Abstract
We sought to perform a comprehensive review of reported cases of conjoined thoracopagus twins with cardiovascular conjunction to determine the overall survival after separation and factors that might be associated with survival. We performed a systematic review of cases of thoracopagus twins with conjoined cardiovascular systems reported in the English medical literature using Embase, PubMed, Web of Science, and Scopus. We employed standard statistical methods to analyze differences among groups. We identified 102 unique cases of thoracopagus twins (69% female) with some degree of cardiovascular conjunction who had undergone surgical separation. We identified 6 distinct types of cardiovascular union. Median age was 47 days (IQR 12, 120). Survival to at least hospital discharge occurred in 51% (105/204). Median age at separation was lower in emergent (9 days, IQR 1, 25) versus elective cases (93 days, IQR 49, 180) (p < 0.0001). Survival to hospital discharge was higher in those who underwent elective separation (70%, 93/132 total children versus 17%, 12/72) (p < 0.0001). Survival was associated with the type of cardiovascular union (p < 0.0001). The separation of thoracopagus twins with cardiovascular conjunction is higher than expected. Increased survival is associated with elective separation, older age, and shared pericardium only. These findings suggest survival could be improved with better delineation of degree of union and delayed separation.
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Abbreviations
- CT:
-
Computed tomography
- IQR:
-
Interquartile range
- MRI:
-
Magnetic resonance imaging
References
Geroulanos S, Jaggi F, Wydler J, Lachat M, Cakmakci M (1993) Thoracopagus symmetricus. On the separation of Siamese twins in the 10th century A. D. by Byzantine physicians. Gesnerus 50 (Pt 3–4):179–200
Al-Arfaj AA, Mitra DK, Kadeeb HS, Al-Ghamdi AA, Abdulghaffar ME, Al-Awary BH (2002) Successful separation of thoracopagus conjoined twins. Saudi Med J 23:1522–1526
Kompanje EJO (2004) The first successful separation of conjoined twins in 1689: some additions and corrections. Twin Res 7:537–541
van der Weiden RMF (2012) The first successful separation of conjoined twins (1689). Twin Res 7:125–127
Spencer R (2000) Theoretical and analytical embryology of conjoined twins: part I: embryogenesis. Clin Anat 13:36–53
Spitz L, Kiely EM (2002) Experience in the management of conjoined twins. Br J Surg 89:1188–1192
Collins RT II, Weinberg PM, Gruber PJ, St John Sutton MG (2012) Conjoined hearts in thoracopagus twins. Pediatr Cardiol 33:252–257
Nichols B, Blattner R, Rudolph A (1967) General clinical management of thoracopagus twins. Birth Defects Orig Artic Ser 3:38–51
Seo JW, Shin SS, Chi JG (1985) Cardiovascular system in conjoined twins: an analysis of 14 Korean cases. Teratology 32:151–161
Wood BC, Sher SR, Mitchell BJ, Oh AK, Rogers GF, Boyajian MJ (2017) Conjoined twin separation: integration of three-dimensional modeling for optimization of surgical planning. J Craniofac Surg 28:4–10
Elixhauser A, Steiner C, Harris DR, Coffey RM (1998) Comorbidity measures for use with administrative data. Med Care 36:8–27
Cywes S, Millar AJ, Rode H, Brown RA (1997) Conjoined twins–the Cape Town experience. Pediatr Surg Int 12:234–248
Izukawa T, Kidd BS, Moes CA, Tyrrell MJ, Ives EJ, Simpson JS, Shandling B (1978) Assessment of the cardiovascular system in conjoined thoracopagus twins. Am J Dis Child 132:19–24
