Thoraco-omphalopagus Twins Research Articles

Two babies, one heart, and no airway

The medical literature on conjoined twins is replete with descriptions of the complexity of surgical separations. However, airway management, although quite challenging, is neglected in literature. We present experience with conjoined twins united anteriorly at the thorax and abdomen sharing one six-chambered heart. Laryngoscopy and intubation recommendations, bronchoscopy techniques, and tracheotomy strategies are outlined for the face-to-face twins. Difficult, unique case report at tertiary care medical center. Conjoined thoraco-omphalopagus twins were successfully intubated at birth using the rigid Seldinger-assisted videotelescopic intubation (SAVI) technique. Flexible airway endoscopy allows for timely management of ventilator-dependent conjoined twins. Tracheotomy was later performed with the twins in the upright position with heads slightly rotated. Meticulous attention to detail, monitoring and vigilance are mandatory. Successful management of conjoined twins relies on close communication and cooperation of all members of the multidisciplinary team. Conjoined thoraco-omphalopagus twins united anteriorly require a challenging airway management strategy. A three-dimensional perspective is utilized for intubation and tracheotomy. The SAVI technique is helpful for difficult intubations in the emergent twin airways.

Two Babies, One Heart, and No Airway

The medical literature on conjoined twins is replete with descriptions of the complexity of surgical separations. However, airway management, although quite challenging, is neglected in literature. We present experience with conjoined twins united anteriorly at the thorax and abdomen sharing one six-chambered heart. Laryngoscopy and intubation recommendations, bronchoscopy techniques, and tracheotomy strategies are outlined for the face-to-face twins. Difficult, unique case report at tertiary care medical center. Conjoined thoraco-omphalopagus twins were successfully intubated at birth using the rigid Seldinger-assisted videotelescopic intubation (SAVI) technique. Flexible airway endoscopy allows for timely management of ventilator-dependent conjoined twins. Tracheotomy was later performed with the twins in the upright position with heads slightly rotated. Meticulous attention to detail, monitoring and vigilance are mandatory. Successful management of conjoined twins relies on close communication and cooperation of all members of the multidisciplinary team. Conjoined thoraco-omphalopagus twins united anteriorly require a challenging airway management strategy. A three-dimensional perspective is utilized for intubation and tracheotomy. The SAVI technique is helpful for difficult intubations in the emergent twin airways.

Prenatal sonographic delineation of the complex cardiac anatomy of thoraco‐omphalopagus twins

We describe a case of thoraco-omphalopagus twins diagnosed at 18 weeks' gestation. Each twin had a separate set of anatomical structures except for a shared heart, upper liver and anterior chest wall. The heart was located at the center of the juncture, and consisted of three atria and two ventricles, each twin having a single ventricle and right atrium but a fused left atrium. A set of great arteries originated from each single ventricle and there was juxtaposition of the aortic and pulmonary valves. One twin had transposition of the great arteries, whereas in the other these were normal in configuration. There was only one umbilical cord which contained four vessels (two arteries and two veins). This case report demonstrates the possibility of using fetal echocardiography to delineate the cardiovascular complex of the conjoined heart.

Successful separation of thoraco-omphalopagus and ischiopagus tetrapus twins in Korea

A pair of male thoraco-omphalopagus twins with common liver, diaphragm, pericardium, and sternum was separated at the age of 59 days after a parasitic relationship had developed between them. Before separation one baby developed acute renal failure during which he had no edema and had normal serum electrolytes, urea nitrogen, and creatinine due to "autodialysis" by the other baby. The boys have now grown normally to the age of 6 years. A pair of female ischiopagus tetrapus twins who had a common terminal ileum and colon with imperforate anus was separated at the age of 20 h. The smaller baby had congenital multiple arthrogryposis of both lower extremities, a fracture at the middle of the left femur, and a double vagina with hydrocolpos due to an imperforate hymen of the right vagina and a rectovaginal fistula on the left. Posterior sagittal rectoplasties were performed at 7 months of age in both babies. They have normal bowel movements. All four children are alive and developing normally. These are the first two case reports of successfully separated conjoined twins in Korea.

Sonographic Prenatal Diagnosis of Congenital Heart Defects in Thoraco‐Omphalopagus

Thoraco-omphalopagus is the most common variety of conjoined twins. About 90% of the conjoined thoraco-omphalopagus twins have a common pericardium, and 75% have conjoined hearts. Options for the treatment of thoraco-omphalopagus are largely dependent on the anatomy of the cardiovascular system. The extent of the conjunction of the heart and the severity of the associated cardiac defects primarily determine the feasibility of successful separation of the infants. Two-dimensional echocardiography and color Doppler examination has become the method used to investigate cardiovascular system of conjoined twins with a high degree of confidence. A case of thoraco-omphalopagus conjoined twins prenatally determined of the extent of cardiac conjunction and diagnosis of associated defects by these methods is presented.

