The bioethics of separating conjoined twins in plastic surgery

The incidence of craniopagus twins approximates four to six per 10 million births. Although rare, surgical separation of conjoined twins poses significant technical and ethical challenges. The present report uses the case of craniopagus twins AD and TD to examine the bioethical issues faced by a multidisciplinary medical team in planning the separation of craniopagus twins. AD and TD are craniopagus twins conjoined at the head. TD's head is conjoined to the back of AD's head. Neurologically, AD has the dominant cerebral circulation. TD has two normal kidneys, whereas AD has none. AD depends on TD's renal function and, on separation, will require either a kidney transplant or lifelong dialysis. This case report reviews one approach to analyzing and solving complex ethical dilemmas in pediatric plastic surgery. The principles reviewed are (1) autonomy and informed consent, focusing especially on the role of children in the informed consent process; (2) beneficence and nonmaleficence, two intricately intertwined principles because separation could potentially cause irreversible harm to one twin while improving the quality of life for the other (as separation is not a life-saving procedure, is it ethical to perform a procedure with unknown surgical risk to improve children's quality of life?); and (3) justice (is it fair to allocate excessive medical resources for the twins' separation?). The present report explores the ethics behind such decisions with respect to the separation of conjoined twins.     

Conjoined twins: theoretical embryologic basis.

A theoretical basis for the embryology of conjoined twins was formulated from clinical experience with ten cases and extensive review of pertinent embryologic and clinical literature, including over 500 cases. Regarding the age old question of fusion or fission, it is concluded that there is no known embryologic process by which conjoined twins can be formed by fission but firm evidence to support fusion in all cases. Whether the fusion occurs between embryos on one embryonic disc or on two is of no consequence since they are all monovular. Intact ectoderm will not fuse to intact ectoderm, and all seven types of conjoined twins are explained by seven possible sites of union in the early embryo. One new term is proposed: parapagus, from the Greek para, meaning "side," combined with pagus, meaning "fixed"; this is the group formerly called dicephalus or diprosopos. These anterolaterally united parapagus twins must result from two nearly parallel notochords in close proximity; craniopagi and pygopagi from fusion at the cranial and caudal neuropores, respectively; cephalopagi and ischiopagi from union at the pharyngeal and cloacal membranes, respectively; thoracopagi from merging of the cardiac anlage; and omphalopagi from fusion of the umbilicus or of the edges of two embryonic discs in any area not including the above sites. Parasitic twins result from embryonic death of one twin, leaving various portions of the body vascularized by the surviving autosite. The rarity of cases (2) not easily explained by the above theories, and the nearly 6% of twins with two umbilical cords arising from the placenta would seem to support these conclusions. Should one wish to learn the methods of a conjurer, he might vainly watch the latter's customary repertoire, and, so long as everything went smoothly, might never obtain a clue to the mysterious performance, baffled by the precision of the manipulations and the complexity of the apparatus; if, however, a single error were made in any part or if a single deviation from the customary method should force the manipulator along an unaccustomed path, it would give the investigator an opportunity to obtain a part or the whole of the secret.(ABSTRACT TRUNCATED AT 400 WORDS)     

Cephalopagus conjoined twins in a leopard cat (Prionailurus bengalensis)

Case reports of conjoined twins ("Siamese twins") in wild mammals are scarce. Most published reports of conjoined twins in mammals concern cases in man and domestic mammals. This article describes a case of cephalopagus conjoined twins in a leopard cat (Prionailurus bengalensis) collected on the island of Sumatra, Indonesia, in the period 1873-76. A review of known cases of conjoined twinning in wild mammals is given.     

The first successful separation of conjoined twins in 1689: some additions and corrections.

The surgical separation of a pair of conjoined twins in the year 1689 by Johannes Fatio was the subject of a recent article in this journal. The reference used as publication of the case was Fatio's book, Der Arzney Doctor, Helvetisch-Vernuftige Wehe-Mutter, published in 1752, although the case was presented in literature by three earlier sources. Two articles were published in the Miscellanea Curiosa sive Ephemeridum Medico-Physicarum Germanicarum Academiae Imperialis Leopoldinae Naturae Curiosorum in 1689 by Emanuel Konig and another in 1690 by Theodor Zwinger who described and illustrated the case in detail. Besides these articles, a Flug Blatt was published on the case between 1689 and 1695. Fatio copied the engraved plate in his book from Konig's engraving. These two sources should be cited as the first publications on the successful separation of Elisabet and Catherina, and not Fatio's book from 1752.     

