There is no record of both separated ischiopagus tetrapus conjoined twins leading normal lives 22 years after separation. We separated a pair of such twins in 1986 and have followed them up till date. Details regarding the technique of separation and the procedures required to ensure normal defecation and micturition and normal musculoskeletal function have been described by us in earlier communications. In this paper we describe their present status and ability to face the world as independent adult females. The outcome reflects the responsibilities and dedication of pediatric surgeons for infant patients with congenital problems after they have become adults, taking a pivotal role to involve surgeons of other disciplines as and when necessary.
Conjoined twins; ischiopagus twins; twins seperation
Asymmetric and parasitic conjoined twins are rarer anomalies of monochorionic monoamniotic twins, consisting of an incomplete twin attached to the fully developed body of the co-twin.
A 30-year-old multigravid woman referred to maternal fetal unit due to polyhydramnios at 28th week of gestation. Sonographic examination revealed a single fetus and polyhydramnios with amniotic fluid index 30 cm. The fetus had normal apparent single head, spine, thorax, abdomen, two upper and two lower limbs, and two relatively well developed rudimentary parasitic lower limbs at sacral region. Lower limbs of the autosite were moving freelly but no movement was detected at the parasite. The parasite contained irregular lower limbs and left foot with three toes. Short and deformed long bones were also present in the parasitic limbs. A Cesarean section was performed at 38th week of gestation and a live female infant weighing 3600 g was delivered. The parasitic lower limbs were totally excised. Post-operative period was uneventful and the newborn was discharged as healthy. Post-natal follow-up was normal at nine-month-old.
Pygopagus tetrapus parasitic twin is a rare form of conjoined twins and in utero diagnosis with ultrasound assists in prenatal management and counselling with parents.
Objective: To summarize our experience in the anesthetic management of conjoined twins undergoing one-stage surgical separation.
Methodology: Medical records of conjoined twins admitted to our hospital for treatment and considered for surgical separation from 1996 to present were retrospectively reviewed. Four cases of conjoined twins underwent one-stage surgical separation under general anesthesia. Preoperative evaluation was performed to determine the extent of anatomical conjunction and associated anomalies. Anesthesia was simultaneously induced in all conjoined twins. The intubation procedure was successfully performed with the head slightly rotated to each baby’s side, followed by the administration of vecuronium. Anesthetic agents were administered according to the estimated weight of each baby. One case of conjoined twins underwent surgical separation with cardiopulmonary bypass due to shared hearts.
Results: All conjoined twins were successfully separated. No significant respiratory or cardiac events occurred during surgery except for one twin, which died after separation because of complicated congenital heart disease.
: Accurate preoperative evaluation, respiratory and circulatory management, and close cooperation of the multidisciplinary team are important aspects of anesthetic management of conjoined twins surgery.
Conjoined twins; Separation; Anesthetic management; Surgery
One of the most interesting congenital malformations is that of conjoined twins. We report echocardiographic features of twin heart in dicephalus, tribrachius, dispinous, thoracoomphalopagus twin. It showed two hearts fused at atrial level. Right-sided heart had single atrial chamber with a single ventricle. A single great vessel, aorta, originated from it. Left-sided heart was well developed with two atria and two ventricles. There was a small mid muscular ventricular septal defect and a small patent ductus arteriosus. Great arteries had normal origins.
Cardiac malformations in twins; conjoined twins; fused heart
Conjoined twins show varying degree of conjoining in either facing or side-by-side fashion. Cephalothoracopagus janiceps is a prototype of facing anomaly in which the two bodies demonstrated a cross symmetry to the midline, that is axial symmetry. Interfacial and intersternal lines crossed at a right angle and no abnormality of situs was associated. Dicephalus dipus dibrachius is a case of side-by-side union, in which the bodies facing nearly the same direction were symmetrical to the middle sagittal plane. Abnormal situs of one was always associated. Other types of conjoined twins as thoracopagus lie between the two extremes of facing and side-by-side union. The three dimensional architectures of the organs in each type would be explained using cross sectional figures of skull, thorax and pelvis. Although the facing twins share the internal organs without fusion, the organs in the side-by-side component are fused with modification of the situs. We postulate sixteen pairs of situs and four manners of division for the explanation of the midline organs and the presence of a dominant co-twin. The splenic locations in a given cardiopulmonary situs are evaluated for the appraisal and applicability of these hypotheses.
Conjoined twins are rare variants of monozygotic twins, which result from an incomplete division of the embryonic disk. Cephalothoracopagus is a rare twin pregnancy described as imperfect fusion of the head and chest, but separated columns, limbs, and pelvis. They occur with incidence rates that range from 1 per 50,000 to 1 per 100,000 births; however, the incidence of the cephalothoracopagus variety is 1 per 58 conjoined twins. In the case of identical and symmetric faces caused by the orientations of the 2 notochordal axes that are perfectly ventroventral, they are called janiceps disymmetros. We present a prenatal diagnosis of a typical case of cephalothoracopagus janiceps disymmetros and the diagnostic confirmation by image and pathology exams.
