Surgical Correction of Ischiopagus Tripus Conjoined Twins with Fused Pelvis: Enhancing Quality of Life through Orthopedic Intervention.

BACKGROUND The rarity of ischiopagus tripus conjoined twins complicates the surgical separation, owing to the lack of cases and high complexity. We aim to report our experience in performing orthopedic correction for ischiopagus tripus twins. CASE REPORT A pair of 3-year-old conjoined boys presented with a fused body at the pelvis region and only 1 umbilicus. There were 2 legs separated by shared genitalia and an anus at the midline, and 1 fused leg, which could be felt and moved by both of the patients. The twins also shared internal organs of the bladder, intestine, and rectum, as visualized through angiography computerized tomography scan. After several team discussions with the institutional review board, the hospital ethics committee, and both parents, it was agreed to perform disarticulation of the fused third limb, followed by correction of the trunk alignment by pelvic closed wedge osteotomy and internal fixation. We successfully reconstructed the pelvis using locking plates and additional 3.5-mm cortical screws and 1.2-mm stainless steel wire. CONCLUSIONS This report describes the presentation and surgical management of a case of ischiopagus tripus conjoined twins. It highlights the challenges involved in surgery and the importance of investigating these infants for other congenital abnormalities. Although surgical approaches for different sets of twins should be individually tailored, interventions aimed to provide optimal outcomes should consider ethical issues and parental/patient expectations. Even in situations in which the twins are inseparable, there is still room for surgical correction to be performed.

Putting our heads together: The future of craniopagus twin separation.

Computer-assisted design and computer-assisted modeling (CAD/CAM), virtual surgical planning (VSP) and augmented/virtual reality (AR/VR) aid our ability to plan and perform complex craniofacial procedures. This study seeks to define the role of the aforementioned techniques in the separation of craniopagus conjoined twins. Six teams were identified who had successfully performed craniopagus twin separation with the use of CAD/CAM, VSP and/or AR/VR. Surgeons involved in separating craniopagus twins have increasingly utilized tools such as CAD/CAM models, VSP and AR/VR to plan and execute successful separation, and these tools are associated with higher success rates than historical controls.

Anterior Chest Wall Reconstruction After Separation of Thoraco-Omphalopagus Conjoined Twins With Cadaveric Rib Grafts and Omental Flap.

BACKGROUND: Anterior chest wall defects have a wide range of etiologies in the pediatric population, ranging from infection, tumor, and trauma to congenital diseases. The reconstructive goals include restoring skeletal stability, obliterating dead space, preserving cardiopulmonary mechanics, and protecting vital underlying mediastinal organs. Although various reconstructive methods have been described in the literature, selecting the optimal method is challenging for the growing pediatric skeleton. Here, we report a case of previously thoraco-omphalopagus twins who underwent successful separation and reconstruction and presented for definitive anterior chest wall reconstruction. METHODS: A pair of previously thoraco-omphalopagus conjoined twins underwent definitive anterior chest wall defect reconstruction using cadaveric ribs and omental flap. Twin A received 2 cadaveric ribs, whereas twin B had a much larger sternal defect that required 3 cadaveric ribs combined with an omental flap for soft tissue chest coverage. Both twins were followed up for 8 months. RESULTS: Twin A's postoperative course was uneventful, and she was discharged on postoperative day 6. Twin B's course was complicated, and she was discharged on supported ventilation on postoperative day 10. At 8 months postoperatively, both twins healed well, and chest radiographs confirmed the stability of the chest reconstructions. The rib grafts in the twin with a tracheostomy were not mobile, and the patient had a solid sternum with adequate pulmonary expansion. The construct initially did not facilitate pulmonary functioning, but after a healing process, it eventually allowed for the twin with the tracheostomy who required pulmonary assistance to no longer need this device. CONCLUSIONS: Cryopreserved cadaveric ribs and omental flaps offer safe and reliable reconstructive methods to successfully reconstruct congenital anterior chest wall skeletal defects in the growing pediatric population. The involvement of multidisciplinary team care is key to optimizing the outcomes.

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.

Incidental Intraoperative Diagnosis of Term Conjoined Twins: A Case Series.

