhydramnios (27.2%), fetal hydrops (9.1%) and rapidly growing tumour (54.0%). The perinatal (PNMR) and infant mortality rates were 333.3 per 1000 births and 285.7 per 1000 live births, respectively. All stillbirths and infant deaths occurred in cases diagnosed prenatally. Factors associated with higher PNMR in registerable births were solid, vascular tumour composition (1000), polyhydramnios (667), premature delivery (667) and rapidly growing tumour (454). In the systematic review, prenatal hydrops fetalis and prematurity were the most morbid association in SCT. Conclusion Prenatal ultrasound was relatively sensitive and specific in diagnosing SCT with good survival rates in live‐born cases. © 2015 John Wiley & Sons, Ltd. What's already known about this topic? Sacrococcygeal teratomas (SCTs) are the most common fetal tumours. The presence of hydrops, polyhydramnios and rapidly growing tumour are associated with high perinatal mortality and morbidity. What does this study add? This population‐based study gives insight into the total prevalence of SCT and outcomes in prenatal versus postnatally detected anomalies. This allows comparison between the findings of this population‐based cohort study and those of a systematic review of the international literature over the same time period of 1995–2012. MRI imaging allowed additional information relating to Altman typing of the prenatal SCT.