Xipho-omphalopagous twins--separation in the newborn.
B Birmole, B Kulkarni, R Shah, S Karapurkar, A Vaidya, M Vaidya, S Borwankar
Dept. of Paediatric Surg, KEM Hospital, Bombay, Maharashtra.
Dept. of Paediatric Surg, KEM Hospital, Bombay, Maharashtra.
Male conjoined twins (thoraco-omphalopagous) were delivered by emergency Caesarean section performed in a full term, 3rd gravida who had presented in labour. On examination one of the twins had gross monstrosity and was threatening the survival of the better twin (twin A). The cannulation and dye studies through single umbilical vein demonstrated significant cross circulation across the connecting bridge. The vein was connected to liver of twin A. An emergency separation was performed to salvage the better twin.
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Birmole B, Kulkarni B, Shah R, Karapurkar S, Vaidya A, Vaidya M, Borwankar S. Xipho-omphalopagous twins--separation in the newborn. J Postgrad Med 1993;39:99-101
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Birmole B, Kulkarni B, Shah R, Karapurkar S, Vaidya A, Vaidya M, Borwankar S. Xipho-omphalopagous twins--separation in the newborn. J Postgrad Med [serial online] 1993 [cited 2020 Mar 29 ];39:99-101
Available from: http://www.jpgmonline.com/text.asp?1993/39/2/99/622
Conjoined twins are a clinical rarity having an incidence that varies from 1 in 50,000 to 1 in 200,000 newborns. More than 600 cases have been reported in the world literature till 1986, most of which are autopsy reports. Although xipho-omphalopagous is the commonest variety with less anatomical complexity as compared to other varieties, the complex physiology and gross congenital malformation of one can be detrimental to the other. We report on separation of xipho-omphalopagous variety of conjoined twins in neonatal period. The indication of neonatal separation, complex anatomy and physiology is discussed.
A full term, 3rd gravida presented in labour to a peripheral Municipal hospital. She was never registered antenatally and did not give family history of twins. Because of prolonged labour and failure of progression of presenting part, an emergency caesarean section was performed. The delivery of twins was difficult because of gross monstrosity of one of the twins which involved megaencephaly, cystic hygroma involving head, neck and upper thorax. The delivery was facilitated by aspiration of cystic hygroma. Two hours later the twins were brought to our department. Both were males, the combined weight being 3.6 kgs and were joined by a bridge extending from xiphisterrium to the umbilicus [Figure:1]. There was a single ubmilical cord, attached on the inferior aspect of the bridge. A soft tissue mass, probably liver tissue could be palpated beneath the skin of the bridge. There were no palpable bowel loops in the isthmus. One of them (Twin A) had good cry activity and, was maintaining colour, temperature and tone. His pulse rate was 140/min and had good air-entry on both sides. His heart sounds were well heard.
The other twin (Twin B) has a gross congenital malformation involving head, neck and the pulse rate was 90/min. There was no cry and no attempt of breathing. Only a slight grimace could be elicitated on stimulating the child. No air entry could be heard and the heart sound were feeble. In spite of this the child was maintaining colour, suggesting significant cross circulation. The puncture mark on twin ‘B’ and injection site on twin ‘A’ were oozing constantly suggesting early DIC. The umbilical cord contained two umbilical veins and two obliterated umbilical arteries. The clinical examination provided ample evidence that twin ‘B’ was dependent on twin ‘A’ and was threatening the survival of twin ‘A’. In order to save time, only few investigations were performed and decision of emergency separation was taken.
Both were started separate intravenous lines and received Vit. K at the onset. Intravenous fluid requirement was calculated considering half of total weight as the individual weight. The neonatologists were called in to assist in the medical management. Plain X-ray revealed a gasless abdomen and chest of twin ‘B’ while the lungs of twin ‘A’ were well aerated and bowels contained gas. [Figure:2]. The ultrasonography of the connecting bridge revealed liver tissues with wide channels in the substance, the doppler study of which showed free circulation. The umbilical vein of twin 'B' could not be cannulated. On cannulating umbilical vein of twin A and injecting water soluble contrast medium, the main flow of dye was found to be towards twin W while the cross circulation through the connecting bridge towards twin 'B' was easily demonstrated [Figure:2]. 2D ECHo and ECG were not done to save time.
