Prenatal findings of serpentine-like syndrome with congenital intrathoracic stomach: differential diagnosis from congenital diaphragmatic hernia
Prenatal findings of serpentine-like syndrome with congenital intrathoracic stomach: differential diagnosis from congenital diaphragmatic hernia
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DOI：
10.1007/s10396-018-0896-8
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发表时间：
2019-04-01
影响因子：
1.8
通讯作者：
Kimura, Tadashi
中科院分区：
文献类型：
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作者：
Mimura, Kazuya;Endo, Masayuki;Kimura, Tadashi
先天性脊柱裂和臂状食道继发胸内胃是一种非常罕见的情况，被描述为蛇纹样综合征。我们报告了一例妊娠18周的胎儿蛇纹样综合征的产前表现。胃被完全抬高到胸部，但肝脏保持其正常位置。我们最初的诊断是孤立性的左侧先天性横隔疝(CDH)。在怀孕20周时，妊娠终止，一名重338克的男性新生儿经阴道分娩。尸检结果显示食道极短，胃位于后纵隔，是先天性食道裂孔疝的结果。从颈椎到胸椎发现了一例严重的脊椎裂。先天性胸腔内胃合并蛇纹样综合征与单纯CDH相比，预后极差。仅靠超声很难做出明确的诊断；然而，不典型的表现，如正常定位的肝脏可见整个胸腔内胃的存在，可能值得进一步探讨。出现类似病例的产科医生和新生儿临床医生应该注意这些产前检查结果的重要性和结局的差异，以确保为父母提供适当的咨询。
Congenital rachischisis and brachioesophagus with secondary intrathoracic stomach, which is described as serpentine-like syndrome, is a very rare condition. We report the prenatal findings of serpentine-like syndrome in a fetus at 18weeks of gestation. The stomach was entirely elevated into the thoracic area, but the liver retained its normal position. Our initial diagnosis was isolated left congenital diaphragmatic hernia (CDH). At 20weeks of gestation, the pregnancy was terminated, and a male newborn weighing 338g was vaginally delivered. The autopsy findings revealed an extremely short esophagus, and the stomach was located in the posterior mediastinum as a result of congenital esophagus hiatal hernia. A severe case of rachischisis was identified from the cervical to thoracic vertebrae. The prognosis of congenital intrathoracic stomach associated with serpentine-like syndrome is extremely poor compared to the prognosis of isolated CDH. It is difficult to make a definite diagnosis by ultrasound alone; however, atypical findings, such as the presence of the entire intrathoracic stomach despite the normally located liver, may warrant further exploration. Obstetricians and neonatal clinicians presented with similar cases should be alerted to the significance of these prenatal findings and the differences in outcomes to ensure appropriate counseling of parents.