Parasitic pygopagus conjoined twins' incidental findings in the obstructed labor of an intrapartum dead baby that had undergone emergency C-section: rare case report in South Papua.

BACKGROUND: Conjoined twins (CT), which used to be historically defined as "monstrous human" and previously so-called Siamese twins in the early eighteenth century, are one of the very rare congenital malformations with an uncertain etiology and complex yet remain inconclusively debatable regarding its pathophysiological mechanisms of fusion and fission theories. Among all types of CT, parasitic CT, especially the pygopagus sub-type, is exceedingly rarer. To the best of the authors' knowledge, no parasitic CT had been reported in Papua, and this is the first finding in South Papua. CASE REPORT: Herein, a 30-year-old multigravida female with 37th-week gestation, previous twice spontaneous miscarriage, and non-adequate antenatal care history is presented with a chief complaint of painful construction and greenish fluid leakage from the vagina, with an examination that showed a cephalic presentation with a "peculiar" big mass at the upper uterus and complete cervical dilation toward second-stage inpartu. Vaginal delivery was performed with a complication of obstructed labor due to uncommon dystocia with a suspected "big mass" below the fetal buttocks and intrapartum dead. Intrapartum transabdominal ultrasound demonstrates a gross anatomically like an organ inside a fluid-filled mass with unidentified parts, leading to a suspected type of congenital malformation at the baby's sacral region. Emergency C-section was done with findings of parasitic pygopagus CT, showing an attachment of a large irregular fluid-filled mass-like incomplete twin (parasite) with palpable soft tissue and bony structure inside to the buttocks of a male autosite twin, and an additional third leg which happened to be an under-developed lower extremity with a sacrum-like structure. CONCLUSIONS: An obstetrician's routine ANC and critical radiological evaluation will increase the odds of identifying CT or other congenital malformations to provide better delivery planning or further management. Increasing maternal health knowledge in society, improving medical skills and knowledge levels for health providers, and advancing supporting facilities and specialists are future strategies for managing and preventing such cases in low-middle-income countries.

The application value of prenatal ultrasound in conjoined twins.

OBJECTIVE: To evaluate the application and effectiveness of prenatal ultrasound in diagnosing and managing conjoined twins. METHODS: A retrospective analysis was conducted on 20 cases of conjoined twins diagnosed at our hospital between January 2016 and December 2022. The types of conjoined twins, ultrasonographic characteristics, and associated anomalies were assessed. RESULTS: The gestational age at diagnosis ranged from 10 to 35 weeks, with an average of 14.21 ± 5.69 weeks. Thirteen cases were detected in the first trimester, five in the early second trimester, one at 23 + 2 weeks, and one at 35 weeks. Thoracopagus was the most common type (11 cases, 55%), followed by omphalopagus (4 cases, 20%), cephalopagus (4 cases, 20%), and parapagus dicephalus (1 case, 5%). In the first trimester, the most common abnormalities observed included increased nuchal translucency (NT), cystic hygroma, hydrops fetalis, and generalized edema. Major birth defects identified in conjoined twins were omphalocele (3 cases), congenital heart malformations (3 cases), neural tube defects (2 cases), urachal cyst (1 case), and umbilical cyst (1 case). Pregnancy was terminated in 18 cases, one case resulted in spontaneous abortion during the second trimester, and one case was delivered by cesarean section at 37 weeks, with successful separation and recovery. CONCLUSION: Prenatal ultrasound is the primary diagnostic tool for conjoined twins. It effectively assesses the extent of twin fusion, provides critical information for clinical decision-making, and aids in the management of obstetric care.

Integrated multiomics reveal the molecular characteristics of conjoined twin fetuses.

RESEARCH QUESTION: Are there any differences between conjoined twin fetuses at the molecular level? DESIGN: Skin tissues were collected from thoracopagus conjoined twins at 15+4 weeks of gestation. The skin tissues were collected from the thigh side of conjoined twins after the abortion procedure. All specimens were obtained after written informed patient consent and were fully anonymized. All relevant ethical regulations were followed. Every specimen underwent multiomics sequencing analysis to determine associations among the DNA methylome, transcriptome and mutations in the exon regions in the conjoined twins. RESULTS: The global methylation pattern was similar in the two fetuses of conjoined twins, while significant differences were seen in local regions such as CpG islands (P = 0.026), enhancers (P < 0.001) and various repetitive elements (P < 0.05), which showed significant differences. The conjoined twins also differed in genes related to growth and development, cellular component morphogenesis and cellular stress, both in terms of DNA methylation levels and gene expression levels. Exon data analysis revealed that the common mutations in conjoined twins mainly occurred in neural development, lipid metabolism and microtubule morphogenesis. Specific mutations were associated with cellular component biosynthesis, behaviour and germ cell development. CONCLUSION: Conjoined twins were similar to each other globally, but there were significant differences related to growth and development, cellular component morphogenesis and cellular stress. The current study reveals the molecular features of conjoined twins for the first time, laying the foundation for future exploration of the mechanism of conjoined twins.