Heteropagus twinning is a rare occurrence. Parasitic and asymmetric conjoined twins are rarer anomalies of monochorionic monoamniotic twins; which consist of an incomplete twin attached to the fully developed body of the co-twin. We present here two such cases of Heteropagus twinning.
Heteropagus; Twins; Omphalocele
Thoracoomphalopagus is the commonest type of conjoined twin where the bodies are fused from upper chest to lower chest. The autopsy done can help counsil the parents for further pregnancies and determine the prognosis depending upon the type of cardiac anomaly by Seo classification when detected antenatally. We describe the detail pathological autopsy of such a case with single heart and single liver.
A detail autopsy was done on the twin fetus.
The twins shared a single heart and sometimes the liver and part of digestive system. The combined weight was 4.1 KG. Both were full-term male babies joined from below the nipple till umbilicus.
Autopsy in conjoined twins helps in deciding the type of fusion of the body and also of the heart and great vessels. It can help in counseling parents about future pregnancies that there is no chance of recurrence of this abnormality and no need to be scared.
Premature xiphoomphalopagus conjoined twins were successfully separated at 7 days of age. The total body weight of the twins before separation was 3502 g. One twin had persistent patent ductus arteriosus and signs of cardiac failure at 4 days of age. This was managed with indomethacin. Thirty-six hours later the second twin became anuric, necessitating early emergency separation. The twins were joined from the lower sternum to the infraumbilical area. The liver was fused, but there was no major vascular connection. There were no other major anomalies. The babies stood the procedure well and were healthy at follow-up 8 months after separation.
An ectopic or accessory limb attached to the back is an extremely rare and strange condition, and there are only a few documented cases in the worldwide literature. The first case was described by Jones and Larkin (1889). There are several theories regarding the origin of this condition. Asymmetric conjoined twinning which is located dorsally in the vertebral column (rachipagus) is the most probable diagnosis of our patient. Conjoined twinning is very rare and the incidence is 1 per 50 000 live births. Rachipagus is even rarer, with no more than 30 case reports so far (Chadha et al. (1993, 2006)). In this report, we present a patient who underwent successful surgical excision of a third arm attached to the back in the midline over the low-dorsal region. Differential diagnoses including teratoma and fetus in fetu are discussed.
This study reports on the experience of one hospital regarding the surgical aspects, anatomic investigation and outcomes of the management of 21 conjoined twin pairs over the past 20 years.
All cases of conjoined twins who were treated during this period were reviewed. A careful imaging evaluation was performed to detail the abdominal anatomy (particularly the liver), inferior vena cava, spleen and pancreas, either to identify the number of organs or to evaluate the degree of organ sharing.
There were eight sets of ischiopagus twins, seven sets of thoracopagus twins, three sets of omphalopagus twins, two sets of thoraco-omphalo-ischiopagus twins and one set of craniopagus twins. Nine pairs of conjoined twins could not be separated due to the complexity of the organs (mainly the liver and heart) that were shared by both twins; these pairs included one set of ischiopagus twins, six sets of thoracopagus twins and one set of thoraco-omphalo-ischiopagus twins. Twelve sets were separated, including seven sets of ischiopagus twins, three sets of omphalopagus twins, one set of thoracopagus twins and one set of craniopagus conjoined twins. The abdominal wall was closed in the majority of patients with the use of mesh instead of the earlier method of using tissue expanders. The surgical survival rate was 66.7%, and one pair of twins who did not undergo separation is currently alive.
A detailed anatomic study of the twins and surgical planning must precede separation. A well-prepared pediatric surgery team is sufficient to surgically manage conjoined twins.
Conjoined Twins; Surgical Separation; Ischiopagus Tripus Conjoined Twins; Omphalopagus Conjoined Twins; Thoracopagus Conjoined Twins
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.
Dicephalic parapagus tribrachius conjoined twin is a very rare condition.
We present a case of 5-days-old male dicephalic parapagus conjoined twins. The conjoined twins were diagnosed in 4th month of pregnancy by ultrasonography. The pregnancy terminates at 36 weeks by cesarean section and triplets were born. The babies were male conjoined twins and another healthy male baby. Many congenital defects of interest can now be detected before birth. Sever form of this malformation precludes postpartum life. It is supposed that with advances in screening methods for prenatal diagnosis these cases are terminated in first or second trimester of pregnancy.
Dicephalic Parapagus Tribrachius; Conjoined Twins; Triplet Pregnancy
To determine the accuracy of prenatal and postnatal echocardiography in delineating the degree of cardiac fusion, intracardiac anatomy (ICA), and ventricular function of 23 sets of conjoined twins with thoracic level fusion presenting to a single centre over a 20 year period.
