Terminology and Classification of Congenital Abnormalities of Urogenital Organs

Abstract

Urogenital organs are classified into the following two groups and fifteen subgroups: Group A, kidney and urinary tract: 1. kidney, 2. calyx and pelvis, 3. UPJ, 4. ureter, 5. UVJ, 6. bladder, 7. trigone and bladder neck, 8. urethra and meatus. Group B, gonad and genital tract: 1. testis, 2. epididymis, 3. seminal duct, 4. seminal vesicle, 5. prostate, 6. penis, 7. scrotum. Their congenital abnormalties may be classified into the following four groups: I. Number; agenesis or aplasia (congenital defect), dupli- or multiplication. II. Position; ectopia, ectopic opening of meatus, malrotation, torsion, transposition. III. Form and structure; fusion, obstructive uropathy, submucous fibrosis, cystic disease. IV. Function; neurogenic dysfunction of urinary tract, hormonal dysfunction of testis. Some abnormalities may belong to several groups in overlap. For example, a fused kidney may be accompanied by the abnormality of number and position of the ureter; renal hypoplasia or dysplasia is often found in case of the ectopic opening of the ureter; bladder exstrophy and epispadias always coexist; testicular agenesis or dysgenesis gives rise to the transformation of internal sexual organs to female type. It is also well known that many congenital abnormalities of urogenital organs, e. g., cryptorchidism, small penis, fused kidney, duplicated renal pelvis and ureter, are often found in various malformation syndromes, e. g., 4p^-, 13-, 18- and 24-trisomies, prune-belly, PradderWilli and crocodile tear syndromes. Congenital abnormalities of urogenital organs are also found frequently in association with simple malformations such as polydactyly and dysplasia of auricle.