Three hours after admission the twins were shifted to the operation theatre with definite clinical evidence of cardiac failure in twin ‘A’ and disseminated intravasular coagulation in both. Awake intubation of twin W could be easily done while twin 'B' could not be intubated. Predicting the death of twin 'B' the surgeons proceeded with separation to save twin ‘A’. The draping was done to expose the bridge. Another operation table was kept ready to reconstruct the other twin separately.
An incision on the anterior surface of the bridge exposed the connecting bridge of liver tissue of the diameter of 2 cm. Both peritoneal cavities were separate extending upto the centre of the isthmus [Figure:3]. Division of the superior part of the bridge did not expose the pleura, pericardium or any defect in the diaphragm. There were two falciform ligaments going away from the umbilicus with the right containing an enlarged umbilical vein. The bowel of twin W contained gas and rest of the viscera was positioned normally. The extrahepatic biliary apparatus in both was separate and patent. There was no abnormality of the urinary system of either of the twins. [Figure:4] is a diagrammatic representation of horizontal section through both twins at the level of isthmus.
Once the peritoneal cavities of both twins were opened, it was possible to pass rubber tubing around the isthmus on either side of midline to act as a tourniquet. Then the isthmus was divided. The major vascular channels were caught and individually ligated. The cut margins of the liver were approximated by multiple interrupted figure of eight sutures. The posterior skin bridge was cut in line with the anterior incision. Inferiorly the cord was left attached to twin 'A'. The abdomen of twin 'A' could be closed easily. Twin 'B' expired immediately after separation while twin 'A' continued to show signs of cardiac failure and DIC. He was put on ventilator and succumbed two hours later.
Post-mortem examination of twin 'B' showed hypoplastic, nonaerated lungs on either side with atretic larynx and malformed heart. Post-mortem of twin 'A' was essentially normal.
Thoraco-omphalopagous forms the commonest variety of conjoined twins. The first reported surgical separation of conjoined twins is by Farius in 1689. Keisewetter has reviewed results of surgical separation of 25 pairs of conjoined twins of various types reported in English Literature, from 1689 to 1963. In 1660, Bohm separated a 2 day old xiphopagous twins with no common organs. After 1660, no other neonatal separation was reported until 1952, when Reitman et al described the separation of 1 day old Xiphopagi with a bridge of skin, subcutaneous tissues and cartilage but without organ or cavity sharing. Only nine cases of surgical separation in newborn period were found in English Literature till 1968 by Gans et al
Most surgeons prefer not to operate on conjoined twins in the newborn period. Surgery later in infancy has been recommended. However, emergency surgery is indicated only in cases, of (1) death or impending death of one twin that threatens the survival of the other; (2) existence of severe malformation that threatens the survival of twins and requires immediate correction and (3) significant damage to connection bridge,.
We did an emergency separation as the survival of twin 'A' was threatened by twin 'B' because the entire circulation of the dying twin was supported by the heart of the healthy twin. This probably induced complex haemodynamic changes in healthy twin leading to cardiac failure. Ligation of connecting channels probably worsened the changes making cardiac failure irreversible. The DIC was probably triggered off by the dying twin. Although cardiac failure in the healthy twin has been an indication for emergency separation, we could not come across any report mentioning DIC as an additional factor. Because of shortage of time we had no laboratory evidence to prove the same. The results of clinical evaluation confirmed by autopsy showed that twin 'B' was indeed a parasite of twin 'A'. Although twin 'A' had remained in a stable condition for sometime, the progressive heart failure and irreversible DIC was the cause of death.
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