Thoraco-omphalopagus Twins Research Articles

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.

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

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. 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. 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%. 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.

First successfully separated set of thoraco-omphalopagus twins in Ilorin, Nigeria: A case report

First successfully separated set of thoraco-omphalopagus twins in Ilorin, Nigeria: A case report

Thoraco-omphalopagus twins with complex biliary tree, pancreatic and intestinal anatomy: Planning behind successful separation, “the Santorini difference”

Here we report the separation of thoraco-omphalopagus twins at week 17th after birth, that are already 11-year-old and performing satisfactorily at the family, school, and social level. At birth, hearts shared a common pericardium and livers were fused along the anterior edge. Small bowel was common from duodenum down to the terminal ileum and invested by a dual mesentery. From the bifurcation bowels were normal. Bile and pancreatic ducts discharged into the duodenum but whether they were fused, or not, and details of the terminal ducts did not show up in the study. Vasculatures were independent. Teams -anesthesia, reconstructive, cardio-surgery, digestive, management-were formed and given specific tasks. Separation, programed for week 24, was performed at week 17 due to an emergency. In the intervention, the central step was the exploration of bile and pancreatic discharges. The duct of Santorini was searched for and identified; it discharged at the minor papilla in the duodenum prior to fusion. Both Wirsungs and the already fused common bile duct discharged in a single major papilla in a fused duodenum. The distance between papillae allowed for a duodenal transection between them. This scenario, considered during planning, commanded the separation. T1 kept the major papilla, while T2, the minor papilla complemented with a cholecystojejunostomy. Careful planning and management were keys to overcome both emergency and incomplete preoperative information.

Successful Surgical Separation of Thoraco-Omphalopagus Symmetrical Conjoined Twins in Iran: Two Case Reports.

Conjoined twins are derived from the division of a single fertilized ovum; a phenomenon accompanied with multiple congenital anomalies. Such twins are identical, of the same sex, and more likely to be female. Most twins die during the embryonic period, and only 18% survive longer than 24 hours. There are complex anomalies in thoraco-omphalopagus twins that makes them unlikely to live long enough to undergo separation. Treatment of this uncommon condition presents both surgical and anesthetic challenges. The management of rare anomalies is difficult even for skilled surgeons. Therefore, it is logical to use the knowledge gained from previous experiences. We herein present the first successful surgical separation of two pairs of thoraco-omphalopagus conjoined twins at the Pediatric Surgery Center of Namazi Hospital (Shiraz, Iran). In both cases, the neonates had separate hearts and common pericardium. Contrast-enhanced computed tomography of two sets of twins showed fusion of sternum, pericardium, diaphragm, and left lobe of liver. Critical steps of the surgical separation were identified and contingency plans were made for possible partial liver donation and cross-circulation between twins. The separation procedure and reconstruction of the abdominal wall were successfully performed. Both pairs of twins, now 6- and 8-year-old, are healthy and have normal growth and are under follow-up.

Successful Separation of Conjoined Twins: Experience of a Tertiary Care Hospital

Background: The separation of conjoined twins presents a unique challenge to many pediatric surgical specialties. Meticulous preoperative investigations, planning and multidisciplinary team approach led by pediatric surgeon contribute to the success of surgical separation. The aim of this study is to share our experiences of one hospital to enhance skill for treating conjoined twins regarding anatomic variations, investigations, surgical aspects, outcomes and rehabilitation.Methodology: All cases of symmetrical conjoined twins who were treated from July 2009 to February 2018 at the Pediatric Surgery Department of Dhaka Medical College Hospital, Dhaka, Bangladesh were included in this prospective type of study. All types of parasitic twins were excluded. Prenatal ultrasound diagnosis, gender, birth weight, anatomy of the twins, the age and weight at the time of the separation surgery, the length of surgery, the duration of anesthesia during the separation surgery, a detailed description of the separation surgery, the type of skin closure, postoperative complications and death were studied.Results: There were three sets of thoraco-omphalo-ischiopagus twins, one set of thoraco-omphalopagus twins, one set of pygopagus twins. Three sets had no prenatal visit or ultrasound evaluation. Two sets of conjoined twins were failed to separate due to the complexity of the organs that were shared by both twins and instability leading to death. Only one set of pygopagus twins was successfully separated. Emergency colostomies were done on pygopagus twins due to the presence of anorectal anomalies. The surgical survival rate was 100%. One set of twins who did not undergo separation due to refusal of treatment was currently alive. One set of twins was under evaluation and currently alive.Conclusion: The overall success of conjoined twin separation depends on the experience and preparedness of treating team led by pediatric surgeon and resources available at the hospital. No single discipline can expect to have all the necessary talents.Bangladesh J Child Health 2018; VOL 42 (2) :73-78

