Abstract
Conjoined twinning is an uncommon condition (1:100,000 live births), in which two genetically identical individuals are joined by a part of their anatomy and eventually share one or more organs. When confronted with one of these sets, pediatric surgeons face one of the most challenging situations in their specialty. This form of twinning raises many interesting issues that we shall address in the following order: history, embryonal mechanisms, anatomical varieties, imaging for definition of the anatomy, ethical issues, technical aspects and results.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Al-Rabeeah A, Zamakhshary M, Al-Namshan M, Al-Jadaan S, Alshaalan H, Al-Qahtani A, Alassiri I (2011) Hepatobiliary anomalies in conjoined twins. J Pediatr Surg 46:888–892
Annas GJ (2001) Conjoined twins--the limits of law at the limits of life. N Engl J Med 344:1104–1108
Barletta A, Pace M, D’Antiga L, Cheli M, Mangili G, Sironi S (2019) Imaging of liver fusion and vascular intra-hepatic shunts in omphalopagus conjoined twins: the role of a double CT study. Clin Imaging 54:25–30
Boer LL, Schepens-Franke AN, Oostra RJ (2019) Two is a crowd: two is a crowd: on the enigmatic etiopathogenesis of conjoined twinning. Clin Anat 32:722–741
Carreker C, De Jong H, Javor V, Fox L, Jacome J, Johns C, Springmeyer K, Davies J (2018) Family support and media considerations with conjoined twins. Semin Perinatol 42:393–401
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 35:459–462
Cuckow P, Mishra P (2015) Urological management. Semin Pediatr Surg 24:237–240
Elizondo RA, Au JK, Roth DR, Olutoye OO, Cass DL, Koh CJ (2017) Urologic considerations in the separation of Thoracoomphalopagus conjoined twins. Urology 99:231–233
Endres L, Wilkins I (2005) Epidemiology and biology of multiple gestations. Clin Perinatol 32:301–314, v
Fallon SC, Olutoye OO (2018) The surgical principles of conjoined twin separation. Semin Perinatol 42:386–392
Fieggen AG, Dunn RN, Pitcher RD, Millar AJ, Rode H, Peter JC (2004) Ischiopagus and pygopagus conjoined twins: neurosurgical considerations. Childs Nerv Syst 20:640–651
Frawley G (2020) Conjoined twins in 2020- state of the art and future directions. Curr Opin Anaesthesiol 33:381–387
Hamdan MA, Begam M, Mirghani HM (2010) Inseparable thoraco-omphalopagus twins. Pediatr Cardiol 31:1252–1253
Hoyle RM, Thomas CG Jr (1989) Twenty-three-year follow-up of separated ischiopagus tetrapus conjoined twins. Ann Surg 210:673–679
Jones D (2015) Orthopedic aspects of separation. Semin Pediatr Surg 24:249–251
Kallberg L (2018) The ethics of separating conjoined twins: two arguments against. Theor Med Bioeth 39:27–56
Kaufman MH (2004) The embryology of conjoined twins. Childs Nerv Syst 20:508–525
Kiely EM, Spitz L (2015) Planning the operation. Semin Pediatr Surg 24:221–223
Lazarus J, Raad J, Rode H, Millar A (2011) Long-term urological outcomes in six sets of conjoined twins. J Pediatr Urol 7:520–525
Liu X, Dong K, Zheng S, Xiao X, Shen C, Dong C, Zhu H, Li H, Bi Y, Ma R (2018) Separation of pygopagus, omphalopagus, and ischiopagus with the aid of three-dimensional models. J Pediatr Surg 53:682–687
Martínez L, Fernández J, Pastor I, García-Guereta L, Lassaletta L, Tovar JA (2003) The contribution of modern imaging to planning separation strategies in conjoined twins. Eur J Pediatr Surg 13:120–124
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 (2009) Epidemiological and clinical analysis of a consecutive series of conjoined twins in Spain. J Pediatr Surg 44:811–820
