Craniopagus Twins: Surgical Anatomy and Embryology and Their Implications
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.39.1.1 on 1 January 1976. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry, 1976, 39, 1-22 Craniopagus twins: surgical anatomy and embryology and their implications J. E. A. O'CONNELL1 From the Department of Neurological Surgery, St. Bartholomew's Hospital, London SYNOPSIS Craniopagus is of two types, partial and total. In the partial form the union is of limited extent, particularly as regards its depth, and separation can be expected to be followed by the survival of both children to lead normal lives. In the total form, of which three varieties can be recognized, the two brains can be regarded as lying within a single cranium and a series of gross intracranial abnormalities develops. These include deformity of the skull base, deformity and displacement of the cerebrum, and a gross circulatory abnormality. It is considered that these and other abnormalities, unlike the primary defect, which is defined, are secondary ones; explanations for them, based on anatomy and embryology, are put forward. The implications of the various anomalies are discussed and the ethical aspects of attempted separation in these major unions considered. Protected by copyright. The birth of conjoined twins is an infrequent future. The practical problems resulting from occurrence. Its incidence is estimated as one in disordered anatomy are discussed and their 50 000 births (Potter, 1961). The union is a solution, complete or partial, described. The cephalic one in 2.0% which makes the incidence abnormal anatomy is analysed and related to of craniopagus twins one in 2.5 million births. embryological development. The nature of the The occurrence is thus a rarity, although, since primary congenital defect is discussed. A classi- numerous cases are not reported in the literature, fication of craniopagus is produced, differentia- it is likely that its frequency is greater than these ting partial from total unions so that confusion figures indicate. Should the twins survive, the in the literature between the different types of possibility of their separation will be raised with case may be avoided in the future. Finally, the a neurosurgeon and if the union is an extensive ethical problems which arise in connection with one, as it commonly is, the problem is a daunt- the surgical treatment of total vertical cranio- ing one. Not only is it unlikely that the surgeon pagus are discussed. http://jnnp.bmj.com/ will have had any previous experience of the con- The material studied has been the records of dition, but until recent times there was little in three personally treated cases of craniopagus the literature to guide him. The first report mentioned above and previously reported. For (Grossman et al., 1953) described the separation the present paper, certain clinical features and of a pair of such twins with the survival of one results of investigation as well as operative of them. Subsequently (O'Connell, 1964; findings are reconsidered. The postmortem O'Connell, 1968) came descriptions of separation studies made in each of the twins of the second on October 1, 2021 by guest. operations in three cases of total vertical and third pairs who died during surgery are craniopagus, one of each pair surviving. This correlated with the other findings. In addition, study of personal material has been made in the through the kindness of colleagues, postmortem certain knowledge that further attempts to studies of another example of craniopagus, not separate craniopagus twins will be made in the seen during life, have been possible. The small but increasing literature dealing with cranio- I Address for reprint requests: Fishing Cottage, ltchen Abbas, near Winchester, Hampshire. pagus has been reviewed and where appropriate (Accepted 16 July 1975.) related to the present work. 1 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.39.1.1 on 1 January 1976. Downloaded from 2 J. E. A. O'Connell CLASSIFICATION OF CRANIOPAGUS much of the dura mater of one twin from that of the other. The result after separation was excellent in According to the site of the cephalic junction, it one child but the other, whose superior sagittal sinus has been customary to classify cases of cranio- was possibly injured at operation, has survived pagus as parietal, frontal, or occipital, over 60% disabled and had repeated operations for cerebro- of them being parietal. This method ofclassifica- spinal fluid fistula and ventricular dilatation (Fig. Ia). tion is inadequate since it makes no distinction The second pair (Baldwin and Dekaban, 1958) had a between unions of limited superficial extent and frontal union (Fig. 1 b); this was an oval area measur- depth and the extensive unions in which the two ing 8.0 cm x 6.5 cm and united the children's right