Rehabilitation and functional outcomes for thoraco-omphalo-ischiopagus conjoined twins five years after separation: A case report.

Conjoined twins are identical twins joined in utero and are a rare phenomenon. This report discusses a case of female thoraco-omphalo-ischiopagus tripus conjoined twins. The twins were separated at age two, and once medically stable, spent one month in inpatient rehabilitation to improve their sitting balance and gross motor skills. This was followed by outpatient physical therapy. The twins initially had customized ZipZac seats, which they were able to wheel independently. After six months of therapy, the girls began walking with posterior walkers and prostheses. The hemipelvectomy prosthesis included a customized thoracolumbosacral orthosis component and was directly attached to a non-articulated pylon. A manual-locking hip joint was added to accommodate sitting. An articulated ankle-foot orthosis was used for the intact leg. Care of formerly conjoined twins requires comprehensive care from a multidisciplinary team involving, but not limited to, a physiatrist, orthopaedic surgeon, physical therapist, and orthotist/prosthetist. Complex congenital limb deficiencies are often a major undertaking for the rehabilitation team as continuous treatment and management are needed throughout the patient's lifetime due to growth, development, and evolving physical demands. Anatomic variations must be examined on a case-by-case basis but often include limb deficiencies, orthopedic abnormalities, and organ comorbidities.

Monochorionic triplet pregnancy complicated by conjoined twins and early twin-twin transfusion syndrome.

BACKGROUND: The condition of monozygotic, monochorionic triplet fetuses with a pair of conjoined twins is extremely rare (close to one in a million births), presents challenges in its management, and with poor prognosis. CASE REPORT: We report a case of monochorionic diamniotic triplet pregnancy, ultrasound at 14 weeks shows a pair of conjoined thoracopagus fetuses, sharing heart, liver, and umbilical cord, in addition to omphalocele. The third fetus, without malformations, presents signs of early heart failure compatible with twin-to-twin transfusion syndrome. It was decided to carry out expectant management where at 18 weeks, intrauterine death of the three fetuses occurs. An abortion is performed by hysterotomy. CONCLUSIONS: The treatment in these cases is discussed, three management options have been proposed: expectant management, selective reduction of the conjoined fetuses, or termination of the pregnancy. A review of the literature found only 12 cases with this combination of pathologies, in which only 3 normal fetuses (25%) survived and none of the conjoined twins survived. To our knowledge, this case is the first of a monochorionic triplet pregnancy with conjoined fetuses complicated with early twin-to-twin transfusion.

A Rare Case of Conjoined Twins.

Identical twins joined in utero are called conjoined twins. They are also called "Siamese twins." It is one of the uncommon variants of twin pregnancy. Our case report aims to demonstrate the significance of prenatal diagnosis and evaluation of conjoined twins due to the increased risk of perinatal morbidity and death. Early prenatal diagnosis and assessment of the degree of joining provide an opportunity for parents to decide whether to continue the pregnancy.

Phenotypically Discordant Anomalies in Conjoined Twins: Quirks of Nature Governed by Molecular Pathways?

A multitude of additional anomalies can be observed in virtually all types of symmetrical conjoined twins. These concomitant defects can be divided into different dysmorphological patterns. Some of these patterns reveal their etiological origin through their topographical location. The so-called shared anomalies are traceable to embryological adjustments and directly linked to the conjoined-twinning mechanism itself, inherently located within the boundaries of the coalescence area. In contrast, discordant patterns are anomalies present in only one of the twin members, intrinsically distant from the area of union. These dysmorphological entities are much more difficult to place in a developmental perspective, as it is presumed that conjoined twins share identical intra-uterine environments and intra-embryonic molecular and genetic footprints. However, their existence testifies that certain developmental fields and their respective developmental pathways take different routes in members of conjoined twins. This observation remains a poorly understood phenomenon. This article describes 69 cases of external discordant patterns within different types of otherwise symmetrical mono-umbilical conjoined twins and places them in a developmental perspective and a molecular framework. Gaining insights into the phenotypes and underlying (biochemical) mechanisms could potentially pave the way and generate novel etiological visions in the formation of conjoined twins itself.

Symmetrical parapagus diprosopus: A comparative, computed tomographic, and pathoanatomical study of a new case in domestic pig.

