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Conjoined twins represent a great challenge for most pediatric specialists including pediatric surgeons, anesthetists, neonatologists, urologists, neurosurgeons, and orthopedic surgeons. This anomaly can be classified according to the type of twins׳ fusion. Various organs can be fused making the separation difficult. Conjoined twins are usually diagnosed antenatally by ultrasound. Detailed fetal echocardiography is necessary to counsel the parents during pregnancy. Postnatally, the majority of the conjoined twins can be thoroughly investigated using various imaging techniques. This allows careful planning of the operation. However, in approximately one-third of the patients an urgent operation is required at birth without a complete assessment of the joining. This is associated with a poorer outcome., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The various stages of the separation are carefully planned but despite this, variations which will change the schedule of the procedure may exist. In general the operation commences on the opposite side from the main procedure and then the twins are turned for the remainder of the operation. Each type of conjoined twin is different but basically thoracopagus involves the hearts, omphalopagus involves the liver and small intestine and ischiopagus involves the large intestine and genito-urinary system. Our results are presented together with interesting cases from which lessons have been learned., (Copyright © 2015. Published by Elsevier Inc.)

The theatre staff would have been made aware of the special requirements for the operation at the planning meetings. Two sets of scrubs nurses will be required but only one set will be needed for the actual separation. The second set of scrub nurses will be needed only when both twins have survived the separation and the second twin is moved to another operating room for closure of the wound., (Copyright © 2015 Elsevier Inc. All rights reserved.)

In dealing with conjoined twins, we have adopted a pragmatic approach in balancing what is possible, parental wishes and the likely outcome for the children. We believe that separation where feasible should be undertaken but appreciate that a few parents may wish their children to remain joined. The advice that we give balances the very real risks of surgery against the type of life the twins will experience if they remain joined. In some types of union such as end to end ischiopagus, the twins will never stand and will be bedridden. With other types of union, independent walking is not possible. Such limitations on living seem intolerable to us, but we have left the final decision to parents., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The management of conjoined twins falls into three distinct groups-non-operative, emergency separation and elective separation. Planning meetings involving all the personnel who will be required during the operation are held. The radiological findings are presented and the anaesthetic, nursing and intensive care requirements are highlighted., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The incidence of conjoined twins is estimated to be around 1 in 250,000 live births. There is a distinct female predominance. In this paper the imaging of conjoined twins both antenatally and postnatally is reviewed, in particular taking into consideration recent advances with multidetector CT. Accurate counselling of parents regarding the likely outcome of the pregnancy and the likelihood of successful separation is dependent on good prenatal imaging with ultrasound and MRI. Planning of postnatal surgical separation is aided by accurate preoperative imaging which, depending on the conjoined area, will encompass many imaging modalities, but often relies heavily on CT scanning.

Objective: To determine the accuracy of prenatal and postnatal echocardiography in delineating the degree of cardiac fusion, intracardiac anatomy (ICA), and ventricular function of 23 sets of conjoined twins with thoracic level fusion presenting to a single centre over a 20 year period., Methods: 13 thoracopagus, 5 thoraco-omphalopagus, and 5 parapagus pairs presenting to the authors' institution between 1985 and 2004 inclusive were assessed. Echocardiographic data were analysed together with operative intervention and outcome. Twins were classified according to the degree of cardiac fusion: separate hearts and pericardium (group A, n = 5), separate hearts and common pericardium (group B, n = 7), fused atria and separate ventricles (group C, n = 2), and fused atria and ventricles (group D, n = 9)., Results: The degree of cardiac fusion was correctly diagnosed in all but one set. ICA was correctly diagnosed in all cases, although the antenatal diagnosis was revised postnatally in three cases. Abnormal ICA was found in one twin only in two group A pairs, one group B pair, and both group C pairs. All group D twins had abnormal anatomy. Ventricular function was good in all twins scanned prenatally, and postnatally function correlated well with clinical condition. Thirteen sets of twins in groups A-C were surgically separated; 16 of 26 survived. None from groups C or D survived., Conclusions: Prenatal and postnatal echocardiography accurately delineates cardiac fusion, ICA, and ventricular function in the majority of twins with thoracic level fusion. It is integral in assessing feasibility of separation. The outcome in twins with fused hearts remains dismal.

