[Cystic tumefaction in the minor pelvis--the agenesis-dysplasia syndrome (case report)].
Ectopic kidney often shows signs of parenchyma maldifferentiation. Multicystic or dysplastic kidney is usually associated with congenital urogenital and skeletal system anomalies. In the Unilateral form of the agenesia-dysplasis syndrome, the kidney, if it is present, is small dysplastic and usually cystically changed. Ipsilateral uterus horn or vaginal agenesia are the most frequently associated anomalies. Case report. A six years old girl was clinically examined due to recurrent urinary tract infections. On ultrasound, the left kidney was enlarged, while the right kidney was absent in normal position. Cystic mass 4x5 cm was seen in the pelvis. The right kidney was not seen on IVP. CT scan showed a cystic formation in the pelvis, described as cystically changed dysplastic kidney. Pelviceal mass was extirpated. Exploration of the genital system revealed vaginal arch blinded in the hypoplastic right uterus horn. On pathohystology tumefaction corresponded to the dysplastic kidney.


CONCLUSIONS
unilateral renal aplasia or dysplasia may indicate genital anomalies having in mind the hereditary character of unilateral form of the agenesia-dysplasia syndrome therefore, it could be helpful in prenatal diagnosis of cystic pelvic mass of fetus in families with this syndrome.
Pavlović
S
S
University Children's Hospital, Belgrade.
Kostić
M
M
Milanović
D
D
Popović-Rolović
M
M
Basta-Jovanović
G
G
Jovanović
O
O
Peco-Antić
A
A
Kruscić
D
D
D000015:Abnormalities, Multiple / Q000175:diagnosis; D002648:Child; D005260:Female; D005836:Genitalia, Female / Q000002:abnormalities*; D006801:Humans; D007668:Kidney / Q000002:abnormalities*
Cisticka tumefakcija u maloj karlici--sindrom agenezije-displazije (prikaz bolesnika)]