cranial cavities are in wide connection. It is frontal regions. There was a dural defect within this area but intact leptomeninges covered the exposed proposed that the cases be placed in two groups, frontal cortex of each cerebrum. Separation and partial and total. In partial craniopagus, the survival of both were achieved without defect and surface area of the heads involved is usually they were developing well seven years later. In the relatively small and the crania may be intact and third pair (Wolfowitz et al., 1968), the union was also simply fused in this area; or there may be a a frontal one but more localized (Fig. ic), so that cranial defect and the dura mater of the twins there was some mobility of one head from the other. be in contact and possibly fused within the The essential of the operative procedure was the defect; or the dura mater may be absent and the separation of a relatively small area of dura mater of leptomeninges covering the related areas of one child from a similar area in the other, and this cerebra are in contact with one another. The was achieved with the survival ofboth children. From what it will be realized that follows, such partial Protected by copyright. abnormality extends no deeper and is, in fact, unions are an entirely different problem from the simply a defect of cerebral coverings and fre- total ones. quently a limited one. It should be noted that occasionally the junction in partial craniopagus In addition to these three cases of localized may appear to be extensive, yet radiological craniopagus treated surgically in recent years, examination reveals that a layer of bone repre- others must clearly have been born, if the senting the fused skulls of the twins extends over incidence of craniopagus twins is one in two- much of the junction. Such a superficial union and-a-half million births. Of a series of 22 cases must, of course, be classified as partial cranio- (Robertson, 1953) two were frontal and five pagus. In total craniopagus there is an extensive occipital. Details are not available, but it is area of surface involvement and the cranial likely that several of these were cases of the cavities are widely connected; indeed, the two partial form. No doubt there have been other brains are contained within a common cranium. reports of cases of localized craniopagus This leads to the very important alterations in throughout the world but the literature is not a cerebral development and circulation which will true guide to the frequency ofsuch abnormalities. be described later in detail. Partial craniopagus http://jnnp.bmj.com/ is thus essentially an extracranial abnormality 2. Total craniopagus and total vertical craniopagus a gross intracranial In this variety the union is extensive. It is one. The partial unions may be frontal, parietal, commonly vertical and described in the literature or occipital; total craniopagus is, from its as parietal. However, very widespread unions nature, likely to be biparietal and, since it is a can occur elsewhere. Klar (1963) described an very wide union, it is thought can better be extensive frontal union and the author has seen described as vertical. radiographs of twins with an extensive parieto- on October 1, 2021 by guest. occipitotemporal union. In some of the reports 1. Partial craniopagus of such extensive unions in situations other than Three pairs of twins of this type have been the vertical one (Sapinski, 1966), it seems possible reported in the literature, all having been that the wide union was vertical at an early treated surgically. developmental stage. Later intrauterine forces The first of these (Voris et al., 1957) was a central altered the relationship of the long axes of the vertical union in which the dura mater of both infants to one another so that a gross angulation children was intact and a thin layer ofskull separated occurred at the site of junction between the J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.39.1.1 on 1 January 1976. Downloaded from Craniopagus twins: surgical anatomy and embryology and their implications 3 4 H.K I ( 1i J ..... .; . _r >' > A Protected by copyright. '^''M-. C--i<>wi;FR $ ....... ..4Fr . / .......... a} X '; 8 Al oV'. ( I-,) (p A Y .; f: VIV_E i .t.fi. +kG 'x2''@} http://jnnp.bmj.com/ rIB on October 1, 2021 by guest. 'C Wv>%'.A.. ( K,. FIG. 1 Partial craniopagus: (a) Voris et al. (1957); (b) Baldwin and Dekaban (1958); (c) Wolfowitz et al. (1968). J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.39.1.1 on 1 January 1976. Downloaded from 4 J. E. A. O'Connell the skull is involved and the appearance is that of a common cranium for the two brains. Examination of personal material suggests that according to the degree of rotation of one twin's head on that of the other, around the longi- tudinal axis, three types of total vertical cranio- pagus can be distinguished.