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Conjoined Twins

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Abstract

Monozygotic, monochorionic, and isosexual twins united by a part of their anatomy are known as Conjoined Twins. This is a rare and fascinating malformation that represents one of the more complex challenges of pediatric surgery. The twins are classified into two main groups: asymmetric and symmetric. Asymmetric ones are acardius acephalus, fetus-in-fetu, or heteropagus twins. In all these, only one component, the autositus supplying circulation, is viable. Symmetric twins are designated craniopagus, thoracopagus, omphalopagus, rachiopagus, ischiopagus, pygopagus, or parapagus according to the location of the joining bridge. This can be large and often contains shared organs. The cardiovascular systems of both components of the set are communicated and the internal environment is also shared to a variable extent.

Mortality is high before and after birth because of frequent and severe associated malformations. Viability of separation is difficult to determine and requires sophisticated imaging studies. Separation not only involves lengthy and complex operations but also difficult ethical decisions with familial, medical, and even court participation.

Separation, when possible, requires various groups of specialists under a strong leadership. Bony parts, nervous system, hearts, great vessels, digestive and genitourinary organs, as well as the skin and musculoskeletal tissues have to be divided and reconstructed to achieve separation with preservation for each component of as much function as possible. Survival is nearly impossible when the hearts are united, but it is possible for one or both twins in all the other forms. Complications are frequent and long-term quality of life is often burdened by fecal and urinary incontinence or by abnormal limbs and genitalia that are the price to pay for separation.

The quality of a pediatric surgical group is heavily put to test by these cases that can only be managed when outstanding expertise is available in the various specialties involved.

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References

  • Abubakar AM, Ahidjo A, Chinda JY, Tahir C, Abubakar S, Adamu SA, Pindiga UH. The epigastric heteropagus conjoined twins. J Pediatr Surg. 2011;46(2):417–20. Epub 2011/02/05

    Article  PubMed  Google Scholar 

  • Arnold J, Luton A, Davies J. Introduction: Unique challenges in the care of conjoined twins. Semin Perinatol. 2018;42(6):319–320.

    Article  PubMed  Google Scholar 

  • Al Rabeeah A. Conjoined twins – past, present, and future. J Pediatr Surg. 2006;41(5):1000–4.

    Article  PubMed  Google Scholar 

  • Andrews RE, McMahon CJ, Yates RW, Cullen S, de Leval MR, Kiely EM, Spitz L, Sullivan ID. Echocardiographic assessment of conjoined twins. Heart. 2006;92(3):382–7.

    Article  CAS  PubMed  Google Scholar 

  • Atkinson L. Ethics and conjoined twins. Childs Nerv Syst. 2004;20(8-9):504–7.

    Article  PubMed  Google Scholar 

  • Baken L, Rousian M, Kompanje EJ, et al. Diagnostic techniques and criteria for first-trimester conjoined twin documentation: a review of the literature illustrated by three recent cases. Obstet Gynecol Surv. 2013;68:743.

    Article  PubMed  Google Scholar 

  • Bhansali M, Sharma DB, Raina VK. Epigastric heteropagus twins: 3 case reports with review of literature. J Pediatr Surg. 2005;40(7):1204–8.

    Article  PubMed  Google Scholar 

  • Boudreaux AM, Tilden SJ. Ethical dilemmas for pediatric surgical patients. Anesthesiol Clin North Am. 2002;20(1):227–40.

    Article  PubMed  Google Scholar 

  • Brizot ML, Liao AW, Lopes LM, Okumura M, Marques MS, Krebs V, Schultz R, Zugaib M. Conjoined twins pregnancies: experience with 36 cases from a single center. Prenat Diagn. 2011;31(12):1120–5. Epub 2011/09/10

    CAS  PubMed  Google Scholar 

  • Browd SR, Goodrich JT, Walker ML. Craniopagus twins. J Neurosurg Pediatr. 2008;1(1):1–20. Epub 2008/03/21

    Article  PubMed  Google Scholar 

  • Carton AJ, Gomez F, Garcia-Guereta L, Martinez L, Tovar JA, Gutierrez-Larraya F. A rhythm by two players: ECG tracing found in conjoined twins. Rev Port Cardiol. 2011;30(11):865–6. Epub 2011/10/28

    Article  PubMed  Google Scholar 

  • Chen Y, Huang ZW, Liu M, Wang JQ, Cai L, Cao CX. Preoperative evaluation of possible cross-circulation in conjoined twins by hepatobiliary scintigraphy. Clin Nucl Med. 2011;36(12):e202–3. Epub 2011/11/09

    Article  PubMed  Google Scholar 

  • Chiu CT, Hou SH, Lai HS, Lee PH, Lin FY, Chen WJ, Chen MT, Lin TW, Chu SH. Separation of thoracopagus conjoined twins. A case report. J Cardiovasc Surg. 1994;35(5):459–62.