Separation of conjoined spinal cords in symmetrical pygopagus twins using intraoperative neuromonitoring: pearls and pitfalls.

Pygopagus twin is a rare congenital malformation with a worldwide incidence of 1in 200,000. Few literature reports are published regarding the matter. In some cases, neuromonitoring is essential for safe surgical separation. We believe it is important to share our challenges and nuances in order to minimize obstacles one might encounter. We utilized neuromonitoring during our separation of both twins, and we planned a multidisciplinary approach and efficient communication system with the other teams in order to plan a successful, safe, and timely separation of the twins. We seek to highlight not our success but rather the obstacles and challenges we encountered during the separation of pygopagus twins in our institute using neuromonitoring for future reference.

The role of intraoperative neuromonitoring in pygopagus separation: two-institution experience in Indonesia.

INTRODUCTION: Pygopagus comprises 17% of all conjoined twin cases. Survival rate is higher compared to other variations of conjoined twins, but separation is a great challenge due to multiorgan involvement. Intraoperative neuromonitoring (IONM) used aims to aid operator in preserving as much function as possible. CASE PRESENTATIONS: The authors reported 2 pairs of pygopagus separation. Intraoperatively, motor-evoked potential (MEP) and sensory-evoked potential (SEP) were used in all patients. Three patients survived in which all had transient motor deficits. Urinary retention was reported in one patient. One patient died 2 weeks after separation as twins only had one kidney which was spared for the healthier twin. DISCUSSION: IONM was used to guide operator in dissecting, identify the ownership of the neural structures, and determine the safest point to separate in pygopagus separation. Despite the normal MEP and SEP recordings, transient motor weakness may still occur transiently. The motor tract development of children is achieved in adolescence, making MEP less accurate. However, the reliability of MEP increases when it is combined with SEP. Autonomic function monitoring such as bulbocavernosus reflex (BCR) could not be assessed due to the unavailability of the probe. CONCLUSION: IONM can aid operator in pygopagus separation during determining the origins of the structure, dissecting, and cutting the neural structures. Normal MEP interpretations are still possible to correlate with transient deficits, but reliability can be improved with the use of SEP. In surgeries involving the lower spine level, BCR monitoring is recommended to avoid autonomic deficits.

Postnatal imaging of conjoined twins: a customized multimodality approach.

Medical imaging plays a crucial role in the management of conjoined twins. The role of imaging is to explore the anatomy, outline the shared organs and determine whether surgery is feasible. It also serves as a roadmap for successful separation. Additionally, imaging helps with counseling parents about prognosis. This review aims to illustrate recent advances in different imaging modalities and their role in the management of the various types of conjoined twins with an emphasis on relevant tips for optimal imaging.

Emergency separation of conjoined twins in a tertiary hospital in Indonesia: three case reports.

BACKGROUND: Emergency separation of conjoined twins is performed when one twin is already dead or dying and threatens the survival of the other. The particular decision to perform an emergency separation of conjoined twins provides an ethical dilemma that needs special attention. Adding to the complexity of surgical and postsurgical management in emergency separation, ethical and sociocultural aspects further complicate decision-making. CASE PRESENTATION: From 1987 to 2022, 18 conjoined twin separations were performed in our centre. This paper describes three conjoined twin emergency separations. In the first case of thoracoomphalopagus babies at nine days of age, one baby was diagnosed with necrotizing enterocolitis with frequent desaturation and seizures, and the other baby was healthy. Emergency separation was performed on the twelfth day of age; unfortunately, neither baby survived the surgery. In the second case, emergency separation was performed on the 110th day of life due to sepsis in one baby. The nonseptic twin passed away six hours after surgery, while the septic twin died 12 days after surgery due to wound dehiscence and abdominal sepsis. The third case was of an omphalopagus conjoined twin with a parasitic twin. The healthy baby was deemed nonviable but found to be healthy upon birth. Immediate emergency separation was performed at 2 h of age. The living baby survived the surgery but passed away two months later. CONCLUSIONS: When separation is deemed necessary to save one twin, it becomes difficult to apply standard ethical medical reasoning. The decision to separate results in most cases in very high-risk surgeries with poor outcomes during surgery and postsurgery. Compounded by the complexity of the case, sociocultural and religious aspects further add to the dynamics of decision-making. A multidisciplinary team must work together with a health ethics committee and navigate through this ethical conundrum with the patient and family at its decision-making centre to decide on the best plan of care.

