Journal of Pathology and Translational Medicine

Case Reports

Acardiac Fetus with Encephalocele: A case report.: Seong Beom Lee, Won Sang Park, Ki Hwa Yang, Jong Chul Shin, Jung Yong Lee, Sang Ho Kim, Choo Soung Kim; Korean J Pathol. 1994;28(6):678-681.

Abstract PDF: The acardiac fetus is a rare type of fetal monster in which, as the name implies, the heart is completely absent. Acardius occurs only in a pair monozygotic twin, and shows various other defects in addition to the absence of the heart. Our autopsy case is acardiac anceps. He weighed 1,980 gm and the height was 33 cm. The brain is poorly developed, 60 gm in weight and similar to reversed snowman (3.5x2.8x2.8, 1.5x1.5x1.3 cm). Encephalocele, 6 cm in diameter, was communicated with the brain by a tract which contains nervous tissue and primitive choroid plexus. The upper extremities were absent, while the vertebrae and lower extremities were relatively well developed. The heart, lungs, stomach, liver, and spleen were absent, but the kidney, genital organs and urinary bladder were present. The intestine was seperated into two segments which were blindly ended, 32 cm and 15 cm in length, respectively.

Meckel-Gruber Syndrome: An autopsy case.: Soong Deok Lee, Chul Woo Kim, Je Geun Chi; Korean J Pathol. 1988;22(4):505-509.

Abstract PDF: We report an autopsy of a male fetus that showed multiple congenital anomalies that could best be designated as Meckel-Gruber syndrome. The fetus was born dead at the gestational age of 38 weeks. His parents denied any history of congenital malformation. And the parity of the mother was 0-0-0-0, but she had the past history of receiving herb medication for common cold. The congenital anomalies found in this case consited of occipital meningoencephalocele, midline cleft palate, bifid epiglottis, hepatic fibrosis, choledochal cyst, bilateral polycystic kidneys, postaxial polydactyly of both hands and feet, aplasia of the left testis, secundum type atrial septal defect and patent ductus arterious. This malformation syndrome is rare and lethal. The prenatal diagnosis should be made by ultrasound study or analysis of the amniotic fluid for alpha-feto protein during intrauterine period. The kidneys showed Potter type III cystic change and there was a characteristic hepatic fibrosis.

Intracranial Encephalocele: an autopsy case of anterior basal type.: Hyun Wook Kang, Je G Chi, Tae Dong Park, Hum Rae Park; Korean J Pathol. 1988;22(4):500-504.

Abstract PDF: Encephalocele is a relatively rare congenital anomaly which is classified into occipital, parietal, anterior syncipital and anterior basal type regarding to the protrusion site through the bony defect of the skull. Anterior basal type of encephalocele is important in view of it's pathogenesis as well as diagnostic difficulty because of invisibility on external appearance. We have experienced a case which could be best fit to anterior basal encephalocele. This type of encephalocele is extremely rare. This report deals with a case of deadborn of 34 weeks of gestation with body weight of 2400gm and head circumference of 32 cm. There was no evidence of protrusion of brain on external examination. On autopsy the normal brain structure was compressed by abnormal mass of brain with normal consistency which was found in the petrous portion of the parietal area and covered partly by the dura. In this case, there were another associated anomalies, such as atrophy of the left optic nerve, hemihypoplasia of the left mandible, patent ductus arteriosus, bilateral hydrocele, and Meckel's diverticulum.

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