13 thoracopagus, 5 thoraco‐omphalopagus, and 5 parapagus pairs presenting to the authors' institution between 1985 and 2004 inclusive were assessed. Echocardiographic data were analysed together with operative intervention and outcome. Twins were classified according to the degree of cardiac fusion: separate hearts and pericardium (group A, n = 5), separate hearts and common pericardium (group B, n = 7), fused atria and separate ventricles (group C, n = 2), and fused atria and ventricles (group D, n = 9).
The degree of cardiac fusion was correctly diagnosed in all but one set. ICA was correctly diagnosed in all cases, although the antenatal diagnosis was revised postnatally in three cases. Abnormal ICA was found in one twin only in two group A pairs, one group B pair, and both group C pairs. All group D twins had abnormal anatomy. Ventricular function was good in all twins scanned prenatally, and postnatally function correlated well with clinical condition. Thirteen sets of twins in groups A–C were surgically separated; 16 of 26 survived. None from groups C or D survived.
Prenatal and postnatal echocardiography accurately delineates cardiac fusion, ICA, and ventricular function in the majority of twins with thoracic level fusion. It is integral in assessing feasibility of separation. The outcome in twins with fused hearts remains dismal.
congenital heart disease; echocardiography; cardiac surgery; prenatal diagnosis
Nearly completed conjoined or fused symmetrical twins are generally called diplopagus. Sheep conjoined twins have been reported less than cow. An apparently female conjoined twin lambs was examined based on external and internal features. In radiology, two vertebral columns and two pairs of the ribs were seen. Only two heads and two necks were separated (thoraco-omphalopygopagus). There were three forelimbs (tribrachius), one of which grew on dorsal region as a notomelus. Teat buds of the monsters differed in number. Only one lamb had umbilicus, including one umbilical vein, and two umbilical arteries locating besides one urinary bladder. This lamb had also one uterus. Two-separated alimentary tracts were observed in a common abdomen. Common thorax and abdominal cavities were separated by a diaphragm. There were two esophageal hiatuses, and two caval foramina but only one aortic hiatus. Two pairs of lungs and two unequal and connected hearts in a common pericardium were observed. Abnormality of the circulatory system might have caused the death of the twins.
Conjoined twins; Diplopagus; Notomelus; Sheep; Tribrachius
12 cases of conjoined twins from West Africa were reported between 1936 and 1978. Eight sets were liveborn and were surgically separated either in local hospitals or abroad. Four were stillborn. Two new cases of stillborn conjoined twins were recently delivered at this hospital. The most common type and the ones most likely to be born alive were the omphalopagi. Surgical separation was successful in 5 cases but the twins separated at Zaria died about a month later. Emergency operations were performed on the pygopagus and ischiopagus, and one member of the former but both of the latter died. The thoracopagus and dicephalus twins were stillborn. However, necropsy findings in one of the thoracopagi indicate that surgical separation would have been feasible had the twins been born alive. The internal mechanical factors causing cardiac defects in such twins may be relevant to the study of the pathogenesis of congenital cardiac malformations.
We explored the prevalence of syringomyelia in a series of 113 cases of fetal dysraphism and hindbrain crowding, of gestational age ranging from 17.5 to 34 weeks with the vast majority less than 26 weeks gestational age. We found syringomyelia in 13 cases of Chiari II malformations, 5 cases of Omphalocele/Exostrophy/Imperforate anus/Spinal abnormality (OEIS), 2 cases of Meckel Gruber syndrome and in a single pair of pyopagus conjoined twins. Secondary injury was not uncommon, with vernicomyelia in Chiari malformations, infarct like histology, or old hemorrhage in 8 cases of syringomyelia. Vernicomyelia did not occur in the absence of syrinx formation. The syringes extended from the sites of dysraphism, in ascending or descending patterns. The syringes were usually in a major proportion anatomically distinct from a dilated or denuded central canal and tended to be dorsal and paramedian or median. We suggest that fetal syringomyelia in Chiari II malformation and other dysraphic states is often established prior to midgestation, has contributions from the primary malformation as well as from secondary in utero injury and is anatomically and pathophysiologically distinct from post natal syringomyelia secondary to hindbrain crowding.
Syringomyelia; Fetal; Chiari II malformation; OEIS; Dysraphism; Spinal cord
Dental anomalies of number and forms may occur in the primary and permanent dentition. Various terms have been used to describe dental twinning anomalies: Germination, fusion, concrescence, double teeth, conjoined teeth, twinned teeth, geminifusion, and vicinifusion. Fused tooth is a developmental anomaly that is seen more frequently in the primary than the permanent dentition. Double tooth involving deciduous anterior teeth is found mostly in the mandible. Very few cases of nonsyndromic double primary molar have been reported in the literature. The succeeding permanent tooth is often found missing congenitally in the same region. This article reports a very rare unilateral occurrence of an anomalous, primary mandibular first macromolar formed by fusion with a dysmorphic premolar like supernumerary tooth in deciduous dentition period. Instead of agenesis of succedaneous tooth, the double tooth has been succeeded by normally developing mandibular first premolar in the same region.
