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目的探讨双胎胸腹联体伴法洛四联症(TOF)婴儿早期外科手术治疗的可行性及其效果。方法术前经X线片、CT、MRI和B超等检查确诊,该联体婴儿共用1个肝脏,心包、膈肌、胸骨下段及第8～10前肋相连,胸膜紧贴,有2个心脏(B婴伴TOF)、各具独立的消化道和胆道系统。经积极术前准备,在营养和肝功能改善后,于出生第43天在多科协作下进行了联体分离手术。手术从一侧胸骨交界处至脐切开皮肤和腹壁进腹探查,术中见腹腔中有各自的消化道,共同肝相连的面积约6cm×5cm,将二婴胆总管作2条纵形线作为离断线,采用局部血流阻断共用肝离断方法,用超声刀进行肝脏分离。胸腔中见二婴儿的心脏共用一个心包,予以切开,直接缝合。胸骨、肋骨亦分别切开。伴TOF的B婴于分体术后0.5a实施Ⅱ期心脏畸形纠治术。术后随访患儿生长发育情况。结果术后二婴儿皆存活,二婴儿于术后7h清醒,分别于术后24和26h拔除气管插管,术后二婴儿分别称体质量,A婴4100g,B婴3900g。B婴0.5a后的心脏畸形纠治手术顺利,恢复良好。术后随访3a,二婴儿生长发育与同龄儿童接近。结论联体婴儿术前经正确评估、正确选用麻醉和手术方法、术后加强监护是手术成功的关键,分体术后0.5a后再行Ⅱ期心血管畸形纠治术是可行的。
Objective To investigate the feasibility and effect of early surgical treatment of infants with twin-thoraco-abdominal combined tetralogy of Fallot (TOF). Methods The preoperative diagnosis was confirmed by X-ray, CT, MRI and B-ultrasound. The conjoined infant shared 1 liver, pericardium, diaphragmatic muscle, lower sternum and anterior ribs of 8-10. The pleura was close and had 2 hearts (B infant with TOF), each with independent digestive tract and biliary system. After positive preoperative preparation, with improved nutrition and improved liver function, we performed combined de-duplication surgery on the 43rd day of life under multidisciplinary collaboration. Surgery from one side of the sternal junction to the umbilical incision of the skin and abdominal wall into the abdomen exploration, see intraperitoneal surgery have their own digestive tract, the common liver connected area of about 6cm × 5cm, the two baby bile duct for two vertical lines As a disconnection, local blood flow was used to block the common liver disconnection method, and an ultrasonic knife was used to separate the liver. See the chest in the second baby’s heart share a pericardium, to be cut, direct suture. Sternum, ribs were cut separately. B with TOF infants in 0.5a after the implementation of phase II correction of cardiac malformations. Postoperative follow-up of children’s growth and development. Results Two infants survived after operation. The two infants were awake at 7h after operation. The tracheal intubation was withdrawn at 24 and 26 h after operation. The two infants were respectively weighed, 4100 g A and 3900 g B infants. B infants after 0.5a correction of heart malformation surgery smoothly and recover well. After follow-up 3a, two infants and young children close to their growth and development. Conclusion The correct evaluation of the preoperative baby, the correct choice of anesthesia and surgical methods, postoperative intensive care is the key to the success of surgery, sub-0.5a after reoperation Ⅱ cardiovascular malformations is feasible.