official journal of Congeital Anomalies Research Association of Japan
Online ISSN : 2433-1503
Print ISSN : 0037-2285
A CASE OF A NEWBORN MALFORMATION ACCOMPANIED WITH CRANIAL DEFORMITIES
Kunikazu KIDOKeizo KAMIYAAkira SUZUKISusumu HAYASHI
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JOURNAL FREE ACCESS

1967 Volume 7 Issue 2 Pages 81-86

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Abstract

A case of newborn malformation with microcephalus, crania bifida through which a mass of substantia cerebrovasculosa protruded, huge bilateral polycystic kidneys and uterus separata duplex et septum vaginae was reported. The mother of the newborn, whose mate was her cousin, is 1 gravida, and 1 para with an anencephalus. In the 2nd stage of labor, the head of the infant was delivered smoothly, but the trunk could not pass through the birth canal due to the huge polycystic kidneys, and the infant died with the enlarged abdomen and legs remaining in the uterine cavity. In order to diminish the body size of the infant, the polycystic kidneys were removed through the birth canal. The necropsy-findings of the newborn are as follows : Age ; 38 weeks of gestation (premature female). Bodyweight ; 2480g. Stature ; 43cm. The head is very small, the abdomen is enormously enlarged, and the brain is 80g, a quarter of the normal one. A part of the occipital bone has a defect of 1.5cm in diameter, and a soft red purple mass of 3cm in length is protruding from there, the base of which is connected to the brain itself. The kidneys, the right one weighing 220g, the left 440g, have the appearances of multiple cysts and seem to have caused the enormous enlargement of the abdomen described above. The microscopic examination revealed that the kidneys were composed of elongated cysts lined with a single layer of low cuboidal cells, mass of immature cells, which were probably of mesenchymal origin, and increased connective tissues, The weight of other organs are as follows : the heart 24g, the right lung 8.6g, the left 6.0g the liver 130g, the spleen 13.5g, the pancreas 3.0g, the thyroid gland 1.0g, the thymus 3.1g, and the right adrenal gland 1.5g, (the left had been lost during the removal of the huge kidneys). In the sex organs is found the complete duplication of the uterus, cervix, and vagina. The right and the left organs are located symmetrically, and almost of the same size.

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© 1967 The Japanese Teratology Society
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