1Center for Maternal, Fetal and Neonatal Medicine, Saitama Medical Center, Saitama Medical University, 2Department of Obstetrics and Gynecology, Shinshu Ueda Medical Center, 3Department of Obstetrics and Gynecology, Saitama Medical Center, Saitama Medical University
Introduction: We report a case that presented with polyhydramnios and resulted in a diagnosis of a very rare anomaly, i.e., otocephaly, at 29 weeks of pregnancy. Case: The patient was 29 years old and gravida 0. She had asthma and was being treated with inhalants including fluticasone propionate. She experienced nausea and was referred to our center from her first physician owing to the polyhydramnios at 29 weeks’ pregnancy. After she was hospitalized for preterm labor, three-dimensional ultrasound precisely demonstrated craniofacial anomalies including agnathia, protuberance of the nose-mouth fusion, and synotia. The fetus was diagnosed with otocephaly. After informed consent was obtained, we decided not to treat her or her baby aggressively. She left the hospital at 29 weeks and 6 days of pregnancy. A male newborn weighing 1,304 g was delivered at 30 weeks’ gestation. Death occurred spontaneously 21 minutes after birth. Discussion: Otocephaly has been reported to occur in fewer than 1 in 70,000 births. It has been reported that theophylline, beclomethasone, and salicylates are causes of otocephaly, but the anomaly strikes suddenly. Three-dimensional ultrasound is useful, and screening at around 20 weeks of pregnancy is recommended for early prenatal diagnosis.