Conjoined twins have been a source of fascination for both the general public and the medical profession since time immemorial. Their birth was initially viewed as an ominous sign of impending disaster. This was followed by a prolonged period through the Middle Ages and into the nineteenth century when they were regarded as freaks or monstrosities and were exhibited with substantial financial reward at circuses and sideshows. Recently, conjoined twins have attracted intense media interest, which has coincided with increasing success in their separation. This article gives an overview of the role of prenatal ultrasound in assessing the prognosis and discusses the post-natal surgical management and outcome., (2005 John Wiley & Sons, Ltd.)

Introduction: Approximately 18% of conjoined twins (1 in 200,000 live births) are joined at the sacrum ("pyopagus"). As the joined structures are not life-threatening, there is generally a good prognosis, with time for investigation and planning of operative separation., Case Report: This paper reports on the management of pyopagus twin girls, the first in the UK delivered at 36 weeks by Caesarean section. The diagnosis had been made by ultrasound at 12 weeks' and confirmed by MRI scan at 26 weeks' gestation. Each twin had wasting and weakness below one knee, but no deficit in the other leg. The perineum had two urethras but only one anus. One infant had a colostomy in the immediate neonatal period. With appropriate further imaging, surgery was planned in two stages. First, a balloon expander was inserted in the bridging area. At 3 months of age a team comprising Plastic, Paediatric and Neurosurgeons undertook the definitive separation., Discussion: Details of the multidisciplinary planning, operative separation and follow-up of these two children will be discussed.

Background: Conjoined twins occur in approximately one in 200 000 live births. Their management is often extremely complex and experience with large numbers is restricted to a few centres worldwide., Methods: An analysis of 17 sets of symmetrical conjoined twins managed by a single team of surgeons over a 16-year period was undertaken., Results: Management could be divided into three groups. Those in group 1 (n = 5) had non-operative management, owing to cardiac fusion or gross anatomical union, and all died. Those in group 2 (n = 7) underwent emergency separation, when one twin was dead or threatening the survival of the sibling or where there was a correctable life-threatening associated anomaly (four of 14 survived). Twins in group 3 (n = 5) underwent elective separation, allowing time for detailed preoperative investigation and planning (eight of ten survived)., Conclusion: Accurate preoperative investigation, a team approach, previous experience, and meticulous operative and postoperative management contributed to the success rate achieved in this series.

Conjoined twins are rare and present a unique challenge to pediatric surgeons and radiologists. Planning of surgical separation is aided by accurate preoperative imaging. Such twins are classified according to the most prominent site of connection: the thorax (thoracopagus), abdomen (omphalopagus), sacrum (pygopagus), pelvis (ischiopagus), skull (craniopagus), face (cephalopagus), or back (rachipagus). The area of fusion largely determines the imaging modalities used. Thoracic conjunction is most common and requires cardiac assessment. Magnetic resonance imaging and computed tomography provide excellent anatomic and bone detail, demonstrating organ position, shared viscera, and limited vascular anatomy. Contrast material radiography allows evaluation of the gastrointestinal and urogenital tracts, and a shared liver requires assessment of anatomy, vascularization, and biliary drainage. Angiography helps define specific vascular supply, which is useful in determining the distribution of shared structures between the twins at surgery. Each set of conjoined twins is unique. An imaging strategy to accurately define anatomic fusion, vascular anomalies, and other associated abnormalities is important for surgical planning and prognostic information.