BACKGROUND: Parapagus diprosopus are conjoined twins characterized by craniofacial duplication and only one body, representing one of the rarest types of these twins. Their occurrence has been recorded in different species of vertebrates, including humans, but few cases have been studied in domestic pigs. CASE: A pair of conjoined twin pigs was studied using x-rays, computed tomography, and necropsy. The abnormalities found were compared with those of the rare swine cases presented in the literature as well as with other species, and the different etiopathogenetic possibilities were addressed. The degree of duplication of the head bones decreased caudally, as did that of the structures of the central nervous system. In the two oral cavities, there was a complete cleft palate. All the cervical vertebrae and thoracic vertebrae up to T3 were partially duplicated. The heart and great vessels were normal, as were the other thoracic and abdominal organs. CONCLUSIONS: The conjoined twin pigs of this study are a case of parapagus diprosopus tetraophthalmus triotus, presenting the same pattern of abnormalities of human diprosopus and that of other species. The scarcity of detailed studies on craniofacial duplication in pigs and the lack of a definitive explanation on the etiology and pathogenesis of conjoined twins shows the need for further research and the publication of more cases.

Overview of Swine Congenital Malformations Associated with Abnormal Twinning.

A review of congenital malformations in swine relating to abnormal twinning was carried out. The aim was to describe and estimate these defects. Among the recorded twins, the most common defect was the syncephalus thoracopagus or cephalothoracopagus. A couple of dicephali and diprosopus congenital anomalies were also registered. At last, some cases of thoraco-omphalopagus piglets were surveyed. There was also a report of an acardiac twin (hemiacardius acephalus) and a case of a conjoined parasitic twin. The pathogenetic mechanisms of this condition, frequently reported in veterinary practice, are discussed. The importance of embryonic imperfect twinning is commonly associated with dystocia.

Technique for liver separation using the modified liver hanging maneuver (LHM) in a case of thoraco-omphalopagus conjoined twins.

INTRODUCTION: Conjoined twins are rare, high-stakes cases requiring complex management. This report presents thoraco-omphalopagus conjoined twins who shared parts of liver and abdominal wall. The main obstacle in separating these patients was liver fusion. PRESENTATION OF CASE: Two-year-old female conjoined twins presented with liver fusion between segments 2 and 3 in twin A and segments 2 and 4A in twin B. Liver separation was performed using the modified liver hanging maneuver. After separating the triangular ligaments and surrounding adhesions, a 12-Fr Foley catheter was inserted under the fused liver, suspending it upward and producing a clear separation plane. Liver separation required only 32 min. No notable bleeding or bile leakage was observed. DISCUSSION: The liver hanging maneuver was originally used as an adjunct technique for liver tumor hepatectomy. We used this technique to separate the fused livers in conjoined twins. The advantages of LHM include potentially reducing excess manipulation, which may result in parenchyma and vascular pedicle injury, and facilitating bleeding control, thereby reducing the operation time. CONCLUSION: The liver hanging maneuver using a conventional Foley catheter is a simple and useful method for separating fused livers in conjoined twins.

Conjoined thoracopagus twins: A systematic review of the anomalies and outcome of surgical separation.

Introduction: Conjoined twin is an extremely rare condition and requires a thorough knowledge of anatomy, and a multidisciplinary approach is essential to successfully separate the twins. Thoracopagus twins lie face to face and are attached from chest to upper abdomen. They are the most common among all the varieties but have a poor survival rate. Materials and Methods: This study is a review of literature from 2019 to the oldest via PubMed and Google Scholar using keywords: Conjoined twins, Thoracopagus twins, Thoracoomphalopagus and Thoraco-omphalopagus twins. The articles were reviewed for the description of the anatomy of shared organs, management and outcome of these twins. Results: One hundred and fifty-eight sets of thoracopagus and thoraco-omphalopagus twins including our twins were included in this study. Out of 158 reported thoracopagus twin sets in literature, with M: F ratio of 1:2.3, 71 sets were found to be non-operable and all of them subsequently expired; 82 sets were operated upon, out of which 83 babies survived, suggesting an overall surgical success rate of about 50%. Conclusion: Thoracopagus twins have a dismal prognosis. The most important decisive parameter for successful separation is the extent of sharing of organs between twins. The role of a motivated multidisciplinary team is also indispensable and cannot be overemphasised.

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.

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.

Craniorachipagus symmetric conjoined twinning: Identification of a fourth case and delineation of the phenotype.

BACKGROUND: Symmetric craniorachipagus is an exceedingly rare type of bi-umbilical conjoined twinning, known from only three scantily described cases. CASE: We identified a fourth, previously described case that was misdiagnosed as janiceps and as pygopagus. It concerned dorsally conjoined twins that were part of a triplet pregnancy, spontaneously born at 22 weeks of gestation. Radiography confirmed union at the occipital craniums and the thoracolumbar vertebral columns. Both twins had their own separate umbilical cords. To delineate the phenotype of craniorachipagus and to differentiate it from rachipagus without cranial involvement, we compared the present case with the previous three reported cases and with the historical literature concerning comparable conditions. Furthermore, we discuss why exceedingly rare conditions such as these are presently underreported in the literature. CONCLUSION: Symmetric craniorachipagus is a type of bi-umbilical conjoined twinning, to date represented by four confirmed cases that share a similar phenotype. This includes dorsal conjunction at the sides of the occipital craniums and the vertebral columns, in the absence of any visceral connections. Details on its etiopathogenesis and apparent lethality await additional case investigations. No unequivocally confirmed cases of symmetric rachipagus without cranial involvement have been reported and its existence in humans has yet to be proven.