Twinning rate in spontaneous abortions.

An unselected series of spontaneous abortions and their mothers were karyotyped with Q-bands to obtain a frequency of twin conceptions lost during the first trimester. Among 661 spontaneous abortions, 15 twin pairs were identified including two sets of conjoined twins. Analysis of Q-band variants permitted the exclusion of cases with two cell lines that could be attributed to maternal contamination or mosaicism. The twinning rate among spontaneous abortions was 1/44 compared with 1/103 live births and stillbirths in the Ontario population. If Weinberg's differential method is applied to these data, the frequency would be as high as 1/30 under the assumption that the incidence of monozygotic twins among abortions is the same as that for live births.     

Cardiovascular system in conjoined twins: an analysis of 14 Korean cases

A new classification of cardiovascular system in conjoined twins is introduced. A special effort has been made to analyze the degree of fusion and symmetry of hearts and great vessels, based on 14 pairs of conjoined twins. The degree of cardiovascular union of the twins is classified into five types. Cases with no vascular union in cardiac, aortic, and inferior vena caval levels were grouped into type I (four cases). Cases with separate hearts and union between aortas or inferior venae cavae were grouped into type II (three cases). Cardiac fusion at the atrial level was grouped into type III (three cases). All of the three cases showed fusion between right atria (subtype IIIa). The theoretical fusion between the left atria or between the left and right atria is put into subtype IIIb. The type IV represents fusion of both atria and ventricles regardless of the number of the chambers (three cases). Type V represents single heart in one of the twins (one case). The external morphologic type and the situs of each twin pair were closely related to the cardiac abnormalities. Dicephalus and thoracopagus were more likely to be associated with abnormal situs and complex cardiac fusion. Abnormal situs was seen in seven out of 14 cases. Three cases with polysplenia and a case with asplenia showed more complex cardiac abnormalities than those with normal situs or situs inversus.     

The conjoined twins of L?wen.

This paper discusses an unusual etching of unlike-sex conjoined twins of the syncephalus type dated 1547. The authenticity of the case is discussed in terms of the biological plausibility of such twins and in the light of mediaeval understanding of the twinning process. Unlike-sex syncephalic twins may occur as a result of dispermic fertilisation, or be an erroneous depiction of an asymmetrical anomaly of the external genitalia of a like-sex pair, mimicking the genitals of the other sex. On the other hand, the L?wen etching conforms with mediaeval beliefs that conjoined twins are divine punishment and therefore were used for ecclesiastical and perhaps commercial purposes. The historical context of the drawing, in which the relation between religion, media, and medicine satisfied the eagerness of the public for curios of Nature, is no longer valid. Thus the L?wen etching opens a window to the past from which modern medicine can be appreciated.     

Is an account of identity necessary for bioethics? What post-genomic biomedicine can teach us

Is a theory of identity necessary for bioethics? In this paper I investigate that question starting from an empirical explication of identity based on post-genomics, in particular on epigenetics. After analysing whether the classic problems a theory of identity has to cope with (fictional transplants; conjoined twins; and definition of death) also affect the proposed epigenetic account of identity, I deal with three topics (the assumption of moral responsibility; decision maintenance in the case of advance directives; and the attribution of value to human beings at given developmental stages) to offer an insight on the relationship between that account and bioethics.     

Über die Hautleistenbefunde bei einem Fall von Thoracopagus

A case of conjoined twins (female thoracopages) has been investigated dermatoglyphically. The variability of characteristics between the twins corresponds with the variability in normal monozygotic twins. The longitudinal configurations in the central part of both left palms are extremely rare. Main clinical details are given.     

The conjoined twins and the limits of rationality in applied ethics

In this article I consider the case of the surgical separation of conjoined twins resulting in the immediate and predictable death of the weaker one. The case was submitted to English law by the hospital, and the operation permitted against the parents' wishes. I consider the relationship between the legal decision and the moral reasons adduced in its support, reasons gaining their force against the framework of much mainstream normative ethical theory. I argue that in a few morally dilemmatic situations, such a legalistic–theoretical approach cannot plausibly accommodate certain irreducible and ineliminable features of the ethical experience of any concrete individual implicated in the situation, and that this failure partly undermines its self–appointed role of guiding such an individual's conduct. For example, the problem as experienced by the judge and by the parents might not be the same problem at all, and some of their respective reasons may be mutually unintelligible or impotent. I certainly do not argue for a rejection of law or of moral theory; I merely challenge their implicit claim to comprehensiveness and their fixation with an idealised and putatively universal rationality modelled on converging scientific enquiry. Finally, I claim that at least in the twins' case there may be insufficient normative robustness to the conclusions reached, or indeed reachable, by the court in a situation where intuitions and moral reasons pull in fundamentally incommensurable directions; as such, there may be room for an acknowledgement of the spiritual, through a humble abstention from making a decision – which is not to be confused with deciding to do nothing.     