Conjoined twins discordant for cleft lip and palate

Female thoraco-omphalopagus twins were of equal size and had similar abnormalities related to the twinning site. However, one twin was more severely affected and also had cleft lip and palate which the co-twin did not have. The implications of this are discussed.

Echo-Doppler assessment of cardiac status in conjoined (thoraco-omphalopagus) twins

Cardiac status was assessed noninvasively in a case of thoraco-omphalopagus conjoined twins by two-dimensional and Doppler echocardiography which showed completely separate hearts and major arteries and veins. The twins were successfully separated surgically, without preoperative invasive cardiac investigation. Echocardiography can provide an accurate assessment of cardiac status in conjoined twins and may obviate the need for cardiac catheterization.

Cholescintigraphy for Assessing the Separation Potential of Thoracoomphalopagus Twins

This case report describes the use of cholescintigraphy in the preoperative evaluation of ventrally conjoined twin girls. Sonography and magnetic resonance imaging disclosed an anatomically conjoined heart and a single apparently fused liver. Cholescintigraphy demonstrated that the hepatic system functioned as two independent livers, gallbladders, and biliary drainage systems that were susceptible to separation.

A method of repair of thoracic cage defects after separation of thoraco-omphalopagus twins

Female thoraco-omphalopagus twins weighing 3,450 g were born by normal, spontaneous vaginal delivery. Though twin A was stable, twin B developed cardiorespiratory distress soon after delivery. Progressive respiratory failure led to separation at the age of 57 days. In order to repair the large thoracic cage defects a sheet of Teflon felt was tailored into two “tents”. Twin A who survived, had primary closure of the chest and abdomen after extensive skin mobilization. Twin B had a Silon chimney inserted in addition to the Teflon felt tent. Both were removed 20 days post-operatively because of infection. She died of respiratory failure 55 days after separation.

Radiological Case of the Month

A gravida 0, para 0, 27-year-old pregnant woman with an unusually large belly at 30 weeks' gestation underwent abdominal ultrasound (Figs 1 and 2). A multiple pregnancy was suspected. <h3>Denouement and Discussion</h3><h3>Thoracoomphalopagus Twins With Big Omphalocele</h3> At a gestational age of 30 weeks, prenatal ultrasonographic diagnosis of thoracoomphalopagus twins was made. They were suspected of having a single heart and an omphalocele. At 34 weeks' gestation, twins were delivered by cesarean section. In the delivery room, the infants were apneic and cyanotic. They were intubated immediately and were ventilated with positive pressure. The combined body weight of the twins was 2280 g; they faced each other and were conjoined at the distal half of the thorax and proximal two thirds of the abdomen. On initial physical examination, twin A had lordosis and a tracheoesophageal fistula, and twin B had kyphosis as well as a left diaphragmatic hernia. The twins

Sonographic Antepartum Diagnosis of Conjoined Twins

The antepartum diagnosis of conjoined twins is difficult but extremely important for maximization of infant viability and reduction of maternal morbidity. Certain characteristic sonographic and radiological features of anteriorly joined twins allow the diagnosis to be made with some certainty. We describe a case of thoraco-omphalopagus twins in which the prepartum diagnosis was made according to these criteria, and we discuss the impact of early diagnosis on obstetric management. (JAMA1982;247:3351-3353)

Asymmetry and discordance for congenital anomalies in conjoined twins: A report of six cases

Six pairs of conjoined twins have been studied. The first case was a pair of 13-week-old omphalopagus fetuses. One was a holoacradius amorphus and the other had rachischisis and anencephaly. The second case was a pair of omphalopagus twins. One of the twins was macerated and corresponded to a developmental age of 13-14 weeks, while the other was developed to 28-30 weeks of gestation and exhibited urogenital and gastrointestinal defects not found in the smaller twin. In the third case, that of a thoracoomphalopagus, one had cleft lip and palate, pulmonic stenosis, and atresia of the ileocecal valve, while the other did not show these anomalies. In the fourth cae, also omphalopagus twins, one had a lumbosacral meningomyelocele and severe gastrointestinal and urogenital anomalies not found in the second twin. The fifth case was a pair of thoracoomphalopagus twins, sharing a common heart with asymmetrical anomalies. The sixth case was a diprosopus anencephalic conjoined twin. The first pairs of conjoined twins were discordant for several abnormalities in nonshared organs, in addition to having abnormalities of the conjoined organs. It seems that discordance in conjoined twins is not a rare finding. The factors that play a role in discordance of anomalies in conjoined twins are probably similar to the factors in monozygotic twins--i.e., environmental, genetic, and abnormal placental and/or fetal circulation.

Computed Tomography in Preoperative Diagnosis of Conjoined Twins

A case of thoracoomphalopagus twins diagnosed preoperatively by computed tomography is described.