Conjoined twins (Siamese twins) represent the rarest form of twin pregnancy. Reported here are two rare cases of conjoined term twins presented to the department of Obstetrics and Gynaecology within 3 months. The first case, 32 years of gravida 6 parity 5 referred from periphery after full trial of labour following multi-organ dysfunction and term intrauterine dead twins. Intraoperatively it was dead conjoined thoraco-omphalopagus females. The patient died after 3 days following multiorgan dysfunction syndrome and disseminated intravascular coagulation. The second case, 22 years gravida 2 parity 1 also referred from periphery in second stage of labour with diagnosis of 39 weeks intrauterine dead twins with obstructed labour, delivered by caesarean with intraoperative conjoined dead females of thoracophagus type. Twins are high-risk pregnancy. This rare diagnosis with complications could have been prevented by regular antenatal checkups, ultrasonography performed by radiologists and early referral antenatally in labour along with multidisciplinary approach. Keywords: conjoined; monozygotic twinning; siamese twins; 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.

Surgical Repair of Complete Common Atrioventricular Canal in a Conjoined Twin Following Separation.

Congenital heart disease (CHD) is common in conjoined twins but reports of surgical repair in this population are rare. We present the case of a conjoined twin diagnosed prenatally with a complete common atrioventricular canal defect (CAVC). Due to wound complications from twin separation, definitive repair was deferred and pulmonary arterial banding was performed initially. At 6 months, a complete CAVC repair was performed. The present case adds to the literature on successful cardiac surgical repairs in conjoined twins.

Impact of Technology on Conjoined Twin Separation: Are We Further Ahead?

SUMMARY: The success of conjoined twin separation has increased since the 1950s, notwithstanding the numerous technical and physiologic challenges encountered in such procedures. Together with improvements in our understanding of conjoined twin physiology and the accumulation of surgical expertise, technological advances have largely paralleled the improvement in outcomes. In particular, advances in imaging modalities, adjuncts to abdominal wall closure such as mesh and tissue expansion, and three-dimensional modeling have been instrumental in advancing care for these patients. Considered together, these domains have allowed multidisciplinary teams to better delineate conjoined anatomy, facilitated improved surgical planning and decision-making, and assisted in overcoming the frequent challenges associated with soft-tissue closure. This review delineates the improvements in these modalities and their relationship to increasingly successful conjoined twin separation in recent years.

Viscoelastic Testing and Factor Concentrate Therapy to Reduce Bleeding and Transfusions in the Surgical Separation of Ischiophagus Twins: A Case Report.

The surgical procedure to separate conjoined twins represents a rare and major challenge. One of the most feared perioperative scenarios is the presence of coagulopathy secondary to bleeding. We present a case of the surgical separation of ischiopagus tetrapus twins using a patient blood management strategy encompassing a tranexamic acid infusion, intraoperative viscoelastic testing, and early fibrinogen supplementation to reduce bleeding and transfusions. This approach allowed early detection and treatment of acquired hypofibrinogenemia, which resulted in minimal exposure to blood products. This case reflects the increasing clinical interest in early avoidance of fibrinogen deficiency in complex noncardiac pediatric surgery.

Heteropagus twins: six cases with systematic review and embryological insights.

PURPOSE: Heteropagus twinning (HT) is a rare anomaly. Six new cases along with a systematic review are described. METHODS: Six cases of HT managed at two tertiary care teaching hospitals over the last 26 years are described. A PubMed search with words: Heteropagus AND/ OR parasitic twins from 2001 to 2021 hit 183 articles. 36 were added from non-PubMed sources. Finally, 120 cases including 114 from 69 articles and 6 new cases were analysed. RESULTS: Of the new cases, 2/6 had an antenatal diagnosis. Five were males. 4 autosites had omphaloceles. Split notochord and 2 parasites attached to a single autosite were encountered. 5/6 autosites survived. On systematic review, the most frequent variant seen was rachipagus (n = 50) followed by omphalopagus (n = 46). Limbs were reported in 75 cases. Congenital heart disease was seen in 17/120(14.2%) autosites. Omphalocele and meningomyelocele were the most common extracardiac anomalies in autosites. Weight along with the anatomy and position of heteropagus twins was a better determinant of the mode of delivery than weight alone. Mortality was reported in 12 cases. CONCLUSION: Autosites in HT generally carry a good prognosis, however, final outcome depends mainly on associated major cardiac anomalies. Meticulous antenatal assessment and preoperative planning are of paramount importance. LEVEL OF EVIDENCE: IV.

Neurosurgical Steps and Lessons Learned in Final Separation of 3-Year-Old Craniopagus Twins.

