Potter 증후근(1부검 증례보고)

Abstract

In 1946 Potter described a series of 20 cases in infants in whom bilateral absence of the kidneys was associated with hypoplasia of the lungs and a chao racteristic face. This combination has been known as Potter's syndrome after the first describer, E.L. Potter. The main facial features she described were an incr· eased space between the eyes, a promnient fold which arises at the inner canthus and sweeps downward and laterally below the eyes, unusual flattening of the nose. excessive recession of the chin. moderate enlargement and decreased chondrification of the ears, and a suggestion of premature senility. The mother of this patient, 28 year old, para 0-01- 0, was admitted to a local clinic with the chief complaint of amenorrhea for 35 weeks in labor and diagnosed as fetal death in utero.There was no known exposure to infection or drug during this pregnancy. She delivered a male baby rather macerated, with cord coiling around the neck twice. Autopsy findings were; crown-rump. 27cm: crown-heel, 42cm; body weight, 2.2kg. The abnormal facial features were low-set and floppy ears, receding chin with dimpling between lower lip and chin, flattened nose, less prominent fold and the appearance of premature senility, The lungs showed hypoplasia with pale color and incomplete lobation, weighing 5.9gm and 6.5gm in the left and the right side, respectively. Microscopically, the alveoli were crowded and revealed tubular structure without specific abnormality except for a general reduction of the parenchymal bulk. There was a bilateral absence of kidneys. and retroperitoneal space was replaced by slightly enlarged and disc-shaped adrenal glands. Both ureters were absent together with hoth renal arteries. The urinary bladder was elongated superoinferiorly to show a tubular structure. and its wall was attenuated in thickness. The left testis was located in the abdomen but the right testis was located in scrotun, embedded in hematoma. There was an extradigit of the right hand.