Thoracopagus conjoined twins.

OBJECTIVE: We present a case report of conjoined twins. CASE REPORT: Secundigravida nullipara, 28-years old, admitted for profuse bleeding at 13 weeks of gestation. Ultrasound confirmed vital pregnancy of conjoined twins - thoracopagus. After prenatal diagnostic consultation the patient decided for termination of pregnancy. Molecular analysis confirmed a female fetus without any chromosomal anomalies. CONCLUSION: The occurrancce of conjoined twins is very rare. Early prenatal ultrasound diagnosis plays an important role. Presented case report describes conjoined twins with poor prognosis because of one shared malformed heart.

Conjoined twins after single blastocyst transfer: a case report including detailed time-lapse recording of the earliest embryogenesis, from zygote to expanded blastocyst.

Conjoined twins are estimated to occur in 1:50 000 pregnancies. Eighteen cases of pregnancies achieved by ART have been published of which three were achieved after single embryo transfer, allowing discussion of embryo characteristics. We report, to the best of our knowledge, the first case of parapagus conjoined twins after ART. Furthermore, this is the first report of conjoined twins with detailed morphokinetics of the earliest embryogenesis from zygote to expanded and hatched blastocyst stage. The case zygote had three refractile bodies, which were all allocated to one blastomere at first cleavage following an asynchronous pronuclei fading. Within 2 h, this blastomere cleaved to four and fragmented. The remaining blastomere cleaved symmetrically and regularly and a blastocyst (score: 4AB) was vitrified 120 h after IVF. Pregnancy was achieved following a frozen-thawed single blastocyst transfer. The etiopathogenetic mechanism of the origin of conjoined twins is unknown and several hypotheses exist. The morphokinetics in the present case and morphology of other reported cases will be discussed in this context.

The role of the University of Padua medical school in the study of conjoined twins between 18th and early 19th century.

The Medical School of Padua (Italy) contributed profoundly to the study of teratology. Many famous physicians and professors of medicine, such as Liceti, Vallisneri, Morgagni, and Malacarne, have studied and investigated these anomalies to better understand the causes and to find a potential explanation, often preserving the specimens for future studies. The present study highlights some historical cases of conjoined twins and a conjoined triplet preserved at the Morgagni Museum of Human Anatomy to show the development of medical theories in the teratological field between the 18th and early 19th century. This approach will provide insights into different study methods and ideas of some of the most famous scholars working in Padua at that time. The current article focuses on rare cases, both human and animal, that were encountered by physicians who worked in the Veneto area in the late 18th and early 19th century. Their detailed descriptions are not only of historical but also of contemporary dysmorphological value.

Cephalothoracoomphalopagus - a rare type of conjoined twins from the pathologists perspective.

Objective: Case description and autopsy fi nding in conjoined twins dia gnosed in the 24th week of pregnancy. Results: We reporta case of a 31-year-old primigravida who was referred to the Department of Gynecology and Obstetrics at our hospital with a presumptive dia gnosis of conjoined twins. The ultrasound examination with subsequent three-dimensional (3D) image reconstruction demonstrated twin gestation complicated by cephalothoracoomphalopagus. Observations demonstrated that the twins were joined over an area that extended from the head to the thoraces down to the central abdomen. In view of multiple congenital malformations incompatible with postnatal life, the pregnancy was terminated. The twins then underwent an autopsy at the Department of Pathology and the autopsy confi rmed previous diagnosis. Conclusion: Cephalothoracoomphalopagus is one of the rarest forms of conjoined twins with unknown incidence due to a very small number of documented cases.

Minimally Jointed Symmetrical Pygopagus Twins - Successful Surgical Separation.