Background/purpose: Conjoined twins often have different body composition and growth rate before separation. This may be because of differences in energy metabolism. The aim of this study was to investigate the energy expenditure, body composition, and calorie intake of thoracopagus conjoined twins with shared hepatic circulation but separate gastrointestinal tracts., Methods: The twins were studied at two periods: (1) before separation (age, 73 days) and (2) after separation (age, 97 days). Calorie intake over the study periods was carefully documented. Respiratory gas exchange was measured by computerized indirect calorimetry. The postseparation weight ratio of twin A to twin B was used to approximate the preseparation weights. Body composition (total body fat) was calculated from skinfold thickness and anthropometric measurements., Results: The body composition of the twins was different: body weight and total body fat were higher in twin B. Resting energy expenditure and calorie intake were markedly different between the conjoined twins before separation. In both twins, the energy expenditure increased after separation. After separation, the resting energy expenditure of the twins was similar., Conclusions: This study illustrates the difference in energy metabolism in a set of thoracopagus conjoined twins. The authors speculate that twin A was supplying nutrients to twin B resulting in increased energy expenditure before separation. This would explain the lower calorie intake and higher fat mass of twin B.

Objectives: To review the urological abnormalities in conjoined twins, a rare congenital abnormality arising from an incomplete separation of a pair of monozygotic twins, and who can be joined from the head to the pelvis with varying degrees of union., Patients and Methods: The records and urological anomalies of conjoined twins presenting from 1985 to 1995 were reviewed retrospectively and the management and surgical reconstruction described., Results: Seven of 11 sets of conjoined twins underwent surgical exploration. Complex urological problems occurred in all twins with omphalo-ischiopagus. The urological anomalies were: Set 1; both twins had one lateral crossed fused renal ectopia with uncrossed ureters draining into a single bladder with one urethra. They had one set of genitalia. Each twin received one crossed fused ectopic kidney, half the bladder and genitalia, with one retaining the urethra and one a vesicostomy. Set 2; these twins had four kidneys (two lateral and two central that were fused), four ureters (two of which crossed) and these drained into two sagittal bladders that emptied by one urethra associated with a single set of external genitalia containing three corpora cavernosa. At separation, the crossed ureters were re-routed, each received a bladder and the genitalia were split. Set 3; there were four kidneys with two crossed ureters draining into two collateral bladders, there was a partial duplication of the urethra and a single hypospadiac penis. After separation, each had kidneys draining into a bladder, one received the urethra and external genitalia, and one a urostomy., Conclusion: Complex urological problems occur in conjoined twins, although restricted to those with pelvic fusion (ischiopagus). Assessment and treatment needs to be individualised and overseen by a surgeon with experience in complex urology.

This report describes separation of thoraco-omphalopagus conjoined twins and management of the hepatobiliary complications that ensued. The livers were fused and, although both infants had gallbladders, the extrahepatic biliary trees were fused. After separation, obstructive jaundice developed in one twin and she required a biliary diversion. The other twin had an external biliary fistula that only closed after resection of a segment of liver with no connection to the ipsilateral biliary tree. Closure of the abdominal wall defects with biodegradable patches proved very satisfactory and eliminated the problems with persistent wound sinuses related to nonabsorbable mesh.

Separation of 3-year-old brachio-thoraco-omphalo-ishiopagus bipus conjoined twin girls is reported. Detailed preoperative assessment and a multidisciplinary approach were essential prerequistes to surgery. The difficulties of managing the extensive body wall defect resulting from separation are discussed. Despite the use of subcutaneous and intraperitoneal tissue expanders, a large area of prosthetic material was required to reconstruct the abdominal wall. Skin cover was achieved using a combination of meshed allogeneic skin, homograft split skin, and autologous cultured keratinocytes. One twin died in the early postoperative period, but the other has recovered well and is awaiting further rehabilitative treatment.

A case of xiphoomphaloischiopagus tripus conjoined twins is reported. Particular emphasis was paid to the large abdominal wall defect that would be produced by separation, and the complications resulting from the use of intraperitoneal Silastic tissue expanders are described. As a result of adequate tissue expansion and the use of a vascularized pedicle graft from the fused limb, primary abdominal wall and pelvic closure was possible without complications of wound healing. The consistent finding of a shared lower urinary tract with one ureter from each infant entering each bladder should be anticipated in this type of twinning.