Dicephalus parapagus conjoined twins discordant for anencephaly: a case report

Usang E Usang1*, Babatunde J Olasode2, Ayi E Archibong1, Jacob J Udo1 and Diana-Abasi U Eduwem1 *Corresponding author: Usang E Usang usangue@yahoo.co.uk Author Affiliations 1 University of Calabar/University of Calabar Teaching Hospital, Calabar, Cross River State, Nigeria 2 Obafemi Awolowo University/Obafemi Awolowo University Teaching Hospitals Complex, Ile Ife, Osun State, Nigeria
Journal of Medical Case Reports 2010, 4:38 doi:10.1186/1752-1947-4-38 The electronic version of this article is the complete one and can be found online at: http://www.jmedicalcasereports.com/content/4/1/38

We recently encountered live dicephalus parapagus conjoined twins discordant for anencephaly who survived for 9 hours after delivery.

Case presentation
9-hour-old female conjoined twins with one torso and two heads were brought into the sick babies unit (SBU) by a 25-year-old Nigerian mother of the Ekoi tribe in Cross Rivers State who just had her first delivery. She had limited antenatal care (ANC) in a primary health centre where no antenatal ultrasonography had been carried out. The pregnancy, which was carried to term, was characterized by regular use of an herbal enema from the onset and polyhydramnios. The delivery had been completed vaginally at home without any obstruction to labour. The normal head presented first. Only the normal twin cried after several minutes of stimulation. The combined weight of the conjoined twins at the time of admission was 2.85 kg. Clinical examination revealed two discordant heads (Figure 1). The normal and anencephalic heads had an occipito-frontal circumference of 34 cm and 24 cm, respectively. There was a single thorax with two neurologically independent upper limbs, single abdomen, one complement of genitalia and an anus as well as two neurologically independent lower limbs. Figure 1. The conjoined twins with discordant heads. At presentation in the SBU, the anencephalic twin was unresponsive to painful stimulus with dilated and unreactive pupils. An orogastric tube was inserted that ended in the neck of the twin. The normal twin remained stable for a short while but soon experienced repeated apneic attacks with cyanosed extremities. Though prompt resuscitative measures were taken, the twin died within 9 hours of birth from cardiopulmonary failure. As a result of their unstable condition and the short duration of life, thorough investigation of the twins was not possible.

Received: Accepted: Published:

5 November 2009 5 February 2010 5 February 2010

Abstract Introduction
Cases of conjoined twins occur so rarely that it is important to learn as much as possible from each case.

Case presentation
We present a case of 9-hour-old, female, Nigerian dicephalus parapagus conjoined twins discordant for anencephaly diagnosed only after the birth of the twins. The anencephalic twin was stillborn while the normal one died within 9 hours of birth from cardiopulmonary failure.

Conclusion
Many congenital defects of interest can now be detected before birth. A severe lesion such as that found in our index case, which is incompatible with postnatal life, requires counselling. If detected early enough during a properly monitored antenatal care, it may indicate termination of pregnancy.

Introduction
Conjoined twinning is a rare phenomenon, occurring in 1 in 50,000 to 100,000. However, since 60% are stillborn or die shortly after, the true incidence is around 1 in 200,000 live births [1]. A rarer form of conjoined twinning is the dicephalus parapagus twins discordant for anencephaly in which the laterally united babies have two heads in one trunk. One of the twins has no cranium or brain tissue, but both have upper limbs and two lower limbs. Whereas the incidence of conjoined twinning in our country is unknown, there have been previous reports from Nigeria [2].

Post-mortem Babygram findings

Post-mortem plain X-ray findings showed a fully developed cranium with normal facial structures continuous with the main body. The second head was devoid of a cranium. Each cranium was connected via a separate spine that terminated abruptly at the fifth lumber vertebrae with no evidence of any sacral component (Figure 2). The ribs on the medial side of each twin were fused with each other creating 12 instead of 24

posterior ribs, but the other ribs had not fused. Each upper limb and clavicle appeared borne by the twin on that side. The lungs and heart were not demonstrable but the single pelvis and lower limbs are clearly defined.
Figure 2. A plain X-ray of the twins showing two separate spines.