Conjoined pygopagus accounts for about 17% of all conjoined twins and commonly share the gluteal region, terminal spine, lower gastrointestinal, urological, and reproductive tracts. This makes their separation a very challenging task. We report herein pygopagus twins who had minimal fusion in coccygeal region and could be separated without a complicated procedure. The case is being reported to add to the literature and emphasize that simple fusion in conjoined twins can be relatively easy to manage.

Cross-cultural representations of conjoined twins.

Conjoined twinning is a rare birth defect estimated to occur in about 1 in 50,000 to 100,000 births. The mechanism of conjoined twinning is not proven. Different forms of conjoined twinning are observed with the thoracopagus form being the most common. The rate of conjoined twinning is similar across all major populations. A dramatic malformation of this type would be an extraordinary occurrence leading people to reflect on the spiritual or supernatural nature of such an event. Therefore, it is not surprising that artifacts that seem to depict different forms of conjoined twins are seen across diverse cultures. In this article, we present a survey of these cultural artifacts including anatomic classification based on external anatomy and an exploration of the cultural and spiritual contexts associated with the artifacts. A key finding is that the most common form of conjoined twinning in the artifacts is parapagus (both dicephalus and diprosopus) in contrast to thoracopagus, the most common form in epidemiologic studies. Potential reasons for this difference are discussed. Evidence is presented to support the speculation that these objects represent artistic impressions of actual conjoined twinning events.

Conjoined twins in dichorionic diamniotic triplet pregnancy: a report of three cases and literature review.

BACKGROUND: Conjoined twins are a rare and serious complication of monochorionic twins. The total incidence is 1.5 per 100,000 births, and about 50% are liveborn. Prenatal screening and diagnosis of conjoined twins is usually performed by ultrasonography. Magnetic resonance imaging can be used to assist in the diagnosis if necessary. Conjoined twins in dichorionic diamniotic triplet pregnancy are extremely rare. CASE PRESENTATION: We reported three cases of dichorionic diamniotic triplet pregnancy with conjoined twins. Due to the poor prognosis of conjoined twins evaluated by multidisciplinary teams, selective termination of conjoined twins was performed in three cases. In case 1, selective reduction of the conjoined twins was performed at 16 gestational weeks, and a healthy female baby weighing 3270 g was delivered at 37 weeks. In case 2, the conjoined twins were selectively terminated at 17 weeks of gestation, and a healthy female baby weighing 2760 g was delivered at 37 weeks and 4 days. In case 3, the conjoined twins were selectively terminated at 15 weeks and 2 days, and a healthy female baby weighing 2450 g was delivered at 33 weeks and 6 days. The babies of all three cases were followed up and are in good health. CONCLUSION(S): Surgical separation is the only treatment for conjoined twins after birth. Early determination of chorionicity and antenatal diagnosis of conjoined twins in triplet gestations are critical for individualized management options and the prognosis of normal triplets. Expecting parents should be extensively counseled by multidisciplinary teams. If there are limitations in successful separation after birth, early selective termination of the conjoined twins by intrathoracic injection of potassium chloride may be a procedure in dichorionic diamniotic triplet pregnancy to improve perinatal outcomes of the normal triplet.

Twin-twin transfusion syndrome complicated by single ventricle physiology: A case report.

Clinically significant extrauterine twin-twin transfusion syndrome in conjoined twins is rare and carries a high risk of perinatal mortality. The ensuing postnatal imbalance in circulation across connecting vessels results in hypovolemia in the donor and hypervolemia in the recipient. Data on management and treatment are sparse especially in the setting of a single ventricle congenital heart defect. We present a case of a pair of omphalopagus conjoined twins, one with a single ventricle physiology (Twin B), who developed twin-twin transfusion syndrome shortly after birth. The resulting pathophysiology in the setting of a single ventricle congenital heart defect created added layers of complexity to their management and expedited surgical separation. Shunting from Twin B to Twin A-with an anatomically normal heart-resulted in mal-perfusion and rapid deterioration jeopardizing the health of both twins. In the preoperative course, steps taken to medically optimize the twins prior to surgery and the anesthetic considerations are detailed in this report.

The Outcomes of Surgical Separation in Thoracopagus Twins with Conjoined Hearts: An Analysis of the Literature.