Zhang Y-Q, Wu L-P, Wu L-J, Chen G-Z, Sun K, Zhang Z-F, Shen R (2009) Echocardiographic assessment of conjoined twins with congenital heart disease in Shanghai. Echocardiography 26:691–698
Al RA (2006) Conjoined twins–past, present, and future. J Pediatr Surg 41:1000–1004
Saguil E, Almonte J, Baltazar W, Acosta A, Caballes A, Catangui A, Villegas C (2009) Conjoined twins in the Philippines: experience of a single institution. Pediatr Surg Int 25:775–780
Trost JG Jr, Lin LO, Clark SJ, Khechoyan DY, Hollier LH Jr, Buchanan EP (2016) Separation of thoraco-omphalo-ischiopagus conjoined twins. Plast Reconstr Surg 138:1064–1072
Able LW (1967) Surgical separation of the Bay City twins. Birth Defects Orig Artic Ser 3:69–74
Allin ES (1967) The conjoined twins of Tofield. Birth Defects Orig Artic Ser 3:143
Andrews RE, McMahon CJ, Yates RWM, Cullen S, de Laval MR, Kiely EM, Spitz L, Sullivan ID (2005) Echocardiographic assessment of conjoined twins. Heart 92:382–387
Bahador A, Nikeghbalain S, Foroughi M, Beizavi Z, Sahmodini MA, Eghbal MH, Vafaei H, Malekhosseini SA, Bahador M (2020) Successful surgical separation of thoraco-omphalopagus symmetrical conjoined twins in Iran: two case reports. Iran J Med Sci 45:143–147
Boles ET, Vassy LE (1979) Thoraco-omphalopagus conjoined twins: successful surgical separation. Surgery 86:485–492
Cordoba RA, Juaneda E, Alday LE (1999) Surgical ligation of a persistent arterial duct in one of conjoined thoracopagus twins prior to surgical separation. Cardiol Young 9:203–206
Graivier L, Jacoby MD (1980) Emergency separation of newborn conjoined (Siamese) twins. Tex Med 76:60–62
Hoshina H, Tanaka O, Obara H, Iwai S (1987) Thoracopagus conjoined twins: management of anesthetic induction and postoperative chest wall defect. Anesthesiology 66:424–426
Jung PM (1997) Successful separation of thoraco-omphalopagus and ischiopagus tetrapus twins in Korea. Pediatr Surg Int 12:607–610
Kato T, Yoshino H, Hebiguchi T, Koyama K (1997) Experience with treatment of three pairs of conjoined twins. Amer J Perinatol 14:25–30
Li D, Lin Y, Lin W, Zhuang F, Shi Z, Guo X, Xu D, Yang X, Lin J, Zhang J, Mo Y, Lai F (2000) Separation of thoracopagus conjoined twins. Zhonghua Wai Ke Za Zhi 38:61–63
Pe-Nyun SH (1972) Thoracoomphalopagous conjoined twins of Burma. J Pediatr Surg 7:691–695
Peterson CG, Hill AJ (1960) Separation of conjoined thoracopagus twins: surgical considerations. Ann Surg 152:717–728
Piasceczna-Piotrowska A, Chilarski A, Krajewski W, Godlewska-Tarka J (2010) Thoraco-omphalopagus twins: different perinatal circumstances, different outcome. Arch Med Sci 6(3):461–463
Ray AK, Mukherjee NN, Mukherjee G, Patra R, Ghosh DK (2004) Separation of thoraco-omphalopagus Siamese twin. Indian J Pediatr 71:755–757
Shi C-R, Cai W, Jin H-M, Chen F, Zhou Y, Zhou D-X (2006) Surgical management to conjoined twins in Shanghai area. Pediatr Surg Int 22:791–795
Thompson JL, Zarroug AE, Matsumoto JMS, Moir CR (2007) Anatomy of successfully separated thoracopagus–omphalopagus conjoined twins. Clin Anat 20:814–818
Towey RM, Kisia AK, Jacobacci S, Muoki M (1979) Anaesthesia for the separation of conjoined twins. Anaesthesia 34:187–192
Watanatittan S, Niramis R, Suwatanaviroj A, Havanonda S (2003) Conjoined twins: surgical separation in 11 cases. J Med Assoc Thai 86(Suppl 3):S633–S643
Zhong H-J, Hong L, Du Z-Y, Huan H, Yang T-D, Qi Y-Y (2013) Anesthetic management of conjoined twins undergoing one-stage surgical separation: a single center experience. Pak J Med Sci 29:1–5
