urogenital malformation

Defect in the organs or tissues of the urinary system or in the sex organs (genitals).

In polycystic kidney disease (an inherited disease), cysts of varying size enlarge one or both kidneys. Kidneys may have abnormal shapes or may be fused. In megaloureter, the ureter's diameter is enlarged. Males may have epispadias or hypospadias, in which the urethra opens on the upper surface or the underside, respectively, of the penis, or cryptorchidism (undescended testicles), in which one or both testes have not descended from the abdomen into the scrotum before birth. Female genital system malformations include agenesis of the ovaries, vagina, or uterus and abnormally shaped uterus.

* * *

▪ pathology

      any defect in the organs and tissues responsible for the formation and excretion of urine or in the sex organs (reproductive system disease) or in both. Some of the more important conditions include:

      1. Multicystic dysplastic kidney, a common type of kidney malformation in newborns in which cysts of varying size enlarge one or both kidneys. Though not necessarily fatal, the condition causes a decrease in the amount of functional kidney tissue, which creates a tendency toward infection.

      2. Abnormal shapes of the kidney, of moderate frequency and including fused kidneys and horseshoe kidney. These organs usually function normally but show an increased tendency toward infection and stone formations.

      3. Megalo-ureter, a disorder in which the passage carrying urine from kidney to bladder is enlarged, sometimes to the size of the small intestine. The cause is usually obstruction of the ureter, bladder, or urethra, which must be treated to avoid kidney damage.

      4. agenesis of the abdominal muscles, occurring mostly in males, involving the failure of either one set of muscles or all to form, in which case the abdomen consists of a loose sack of skin. Because there is no support for the viscera, various malfunctions, especially of the urogenital system, occur. Treatment consists of supporting the abdominal wall by the use of a corset and by symptomatic care of malfunctions already present.

      5. Epispadias, an uncommon malformation of the male genital system in which the urethra opens on the upper surface of the penis. In hypospadias, often familial, the urethra opens on the underside of the penis. Plastic surgery can repair both anomalies.

      6. cryptorchidism (q.v.), or undescended testicles, a common disorder in males in which one or both of the testes (testis), which usually descend from the abdomen into the scrotum during the ninth month of fetal life, fail to descend because of mechanical difficulty or hormonal defect. Spontaneous descent usually occurs within a few years; if not, hormone treatment or surgery is employed.

      7. Female genital system malformations, mostly either agenesis of the ovaries, vagina, or uterus or abnormally shaped uterus. The former results in sterility or infertility, and the latter may interfere with the ability to carry an infant to term.

* * *