    CAS  Google Scholar 

  • Cywes S, Millar AJ, Rode H, Brown RA. Conjoined twins – the Cape Town experience. Pediatr Surg Int. 1997;12(4):234–48.

    CAS  PubMed  Google Scholar 

  • el Gohary MA. Siamese twins in the United Arab Emirates. Pediatr Surg Int. 1998;13(2-3):154–7.

    Article  PubMed  Google Scholar 

  • Fieggen AG, Dunn RN, Pitcher RD, Millar AJ, Rode H, Peter JC. Ischiopagus and pygopagus conjoined twins: neurosurgical considerations. Childs Nerv Syst. 2004;20(8–9):640–51.

    PubMed  Google Scholar 

  • Fishman SJ, Puder M, Geva T, Jenkins K, Ziegler MM, Shamberger RC. Cardiac relocation and chest wall reconstruction after separation of thoracopagus conjoined twins with a single heart. J Pediatr Surg. 2002;37(3):515–7.

    Article  PubMed  Google Scholar 

  • Gillon R. Imposed separation of conjoined twins – moral hubris by the English courts? J Med Ethics. 2001;27(1):3–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Holcomb 3rd GW, Keating MA, Hollowell JG, Murphy JP, Duckett JW. Continent urinary reconstruction in ischiopagus tripus conjoined twins. J Urol. 1989;141(1):100–2.

    Article  PubMed  Google Scholar 

  • Kaufman MH. The embryology of conjoined twins. Childs Nerv Syst. 2004;20(8-9):508–25.

    Article  CAS  PubMed  Google Scholar 

  • Kiely EM, Spitz L. The separation procedure. Semin Pediatr Surg. 2015;24(5):231–6.

    Article  PubMed  Google Scholar 

  • Kim SS, Waldhausen JH, Weidner BC, Grady R, Mitchell M, Sawin R. Perineal reconstruction of female conjoined twins. J Pediatr Surg. 2002;37(12):1740–3.

    Article  PubMed  Google Scholar 

  • Kingston CA, McHugh K, Kumaradevan J, Kiely EM, Spitz L. Imaging in the preoperative assessment of conjoined twins. Radiographics. 2001;21(5):1187–208.

    Article  CAS  PubMed  Google Scholar 

  • Kobylarz K. History of treatment of conjoined twins. Anaesthesiol Intensive Ther. 2014;46(2):116–123.

    Article  PubMed  Google Scholar 

  • Lai HS, Chu SH, Lee PH, Chen WJ. Unbalanced cross circulation in conjoined twins. Surgery. 1997;121(5):591–2.

    Article  CAS  PubMed  Google Scholar 

  • Lazarus J, Raad J, Rode H, Millar A. Long-term urological outcomes in six sets of conjoined twins. J Pediatr Urol. 2011;7(5):520–5. Epub 2010/10/12

    Article  PubMed  Google Scholar 

  • Lee M, Gosain AK, Becker D. The bioethics of separating conjoined twins in plastic surgery. Plast Reconstr Surg. 2011;128(4):328e–34e. Epub 2011/09/17

    Article  CAS  PubMed  Google Scholar 

  • Logrono R, Garcia-Lithgow C, Harris C, Kent M, Meisner L. Heteropagus conjoined twins due to fusion of two embryos: report and review. Am J Med Genet. 1997;73(3):239–43.

    Article  CAS  PubMed  Google Scholar 

  • Lopes LM, Brizot ML, Schultz R, Liao AW, Krebs VL, Francisco RP, Zugaib M. Twenty-five years of fetal echocardiography in conjoined twins: lessons learned. J Am Soc Echocardiogr. 2013;26(5):530–8. Epub 2013/04/09

    Article  PubMed  Google Scholar 

  • Marin-Padilla M, Chin AJ, Marin-Padilla TM. Cardiovascular abnormalities in thoracopagus twins. Teratology. 1981;23(1):101–13.

    Article  CAS  PubMed  Google Scholar 

  • Martinez L, Fernandez J, Pastor I, Garcia-Guereta L, Lassaletta L, Tovar JA. The contribution of modern imaging to planning separation strategies in conjoined twins. Eur J Pediatr Surg. 2003;13(2):120–4.

