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PubMed2002

Perineal reconstruction of female conjoined twins.

BACKGROUND/PURPOSE: Conjoined twins are some of the most challenging patients faced by surgeons. Pygopagus and ischiopagus twins present particular gastrointestinal and genitourinary reconstructive challenges. This study reviews the authors' experience with the perineal reconstruction of these types of conjoined twins. METHODS: Retrospective analysis was performed for 3 sets of female conjoined twins undergoing separation between 1999 and 2001. Particular attention was given to the separation and reconstruction of the distal gastrointestinal and urogenital structures. RESULTS: Three sets of female conjoined twins underwent successful separation 2 pygopagus, one ischiopagus tripus) with 5 surviving infants. The sixth infant died of congenital anomalies incompatible with life. Four of the 5 surviving infants had diverting enterostomies. Two of these enterostomies have been closed. Perineal reconstruction consisted of anoplasty (5 of 5), vaginoplasty (4 of 5), and urethroplasty (4 of 5). Although fecal and urinary continence are not completely measurable at this age (<3 years), all 5 survivors void spontaneously. Three infants with intestinal continuity have apparently normal defecation without the need of a bowel regimen. CONCLUSIONS: With careful preoperative planning and a multidisciplinary team of pediatric surgeons and urologists, satisfactory reconstruction and functional outcome of the female perineum can be achieved in conjoined twins.

Feasibility Studies↗
PubMed1999

Differential energy metabolism in conjoined twins.

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.

Energy Metabolism↗
PubMed1987

Conjoined twins, right atrial isomerism, and sequential segmental analysis.

Three cases of twins conjoined across the chest were examined. In one set, one twin showed right atrial isomerism and the other showed the usual atrial arrangement. The other two sets showed the usual atrial arrangement in both twins. The associated cardiac anomalies were diverse, ranging from atrioventricular septal defects to absent atrioventricular connections. Sequential segmental analysis was used successfully in each case to classify the complex cardiac anomalies. The first step was the recognition of the atrial arrangement. This was easily achieved despite the complexity of the intracardiac morphology.

Female↗
PubMed2002

Experience in the management of conjoined twins.

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.

Child, Preschool↗
PubMed1983

Early cardiac evaluation of thoracically conjoined twins.

Thoracopagus conjoined twins were delivered at 31 weeks gestation by cesarean section. Although there was a single heart, the possibility of salvaging one twin only was contemplated. Echocardiography revealed an A-V canal and suspected transposition of the great vessels. To determine the possibility of surgery, cardiac catheterization was performed to rule out other congenital cardiac malformations incompatible with life, and also to delineate the exact anatomy of the great vessels of the heart. Unfortunately, complex congenital abnormalities were found, thus excluding the possibility of surgery. Newly delivered conjoined twins should be regarded as potentially correctable and should be promptly investigated while they are yet in good clinical condition.

Female↗
PubMed1998

Expansion and division of conjoined twins.

Omphalopagus conjoined twins were born by cesarean section after their 24-year-old gravida 2, para 1 mother went into labor at 35 weeks' gestation. Initial studies included roentenograms, an upper gastrointestinal study, small bowel follow-through, and computerized tomography with intravenous contrast from neck through pelvis. Results of these studies showed that the twins had very little shared vasculature, but did share a pericardium and liver parenchyma. The infants were allowed to gain weight and grow. After 3 weeks, tissue expanders were placed subcutaneously and were rapidly expanded in 2 weeks. The infants were then separated. An Argon Beam Coagulator was used to aid the separation of the liver. The abdominal fascia was closed primarily on each infant, and the skin easily closed without tension. During the first 36 hours of the postoperative course, both infants required significant ventilatory support including inhaled nitric oxide. After this period, the ventilatory support decreased dramatically and rapidly. The infants were discharged 9 days after their surgical separation. Conjoined omphalopagus twins, the use of tissue expanders and the Argon Beam Coagulator, and the postoperative course are discussed.

Contrast Media↗
PubMed2005

Acute abdominal pain in a separated conjoined twin.

Complications of conjoined twins are typically events surrounding the birth and surgical separation. We report an unusual late-onset complication, a grade IV duodenal disruption of the accessory system in a formerly conjoined twin. Because conjoined twins have unique anatomy based on the junction and division of organs, one must always consider a multitude of causes for common complaints. Basic operative principles apply: the acute abdomen must be explored. Finally, to avoid these complications, removal of all or nearly all parasitic structures must be considered at the initial separation.

