Yan Bin,Yu Jiakang,Zhong Wei,et al.Preoperative evaluation strategy and operative opportunity of 4 cases of thoracoabdominal conjoined infant[J].Journal of Clinical Pediatric Surgery,2019,18(04):304-309,314.[doi:10.3969/j.issn.1671-6353.2019.04.011]
4例胸腹联体婴儿术前评估策略及手术时机探讨
- Title:
- Preoperative evaluation strategy and operative opportunity of 4 cases of thoracoabdominal conjoined infant
- Keywords:
- Twins; Conjoined/SU; Heart Defects; Congenital/DI
- 分类号:
- R729;R619
- 摘要:
- 目的 总结胸腹联体婴的治疗经验,探讨联体婴儿的术前评估要点及治疗策略。方法 回顾2009—2016年广州市妇女儿童医疗中心收治的4例胸腹联体婴儿的临床资料,分析影像学评估策略,并发畸形对手术时机的影响,手术分离技巧以及胸腹联体婴儿诊治流程。结果 联体婴儿男童2例,女童2例,入院后均接受全面评估,4例均诊断为胸腹联体畸形。4例联体儿体桥内均可见肝脏相连,但有各自独立肝门、胆管及胆囊;胃肠道系统、泌尿系统完全独立无异常;均合并严重的先天性心脏畸形。1例因共用单个四腔心脏,放弃治疗后死亡;其余3例因循环不稳定导致肺炎,符合提前行分离手术指征,经多学科会诊后制定治疗方案;应用带蒂皮瓣转移联合补片方法修补体桥分离后的腹壁缺损,手术平均年龄为(35±6)d。成功分离6名个体,4名患儿术后出现伤口愈合不良,1名患儿于分离术后25 d行心脏畸形矫治术,平均住院时间(79±9)d。随访6个月至9年,1名于出院后半年因心脏畸形并发症死亡,其余5名患儿存活。结论 通过对胸腹联体婴进行全面评估,多学科协作,严格掌握手术提前和急诊手术指征,制定精细体桥分离方案和序贯治疗并发畸形,可提高胸腹联体婴双胎存活率。
- Abstract:
- Objective To summarize the experiences of treating thoracoabdominal conjoined infants and discuss the preoperative evaluations and treatment strategies of conjoined infants.Methods The clinical data of 4 hospitalized cases of thoracoabdominal conjoined infants from 2009 to 2016 were reviewed.The imaging evaluation strategy,influence of complicated malformation on operative opportunity,surgical separation techniques and diagnosis and treatment process of thoracoabdominal conjoined infants were analyzed.Results There were 2 male and 2 female conjoined babies.All of them were evaluated after admission.All four cases were all diagnosed as thoracoabdominal conjoined deformity.Liver connections in body bridges were independent of hepatic portal,bile duct and gallbladder.And gastrointestinal tract system and urinary tract were completely independent and showed no abnormalities.All of them were combined with severe congenital heart malformations.One case died after giving up treatment because of sharing a single four-chamber heart.Another 3 cases had pneumonia due to unstable circulation and fulfilled the indication of early separation.Treatment plan was formulated after multi-disciplinary (MDT) consultation.Abdominal wall defect was repaired after body bridge separation by pedicle flap transfer plus patching.The mean operative age was (35±6) days.Six patients were successfully separated and 4 children had poor wound healing after operation.One patient underwent cardiac deformity correction at 25 days after separation.The average hospitalization time was (79±9) days.One patient died of complications of cardiac malformation and another 5 children survived during a follow-up period of 6-9 years after discharge.Conclusion The survival rate of thoracoabdominal conjoined twins may be improved by comprehensive evaluations of thoracoabdominal conjoined infants,multidisciplinary cooperation,strict grasp of early and emergency surgical indications,establishment of fine body-bridge separation scheme and sequential treatment of concomitant malformations.
参考文献/References:
1 Spitz L,Kiely EM.Conjoined twins[J].Current Paediatrics,2001,11(5):386-389.DOI:10.1054/cupe.2001.0216.
2 邹华新,李林.胸腹联体畸形的诊断与治疗进展[J].临床小儿外科杂志,2007,6(1):57-59.DOI:10.3969/j.issn.1671-6353.2007.01.024. Zou XH,Li L.Clinical achievements in diagnosis and therapy of omphalopagus conjoined twins[J].J Clin Ped Sur,2007,6(1):57-59.DOI:10.3969/j.issn.1671-6353.2007.01.024.
3 Richardson RJ,Applebaum H,Taber P,et al.Use of magnetic resonance imaging in planning the separation of omphalopagus conjoined twins[J].J Pediatr Surg,1989,24(7):683.
4 张金哲,潘少川,黄澄如.实用小儿外科学(上册)[M].浙江科学技术出版社,2003:251-261. Zhang JZ,Pan SC,Huang CR.Clin Ped Sur,2007,Volume One,2003[J].Press of Zhejiang Science and Technology,2003:251-261.
5 Spencer R.Theoretical and analytical embryology of conjoined twins:Part Ⅱ:Adjustments to union[J].Clinical Anatomy,2000,13(2):97-120.DOI:10.1002/1098-2353(200103)14:2<165::AID-CA1027>3.0.CO;2-S.
6 Khan ZH,Hamidi S,Miri SM.Craniopagus,Laleh and Ladan twins,sagital sinus[J].Turk Neurosurg,2007,17(1):27-32 DOI:10.1108/17505931211241369.
7 Campbell S,Theile R,Stuart G,et al.Separation of craniopagus joined at the occiput.Case report[J].J Neurosurg,2002,97(4):983-987.DOI:10.3171/jns.2002.97.4.0983.