Autopsy findings
The head with normal calvaria contained a wellformed brain whereas the anencephalic head had no forebrain. Two complements of neck organs and two vertebral columns were demonstrable. The right trachea continued to a right-sided pair of normal lungs while the left trachea joined a pair of collapsed and hypoplastic lungs. A single intestinal tract opened to the exterior as a well-formed anal canal. The other abdominal and pelvic organs were not duplicated. Two pairs of great vessels (Figure 3; arrows), two aortic and two superior vena cavae entered the single heart. There were two atria, two rudimentary auricular appendages and two ventricles.
Figure 3. The twins' single heart with paired great vessels (arrows).

Discussion
Prenatally diagnosed dicephalus conjoined twins discordant for anencephaly has been reported but is rare [3]. It is also rare for such an anomaly to escape antepartum diagnosis and only present at birth, as in the case of our patients, with the current antenatal screening tests carried out in developed countries. The relationship between conjoined twinning and anencephaly is not well understood. However, it has been observed that the incidence of congenital malformations is significantly increased in conjoined twinning, probably due to the later incomplete fission of the monozygotic embryo during embryogenesis (fission theory) or due to secondary union of two originally separate monovular embryonic discs (fusion theory) [4]. For this reason, it is claimed that the same aetiological factor could be responsible for both the conjoining process and congenital malformations [5]. Consequently, there is failure of the neural tube at the cranial end during the fourth week of development [6] causing the forebrain primordium to be abnormal and the development of the calvaria to be defective. This gives rise to anencephaly which is a fatal disorder. While 50% of cases result in fetal demise, the rest die at birth or shortly thereafter as was the case with our discordant conjoined twins. This disorder is also associated with a high risk of preterm delivery before 32 weeks due to the development of polyhydramnios [7], possibly due to the fetuses lacking the neural control necessary for swallowing amniotic fluid. This is probably responsible for the few reported cases of the anomaly in the literature. Spitz [8], in a study of conjoined twins, concluded that one third of those born alive have severe

defects for which surgery is not possible. Similarly, Golladay et al. [9] observed that surgical separation is feasible only when the upper portions of the trunks are sufficiently separate to provide a stable rib cage for each infant. We agree with these authors because the clinical, radiologic and morbid study of our twins showed that separation was impossible. In the case of monozygotic twins discordant for anencephaly, selective termination by occlusion of the umbilical vessels of the abnormal fetus [10] would be the optimal management for the future. This prevents transplacental passage of injurious agents through the common placenta to the normal co-twin, which would occur if this selective termination was achieved by intracardiac injection of potassium chloride [11]. However, when twins are conjoined, as in the case of our patients, they not only share one placenta but have a single umbilical cord through which umbilical vessels are shared [12], therefore selective termination is impossible. We therefore agree with Owolabi et al. [13] that termination of pregnancy should be advised in cases where dicephalic twins are detected early in utero, especially if there is discordance for anencephaly as in the case of our patients. Screening the serum of pregnant women at 16 to 18 weeks' gestation for alpha-fetoprotein can result in the detection of about 80% of fetuses with anencephaly and other neural tube defects[14]. If a woman has a high alphafetoprotein level, ultrasonography is performed to determine whether an abnormality is present. With the advent of high resolution ultrasonography, conjoined twins can be picked up as early as the 8th week of gestation and with fetal echocardiography as well as ultra fast magnetic resonance imaging, evaluated for possibility of postnatal survival [15]. However, most of these facilities are lacking in many of our country's institutions. Moreover, many of the patients do not register for ANC due to poverty and being ill-informed, as in our index case. As a result, prenatal diagnosis of congenital anomalies is unlikely in our region.