Abdomen, Acute↗
PubMed1995

Extrauterine twin-twin transfusion syndrome after birth in conjoined twins.

Cross-circulation of conjoined twins in utero has been demonstrated and the extent of its severity has varied. Significant 'extrauterine' twin-twin transfusion syndrome after birth, however, caused by an unbalanced shunting within the circulation has not been reported. In a case of omphalopagus conjoined twins complicated by hypovolemia in one twin and hypervolemia in the other, we demonstrated by color Doppler imaging an aberrant vessel from the hepatic artery of one twin to the hepatic vein of the other. Unbalanced shunting through this aberrant vessel was suspected and urgent separation of the conjoined twins was undertaken. Circulatory changes after birth leading to 'extrauterine twin-twin transfusion syndrome' are discussed. A possible explanation of the pathophysiology of intrauterine twin-twin transfusion syndrome is also given.

Adult↗
PubMed1985

A new technique for evaluating cutaneous vascularity in complicated conjoined twins.

Ischiopagus tripus conjoined twins were recently encountered. All organ systems were thoroughly evaluated using radiographic, angiographic, radionuclide, and CT imaging techniques. None of these studies were capable of accurately assessing the vascular territories of the skin at the level of the pelvis, the most critical area in terms of separation. Qualitative visual assessment of tissue fluorescein delivery under ultraviolet illumination is subject to subjective errors. Perfusion fluorometry is a recently developed technique whereby tissue fluorescence can be quantitated over time in very small amounts, even with repeated injections. In these conjoined twins, using sequential fluorescein injections in each twin, it was possible to accurately determine which portions of the pelvis and the shared leg belonged to which twin and exactly where the skin incisions should be made. Additionally, during operation it was possible to accurately predict the viability of skin flaps used for closure. Both twins survived. This is the first time it has been possible to accurately assess vascular territories of the skin in a complicated form of conjoined twinning. The technique is also useful in the evaluation of flow patterns in various other parts of the body.

Aortography↗
PubMed2006

Surgical management to conjoined twins in Shanghai area.

Conjoined twins are very rare congenital malformation. The aim of this study was to summarize our experiences of surgical separation on seven sets of conjoined twins, and improve the treatment of conjoined twins in the future. A retrospective review of surgical separation included data of prenatal diagnosis, associated malformation, timing of separation, intra- and postoperative management, and follow-up of six sets of conjoined twins at Shanghai Xin-Hua Hospital from 1980 to 2005 and one set at Shanghai Children's Hospital in 2002. Surgical separation was performed on seven sets of conjoined twins; six sets of thoracopagus-omphalapagus (including four sets of xipho-omphalopagus) and one set of ischiopagus. All sets presented varying degrees of severity of congenital cardiac malformations. Four sets were diagnosed prenatally by ultrasonography. Two sets of conjoined twins (case 2 and case 3) required emergent separation within 7 days after birth; both members of case 2 died within 2 days post operation, one member of case 3 died during operation while the other member survived. Five sets had scheduled separation undertaken more than 30 days after birth. One member of a set (case 6B) died 13 days after operation due to severe congenital cardiovascular anomalies. All other members of conjoined twins survived. Case 6A had a severe defect of the anterior thoracic cage and prosthesis of titanium alloy scaffold filled with silicone rubber was used to repair the defects successfully. Following up from 1980 to 2005, one member of a set (case 1A) died 4 years after operation due to pneumonia. Contact was lost to the surviving member of case 3 (ischiopagus). Other survivors of the separations had normal development. (1) Timing of operation and separation plan should be given according to the circumstances and the nature of the organ shared in each individual set of twins. (2) Prosthesis of titanium alloy scaffolds filled with silicone rubber may become one of viable methods for repairing severe defects of the thoracic cage.

Abnormalities, Multiple↗
PubMed2003

The lesser of two evils: a contextual view of the English case of the conjoined twins.

In 2000 conjoined twin girls were born in Manchester, England. They were joined in such a way that it was impossible to separate them to enable both twins to survive. Their bodies were fused at the lower abdomen and they shared an aorta and a bladder. Their arms and legs were at right angles to their conjoined trunk. The situation of the conjoined twins is of supreme importance as a private tragedy for their family. The resulting litigation also presents an important landmark in English law. In deciding the fate of the two children, the Court of Appeal provided an authoritative review, analysis and application of family law and medical law with regard to neonates. The most significant legal legacy of the case, however, may well be in the field of criminal law by way of the court's interpretation and application of the defences of necessity and self-defence.