McMahon CJ, Spencer R (2006) Congenital heart defects in conjoined twins: outcome after surgical separation of thoracopagus. Pediatr Cardiol 27:1–12
Meyers RL, Matlak ME (2002) Biliary tract anomalies in thoraco-omphalopagus conjoined twins. J Pediatr Surg 37:1716–1719
Mian A, Gabra NI, Sharma T, Topale N, Gielecki J, Tubbs RS, Loukas M (2017) Conjoined twins: from conception to separation, a review. Clin Anat 30:385–396
Mutchinick OM, Luna-Muñoz L, Amar E, Bakker MK, Clementi M, Cocchi G, da Graça DM, Feldkamp ML, Landau D, Leoncini E, Li Z, Lowry B, Marengo LK, Martínez-Frías ML, Mastroiacovo P, Métneki J, Morgan M, Pierini A, Rissman A, Ritvanen A, Scarano G, Siffel C, Szabova E, Arteaga-Vázquez J (2011) 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 157c:274–287
Nejat F, Habibi Z, Goudarzi M, Azad MS, Moradi E, Heidari V, Kadivar M, Soltani ZE, Kouchakzadeh L (2017) Emergency separation of craniopagus twins: case report. J Neurosurg Pediatr 20:307–313
O’Brien P, Nugent M, Khalil A (2015) Prenatal diagnosis and obstetric management. Semin Pediatr Surg 24:203–206
Parmekar S, McMullen L, Washington C, Arnold JL (2018) Role of simulation in preparation for the care of conjoined twins-prenatal preparation to separation. Semin Perinatol 42:329–339
Pierro A, Kiely EM, Spitz L (2015) Classification and clinical evaluation. Semin Pediatr Surg 24:207–211
Saranrittichai S, Niramis R, Siripornadulsilp S, Thianjaruwatthana W, Hompleum T, Tipsunthonsak N, Pulnitiporn A, Vasanawathana S, Jarupoonphol V (2007) Successful separation of thoracopagus conjoined twins with a single extra-hepatic biliary system. J Med Assoc Thail 90:994–1000
Shapiro E, Fair WR, Ternberg JL, Siegel MJ, Bell MJ, Manley CB (1991) Ischiopagus tetrapus twins: urological aspects of separation and 10-year followup. J Urol 145:120–125
Spencer R (2000) Theoretical and analytical embryology of conjoined twins: part I: embryogenesis. Clin Anat 13:36–53
Spitz L (2005) Conjoined twins. Prenat Diagn 25:814–819
Spitz L (2015) Ethics in the management of conjoined twins. Semin Pediatr Surg 24:263–264
Staffenberg DA, Goodrich JT (2012) Separation of craniopagus conjoined twins with a staged approach. J Craniofac Surg 23:2004–2010
Thomas A, Johnson K, Placencia FX (2018) An ethically-justifiable, practical approach to decision-making surrounding conjoined-twin separation. Semin Perinatol 42:381–385
Tovar JA (2009) Conjoined twins. In: Puri P, Höllwarth M (eds) Pediatric surgery. Springer, Berlin, pp 646–655
Walton JM, Gillis DA, Giacomantonio JM, Hayashi AH, Lau HY (1991) Emergency separation of conjoined twins. J Pediatr Surg 26:1337–1340
Wasserman D (2001) Killing Mary to save Jodie: conjoined twins and individual rights. Philos Public Policy Q 21:9–14
Watson SG, McHugh K (2015) Conjoined twins: radiological experience. Semin Pediatr Surg 24:212–216
Yokomori K, Ohkura M, Kitano Y, Nakajo T, Harii K, Tanikaze S (1993) Comprehensive planning of operative strategy for separation of ischiopagus tripus twins with particular reference to quality of life. J Pediatr Surg 28:833–837
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2023 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Tovar, J.A., Martinez, L. (2023). Conjoined Twins. In: Puri, P., Höllwarth, M.E. (eds) Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-81488-5_44
Download citation
DOI: https://doi.org/10.1007/978-3-030-81488-5_44
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-81487-8
Online ISBN: 978-3-030-81488-5
eBook Packages: MedicineMedicine (R0)