The only published paper1 describing the separation of the conjoined twins did not describe the novel steps and techniques of the microsurgery part, which lasted 26 hours. That paper did not include the neurosurgical video, either. The massive intracerebral hemorrhage that occurred on postoperative day 33 was not reported, and its potential causes have not been scrutinized. The strategy of final separation was developed during day-by-day microsurgical practice on cadavers and did lead us to novel surgical solutions as the introduction of the application of hinge and distractors. One of the twins advanced to GOS 5 status during first 5 postoperative months. The other twin slowly advanced to GOS 3 status and remained at that functional level. The latter suffered the massive brain hemorrhage on postoperative day 33, which led to delayed rehabilitation and interfered with a potentially better outcome. The thorough analysis of the potential causative factors revealed the possible pathophysiologic mechanism behind that complication.2 Two major factors have been identified, one of which is the traction-related traumatic brain injury that evolved during supine position. Another probable theory is the lack of sufficient reconstruction of the posterior part of the skull, which caused a recurring tactile microtrauma of the brain due to the supine position. The hemorrhage may have been facilitated by the fact that their coagulation tests were on the lower edge of normal parameters. It was a mistake that this part of the surgery was not performed by the neurosurgeons who designed it and practiced extensively on fresh cadavers using 3-dimensional models. Sufficient cranioplasty was performed 3 months later. It is impossible to say with certainty the cause of the hemorrhage, since there may have been other unknown causes (e.g., blood pressure spike). As a contributing factor, congestion of the veins is also possible.3 We concluded that closer monitoring, a stricter management of the interdisciplinary team work, and realizing the seriousness of the lack of sufficient structural support earlier could have prevented this unfortunate complication. In a similar case we advise future professionals to use a halo ring postoperatively for posterior protection until a sufficient cranioplasty can safely be done.

A Century With Craniopagus Twin Separation Surgeries: Nihilism to Optimism.

Craniopagus conjoined twins are extremely rare, reported 1 in 2.5 million live births. To date, 62 separation attempts in 69 well-documented cases of craniopagus twins have been made. Of these, 34 were performed in a single-stage approach, and 28 were attempted in a multistage approach. One or both twins died of massive intraoperative blood loss and cardiac arrest in 14 cases. We report our surgical experience with conjoined craniopagus twins (JB) with type III total vertical joining and shared circumferential/circular sinus with left-sided dominance. A brief review of the literature is also provided. In our twins, the meticulous preoperative study and planning by the multidisciplinary team consisting of 125-member, first-staged surgical separation consisted of creation of venous conduit to bypass part of shared circumferential sinus and partial hemispheric disconnection. Six weeks later, twin J manifested acute cardiac overload because of one-way fistula development from blocked venous bypass graft necessitating emergency final separation surgery. Unique perioperative issues were abnormal anatomy, hemodynamic sequelae from one-way fistula development after venous bypass graft thrombosis, cardiac arrest after massive venous air embolism requiring prolonged cardiopulmonary resuscitation, and return of spontaneous circulation at 15 minutes immediately after separation. This is the first Indian craniopagus separation surgery in a complex total vertical craniopagus twin reported by a single-center multidisciplinary team. Both twins could be sent home, but one remained severely handicapped. Adequate perioperative planning and multidisciplinary team approach are vital in craniopagus twin separation surgeries.

Conjoined Twin Separation: Review of 30-Year Case Experience and Lessons Learned.

BACKGROUND: Conjoined twinning is a rare medical phenomenon, and numerous challenges remain with respect to surgical separation and reconstruction. The purpose of this study is to present a detailed discussion of the authors' institutional experience with eight conjoined twin separations over the past three decades, focusing on challenges and lessons gleaned from these cases. METHODS: The records of all patients who underwent conjoined twin separation at The Hospital for Sick Children in Toronto, Ontario, Canada, from 1984 to 2018 were retrospectively reviewed. RESULTS: Eight sets of conjoined twins were analyzed. Half of the sets [ n = 4 (50 percent)] were female. There were four sets (50 percent) of ischiopagus twins, three sets (37.5 percent) of omphalopagus twins, and one set (12.5 percent) of craniopagus twins. The median age at separation was 6.75 months. The mean durations of intensive care unit and hospital stay were 14.1 ± 12.9 days and 4.9 ± 4.8 months, respectively. Mean length of follow-up was 6.7 ± 4.4 years. Three deaths occurred in our series, with an overall survival rate of 81 percent. Two sets of twins experienced expander-related complications such as infection and bowel perforation. Three twins required reoperation because of flap necrosis or dehiscence after separation. CONCLUSIONS: The authors' results highlight the unique nature of each operation and the great ingenuity required in managing the particular considerations of each case and also adhering to a systematic approach of evaluation and planning. A number of novel strategies were used at the authors' center and have now become commonplace. The lessons learned from such procedures may improve care for future generations of patients. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.