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Congenital Urethral and Testes Anomalies

Congenital Urethral and Testes Anomalies

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Typical anatomy:
The penile urethra passes through the penis and opens at its distal end. Dorsal surface of penis faces abdomen; ventral surface faces the legs.
Urethral defects
Be aware that urethral displacement can also occur in female embryos, but is more common in males.
Hypospadias are defects on the ventral aspect (the underbelly) of the urethra
  • "Hypo" means "below"
  • Occurs when urtheral fold fusion is interrupted, leaving openings in the ventral urethra.
— Current thinking is that hormonal abnormalities lead to this defect.
  • Hypospadias is problematic because of the cosmetic abnormality, the voiding abnormalities (which are of varying severity), and the associated chordee (the bent penis) that can accompany it (we'll explore it further in a moment).
— Corrective surgery is typically performed for hypospadias.
  • Hypospadia variants:
— Perineal hypospadias lie in the perineum — Scrotal hypospadias pass through the scrotal tissue — Penile hypospadias occur along the shaft of the penis — Glanular hypospadias pass through the ventral surface of the glans, aka, tip, of the penis
Epispadias are mis-placed openings in the dorsal surface of the urethra.
  • "Epi" means "above"
  • Associated with dorsal chordee
  • Variants:
— Penopubic (occurs when the pubic bones do not fuse and the urethra remains open) — Penile epispadia — Glanular epispadia
  • Epispadias can be associated with bladder exstrophy:
— The flattened urinary bladder protrudes from the abdomen and the unfused pubic bones are widely apart. — Penis is superiorly displaced.
Testicular defects
Normal location:
  • Within the scrotum outside of the body, where temperature is held slightly lower than that of the body.
Cryptorchidism
  • Undescended testes; can be found anywhere along the typical migratory path:
— Abdomen — Inguinal canal — Just superior to the scrotum (aka, suprascrotal)
  • Can be uni- or bilateral, and tends to resolve itself within a few months after birth.
— However, if it does not, surgery is usually required to avoid infertility, increased risk of testicular cancer, and increased risk of testicular torsion (see reference below) (recall that sperm function only when kept slightly cooler than body temperature; it is thought that, when the temperature is chronically high, cancerous cells are more likely to proliferate).
Hydrocele
  • Fluid accumulates in the tunica vaginalis, which surrounds testis in scrotum.
  • Recall that the tunica vaginalis forms a sac and precedes the testis into the scrotum during testicular migration; typically, the fluid within the sac is reabsorbed and the tunica vaginalis loses its attachment to the abdominal peritoneum.
  • In some cases, peritoneal attachment is lost but the fluid is not reabsorbed, creating a non-communicating hydrocele.
  • Such hydroceles form visible swellings within the scrotum and are typically benign; they often resolve themselves over time.
  • However, in other cases, the connection between the tunica vaginalis and abdominal cavity persists, allowing fluid to accumulate in a "communicating hydrocele."
— Because this persistent communication can lead to intestinal herniation, surgery is often necessary.