Conclusion
This case emphasizes the need for ANC with prenatal ultrasound monitoring of high-risk pregnancies in order to determine the nature of the perinatal management required. When serious malformations that are incompatible with postnatal life are diagnosed early enough, the family has the option of terminating the pregnancy. Therefore, there is a need to improve our health care delivery system to make such services available and accessible to all our pregnant women. Similarly, it is important to educate the women and their spouses on the need for proper ANC.

Consent
Written informed consent was obtained from the parents for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the journal's Editor-in-Chief.

ISRN Obstetrics and Gynecology Volume 2011 (2011), Article ID 238360, 3 pages doi:10.5402/2011/238360

Case Report

Thoracopagus Conjoined Twins: A Case Report

Mehmet A. Osmanaaolu, Turhan Aran, Sleyman Gven, Cavit Kart, zgr zdemir, and Hasan Bozkaya
Department of Obstetrics and Gynecology, Karadeniz Technical University, Trabzon 61080, Turkey Received 21 September 2010; Accepted 3 November 2010 Academic Editor: S. San Martin Copyright 2011 Mehmet A. Osmanaaolu et al. This is an open access article distributed under theCreative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

(Figures 2(a) and 2(b)), and the parents were informed about the malformation and the twins poor chance for survival. The parents decided to terminate the pregnancy. A written informed consent was taken from the family, and the termination of pregnancy was approved by the Medical Ethics Committee. The next two days, after induction of labor with prostaglandin, a vaginal delivery of the conjoined twins was achieved without complication. Figure 1: Image of conjoined twins (thoracopagus) at 11 weeks gestation. Figure 2: Image of conjoined twins after vaginal delivery at 11 weeks gestation. 3. Discussion Conjoined twins are classified according to the most prominent site of conjunction: thorax (thoracopagus), abdomen (omphalopagus), sacrum (pygopagus), pelvis (ischiopagus), skull (cephalopagus), and back (rachipagus). Depending on the aspect of the embryonic disc, the most common types are thoracopagus (19%) [3] (Table 1). Its etiology is unknown, but an incomplete division of the zygote between 13th and 15th days after fertilization probably occurs [4]. The overall survival rate for conjoined twins is approximately 25% [5]. The condition is more frequently found among females, with a ratio of 3:1 [4]. Two theories have been proposed to explain this observation: the process of X-inactivation overlaps with the timing of monozygotic twinning and thus may directly contribute to development of monozygotic twins, and the XX karyotype may confer a survival benefit [2]. Two contradicting theories exist to explain the origins of conjoined twins. The traditional theory is fission, in which the fertilized egg splits partially and conjoined twins represent delayed separation of the embryonic mass after day 12 of fertilization. The second theory is fusion, in which a fertilized egg completely separates, but stem cells (which search for similar cells) find like-stem cells on the other twin and fuse the twins together [4, 6, 7]. Conjoined twins share a single common chorion, placenta, and amniotic sac, although these characteristics are not exclusive to conjoined twins as there are some monozygotic but nonconjoined twins that also share these structures in utero [4, 6]. Early diagnosis of conjoined twins was previously reported, but not before the 10th week of gestation [8]. On careful transvaginal sonography and serial scanning, there appears to be an inability to separate between the anatomical parts of the fetuses. Once conjoined twins have been diagnosed, characterization of the type and severity of the abnormality can be performed with ultrasound, three-dimensional ultrasound, computed tomography, or magnetic resonance imaging [9, 10].