Zubowicz VN, Ricketts R (1988) Use of skin expansion in separation of conjoined twins. Ann Plast Surg 1988:272–276
Benjamin LC, Nahar J, Sable C, Midgley FM, Di Russo GB (2005) Separation of thoracopagus-cardiopagus twins joined by a myocardial bridge. J Thorac Cardiovasc Surg 130:1212–1213
Sun J, Zhang Y, Ruan Q, Tang S, Sun Z, Zeng R (2008) Gains from the separation of two cases of conjoined twins. J Plast Reconstr Aesthet Surg 61:552–556
McMahon CJ, Mullins CE, Vick GW, Caville CC, Bezold LI, Nihill MR (2000) Cardiac catheterization in diagnosis and management of congenital heart disease in thoracopagus conjoined twins. Cathet Cardiovasc Intervent 51:159–167
Chiu CT, Hou SH, Lai HS, Lee PH, Lin FY, Chen WJ, Chen MT, Lin TW, Chu SH (1994) Separation of thoracopagus conjoined twins: a case report. J Cardiovasc Surg (Torino) 35:459–462
Tannuri AC, Batatinha JA, Velhote MC, Tannuri U (2013) Conjoined twins—twenty years’ experience at a reference center in Brazil. Clinics 68:371–377
Brizot ML, Liao AW, Lopes LM, Okumura M, Marques MS, Krebs V et al (2011) Conjoined twins pregnancies: experience with 36 cases from a single center. Prenat Diagn 31(12):1120–1125
Bloch EC, Karis JH (1980) Cardiopagus in neonatal thoracopagus twins: anesthetic management. Anesth Analg 49(4):304–307
El-Gohary MA (1998) Siamese twins in the United Arab Emirates. Pediatr Surg Int 13:154–157
Goo HW, Park JJ, Kim EA, Won HS (2014) Cardiac fusion and complex congenital cardiac defects in thoracopagus twins: diagnostic value of cardiac CT. Pediatr Radiol 44(9):1169–1174
Juhnke B, Mattson AR, Saltzman D, Azakie A, Hoggard E, Ambrose M, Iaizzo PA, Erdman A, Fischer G (2019) Use of virtual reality for pre-surgical planning in separation of conjoined twins: a case report. Proc Inst Mech Eng H 233:1327–1332
Messmer B, Hornchen H, Kosters C (1981) Surgical separation of conjoined (Siamese) xiphopagus twins. Surgery 89:622–625
Patel R, Fox K, Dawson J, Taylor JF, Graham GR (1977) Cardiovascular anomalies in thoracopagus twins and the importance of preoperative cardiac evaluation. Heart 39:1254–1258
Ramp JB, Baylor JR, Casamento VK, Gerald AL, Golczynski CD, McComiskey CA (1989) Conjoined twins: a multidisciplinary approach. Neonatal Netw 8:29–39
Synhorst D, Matlak M, Roan Y, Johnson D, Byrne J, McGough E (1979) Separation of conjoined thoracopagous twins joined at the right atrial. Am J Cardiol 43:662–665
Watanatittan S, Niramis R, Suwatanaviroj A, Havanonda S (2003) Conjoined twins: surgical separation in 11 cases. J Med Assoc Thai 86(S3):S633–S643
Wen X, Parajuly SS, Lu Q, Xiang B, Jiang X, Tang H, Luo Y (2013) Thoraco-omphalopagus conjoined twins: impact of ultrasound assessment on successful surgical separation. J Clin Imaging 37:138–142
Suan C, Ojeda R, García-Perla JL, Cerro J, Romero D, Gilabert J, González A, Casas G, Sánchez-Felipe L, Peñasco F, Repullo J (1998) Anaesthetic management of the surgical separation of a pair of thoracopagus-cardiopagus twins. Pediatr Anesth 8:255–257
Rode H, Fieggen AG, Brown RA, Cywes S, Davies MRQ, Hewitson JP, Hoffman EB, Jee LD, Lawrenson J, Mann MD, Matthews LS, Millar AJW, Numanoglu A, Peter JC, Thomas J, Wainwright H (2006) Four decades of conjoined twins at Red Cross Children’s Hospital–lessons learned. S Afr Med J 96:931–940
O’Neill JA, Holcomb GW, Schnaufer L, Templeton JM, Bishop HC, Ross AJ, Duckett JW, Norwood WI, Ziegler MM, Koop CE (1988) Surgical experience with thirteen conjoined twins. Ann Surg 208:299–312