In detail.

Two families with twins - one in which adult DZ twins continue to face the risk of frontotemporal dementia, and the other in which a mother copes with brittle bones in a newborn infant MZ pair - are featured. The nature and progress of the disorders are reviewed and personal perspectives from the twins and other family members are variously provided. Next, four current twin studies are briefly summarized. Topics include sex differences in social support systems, new reproductive technologies, comparative twin-singleton frequencies of placenta previa, and the nature and complications of heterotopic pregnancies. This article concludes with a brief sampling of human interest stories involving twins: a different looking DZ pair, newborn sextuplets, conjoined twins and opposite-sex twin athletes.     

The first successful separation of conjoined twins (1689).

Reports on the first successful separation of conjoined twins in 1689 and especially the publication of this achievement, as late as 1752, in the Helvetisch-vernünftiche Wehe-Mutter by Johannes Fatio are discussed in this paper with the intention to correct several historical inaccuracies.     

The Development of Francis Galton's Ideas on the Mechanism of Heredity

Galton greeted Darwin's theory of pangenesis with enthusiasm, and tried to test the assumption that the hereditary particles circulate in the blood by transfusion experiments on rabbits. The failure of these experiments led him to reject this assumption, and in the 1870s he developed an alternative theory of heredity, which incorporated those parts of Darwin's theory that did not involve the transportation of hereditary particles throughout the system. He supposed that the fertilized ovum contains a large number of hereditary elements, which he collectively called the “stirp,” a few of which are patent, developing into particular cell types, while the rest remain latent; the latent elements can be transmitted to the next generation, while the patent elements, with rare exceptions, cannot since they have developed into cells. The problem with this theory is that it does not explain the similarity between parent and child unless there is a high correlation between latent and patent elements. Galton probably came to realize this problem during his subsequent statistical work on heredity, and he quietly dropped the idea that patent elements are not transmitted in Natural Inheritance (1889). Galton thought that brothers and sisters had identical stirps, and he attributed differences between them to variability in the choice of patent elements from the stirp, that is to say to developmental variability. He attributed the likeness of monozygotic twins to the similarity of their developmental environment. Galton's twin method was to track the life history changes of twins to see whether twins who were similar at birth diverged in dissimilar environments or whether twins who were dissimilar at birth converged in similar environments. It is quite different from the modern twin method of comparing the similarities between monozygotic and dizygotic twins, on the assumption that monozygotic twins are genetically identical whereas dizygotic twins are not. It has been argued that Galton foreshadowed Weismann's theory of the continuity of the germ-plasm, but this is only true in a weak sense. They both believed that the inheritance of acquired characters was either rare or impossible, but Galton did not forestall the essential part of Weismann's theory, that the germ-plasm of the zygote is doubled, with one part being reserved for the formation of the germ-cells. This revised version was published online in July 2006 with corrections to the Cover Date.     

Brief communication: Conjoined twins at angel mounds? an ancient DNA perspective

Conjoined twins are born when a single fertilized egg partially splits into two fetuses. A hypothetical case of infant conjoined twins from Angel Mounds, a Middle Mississippian site (A.D. 1050-1400) on the Ohio River near Evansville, Indiana, was discovered in 1941. Morphological analysis does not rule out the field interpretation of this double burial as twins. Ancient mitochondrial DNA recovered from both infants demonstrates that they were not maternal relatives, and hence that they cannot have been conjoined twins.     

High-frequency twinning of Xenopus laevis embryos from eggs centrifuged before first cleavage