Abstract Objective. Conjoined twin is a rarely seen congenital anomaly together with severe mortality and morbidity. The more common types of conjoined twins include the thoracopagus type, where the fusion is anterior, at the chest, and involves the heart. We are reporting one case of conjoined thoracopagus twins diagnosed by ultrasonography at 11 weeks. Case Report. In a multigravid pregnant woman who has been admitted to our clinic with a diagnosis of conjoined twins, thoracopagus, by ultrasonography at an 11-week gestation, termination of the pregnancy was performed. Conclusion. Making an early diagnosis with ultrasonographic examination gives the parents a chance to elect pregnancy termination. 1. Introduction Conjoined twins represent one of the rarest forms of twin gestation. They occur in roughly 1 in every 200 identical twin pregnancies and are always identical. The incidence ranges from 1 in 50000 to 1 in 100000 live births [1]. Because this situation carries high risk, early diagnosis and management of delivery is extremely important. The role of ultrasound in early diagnosis and management are discussed. 2. Case Report A 31-year-old multigravid woman was referred to our university hospital at an 11-week gestation because of a conjoined twin (thoracopagus) diagnosed by ultrasonography. Her last menstrual date was unknown. She had no personal or family history of twins. Sonography was performed and two fetuses with 2 arms, 2 legs, and 2 heads were visualized. The twins were joined at the thorax and upper abdomen. There was a single umbilical cord, and only one fetal heart was observed (Figure 1). The placenta was localized anteriorly, and one artery and one vein were seen in the umbilical cord. On the basis of these findings, the diagnosis of terata anacatadidyma, thoracopagus, conjoined twins was made

Termination of pregnancy can be offered to the family. In the present study, the diagnosis has been performed in the first trimester, and because the family has chosen termination of this pregnancy, further diagnostic intervention has not been considered. Surgery to separate conjoined twins may range from relatively simple to extremely complex, depending on the point of attachment and the internal parts that are shared. Most cases of separation are extremely risky and life-threatening. In conclusion, conjoined twins are associated with a high perinatal mortality; therefore, making an early diagnosis with ultrasonographic examination of conjoined twins gives the parents a chance to elect pregnancy termination. Acknowledgment This manuscript is presented as a poster at the VIII. National Gynecology and Obstetrics Congress, Antalya, Turkey, 2010. References A. E. J. Rees, G. M. Vujanic, and W. M. Williams, Epidemic of conjoined twins in Cardiff, British Journal of Obstetrics and Gynaecology, vol. 100, no. 4, pp. 388 391, 1993. S. Chitnis, C. Derom, R. Vlietinck, R. Derom, J. Monteiro, and P. K. Gregersen, X chromosome-inactivation patterns confirm the late timing of monoamniotic-MZ twimming, American Journal of Human Genetics, vol. 65, no. 2, pp. 570571, 1999. View at Publisher View at Google Scholar View at PubMed L. Schnaufer, Conjoined twins, in Swenson's Pediatric Surgery, J. G. Raffensperger, Ed., pp. 910920, Appleton Century-Crofts, New York, NY, USA, 4th edition, 1980. T. Abossolo, P. Dancoisne, J. Tuaillon, E. Orvain, J. C. Sommer, and J. P. Rivire, Early prenatal diagnosis of asymmetric cephalothoracopagus twins, Journal de gyncologie, obsttrique et biologie de la reproduction, vol. 23, no. 1, pp. 7984, 1994. J. L. Stone and J. T. Goodrich, The craniopagus malformation: classification and implications for surgical separation, Brain, vol. 129, no. 5, pp. 10841095, 2006. View at Publisher View at Google Scholar View at PubMed R. Spencer, Theoretical and analytical embryology of conjoined twins: part I: embryogenesis,Clinical Anatomy, vol. 13, no. 1, pp. 3653, 2000. View at Publisher View at Google Scholar R. Spencer, Theoretical and analytical embryology of conjoined twins: part II: adjustments to union, Clinical Anatomy, vol. 13, no. 2, pp. 97120, 2000. View at Publisher View at Google Scholar C. Hubinont, P. Kollmann, V. Malvaux, J. Donnez, and P. Bernard, First-trimester diagnosis of conjoined twins, Fetal Diagnosis and Therapy, vol. 12, no. 3, pp. 185187, 1997. K. Kuroda, Y. Kamei, S. Kozuma et al., Prenatal evaluation of cephalopagus conjoined twins by means of three-dimensional ultrasound at 13 weeks of pregnancy, Ultrasound in Obstetrics and Gynecology, vol. 16, no. 3, pp. 264266, 2000. View at Publisher View at Google Scholar View at PubMed

10. C. A. Kingston, K. McHugh, J. Kumaradevan, E. M. Kiely, and L. Spitz, Imaging in the preoperative assessment of conjoined twins, Radiographics, vol. 21, no. 5, pp. 11871208, 2001.

