Cazeneuve C, Nihoul-Fékété C, Adafer M, Yassine B, Boury R, Wahhabi M, Lajarrige C, Dumez Y, Aubry M C, Moriette G
Pédiatrie et maternité, centre hospitalier, Laon, France.
Arch Pediatr. 1995 May;2(5):452-5. doi: 10.1016/0929-693x(96)81181-x.
Conjoined (siamese) twins represent a rare situation which may occur in 1 of every 50,000 births. A prenatal diagnosis usually leads to stopping pregnancy. We report a case with successful surgical separation at the age of 15 days.
Ultrasonographic examination at 20 weeks of gestation showed omphalopagus siamese joined at the abdomen from the xiphoid process to the umbilicus. Conjoined structures included liver. There was a multicystic right kidney in one twin, without other malformation. Karyotype was normal, 46XX. The mother refused interruption of her pregnancy. Both girls were born by cesarean section. Angiography, magnetic resonance imaging and intravenous urography confirmed the ultrasound examination. There was no cross circulation into the liver and the gastrointestinal tract was not conjoined. The twins were separated at 15 days of age with right heminephrectomy of the multicystic kidney. The girls are now 16 months old and are in very good health.
Prognosis of siamese twins depends on the nature of joined structures and presence of malformations. Ultrasonographic examination during pregnancy shows the possibility of surgical separation, which is performed by a double anesthetic and surgical pediatric team. Preoperative investigations must include MRI.
联体（暹罗）双胎是一种罕见情况，每50000例分娩中可能出现1例。产前诊断通常会导致终止妊娠。我们报告1例在出生15天时成功进行手术分离的病例。
妊娠20周时的超声检查显示胸腹联体双胎，从剑突至脐部在腹部相连。联体结构包括肝脏。其中一个双胎有一个多囊性右肾，无其他畸形。核型正常，46XX。母亲拒绝终止妊娠。两个女孩均通过剖宫产出生。血管造影、磁共振成像和静脉肾盂造影证实了超声检查结果。肝脏无交叉循环，胃肠道未联体。双胎在15日龄时进行了分离，对多囊性肾实施了右侧半肾切除术。这两个女孩现在16个月大，身体健康。
联体双胎的预后取决于联体结构的性质和有无畸形。孕期超声检查显示了手术分离的可能性，手术由小儿麻醉和手术团队实施。术前检查必须包括磁共振成像。