We sought to perform a comprehensive review of reported cases of conjoined thoracopagus twins with cardiovascular conjunction to determine the overall survival after separation and factors that might be associated with survival. We performed a systematic review of cases of thoracopagus twins with conjoined cardiovascular systems reported in the English medical literature using Embase, PubMed, Web of Science, and Scopus. We employed standard statistical methods to analyze differences among groups. We identified 102 unique cases of thoracopagus twins (69% female) with some degree of cardiovascular conjunction who had undergone surgical separation. We identified 6 distinct types of cardiovascular union. Median age was 47 days (IQR 12, 120). Survival to at least hospital discharge occurred in 51% (105/204). Median age at separation was lower in emergent (9 days, IQR 1, 25) versus elective cases (93 days, IQR 49, 180) (p < 0.0001). Survival to hospital discharge was higher in those who underwent elective separation (70%, 93/132 total children versus 17%, 12/72) (p < 0.0001). Survival was associated with the type of cardiovascular union (p < 0.0001). The separation of thoracopagus twins with cardiovascular conjunction is higher than expected. Increased survival is associated with elective separation, older age, and shared pericardium only. These findings suggest survival could be improved with better delineation of degree of union and delayed separation.

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons.

Shared anomalies, always located close to the area of coalescence and observable in virtually every type of conjoined twinning, are currently seen as separate anomalies caused by mostly unknown and seemingly unrelated pathways rather than being connected to the twinning mechanism itself. Therefore, most (case) reports about conjoined twins are mere descriptions of (external) dysmorphologies lacking reflections on the possible origin of their concomitant anomalies. As we will demonstrate in this article, shared anomalies are influenced, and in some cases solely and sequentially explained, by interaction aplasia and neo-axial orientation; two embryological mechanisms to which each set of conjoined twins is subjected and are responsible for their ultimate phenotypical fate. In this review, we consider how the ventral, lateral and caudal conjunction types and their intermediates determine the phenotypic presentation of the twins, including patterns of shared malformations and anomalies, which in themselves can be indistinguishable from those encountered in singleton cases. Hence, it can be hypothesized that certain anomalies in singletons originate in a fashion similar to that in conjoined twins.

Conjoined Thoracopagus Twins - Our Experience of Successful Separation.

Conjoined twin is an extremely rare condition and needs a thorough knowledge of anatomy and a multidisciplinary approach is essential to successfully separate, the twins. Thoracopagus are the twins attached by chest and umbilicus and are the commonest among all the varieties but carries a poor survival rate. We describe our approach and experience of management of thoracopagus twins who were separated at eighty-three day of life and are alive and well after 4 years of follow up. The most important decisive parameter for successful separation is the extent of sharing of organs between twins but the role of a motivated multidisciplinary team is also indispensable.

Three-dimensional ultrasonographic features of diamniotic conjoined twins with body stalk anomaly.

BACKGROUND: Since conjoined twins were thought to be monoamniotic in the past, diamniotic conjoined twins would be improbable theoretically. Body stalk anomaly is a severe defect of the body wall, which is rare among twins. Body stalk anomaly in monochorionic diamniotic conjoined twins has never been reported prenatally so far as we know. CASE PRESENTATION: Here we present an extremely rare case of concordant body stalk anomaly in monochorionic diamniotic conjoined twins. Ultrasonography at 9 + 5 weeks revealed one chorionic and two amniotic cavities, close apposition of abdomen, limited movement, and common umbilical vessels. Follow-up ultrasonography at 11 + 6 weeks and 13 + 2 weeks showed close apposition of the lower abdominal region with cystic structures and a small bowel-like mass between the two fetuses. Three-dimensional ultrasonography assisted in observing the entire appearance of both twins in earlier first trimester, including amnioticity, conjoined region and umbilical vessels. We attribute this diamniotic conjoined twin in our case to the fusion theory. A single yolk sac was observed, challenging the idea that yolk sac number predicts amnionicity. Identification of single yolk sac and its allantois may form a common body stalk during this fusion, leading to concordant body stalk anomaly in monochorionic diamniotic twins. CONCLUSIONS: Our case may provide important insights into both ultrasonographic features and embryogenesis of this extremely rare anomaly.

Lessons from an historical museum of anatomical pathology: thoraco-abdominopagus fetus.

Historical Museums of Anatomical Pathology are a relevant teaching tool for medical undergraduate students and postgraduate residents. The visualization of real specimens allow a deeper comprehension of diseases, namely of rare conditions as in Teratology. This article emphasizes the need to preserve and use Universities Museums, by presenting a XIXth Thoraco-Abdominopagus Fetus.