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One of the most interesting congenital malformations is a conjoined twin. Conjoined twins are a rare occurrence in obstetric practice. More commonly known as Siamese twins, this phenomenon is shrouded in mystery and considered a curiosity by general public. Current technology is providing a basis for earlier diagnosis and a better prognosis. Frequently, the twins are born dead, but there are few cases in which the twins survive. We present a case of Dicephalus Dipus Conjoined Twins; a rare type of conjoined twins.

share the same chorion and amnion. This is a very rare condition and accounts for 1-2% of monozygotic twins. The embryonic disk starts to differentiate on the 13th day. If the split occurs after day 13, then the twins will share body parts in addition to sharing their chorion and amnion. [1] The classification of conjoined twins is based on the site of union. The suffix-pagus is used meaning fastened. Thoracopagus - shared thorax. 90% have a shared heart. Omphalopagus - shared abdomen. Thoraco-omphalopagus - one of the most common types. Ileopagus - connected at the iliac bone. When the twins are extensively connected then the duplicated part is named. For example, dicephalus refers to two heads with one body. [1] An attempt to standardise and classify conjoined twins according to the external forms of conjunction has been proposed by Leacham [Table 1] [2] Regardless of the site of union, variations occur with regard to the internal organs. Certain organs may be common to both twins or these may be separate. In the thoracophagus, the heart is often conjoined with associated cardiac anomalies and in omphalopagus the liver is often conjoined. Prognosis, obstetric management and treatment planning are determined by degree of fusion and extent of joining of fetal organs. Cesarean section is recommended in most third-trimester deliveries because of the high incidence of dystocia and resultant fetal damage. [3] Antenatal diagnosis by ultrasound is possible in modern day obstetrics. Ultrasonographic identification of any of the following classical signs may suggest the diagnosis: both fetal heads in the same plane, unusual backward flexion of the cervical spine, no change in the relative position after maternal movement and manual manipulations and inability to separate fetal bodies after careful observation. [4] Dicephalus Dipus Conjoined Twins form a rare variant of conjoined twins. Such variants are usually stillborn or die immediately after birth, but some including the Scottish brothers of James III have lived for a number of years. As most of the studies concentrate on the obstetrical problems, the anatomical description of the dicephalus twins is often incomplete. A conjoint twin is frequently the mirror image of its partner and this is particularly true in dicephalus twins. The right set of lungs may be a mirror image of the normal and the right aorta usually has a right aortic arch. The liver is always single and there is usually one spleen and that is on left. The right stomach may be atrophic but is usually present and the intestines almost always join either just distal to the duodenum or at the level of Meckel's diverticulum. Diaphragmatic hernia are also frequent. The right sided twin of the dicephalus dipus twin will usually have complex cardiovascular anomalies not amenable to surgical correction. This should be taken into account if the surgical separation of this variant of conjoined twins is planned. [5]