In embryos of the frog Xenopus laevis, the dorsal structures normally develop from regions of the egg opposite the side of sperm entry. Gravity and centrifugal force, applied at an angle of 90 degrees to the animal-vegetal axis of the egg, can override this topographic relationship and can cause the dorsal structures to be positioned according to the force vector (S. Black and J. Gerhart, 1985, Dev. Biol. 108, 310-324). We report here that at time 0.40 (40% of the first cleavage interval), an average of 60% of eggs centrifuged at 30g for 4 min in this orientation form conjoined twins with one body axis arising from the centripetal side of the egg and one arising from the centrifugal side of the egg. This positioning is observed regardless of the orientation of the side of sperm entry in the centrifugal field. If, after the 0.40 centrifugation, the eggs are inclined with the centripetal side up, they do not make twins; instead, they make only a single axis at the centripetal side. This indicates that the second axis in twins is caused to form by postcentrifugation gravity-driven internal rearrangements of materials that were displaced by the centrifugation. Twins also form at high frequency in eggs centrifuged twice, first at an inclination of 90 degrees, and then at an inclination of 0 degrees. The second centrifugation yields secondary axes even when it is begun midway in the second cell cycle, well after the time of grey crescent formation. Double centrifugation also causes twinning ("double rescue") of uv-irradiated eggs which otherwise would not develop axial structures. This suggests that the internal displacements caused by the centrifugations can substitute for a step in the normal axis specification process that is impaired in irradiated eggs.     

Cranio-dirachischisis totalis in cephalothoracopagus twins

A set of conjoined 13-day-old male hamster twins is described. The twins were joined at the head and thorax. The brain was exencephalic and the neural plate was completely open throughout its length. The notochord was duplicated throughout its entire length. Partial twinning of the neural plate as indicated by histologic reconstruction is suggested with fusion or non-separation of the neural plates in the medial alar wing area. There was complete separation of the neural plates together with duplication of the lower extremities and tails in the caudal region of this specimen.     

Salmonid fish: model organisms to study cardiovascular morphogenesis in conjoined twins?

Background

There is a gap in knowledge regarding the cardiovascular system in fish conjoined twins, and regarding the cardiovascular morphogenesis of conjoined twins in general. We examined the cardiovascular system in a pair of fully developed ventrally conjoined salmonid twins (45.5 g body weight), and the arrangement of the blood vessels during early development in ventrally conjoined yolk sac larvae salmonid twins (<0.5 g body weight).

Results

In the fully developed twins, one twin was normal, while the other was small and severely malformed. The mouth of the small twin was blocked, inhibiting respiration and feeding. Both twins had hearts, but these were connected through a common circulatory system. They were joined by the following blood vessels: (i) arteria iliaca running from arteria caudalis of the large twin to the kidney of the small twin; (ii) arteria subclavia running from aorta dorsalis of the large twin to aorta dorsalis of the small twin; (iii) vena hepatica running from the liver of the small twin into the sinus venosus of the large twin. Among the yolk sac larvae twins investigated, distinct vascular connections were found in some individuals through a joined v. vitellina hepatica.

Conclusions

Ventrally conjoined fish twins can develop cardiovascular connections during early development, enabling a normal superior twin to supply a malfunctioning twin with oxygen and nutrients. Since the yolk sac in salmonids is transparent, twinning in salmonids may be a useful model in which to study cardiovascular morphogenesis in conjoined twins.

     

Unique anomalies in cephalothoracopagus janiceps conjoined twins with implications for multiple mechanisms in the abnormal embryogenesis

The anatomic features of female conjoined twins with the Janiceps type of cephalothoracopagus are described. Abnormalities included bilateral clefts of the alveolar arches, shared rudimentary mandible, high, arched clavicles, multiple rib deformities, single shared foregut and small intestine, absent large intestines, omphalocele, multicystic kidneys, hypoplastic lungs, interconnected aortas and neck vessels, single ovary with elongated uterus in each twin, displaced labia, abnormal segmentation of the vertebrae, spinal dysraphism, diastasis of the symphysis pubis, malrotated lower extremity, bilateral posterior dislocation of the hips, and club feet. There were two hearts with internal anomalies. Both spinal cords had a myelocele in the lumbar region. The abnormalities noted in previous reports of conjoined twins of this type are reviewed and compared. We propose that factors associated with conjoining, dysgenetic (developmental) defects, and deformations resulting from crowding in utero all may have been important in the abnormal development in this case.     

Upsetting the balance on sex selection

It is widely assumed that the strongest case for permitting non‐medical sex selection is where parents aim at family balance. This piece criticizes one representative attempt to justify sex selection for family balance. Kluge (2007) assumes that some couples may seek sex selection because they hold discriminatory values, but this need not impugn those who merely have preferences, without evaluative commitments, for a particular sex. This is disputed by those who see any sex selection as inherently sexist because it upholds stereotypes about the sexes. This article takes an alternative approach. I argue that, even if we accept that preference‐based selection is unobjectionable, a policy permitting selection for family balancing does a poor job of distinguishing between value‐based and preference‐based selection. If we wish to permit only preference‐based sex selection we should seek to identify parents’ motives. If we wish to justify a family balancing policy, other arguments are needed.