Monosymmetros Cephalothoracopagus Tetrabrachius and Tetrapus Piglets with Syndromic Evolution.

Conjoined twins are rare congenital malformations that have been reported in mammals. Two different cases are presented in this study. Case No. 1 features monocephalic, thoracopagus-conjoined twin piglets with anencephaly and palatoschisis of the Pietrain breed, and case No. 2 features monocephalic, thoracopagus conjoined twin piglets with palatoschisis and bifid root tongue of a mixed breed. These cases were examined using post-mortem and computed tomography (CT) examinations. In both cases, the conjoined symmetrical twins had a single head, one neck, and fused thoracic cavities, while the abdominal cavities were separated. Similarly, in both cases, they had four forelimbs and four hindlimbs and duplicated foramen magnum. During CT examination, in case No. 1, severe abnormalities were observed in the skull and vertebral column. In the left twin, occult dysraphism was seen from the C2 vertebra until the end of the vertebral column, and in the right twin, from the C3 vertebra until the end of the state vertebral level. In case No. 2, the oral cavity contained a tongue with a bifid root connected with one hyoid bone, and the soft palate presented a small cleft. During CT examination, the parietal bone and the occipital bones were partially duplicated. This case also presented occult dysraphism, but only in the cervical vertebrae, C1-C6 for the left twin and C1-C5 for the right twin. In both cases, abnormalities of the internal organs were revealed during necropsy. Conjoined twins with multiple congenital anomalies presented here enhance our understanding of the various clinical forms of conjoined cases in veterinary medicine.

Twin fetuses associated with double amniotic sacs diagnosed using transvaginal ultrasonography: A case report.

BACKGROUND: Conjoined twins are a rare twin malformation commonly presenting as single amniotic sac twinning, with double amniotic sac twinning being extremely rare and poorly reported. Most conjoined twins are females. CASE SUMMARY: A woman of childbearing age conceived naturally, and at 8 wk of gestation, transvaginal ultrasonography showed an embryo and cardiac tube pulsation in both amniotic sacs. On dynamic observation, the two embryos were connected in the lower abdomen, with restricted movement. A repeat transvaginal ultrasound at 11 wk showed that the intestinal tubes of both fetuses were connected in the lower abdomen. The pregnancy was terminated and labor was induced. CONCLUSION: Transvaginal ultrasound may detect conjoined twin malformations in an early stage. Our case provides diagnostic insights for ultrasonographers and can help develop early therapeutic interventions.

Antenatal imaging diagnosis of thoraco-omphalopagus conjoined twins.

Conjoined twins occur in an estimated one in every 200,000 live births. The etiology remains largely speculative, with genetic and environmental factors being considered. The thoraco-omphalopagus type accounts for approximately 40% of cases, making it a focal point for clinical and radiological research. Radiological imaging plays a pivotal role in delineating anatomical details, offering insights into the feasibility of surgical interventions and informing parental counselling regarding prognosis and management options. We present a case of thoracoomphalogus conjoined twins diagnosed during the third trimester of pregnancy in a 19-year-old woman. The detailed radiological assessment using ultra-sound and MRI provided crucial information on organ sharing and vascular anatomy, which is critical for management strategies. This case underscores the critical role of prenatal imaging in detecting complex congenital anomalies, facilitating informed decision-making by healthcare providers and families.

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.

Thoraco-Omphalopagus Conjoined Twins: A Rare Presentation in the Dominican Republic.

The case involves a 23-year-old Dominican woman's admission to Hospital Docente Nuestra Señora De la Altagracia for an elective cesarean section at 38 weeks gestation with conjoined twins. Despite effective treatment for syphilis in the third trimester, her medical history complicated the situation. The twins, thoraco-omphalopagus conjoined, share vital organs and exhibit congenital anomalies, posing unique diagnostic and management challenges. This case contributes to the scarce literature on conjoined twins, especially in the Dominican Republic. It highlights the complexities of diagnosis, prognosis, and management strategies for such rare cases. This emphasizes the importance of ongoing research and medical intervention in addressing these challenges.

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.

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.

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.

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.