LDT classification and therapeutic strategy of congenital body wall defects

Repairing body wall defects is a critical step in the treatment of some congenital deformities, and this procedure may need the help from plastic surgeons. Although there are many articles about congenital deformities, body wall defects of these malformations are rarely studied as independent targets. In this article, the authors present an LDT classification for congenital body wall defects according to the position of the defects, the tissue layers involved, and the surgical urgency, each of which is represented by letters L, D, and T, respectively. That is, the defects in different areas (L), full-thickness (D1), or partial (D0A, D0B) defects, defects needing instant repair (T2), semi-elective repair (T1), or elective repair (T0). Based on this classification system, the authors have performed body wall reconstruction on two pairs of thoraco-omphalopagus twins, one pair of ischiopagus tetrapus twins, and an infant and an adult, both of whom were diagnosed with pentalogy of Cantrell associated with ectopia cordis. Except for one pair of thoraco-omphalopagus twins who died after emergency separation, all the other patients survived. Another pair of thoraco-omphalopagus twins suffered from wound dehiscence and partial flap necrosis, respectively, after surgery. An expanded polytetrafluoroethylene mesh in one sister of the ischiopagus twins was removed because of infection. LDT classification not only can help doctors categorise different congenital body wall defects rapidly and easily, but can also guide the reconstruction of these defects. It may have clinical value to plastic surgeons to some extent.

Peripherally Inserted Central Catheter Insertion in Conjoined Thoraco-omphalopagus Twins

Peripherally Inserted Central Catheter Insertion in Conjoined Thoraco-omphalopagus Twins

Conjoined Twin Separation: Integration of Three-Dimensional Modeling for Optimization of Surgical Planning.

Conjoined twinning is a rare anomaly, with an incidence of approximately 1 in 100,000 live births. There is a high perinatal mortality rate, but twins who survive pose reconstructive challenges that require meticulous preoperative planning. The authors describe the senior surgeon's career experience with conjoined twin separation, and the evolution of medical modeling and 3-dimensional imaging as a critical component in presurgical planning.The authors performed a retrospective review of all consecutive patients of conjoined twin separation at a single institution from January 2004 to December 2013. Data were collected related to patient demographics, comorbidities, operative technique, perioperative complications, survival, long-term outcomes, and the type of medical modeling system used for preoperative planning.Five sets of conjoined twins underwent separation during the 10-year study period. There were 3 sets of thoraco-omphalopagus twins, 1 set of pyopagus twins, and 1 set of ischiopagus tetrapus twins. The mean age at separation was 70 days, with a mean of 3.5 surgical procedures performed per patient during the first year of life. One set of twins experienced postseparation complications that warranted immediate return to the operating room. The overall survival rate after separation was 70%. The imaging methods used were computed tomography scan with 3-dimensional reconstruction, plaster molds, medical modeling with composite printing, and virtual surgical planning.The use of imaging and medical modeling in presurgical planning has proven to be an important element in optimizing the outcomes for patients with this rare anomaly.

Anatomical basis of femoral component sizing of total knee arthroplasty

Conjoined twins are a rare congenital anomaly of twinning which borders on monstrosity. Of the various types of conjoined twins, thoraco-omphalopagus is a commonly occurring type. A recent theory postulates fusion of two embryonic discs as more of a cause for conjoined twinning than fission of a single embryonic plate. In this paper, the findings of a pair thoraco-omphalopagus twins are outlined with a review of the embryological theories that surround their formation.

Conjoined twins – Thoraco-omphalopagus: A case report

Conjoined twins are a rare congenital anomaly of twinning which borders on monstrosity. Of the various types of conjoined twins, thoraco-omphalopagus is a commonly occurring type. A recent theory postulates fusion of two embryonic discs as more of a cause for conjoined twinning than fission of a single embryonic plate. In this paper, the findings of a pair thoraco-omphalopagus twins are outlined with a review of the embryological theories that surround their formation.