Fusion; nonsyndromic; supernumerary
This paper is written in response to controversial judicial decisions following separation surgery on conjoined twins "Jodie" and "Mary". The courts, it is argued, seem to have conceptualised the twins as "entangled singletons" requiring medical intervention to render them physically separate and thus "as they were meant to be", notwithstanding the death of the weaker twin, "Mary". In contrast, we argue that certain notions, philosophical and biological, of what human beings are intended to be, are problematic. We consider three compelling conceptualisations of conjoined twins and advocate a model that conceives them as two psychologically separate individuals who happen to share a body, the sharing of a body being integral to the individuality of each twin. While we reject an "essentialist" view of the conjoined state, a view which might render separation surgery unthinkable in all cases, we nevertheless argue against an "adversarial" interpretation of conjoined twins' respective best interests. We maintain that the physical entanglement should be regarded as a shared problem rather than one posed by one twin to the other. And if, after deliberation, separation surgery is deemed the "least detrimental alternative" or the "lesser of two evils", then there should be recognition of what conjoined twins will lose, as well as gain, through separation. The current drive to separate twins at all costs may evince a deeper unease with bodily configurations that appear to threaten the premium that the Western ethical and legal tradition places on personal sovereignty, and the physical circumscription that such sovereignty assumes.
Cases of conjoined twins occur so rarely that it is important to learn as much as possible from each case.
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.
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.
Epigastric heteropagus is an extremely rare congenital anomaly, in which there is asymmetrical conjoined twinning, with the parasitic twin attached to the epigastrium. A 2-day-old male, with epigastric heteropagus and omphalocele, was operated in our institution. After excision of the parasitic twin, omphalocele was covered with a gluteal skin flap available from the parasitic twin. Post-operative course was uneventful, except for infection along the edges of the skin flaps, which was managed conservatively. Only 44 cases of epigastric heteropagus twins have been reported previously in world literature. We present a novel surgical approach for the repair of the omphalocele in a case of epigastric heteropagus twins, probably the 45th case to be reported in the world literature.
Conjoined twins; epigastric heteropagus; gluteal skin flap
We present a case of female cephalothoracoomphalopagus conjoind twin, which is extremely rare type of conjoined twins. We also review the contemprory knowledge regarding incidence, etiopathogenesis, antenatal diagnosis and outcone or the prognosis of conjoint twins. The case belong to hindu female, no history of consanguineous marriage, ingestion of drugs or exposure to any radiation. History of one abortion and one twin delivery present. Conjoind twin was cephalothoracoomphalopagus type, delivered vaginaly at 30 wks of gestion to a 25 yrs old multigravida. Management of conjoind twin still remain challenges because of multiple congenital anomalies and poor outcome is seen.
Cephalothoracoomphalopagus; conjoind twin; outcome
Prior studies have indicated that marriage is negatively associated with male antisocial behavior. Although often interpreted as a causal association, marriage is not a random event. As such, the association may stem from selection processes, whereby men less inclined towards antisocial behavior are more likely to marry.
To evaluate selection versus causation explanations of the association between marriage and desistence from antisocial behavior.
Co-twin control analyses in a prospective twin study provided an analog of the idealized counterfactual model of causation. The co-twin control design uses the unmarried co-twin of a married twin to estimate what the married twin would have looked like had he remained unmarried. Discordant monozygotic (MZ) twins are particularly informative as they share a common genotype and rearing environment.
289 male-male twin pairs (65% MZ) from the Minnesota Twin Family Study assessed at ages 17, 20, 24, and 29 years. None of the participants were married at age 17, and 2.6% were married at age 20. By age 29, 58.8% of the participants were or had been married.
Main Outcome Measure
A tally of Criterion C symptoms of DSM-III-R Antisocial Personality Disorder, as assessed via structured clinical interview.
Mean differences in antisocial behavior across marital status were present even at ages 17 and 20, suggesting a selection process. However, the within-pair effect of marriage was significant for MZ twins, such that the married twin engaged in less antisocial behavior than his unmarried co-twin. Results were equivalent to those in dizygotic twins and persisted when controlling for prior antisocial behavior.
Results indicate an initial selection effect, whereby men with lower levels of antisocial behavior are more likely to marry. However, this tendency to refrain from antisocial behavior appears to be accentuated by the state of marriage.
antisocial personality disorder; marriage; desistence; twin study; co-twin control analyses