Anatomical, Embryological and Ethical Aspect of Conjoined (Siamese) Twins
Authors:
E. Kubíková 1; I. Varga 2,3
; H. El Falougy 1; Š. Polák 2; J. Beňuška 1
Authors place of work:
Anatomický ústav, Lekárska fakulta, Univerzita Komenského, Bratislava
prednosta prof. MUDr. J. Beňuška, PhD.
1; Ústav histológie a embryológie, Lekárska fakulta, Univerzita Komenského, Bratislava
prednosta doc. MUDr. Š. Polák, CSc.
Ústav fyziológie a patofyziológie, Fakulta zdravotníckych špecializačných štúdií
2; Slovenská zdravotnícka univerzita, Bratislava
prednosta doc. MUDr. I. Béder, CSc.
3
Published in the journal:
Čes-slov Pediat 2009; 64 (1): 33-39.
Category:
Review
Summary
Conjoined (Siamese) twins are rare developmental anomalies, which draws attention not only in the scientific community, but in the whole society. Siamese twins are rare result of fission of the monozygotic twins, because the separation of embryos occurs in the stage of the primitive streak (in the 3rd week of development). In this stage the three germ layers give rise to the primitive organs.
Fission of embryos in the stage of the primitive streak in general is not complete; the cells are not equally separated in two embryos. However other theory explains the formation of conjoined twins on the base of fission and later fusion of two germ discs originating from the same zygote. This theory confirms the fact, that if the embryo fissioned before the development of the notochord, each Siamese twin will have its own notochord.
In this work authors review the knowledge about the etiology and pathogenesis of the conjoined twins. They present the incidence and the clinico-anatomical classification of this developmental anomaly. The work summarizes the information about the cases of conjoined twins in Slovakia. In the last 40 years were born 6 conjoined twins in Slovakia; 3 cases were separated by surgical procedure. The experience of the Slovak physicians confirms that accession to the surgical separation depends on the clinical findings, viability of both children, and on their basic vital functions. In convenient conditions the surgical separation can be postponed for weeks, months or years (Slovak cases from the years 2000 and 2007). In the case of life threatening or death of one of the conjoined twin, an urgent surgical separation has to be performed to save the more viable twin (Slovak case from the year 1986).
The ethical and moral problems related with the decision of surgical separation still unanswered, mainly in the case of unequal viability of both twins. In this work authors review the moral and ethical aspects in relation with the known cases of separated conjoined twins.
Key words:
conjoined (Siamese) twins, etiology, classification, occurrence in Slovak Republic, medical ethic
Zdroje
1. Alexander E. The original Siamese twins: we know why Chang died, but why died Eng? N. C. Med. J. 2001;62: 66–68.
2. Baldwin VJ. Pathology of Multiple Pregnancy. New York: Springer Verlag, 1994:1–409.
3. Baron BW, Shermeta DW, Ismail MA, Ben-Ami T, Yousefzadeh D, Carlson N, Amarose AP, Esterly JR. Unique anomalies in cephalothoracopagus janiceps conjoined twins with implication for multiple mechanisms in the abnormal embryogenesis. Teratology 1990;41: 9–22.
4. Bondeson J. The biddended maids: a curious chapter in the history of conjoined twins. J. R. Soc. Med. 1992;85: 217–221.
5. Derom C, Vlientinek R, Derom R, Van den Berghe H, Thiery M. Population-based study of sex proportion in monoamniotic twins. N. Engl. J. Med. 1988;319: 119–120.
6. Geroulanos S, Jaggi F, Wydler J, et al. Thoracopagus symmetricus. Gesnerus 1994;50: 179–200.
7. Gilbert-Barness E, Debich-Spicer D, Opitz JM. Conjoined twins: morphogenesis of the heart and a review. Am. J. Med. Genet. 2003;120A: 568 – 582.
8. Hall JG. Twins and twinning. In Rimoin DI, Connor JM, Pyeritz RE (eds). Emery and Rimoin´s Principles and Practice of Medical Genetics. Vol. 1, 4th ed. New York: Churchill Livingstone, 2001: 501–513.
9. Hall JG. Twinning. Lancet 2003;362: 735–743.
10. Hoyle RM. Surgical separation of conjoined twins. Surg. Gynecol. Obstet. 1990;170: 549–562.
11. Chiu CT, Hou SH, Lai HS, Lee PH, Chen WJ, Lin TW, Chu SH. Separation of thoracopagus conjoined twins: A case report. J. Cardiovasc. Surg. 1994;35(5): 459–462.
12. James WH. Sex ratio and placentation in twins. Ann. Hum. Biol. 1980;7: 273–276.
13. Janec M, Vojtko M, Kubíková E. Xiphoomphalophagus – siamské dvojčatá s mnohopočetnými malformáciami GIT. Rozhl. Chir. 1989;68(8–9): 616–620.