Jenkins KJ, Newburger JW, Lock JE, Davis RB, Coffman GA, Iezzoni LI (1995) In-hospital mortality for surgical repair of congenital heart defects: preliminary observations of variation by hospital caseload. Pediatrics 95:323–330
Brown DL, Holubec DM, Towle DJ, Bodary AK, Patterson AR, Mack J (1985) Anesthetic management of thoracopagus twins undergoing cardiopagus separation. Anesthesiology 62:679–682
Cohen HL, Shapiro ML, Haller JO, Schwartz D (1992) The multivessel umbilical cord: an antenatal indicator of possible conjoined twinning. J Clin Ultrasound 20:278–282
Johnson NJ, Doherty JE (1957) Simultaneous electrocardiograms in thoracopagus twins. Am Heart J 53:151–156
Kelekis L, Kelekis D, Mameletzis K, Kelemouridis B, Artopoulos J (1980) Thoracopagus twins studied with computed tomography. J Comput Assist Tomogr 4:405–406
Kobylarz K (2014) Anaesthesia of conjoined twins - case series. Anaesthesiol Intensive Ther 46:65–77
Micheli JL, Sadeghi H, Freeman J, Bozic C, Queloz J, Genton N (1978) An attempt to separate xiphopagus twins sharing a common heart, liver, and duodenum. J Pediatr Surg 13:139–142
Riker W, Traisman H (1964) Conjoined twins: report of three cases. Illinois Med J 126:450–454
Bansal R, Paliwal N, Karnawat R, Kothari A (2018) Anesthetic management of parasitic conjoined twins’ separation surgery. Saudi J Anaesth 12:485–494
Cummings BM, Gee MS, Benavidez OJ, Shank ES, Bojovic B, Raskin KA, Goldstein AM (2017) Case 33–2017. 22-month-old conjoined twins. N Engl J Med 377:1667–1677
Fishman SJ, Puder M, Geva T, Jenkins K, Ziegler MM, Shamberger RC (2002) Cardiac relocation and chest wall reconstruction after separation of thoracopagus conjoined twins with a single heart. J Pediatr Surg 37:515–517
Norwitz ER, Hoyte LP, Jenkins KJ, van der Velde ME, Ratiu P, Rodriguez-Thompson D, Wilkins-Haug L, Tempany CM, Fishman SJ (2000) Separation of conjoined twins with the twin reversed-arterial-perfusion sequence after prenatal planning with three-dimensional modeling. N Engl J Med 343:399–402
Leachman RD, Latson JR, Kohler CM, McNamara DG (1967) Cardiovascular evaluation of conjoined twins. Birth Defects Orig Artic Ser 3:52–65
Mehollin-Ray AR (2018) Prenatal and postnatal radiologic evaluation of conjoined twins. Semin Perinatol 42:369–380
Liu H-C, Lo C-W, Weng Z-C, Hwang B, Lee P-C (2011) Various modalities for evaluation of a fused heart in conjoined twins. Pediatr Cardiol 33:192–200
Fallon SC, Olutoye OO (2018) The surgical principles of conjoined twin separation. Semin Perinatol 42:386–392
Sager EC, Thomas A, Sundgren NC (2018) Conjoined twins: pre-birth management, changes to NRP, and transport. Semin Perinatol 42:321–328
Wu S, Guo K, Xiao P, Yang J, Sun J (2018) Body wall reconstruction for conjoined twins. Ann Plast Surg 81:S66–S70
Edwards WD, Hagel DR, Thompson J, Whorton CM, Edwards JE (1977) Conjoined thoracopagus twins. Circulation 56:491–497
Spencer R (2000) Theoretical and analytical embryology of conjoined twins: part II: adjustments to union. Clin Anat 13:97–120
MacKenzie TC, Crombleholme TM, Johnson MP, Schnaufer L, Flake AW, Hedrick HL, Howell LJ, Adzick NS (2002) The natural history of prenatally diagnosed conjoined twins. J Pediatr Surg 37:303–309
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Collins, R.T., O’Connor, M.J. The Outcomes of Surgical Separation in Thoracopagus Twins with Conjoined Hearts: An Analysis of the Literature. Pediatr Cardiol 42, 875–882 (2021). https://doi.org/10.1007/s00246-021-02555-8
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DOI: https://doi.org/10.1007/s00246-021-02555-8