유합쌍태아 중 하나에서 나타난 다비증

Abstract

The term "polysplenia" is used to indicate a characteristic constellation of visceral anomalies. of which the outstanding feature is a strong tendency for normally asymmetric organs to develop more or less symmetrically. Associated anomalies of the polysplenia include various cardiovascular anomalies, symmetrically bilabed lungs, and symmetrical hy poarterial bronchi, while asplenia is accompanied by cardiac malformations, symmetrically trilobed lungs and epiarterial bronchi. There is no reported case of polysplenia in Korean literature, although a few cases of asplenia have been reported. We studied a case of polysplenia in one of conjoined twins, and this unusual combination of rare anomalies is presented. This monoamniotic, monochorionic conjoined twins were delivered by caesarian section to a 29-year-old mother after 36 weeks of gestation, Prenatal diagnosis was twin pregnancy with breech presentation and premature rupture of membrane. The conjoined twins had two heads, two legs and two arms connected side by side (dicephalus dipus dibr-achius). In one individual (A) there were one heart, two lungs, one spleen and one kidney. In the other one (B) of conjoined twins, there were two bilobed lungs without heart, two spleens and one kidney. The spleens were of same size, connected by loose connective tissue. They were located both in the left upper quadrant of the body and weighed 8gm together. Both of them had caudal indentations and showed normal red and white pulps microscopically. As a whole, gastrointestinal tract was duplicated down to the level of distal ileum, where Meckel's diverticulum was found. There were one liver with two gallbladders and three adrenals. The anomalies of heart and great vessels of the individual (A) were hypoplasia of left atrium and ventricle, three superior vena cavae, atrial septal defect, partial anomalous pulmonary venous return, and vascular ring caused by aorta and pulmonary artery. The individual (B) had no heart but was connected to the cardiovascular system of individual (A) via the descending aorta. Special interest is put in this case on the concurrence of poly splenia and conjoined twins. The heart and the spleen develop at the same embryologic period, during the gestation age from the .il st to the 10th day. It is considered that ucurrence of acardia and polysplcnia only in one side of the conjoined twins implies the importance of intrauterine environmental factors related to genesis of the anomalies of the heart and the spleen.