An Interesting Case of Undiagnosed Conjoined Twins

ABSTRACT A 20 years old primigravida who presented to us at 36 weeks 3 days gestation with labor pains. She was perceiving fetal movements well, booked and immunized at a private hospital in Hosur. On examination, she was 7 cm dilated, well effaced, breech at 0 station. She was taken up for emergency lower segment cesarean section (LSCS) in view of primi with breech and mother not willing for vaginal delivery. An alive term 3.5 kg female paraphagus dicephalus twins was extracted. Extraction was difficult. Apgar was 3 at 1 minute and 5 at 5 minutes. The babies died after 45 minute. External examination showed that the twins were conjoined from the level of thorax downward. The twins had two heads, two upper limbs and a shared thorax, abdomen, pelvis and a single female external genitalia and single pair of lower limbs with CTEV (dicephalus, dibrachius, dispinous, thoraco-omphalopagus twins). How to cite this article Kirtan K, Doopadapalli D, Shailaja N, Krishna L, Bhat BS. An Interesting Case of Undiagnosed Conjoined Twins. Donald School J Ultrasound Obstet Gynecol 2015;9(3):327-330.

Conjoined twins in Edo state of Nigeria; a report of the first surviving set

The term conjoined twins refers to babies who are physically joined at some point. It is a rare condition with an estimated incidence of 1 per 200,000 live births. We report our experience with conjoined twins over a twelve year period in tertiary hospital in Nigeria and a case of the first set of conjoined twin survivors in Benin City, Nigeria. Over the last twelve years (1999-2011), three cases of conjoined twin have been recorded in our teaching hospital. A set of thoracoomphalopagus twins (females) were delivered in 1999 and they survived for only 36hrs. Another set of female omphalopagus twins were delivered in 2009 and survived a separation surgery. A third set of female thoracoomphalopagus was delivered in another institution same year and referred to our unit but they only survived for 48 hours. The first surviving twins were omphalopagus, sharing a single liver, and common bile duct emptying into a common duodenum. The stomach, as well as the jejunum was normal and unshared. Surgical separation of the liver was done and biliary reconstruction procedure performed for twin II. A three -year follow up showed good outcome.

Thoraco-omphalopagus conjoined twins: impact of ultrasound assessment on successful surgical separation

In this case report, we present a case of thoraco-omphalopagus conjoined twins who were successfully separated and had long-term survival. We performed ultrasound assessment and found that the twins shared the liver and that the fusion site of their liver had gross communication of blood vessel. Ultrasound cardiogram examination showed that the anomalous vessels that connected their hearts drained from the number 1 infant to the number 2 infant. The ultrasound assessment has significant impact on the successful surgical separation of the thoraco-omphalopagus twins.

Separation of thoraco-omphalopagus twins in a rural secondary hospital: Perioperative management

Providing anaesthesia for the separation surgery of conjoined twins presents unique challenges to the managing anaesthesiologists. The low incidence of such surgeries and anatomical variations in each type of conjoined twins makes each separation surgery a unique experience. This report features the anaesthetic plan and challenges faced in performing the separation surgery of a set of thoraco-omphalopagus twins in a rural secondary hospital in a remote location in India.

A Rare Multiple-Gestation Anomaly

Conjoined twins are a rare anomaly. The classification of twinning that accounts for the majority of conjoined twins is thoraco-omphalopagus. This report discusses a case of female thoraco-omphalopagus twins who share a single cardiac system.