Aorta, Abdominal↗
PubMed1988

Use of skin expansion in separation of conjoined twins.

Successful separation of conjoined twins attached at the thorax and upper abdomen was greatly facilitated by preliminary skin expansion. Recognized problems encountered in traditional methods of wound closure at the separation (including wound breakdown, infection, ventilatory compromise, and obstruction to venous return resulting from tight abdominal skin closure) were avoided through the use of this technique. Technical aspects of expander placement and inflation in conjoined infants are discussed. Skin expansion may represent a major advance in wound management of conjoined twins after surgical separation. This is the second report on the use of skin expansion in conjoined twins and the first involving thoracopagus (twins conjoined at the chest and abdomen).

Dermatologic Surgical Procedures↗
PubMed1995

Experience with urogenital reconstruction of ischiopagus conjoined twins.

PURPOSE: Ischiopagus conjoined twins are joined at the lower chest or abdomen down to the pelvis. We review our experience with such patients at Children's Hospital of Philadelphia. MATERIALS AND METHODS: Six sets of ischiopagus twins were separated and their treatment is discussed. In addition, reports on 36 sets of ischiopagus twins are reviewed. RESULTS: If a shared bladder is present, 1 twin retains it while the other receives a temporary urinary drainage system. Later, the twin without a bladder undergoes reconstruction to create a continent catheterizable pouch. When 4 kidneys are present the opposite ureter is reimplanted or crossed with transureteroureterostomy. Twins with 2 sets of genitalia can usually undergo separation and reconstruction appropriately. Occasionally single external genitalia are present and secondary reconstructive genitoplasty is required. CONCLUSIONS: Multiple staged genital reconstructions are required but with proper planning satisfactory outcomes will result.

Decision Trees↗
PubMed1991

Emergency separation of conjoined twins.

Female omphalopagus conjoined twins were successfully delivered vaginally and required emergency surgical separation shortly after birth for gastroschisis. Shared tissue included conjoined bowel; one twin also had a complex cloacal abnormality and patent urachus. A 2-year follow-up is presented. A review of the relevant literature confirms that this is the first example of gastroschisis conjoining omphalopagus twins.

Abdominal Muscles↗
PubMed1998

Parapagus conjoined twin Holstein calf.

BACKGROUND: Conjoined twins with a doubling of all cranial structures, having two vertebral columns along their entire body length yet with a single pelvis, have not been described in the literature. The cause of conjoined twinning is incompletely understood; however, two main theories (fission and fusion) have been proposed and disputed. METHODS: A dicephalus, tetrabrachius (parapagus) conjoined twin Holstein heifer calf was studied. RESULTS: Two normal heads were present on two necks. The twins were fused in the thoracic region. There were four forelimbs and an abnormal orientation of ribs. Two separate vertebral columns along the length of the animal ended with two tails. There was a single pelvis, and only two hindlimbs were present. The musculature of the medial forelimbs was complete but abnormally positioned. Some medial structures, caudal to the thorax, failed to develop in these twins. There were two hearts, each one supplying one half of the body. The cranial vasculature was doubled and normal. Caudally there were two aortas, each supplying the respective half of the twin. The right caudal vena cava drained all caudal parts of the body while the left caudal vena cava drained only the liver. There were two sets of lungs. Each twin had a separate esophagus that entered a separate stomach. The right abomasum (fourth stomach chamber) was herniated through the diaphragm into the thoracic cavity. The two duodenums from each stomach fused distal to the pyloric sphincters. Caudal to this point of fusion, all structures of the digestive and urogenital systems were single. The calf had a single anus and vulva. CONCLUSIONS: The anatomical findings in this twin suggest a fission event followed by fusion of parallel embryonic axes.

Abnormalities, Multiple↗
PubMed1986

Obstetric management of conjoined twins.

Three cases of conjoined twins are presented: two thorocopagus and one craniopagus. The diagnosis of conjoining varied from late second trimester to time of term delivery. Delivery mode was both by vaginal and cesarean routes, and occurred at both level one and level three hospitals. Antenatal diagnostic procedures and issues in obstetric management are discussed. A suggested obstetric management sequence for conjoined twins is proposed.

Adult↗