Cojoined twins thoracoonfalophagus separation and liver bipartition (case report)

Abstract

Background: Cojoined twins are a rare phenomenon of monochorionic and monoamniotic twins. The incidence varies from 1 in 50,000 to 1 in 200,000 live births. Female fetuses are more commonly affected, with a ratio of men to women being 1:3, particularly in the thoracic type. The thoraco onfalophagus twins are joined and can share body part as the respiratory, cardiovascular and gastrointestinal systems, besides of thoracoabdominal wall. in the present case shared thoracoabdominal wall and livers and one of the twins has congenital complex cardiac malformation. These types of twins have better chances of survival if they are separated successfully. Methods: We present a rare case of male thoraco onfalóphagus twins successfully separated at 28 days old. We reviewed the postnatal study, the surgical technical aspects of the separation and the postoperative evolution of both twins. These twins are the product of primigesta of 22 years (G1P1001), without significant antecedents, with prenatal diagnosis of double gestation type Siameses thoraco onfalóphagus, the delivery was by caesarean section at 34 weeks of gestation for acute fetal distress. and we confirm that the Siameses are joined by the thoracoabdominal wall; Ultrasound studies and AngioCtScan showed that shared the visceras: heart and liver, the separation surgery was successfully performed with a multidisciplinary team of anesthesiologists, hepatobiliary surgeons, pediatric cardiovascular surgeons, plastic surgeons, pediatric surgeons, Results: The intraoperative findings, hearts and independent great vessels were seen, the pericardium, diaphragm and costal arches were shared, absence of sternum, the livers were joined by a bridge with an area of approximately 7 cm2 of parenchyma and maintained independence from the portal systems, A left portal branch coming from siamese 2 was common, veins cavas and suprahepatic veins as well as the biliary system were independent, there was a complex cardiac malformation in the Siames 1 (double exit route from the right ventricle, subvalvular and pulmonary valve stenosis, double-related ventricular septal defect) and malformation cardiac failure in the Siamese 2 (interatrial communication without hemodynamic repercussion), intestinal malrotation in the Siamese 1. Successful separation of the Cojoined twins was achieved through hepatic transection with ultrasonic dissector (CUSA), in addition to the use of monopolar and bipolar electrocautery, the shared portal vein was sutured; The separation of the thoracic and abdominal wall components described was carried out. In siamese 1 the total closure of the chest wall was not achieved and it was covered with a dermal patch that guarantees the posterior reconstruction, the abdominal wall was closed satisfactorily with lateral incisions of discharge. In siamese 2, satisfactory closure of the chest wall and abdominal wall with lateral discharge incisions was achieved. Conclusion: The management of cojoined twins together remains a medical challenge, due to multiple congenital anomalies associated, as in this case they lead to low success rate. The specialized and multidisciplinary integral management of these cojoined twins is necessary to guarantee success in terms of reducing complications and increasing survival. In this particular case, we performed the separation of thoracoonfalophagus and liver Bipartition succesfully and the current evolution is satisfactory, with emphasis on the subsequent correction of chest wall defects and associated cardiac congenital malformations.