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.

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.

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.

Is a classical incision the only way to perform a near-term conjoined twins' cesarean section? Thoracopagus conjoined twin delivery by a low-segment transverse incision.

Conjoined twin pregnancies are rare, usually occurring in cases of monochorionic monoamniotic twin pregnancies. The most common type of conjoined twins, thoracopagus (42%), is difficult to deliver via a low-segment transverse incision hysterotomy after 35 weeks of gestation. Therefore, conjoined twin cesarean deliveries are typically performed using the classical incision method. However, this often leads to an increased risk of postoperative maternal morbidity and uterine rupture during a subsequent pregnancy. Because of the low survival rate of conjoined twins, subsequent pregnancies are often desired. Hence, minimizing trauma to the uterus is a primary concern. A technique for delivering conjoined twins at 35 weeks of gestation by cesarean delivery with a low-segment transverse incision hysterotomy is proposed here. A video is included to explain and demonstrate these procedures. This method can minimize uterine trauma and maximize the chances of a successful subsequent pregnancy.

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.

A Case of Thoraco-Omphalopagus Conjoined Twins: Clinical Imaging and Anatomical Classification.

Conjoined twins are a rare phenomenon estimated to occur in a range between 1 in 49,000 births and 1 in 189,000 births. As a product of monochorionic-monoamniotic pregnancies, they are currently believed to result from late, incomplete fission of the bilaminar embryonic disk at 13-15 days gestation. Conjoined twins are typically classified by the point at which their bodies are joined, with 15 recognized types, five of which account for more than 70% of cases. Fusion of the thorax and upper abdomen (thoraco-omphalopagus) accounts for 28% of all cases. Mortality and morbidity rates remain high irrespective of the point of fusion, with 40-60% of cases being lost to miscarriage and stillbirth, and only about 18% of live births surviving more than 24 hours. Given this prognosis, knowledge of underlying anatomy and clinical imaging is paramount to antenatal diagnosis, assessment of viability, and subsequent management of conjoined twins. A case of thoraco-omphalopagus twins with a single heart and single liver discovered on routine ultrasound at 12 weeks gestation is described.

A Detailed Description and Discussion on Conjoined Twins.

Conjoined twins are described as having been physically fused during pregnancy and delivery. They were first mentioned a long time ago when there was not much known about this. They share some organs that are vital for survival, like the heart; these twins are almost impossible to save, but there are some cases wherein there is evidence of their survival. The article aims to present a unique discussion on conjoined twins. This article talks about the formation of conjoint twins, their types and nomenclatures, embryological concepts, past history/traditional tales, case studies, and the medical enhancements happening in this area. Both fission and fusion are thought to contribute to the disease. A monozygotic twin pregnancy cleaves when it occurs more than thirteen days after fertilization. There is just one placenta and one womb for conjoined twins (one amniotic sac). The twins that are born and stay alive after delivery usually stay alive for a few days or weeks. It's pretty rare for them to live a long prosperous life, but this article shows the otherwise, too, like the Siamese twins, which is a unique example of conjoint twins who lived for a long time. This kind of pregnancy is a complex procedure that needs to be managed by a team of professionals.

Thoraco-omphalopagus conjoined twin: A rare case report.

INTRODUCTION AND IMPORTANCE: Conjoined twins represent a rare phenomenon and the etiology has not been clarified yet. There is a high rate of stillbirth and neonatal deaths resulting in very few cases surviving long enough for surgical separation. CASE PRESENTATION: A 33-year-old gravida 2 para 1 mother without any first and second trimester antenatal care visits was diagnosed to have conjoined twins in the third trimester. Mother and her family chose to terminate the pregnancy for which elective lower section cesarean section was done with the delivery of female conjoined twins, both of them subsequently declared dead within 4 h of birth. CLINICAL DISCUSSION: A conjoined twin gestation provides inimitable intricacy for obstetric management irrespective of the patient's areas of care. Early diagnosis through ultrasonography can be done and detailed evaluation is necessary along with fetal echocardiography regardless of site of fusion. Cesarean section is the recommended mode of delivery as this reduces various complications. CONCLUSION: The obstetricians' role in timely prenatal diagnosis, counseling, and organization of interdisciplinary medical care is indispensable in cases of conjoined twins.