41 Duodenal atresia and sudden fetal death. Running head: Prenatal diagnosis of fetal duodenal atresia and risk of fetal adverse outcome Giuseppe Trojano 1 , Lorella Battini 1 , Pietro Bottone 1 , Veronica Tosi 1 , Chiara Nanini 1 , Arianna Carmignani 1 , Maria Giovanna Salerno 1 1 Azienda Ospedaliera Universitaria Pisana Division of Obstetrics and Gynaecology Pisa, Italy ABSTRACT Duodenal atresia occurs in approximately 1:10.000 live births. The outcome of congenital duodenal obstructions, in terms of mortality rate, has improved over the last decades, mainly attributable to the improvement in the quality of prenatal diagnosis and neonatal intensive care. Nevertheless, several cases of sudden intrauterine fetal death (IUFD) in fetuses with duodenal obstruction have been described in the Literature. We report a case of a fetus with duodenal atresia and sudden IUFD at 32 weeks’ gestation and the review of literature. We speculate that the fetal demise was due to a vaso-vagal reaction initiated by esophageal dysmotility. This report is intended to describe the features of duodenal obstruction on prenatal ultrasonography and alert the obsterician to the possible association with sudden IUFD. Key Words: Duodenal atresia SOMMARIO L’atresia dudenale ha un’incidenza di circa 1:10000 nati vivi. L’outcome dei pazienti con ostruzione duodenale, in termini di tasso di mortalità, risulta migliorato nelle ultime decadi, soprattutto grazie al miglioramento della qualità della diagnosi prenatale e dell’assistenza neonatale. Diversi casi di morte intrauterina fetale improvvisa (IUFD) in feti con ostruzione duodenale sono stati descritti in letteratura. Riportiamo il caso di un feto con atresia duodenale e improvvisa IUFD a 32 settimane e la revisione della letteratura. Supponiamo che la morte fetale sia da attribuire ad una reazione vaso-vagale iniziata con alterazione della peristalsi esofagea. Questo report si propone di descrivere i segni ecografci di ostruzione duodenale al fne di allertare l’ostetrico della possibile associazione con la morte improvvisa fetale. Parole chiave: Atresia duodenale CASE REPORT A 32-year-old Caucasian woman with a spontaneous physiologic pregnancy and unremarkable obstetric history was referred to our Unit for a third trimester routine scan. A fetal duodenal obstruction was suspected by sonographic examination at 32 weeks’ gestation. Detailed fetal survey showed a gastric “double bubble” sign, suggestive for fetal duodenal stenosis or atresia (Figure 1-up), associated with polyhydramnios (deepest vertical pocket of 10 cm) and fetal vomiting-like movements. No other fetal anomalies were detected. The fetal growth was regular and the umbilical artery Doppler- velocimetry was normal (PI 0.90). After the family had been counseled, a further sonographic examination was performed two days later, showing the fetal demise. A stillborn 1850 g baby-girl was vaginally delivered 4 days later, following pharmacological induction of labor with vaginal prostaglandins. Fetal karyotype on a sample from the neonate skin was found to be normal. Fetal autopsy and histological examination Figure 1. Ultrasound: Double Bubble sign (up); Pathology specimen: Atresia of the distal portion of the duodenum (down). of the placenta were performed. Atresia of the distal portion of the duodenum, due to failed recanalization of the lumen, was detected 3 cm from the pylorus (Figure 1-down). No pathological Correspondence to: giustroiano@gmail.com Copyright 2014, Partner-Graf srl, Prato