    Article  CAS  PubMed  Google Scholar 

  • Martinez-Frias ML. Conjoined twins presenting with different sex: description of a second case that truly represents the earliest historical evidence in humans. Am J Med Genet A. 2009;149A(7):1595–6. Epub 2009/06/18

    Article  PubMed  Google Scholar 

  • Martinez-Frias ML, Bermejo E, Mendioroz J, Rodriguez-Pinilla E, Blanco M, Egues J, Felix V, Garcia A, Huertas H, Nieto C, Lopez JA, Lopez S, Paisan L, Rosa A, Vazquez MS. Epidemiological and clinical analysis of a consecutive series of conjoined twins in Spain. J Pediatr Surg. 2009;44(4):811–20. Epub 2009/04/14

    Article  PubMed  Google Scholar 

  • Mathew RP, Francis S, Basti RS, et al. Conjoined twins – role of imaging and recent advances. J Ultrason. 2017;17(71):259–266.

    Article  PubMed  PubMed Central  Google Scholar 

  • Mian A, Gabra NI, Sharma T, et al. Conjoined twins: from conception to separation, a review. Clin Anat. 2017;30(3):385–96.

    Article  PubMed  Google Scholar 

  • McHugh K, Kiely EM, Spitz L. Imaging of conjoined twins. Pediatr Radiol. 2006;36(9):899–910.

    Article  PubMed  Google Scholar 

  • McLorie GA, Khoury AE, Alphin T. Ischiopagus twins: an outcome analysis of urological aspects of repair in 3 sets of twins. J Urol. 1997;157(2):650–3.

    Article  CAS  PubMed  Google Scholar 

  • McMahon CJ, Spencer R. Congenital heart defects in conjoined twins: outcome after surgical separation of thoracopagus. Pediatr Cardiol. 2006;27(1):1–12.

    Article  CAS  PubMed  Google Scholar 

  • Melo A, Dinis R, Portugal A, et al. Early prenatal diagnosis of parapagus conjoined twins. Clin Pract. 2018;8(2):1039.

    PubMed  PubMed Central  Google Scholar 

  • Meyers RL, Matlak ME. Biliary tract anomalies in thoraco-omphalopagus conjoined twins. J Pediatr Surg. 2002;37(12):1716–9.

    Article  PubMed  Google Scholar 

  • Mutchinick OM, Luna-Munoz L, Amar E, Bakker MK, Clementi M, Cocchi G, da Graca Dutra M, Feldkamp ML, Landau D, Leoncini E, Li Z, Lowry B, Marengo LK, Martinez-Frias ML, Mastroiacovo P, Metneki J, Morgan M, Pierini A, Rissman A, Ritvanen A, Scarano G, Siffel C, Szabova E, Arteaga-Vazquez J. Conjoined twins: a worldwide collaborative epidemiological study of the international clearinghouse for birth defects surveillance and research. Am J Med Genet C Semin Med Genet. 2011;157C(4):274–87. Epub 2011/10/18

    Article  PubMed  Google Scholar 

  • Ohashi A, Tsuji S, Kuroyanagi Y, Kinoshita Y, Kaneko K, Mine K, Hamada Y, Inagaki T. Multidetector computed tomography angiography for successful surgical separation in pygopagus conjoined twins. Pediatr Int. 2012;54(1):150–2. Epub 2012/02/18

    Article  PubMed  Google Scholar 

  • Pierro A, Kiely EM, Spitz L. Classification and clinical evaluation. Semin Pediatr Surg. 2015;24(5):207–11.

    Article  PubMed  Google Scholar 

  • Powis M, Spitz L, Pierro A. Differential energy metabolism in conjoined twins. J Pediatr Surg. 1999;34(7):1115–7.

    Article  CAS  PubMed  Google Scholar 

  • Rode H, Fieggen AG, Brown RA, Cywes S, Davies MR, Hewitson JP, Hoffman EB, Jee LD, Lawrenson J, Mann MD, Matthews LS, Millar AJ, Numanoglu A, Peter JC, Thomas J, Wainwright H. Four decades of conjoined twins at Red Cross Children’s Hospital – lessons learned. S Afr Med J. 2006;96(9 Pt 2):931–40.