8 Tsai FD.Ancient Chinese medical ethics and the four principles of biomedical ethics[J].J Med Ethics,1999,25(4):315-321.DOI:10.1136/jme.25.4.315.
9 王文献,戴书华,孙清荣,等.影像学综合评估联体婴儿分离术的意义[J].中国医学影像学杂志,2002,10(3):161-163.DOI:10.3969/j.issn.1005-5185.2002.03.001. Wang WX,Dai SH,Sun QR,et al.Imaging comprehensive evaluation for preoperative planning for surgical separation of conjoined twins[J].Chinese J Med Imaging,2002,10(3):161-163.DOI:10.3969/j.issn.1005-5185.2002.03.001.
10 Shi CR,Cai W,Jin HM,et al.Surgical management to conjoined twins in Shanghai area[J].Pediatr Surg Int,2006,22(10):791-795.DOI:10.1007/s00383-006-1745-1.
11 Martínezfrías ML,Bermejo E,Mendioroz J,et al.Epidemiological and clinical analysis of a consecutive series of conjoined twins in Spain[J].Pediatr Surg,2009,44(4):811-820.DOI:10.1016/j.jpedsurg.2008.07.002.
12 De Ugarte DA,Boechat MI,Shaw WW,et al.Parasitic omphalopagus complicated by omphalocele and congenital heart disease[J].J Pediatr Surg,2002,37(9):1357-1358.DOI:10.1016/j.jpedsurg.2008.07.002.
13 Spencer R,Robichaux WH,Superneau DW,et al.Unusual cardiac malformations in conjoined twins:thoracopagus twins with conjoined pentalogy of Cantrell and an omphalopagus twin with atretic ventricles[J].Pediatr Cardiol,2002,23(6):631-638.DOI:10.1007/s00246-002-0162-z.
14 Spitz L,Kiely EM,Pierro A.Conjoined Twins[M]//Pediatric Surgery.Elsevier Inc.2012:1725-1738.
15 Walton JM,Gillis DA,Giacomantonio JM,et al.Emergency separation of conjoined twins[J].J Pediatr Surg,1991,26(11):1337-1340.DOI:10.1016/0022-3468(91)90615-Z.
16 杨彤翰,李靖,黄小兵,等.联体畸形婴儿的手术时机与共用肝的分离方法[J].中华肝胆外科杂志,2010,16(3):161-163.DOI:10.3760/cma.j.issn.1007-8118.2010.03.001 Yang TH,Li J,Huang XB,et al.Operative timing and methods of separation surgery for joined liver of conjoined twins:a report of 3 cases[J].Chin J Hepatobiliary Surg,2010,16(3):161-163.DOI:10.3760/cma.j.issn.1007-8118.2010.03.001.
17 余家康,夏慧敏,钟微,等.2例胸腹联体婴成功分离的临床经验总结[J].中国普外基础与临床杂志,2011,18(7):741-744.Yu JK,Xia HM,Zhong W,et al.Clinical experience on separation surgery for thoracoventropagus conjoined twins:a report of 2 cases[J].Chin J Bases Clin General Surg,2011,18(7):741-744.
18 Kompanje EJ.The first successful separation of conjoined twins in 1689:some additions and corrections[J].Twin Res,2004,7(6):537-541.DOI:10.1375/1369052042663760.
19 孙家明,阮庆兰,孙宗全,等.胸腹联体婴儿分离术二例[J].中华外科杂志,2007,45(9):623-625.DOI:10.3760/j.issn:0529-5815.2007.09.014. Sun JM,Ruan QL,Sun ZQ,et al.Separation of thoracoventropagus conjoined twins:2 cases report[J].Chin I Surg,2007,45(9):623-625.DOI:10.3760/j.issn:0529-5815.2007.09.014.
20 邹婵娟,李碧香,高喜容,等.联体双胎分离手术4例[J].临床小儿外科杂志,2012,11(6):404-408.DOI:10.3969/j.issn.1671-6353.2012.06.002. Zou CJ,Li BX,Gao XR,et al.Experience of surgical treatment of conjoined twins-4 cases report[J].J Clin Ped Sur,2012,11(6):404-408.DOI:10.3969/j.issn.1671-6353.2012.06.002.
21 Karpelowsky JS,Millar AJ.Porcine dermal collagen (Perma-col) for chest and abdominal waIl reconstruction in thoracoomphaIopagus conjoined twin separation[J].Pediatr Surg Int,2010,26(3):315-318.DOI:10.1007/s00383-009-2501-0.
22 Fishman SJ,Puder M,Geva T,et al.Cardiac relocation and chest wall reconstruction after separation of thoracopagusconjoined twins with a with a single heart[J].J Pediatr Surg,2002,37(3):515-517.DOI:10.1053/jpsu.2002.30867.
23 曹霖,汪晓东,李立.多学科协作诊治模式的会诊流程探讨(一)[J].中国普外基础与临床杂志,2007,14(3):343-345.DOI:10.3969/j.issn.1007-9424.2007.03.030. Cao L,Wang XD,Li L.Consultation model of multi-disciplinary team for colorectal cancer.Chin J Bases Clin General Surg,2007,14(3):343-345.DOI:10.3969/j.issn.1007-9424.2007.03.030.
相似文献/References:
[1]邹婵娟 李碧香 高喜容. 联体双胎分离手术4例[J].临床小儿外科杂志,2012,11(06):404.
备注/Memo
收稿日期:2018-05-08。
基金项目:广东省科技计划项目(编号:2014A020212022)
通讯作者:钟微,Email:manr68@126.com