유합쌍태아 중 하나에서 나타난 다비증
Polysplenia in one of conjoined twins

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신성식; 지제근
Issue Date
서울대학교 의과대학
Seoul J Med 1983;24(1):147-151
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.
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 24 No.1 (1983)
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