    CAS  PubMed  Google Scholar 

  • Rubini G, Paradies G, Leggio A, D’Addabbo A. Scintigraphy in assessment of the feasibility of separation of a set of xipho-omphalopagous conjoined twins. Clin Nucl Med. 1995;20(12):1074–8.

    Article  CAS  PubMed  Google Scholar 

  • Spencer R. Anatomic description of conjoined twins: a plea for standardized terminology. J Pediatr Surg. 1996;31(7):941.

    Article  CAS  PubMed  Google Scholar 

  • Spencer R. Theoretical and analytical embryology of conjoined twins: part I: embryogenesis. Clin Anat. 2000a;13(1):36–53.

    Article  CAS  PubMed  Google Scholar 

  • Spencer R. Theoretical and analytical embryology of conjoined twins: part II: adjustments to union. Clin Anat. 2000b;13(2):97–120.

    Article  CAS  PubMed  Google Scholar 

  • Spencer R. Parasitic conjoined twins: external, internal (fetuses in fetu and teratomas), and detached (acardiacs). Clin Anat. 2001;14(6):428–44.

    Article  CAS  PubMed  Google Scholar 

  • Spencer R. Conjoined twins. Developmental malformations and clinical implications. Baltimore and London: The Johns Hopkins University Press; 2003. 476 p.

    Google Scholar 

  • Spitz L, Kiely EM. Conjoined twins. JAMA. 2003;289(10):1307–10.

    Article  PubMed  Google Scholar 

  • Spitz L. Ethics in the management of conjoined twins. Semin Pediatr Surg. 2015;24(5):263–4.

    Article  PubMed  Google Scholar 

  • Staffenberg DA, Goodrich JT. Separation of craniopagus conjoined twins with a staged approach. J Craniofac Surg. 2012;23(7 Suppl 1):2004–10. Epub 2012/11/21

    PubMed  Google Scholar 

  • Szmuk P, Rabb MF, Curry B, Smith KJ, Lantin-Hermoso MR, Ezri T. Anaesthetic management of thoracopagus twins with complex cyanotic heart disease for cardiac assessment: special considerations related to ventilation and cross-circulation. Br J Anaesth. 2006;96(3):341–5.

    Article  CAS  PubMed  Google Scholar 

  • Tannuri AC, Batatinha JA, Velhote MC, Tannuri U. Conjoined twins: twenty years’ experience at a reference center in Brazil. Clinics (Sao Paulo). 2013;68(3):371–7. Epub 2013/05/07

    Article  Google Scholar 

  • Thomas Collins 2nd R, Weinberg PM, Gruber PJ, St John Sutton MG. Conjoined hearts in thoracopagus twins. Pediatr Cardiol. 2012;33(2):252–7. Epub 2012/01/25

    Article  PubMed  Google Scholar 

  • Thomas JM, Lopez JT. Conjoined twins – the anaesthetic management of 15 sets from 1991-2002. Paediatr Anaesth. 2004;14(2):117–29.

    Article  PubMed  Google Scholar 

  • Tovar JA. Conjoined twins. In: Puri P, Höllwarth ME, editors. PediatricSurgery: diagnosis and management. Heidelberg: Springer; 2009.

    Chapter  Google Scholar 

  • Tovar JA. Conjoined twins. In: Puri P, editor. Newborn surgery. 3rd ed. London. Hoddar Arnold; 2011.

    Chapter  Google Scholar 

  • van der Weiden RM. The first successful separation of conjoined twins (1689). Twin Res. 2004;7(2):125–7.

    Article  PubMed  Google Scholar 

  • Votteler TP, Lipsky K. Long-term results of 10 conjoined twin separations. J Pediatr Surg. 2005;40(4):618–29.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Surgery, Hospital Universitario La Paz, Universidad Autonoma de Madrid, Madrid, Spain

    Juan A. Tovar

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  1. Juan A. Tovar
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Correspondence to Juan A. Tovar .

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  1. Our Lady’s Children's Hospital, National Children’s Research Centre, Dublin, Ireland

    Prem Puri

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Tovar, J.A. (2019). Conjoined Twins. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_86-2

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  • DOI: https://doi.org/10.1007/978-3-642-38482-0_86-2

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  • Print ISBN: 978-3-642-38482-0

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Chapter history

  1. Latest

    Conjoined Twins
    Published:
    12 January 2019

    DOI: https://doi.org/10.1007/978-3-642-38482-0_86-2

  2. Original

    Conjoined Twins
    Published:
    11 April 2017

    DOI: https://doi.org/10.1007/978-3-642-38482-0_86-1

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