Successful Tracheal Intubation Using the Airtraq in Thoraco-omphalopagus Twins

To the Editor Tracheal intubation in conjoined twins, especially thoraco-omphalopagus twins, represents a unique challenge because of the varying extent of anatomical development and awkward positioning.1 We successfully intubated the tracheas of thoraco-omphalopagus twins using the Airtraq® (Prodol Meditec S.A., Vizcaya, Spain) (Fig. 1).Figure 1: Thoraco-omphalopagus twins after intubation (identified as A, on the right, and B, on the left).Female thoraco-omphalopagus conjoined twins, 40 days old, weighing 6.5 kg, were scheduled for surgical separation. The use of Airtraq optical laryngoscope in children was approved by the Human Ethics Committee of West China Hospital, and the parents provided informed consent. Preoperative studies showed a large cross-abdominal liver and separate hearts in a single pericardial sac combined with a left-sided superior vena cava of twin A draining into the coronary sinus of twin B. Both twins presented with micrognathism, high-vaulted arch, and short neck, and therefore difficult airway management was anticipated. After they were placed on the operating table with their necks slightly hyperextended, routine monitoring was established. Anesthesia was induced with sevoflurane, midozalam, and low-dose fentanyl with the intention to intubate the tracheas under deep sedation while maintaining spontaneous respiration. After the disappearance of eyelash reflex, tracheal intubation in twin B using a Macintosh laryngoscope blade size 1 was attempted by an experienced pediatric anesthesiologist with the twins in the lateral position to avoid significant hemodynamic compromise. Only the tip of the epiglottis was viewed and Cormack and Lehane grade III difficulty of intubation confirmed. Withdrawing the laryngoscope resulted in laryngospasm similar to that described by Shank1 and was resolved by administration of propofol. According to our preoperative protocol, we planned that the trachea of the second twin should be intubated. Therefore, while twin B underwent mask ventilation, the trachea of twin A was intubated without difficulty using an Airtraq optical laryngoscope (AOL; Prodol, Vizcaya, Spain), directly after which twin B was given succinylcholine 3 mg IV and tracheal intubation performed for twin B in a similar fashion. Although thoraco-omphalopagus twins usually present with an inflexible position, tracheal intubation can be achieved with the twins in the lateral and partly rotated position in most cases, rather than holding one baby above the other.2,3 We prefer deep sedation, maintaining spontaneous breathing in the management of anticipated difficult intubation. Transient laryngospasm occurring in our case was probably due to inadequate depth of anesthesia. Had our attempt using video-laryngscopy not been successful, we were prepared to use fiberoptic and rigid Seldinger-assisted video-telescopic intubation, as successfully applied in other similar cases.4–6 Jing Lin, MD Bin Liu, MD Ling Tan, MD Yun-Xia Zuo, MD, PhD Department of Anesthesiology West China Hospital Sichuan University Chengdu, Sichuan People's Republic of China [email protected]

Anaesthesia for the separation of conjoined twins

Thoraco-omphalopagus is one of the most common type of conjoint twins accounting for 74% cases of conjoint twins. We report the anaesthetic management for successful separation of thoraco-omphalopagus conjoint twins, both of them surviving till date. We highlight the responsibility of anaesthesia team in anaesthetising the two individual patients simultaneously, need of careful monitoring and anticipation of complications like massive blood loss, hypotension, hypokalemia, hypoxia and hypercabia. Detailed description of successful management is reported.

Thoraco-omphalopagus twins: different perinatal circumstances, different outcome

Two pairs of omphalopagus twins were encountered at the Polish Mother's Health Institution in Lodz, Poland during the past 15 years. In the first set the prenatal diagnosis was not established and the delivery of the twins in a regional hospital was a complete surprise. Both babies died. In the second case the conjoined twins were diagnosed prenatally, surgical separation was successful, and both twins survived. The prenatal identification of conjoined twins is of cardinal importance for the planning of delivery and possible separation.

Route of delivery of thoracoomphalopagus twins: analysis of three cases

The management of thoracoomphalopagus twins depends on gestational age and life expectancy of the fetuses. Vaginal route of delivery is limited to small nonviable fetuses. Cesarean section should be reserved for larger fetuses or when the life expectancy of the fetuses is of concern. The first case was that of heavily malformed fetuses of 28 weeks in preterm labor, delivered vaginally according to their parent's request, but the fusion between fetuses was torn cephalad. The second case was diagnosed and underwent cesarean section at her 29th gestational week, but her babies were lost after birth due to cardiorespiratory arrest. The third case was 34 weeks pregnant and not diagnosed until birth. Presentation of the first fetus was vertex, while the other was breech. Totally 3,700 g twins were delivered by vaginal route without any serious complication. Successful vaginal delivery of the third trimester fetuses is possible. The technique of the delivery is important.