14. Kapeller K, Pospíšilová V. Embryológia človeka. Martin: Osveta, 2001: 1–371.
15. Kato T, Yyoshino H, Hebiguchi T, Koyama K. Experience with treatment of three pairs of conjoined twins. Am. J. Perinatol. 1997;14(1): 25–30.
16. Kennedy GE. The 3,000 year history of conjoined twins. West. J. Med. 2001;175(3): 176–177.
17. Kompanje JE. The first successful separation of conjoined twins in 1689, some addition and corrections. Twin. Res. 2004;7: 537–541.
18. Lammer SE. Tragedies and medical choices. Christ Century 1993;8–15: 845–846.
19. Lai HS, Lee PH, Chu SH, Chen MT, Lin TW, Duh YC, Chen WJ. Successful early separation of a premature xipho-omphalopagus conjoined twins: a case report. Can. J. Surg. 1997;40(2): 139–142.
20. Mainous RO. Conjoined twins: Whose best interest should prevail? An argument for separation. Pediatr. Nurs. 2002;28(5): 525–529.
21. Mitchell S. Exhibiting monstrosity: Chang and Eng, the „original“ Siamese twins. Endeavour 2003;27(4): 150–154.
22. Métneki J, Czeizel A. Conjoined twins in Hungary, 1970–1986. Acta Genet. Med. Gemellol. 1989;38: 285–299.
23. Paris JJ, Elias-Jones AC. „Do we murder Mary to save Jodie?“ An ethical analysis of the separation of the Manchester conjoined twins. Postgrad. Med. J. 2001;77: 593–598.
24. Porter M. Tlatilco and the preclassis cultures of the New World. Viking. Fund. Pub. Anthro. 1953;No. 19.
25. Pospíšilová V, Mikušová R, Polák Š, Varga I. Embryológia človeka. Učebnica pre bakalárske a magisterské zdravotnícke odbory. Bratislava: Univerzita Komenského, 2007: 1–100.
26. Sakcharoen N, Wannakratoirot P. Sonographic prenatal diagnosis of congenital heart defects in thoraco-omphalopagus. Asia Oceania J. Obstet. Gynaecol. 1993;19(1): 43–49.
27. Siman J, Payer J, Brozman M, Tanuška D, Pevalová L, Macek M, Babala J, Jursová M, Cingel V, Duchaj B. Separation of Siamese twins in Bratislava. Bratisl. lek. Listy 2004;105(2): 37–44.
28. Smith D, Jeraj M. Diagnostic imaging of conjoined twins. Can. J. Med. Radiat. Technol. 1996;27(1): 31–39.
29. Spencer R. Conjoined twins: Theoretical embryologic basis. Teratology 1992;45: 591–602.
30. Spencer R. Anatomic description of conjoined twins: A plea for standardized terminology. J. Pediatr. Surg. 1996;31(7): 941–944.
31. Spencer R. Theoretical and analytical embryology of conjoined twins: Part 1: Embryogenesis. Clin. Anat. 2000;13: 36–53.
32. Spitz L, Kiely EM. Experience in the management of conjoined twins. Br. J. Surg. 2002;89: 1188–1192.
33. Spitz L. Conjoined twins. Prenat. Diag. 2005;25: 814–819.
34. Strocker A, Ford R, Patel S, Ayad I, Shapiro N. Airway evaluation of conjoined twins. Ann. Otol. Rhinol. Laryngol. 2005;114: 15–18.
35. Thomas JM, Lopez JT. Conjoined twins – the anaesthetic management of 15 sets from 1991–2002. Pediatr. Anaesth. 2004;14: 117–129.
36. Thomasma DC, Muraskas J, Marshall PA, Myers T, Tomich P, O´Neill JA. The etics of caring for conjoined twins: The Lakeberg twins. Hastings Cent. Rep. 1996;26(4): 4–12.
37. Tomo I, Galbavý Š. K výskytu siamských dvojčiat na Slovensku. Diagnóza 1996;1: 26–27.
38. Varga I, Tomo IM, Polák Š, Tóth F, Kubíková E. Historicko-medicínsky pohľad na siamské dvojčatá a ich známe prípady výskytu na území Slovenska. In Polák Š, Pospíšilová V, Varga I (eds). Morfológia v súčasnosti. Bratislava: Univerzita Komenského, 2006: 80–89.
39. Varga I, Polák Š, Tomo IM, Tóth F, Kubíková E. Siamské dvojčatá z pohľadu embryológie a súčasnej medicíny. Biológia Ekológia Chémia 2006;11(3): 18–22.
40. Wallace I, Wallace A. The Two. New York: Simon and Schuster, 1978: 1–352.
41. Weston PJ, Ives EJ, Honore RLH, Lees GM, Sinclair DB, Schiff D. Monochorionic diamniotic minimally conjoined twins: A case report. Am. J. Med. Genet. 1990;37: 558–561.
Štítky
Neonatology Paediatrics General practitioner for children and adolescentsČlánok vyšiel v časopise
Czech-Slovak Pediatrics
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