Case report A 29 year old women, gravida3, para 2 Last childbirth FTNVD male baby. She had regular menstrual periods before getting pregnant. Her grandmother had a history of twins. She was an unbooked case with no antenatal Ultrasound report. She presented to our hospital at term in the second stage of labour and underwent a vaginal delivery. Twins were delivered as breech. Twins had single male external genitalia, weighing 3 kg together. Placenta was single. On clinical examination it was noted that the twins were conjoined from level of xiphisternum downwards. The twins had two heads, two pairs of upper limbs, a shared pelvis and a single pair of lower limbs (dicephalus, tetrabrachius, dipus twins). Twins had single male external genitalia. Two separate heartbeats were auscultated with heart rate of around 146/min, respiratory rate being 45 for right twin and 52 for the left twin. No murmur was evident clinically. An infantogram was done to further assess the twins; findings of clinical examination were confirmed. Twins had a two heads, two thoraces, two pair of upper limbs and a shared abdomen, pelvis and a single pair of lower limbs. X-ray revealed the twins had two separate thoraces, two hearts and two vertebral columns. Echocardiography revealed that both the hearts were morphologically grossly normal, with no evidence of any gross cardiac anomaly. Ultrasonography of the abdomen revealed a shared liver, a single spleen on the left side and a single pair of kidneys. There was no evidence of any hydronephrosis. Twins were referred to a higher center for surgical management.

Discussion

EMBRYOLOGICAL

BASIS

Four days after fertilization the trophoblast (chorion) differentiates. If the split occurs before this time the monozygotic twins will implant as separate blastocysts each with their own chorion and amnion. Eight days after fertilization the amnion differentiates. If the split occurs between the 4th and 8th days, then the twins will share the same chorion but have separate amnions. If a split occurs after the 8th day and before the 13th day, then twins will

Separation of conjoined twins is complicated procedure. The importance of multidiscipline team with rehearsal of all aspects (surgical, anesthetic and nursing) of the operative procedure cannot be overemphasized. Although the outcome is influenced by careful planning and organization from all participants, the prognosis is often predetermined by the underlying anatomy which may preclude successful separation. [6] Although, conjoined twins are rare, occurring in approximately 1 per 50,000 to 1 per 100,000; it should be suspected in all monochorionic, monoamniotic twin pregnancies and careful sonographic assessment should be performed to identify the presence of shared fetal organs.[7] Siamese twins or double monsters have always been a subject of curiousty and mystery for the general public. The present case highlights morphological features of an antenataly undiagnosed Dicephalus Dipus Conjoined Twins, a rare form of conjoined twins. Indirectly emphasizing the importance of careful antenatal sonographic assessment in all monochorionic, monoamniotic twin pregnancies to rule out conjoined twins. Early prenatal diagnosis and precise characterization of conjoined twins are essential for optimal obstetric, interventional and postnatal management as well as to reduce psychological trauma to the parents.

7. Levi CS, Lyons EA, Martel MJ, Dashefsky SM, Holt

SC. Sonography in the diagnosis and management of Multifetal Pregnancy. In Rumack's Diagnostic Ultrasound: St. Louis, Mosby- Year Book Inc,1997;Chapter 35: 1062-1065.

References 1. Finberg HJ. Ultrasound Evaluation In Multiple Gestation. In Callen's Ultrasonography in Obstetrics and Gynecology:Harcourt Publishers 3rd edition,1994;Chapter 8:121-124. 2. Gerlis LM, Seo JW, Ho SY, Chi JG. Morphology of the cardiovascular system in conjoined twins: spatial and sequential segmental arrangements in 36 cases. Teratology. 1993; 47:91- 108. 3. Tandon R, Sterns LP, Edwards JE. Thoracopagus Twins. Arch Pathol 1974; 98: 248-251. [PUBMED] 4. Kalchbrenner M, Weiner S, Templeton J, Losure TA. Prenatal Ultrasound Diagnosis of Thoracophagus Conjoined Twins. J Clin Ultrasound 1987; 15: 59-63. [PUBMED] 5. Golladay ES, Williams GD, Seibert JJ,Dungan WT, Shenefelt R. Dicephalus Dipus Conjoined Twins: A surgical separation and review of previously reported cases. Journal Of Pediatric Surgery 1982; 17(3): 259264. 6. Miller D, Colobani P, Buck JR, Dudgeon DL, Haller JA. New techniques in the diagnosis and operative management of Siamese twins.. Journal Of Pediatric Surgery 1983; 18(4): 373- 376.