• Clinical science

Bladder exstrophy, abnormalities of the male urethra, and webbed penis

Abstract

Bladder exstrophy is a rare condition caused by an embryonal malformation resulting in failed closure of the anterior abdominal wall and an exposed urinary bladder. Surgery, which involves bladder and abdominal wall closure, is necessary and should be performed within the first days of life. Even with early treatment, life-long care is needed to prevent further complications. Abnormalities of the male urethra, on the other hand, are common and the need for treatment depends on the condition and the symptoms. Epispadias and hypospadias may result in difficulty urinating and infections, and surgical repair is indicated for restoring functionality and reconstruction of external genitalia. Posterior urethral valves are the most common cause of urinary obstruction in newborn boys and can be associated with hydronephrosis, renal failure, and pulmonary hypoplasia. Early identification by prenatal ultrasound with diagnosis by voiding cystourethrogram allows for cystoscopic valve ablation and relief of the obstruction. However, despite treatment, many patients will develop chronic kidney disease.

Bladder exstrophy

References:[1][2][3]

Epispadias

  • Definition
    • Embryonic malformation due to malpositioning of the genital tubercle → causes incomplete urethral tubularization on the dorsal penis when associated with bladder exstrophy, although in an attenuated form
    • May also be due to chronic pressure, e.g., as a result of a transurethral indwelling catheter eroding the urethra.
  • Epidemiology: incidence is about 1/200,000 live births (isolated hypospadias)
  • Symptoms/clinical features
  • Diagnostics: clinical
  • Treatment
    • In young patients: operative closure with urethroplasty and reconstruction of genitalia if indicated
    • In patients with long-term indwelling catheters: insertion of a suprapubic indwelling catheter (reconstructive treatment is usually not necessary)
  • Complications

References:[1][2][4]

Hypospadias

  • Definition: common congenital malformation; with incorrect positioning of the urethral meatus due to failure of urethral folds and foreskin to fuse on ventral penis
  • Etiology: : Genetic (e.g., disorders of sex development; ), endocrine (e.g., 5-alpha reductase deficiency) and environmental factors have been implicated
  • Epidemiology: incidence is 1/250 male births
  • Symptoms/clinical features:
  • Diagnostics: clinical
  • Treatment
    • Mild cases do not necessarily require surgery
    • Significant displacement or symptomatic micturition warrants surgical repair
      • Reconstruction of urethra, penis, and scrotum at 6 months of age: urethroplasty and orthoplasty
      • Severe cases require two-stage repair with correction of penile curvature first followed by urethroplasty at least 6 months later
    • Complications following repair:
      • Urethral fistula
      • Meatal stenosis
        • Narrowing of ventral meatus causes thin urinary stream and straining with urination
        • Most commonly caused by circumcision
        • Treatment consists of surgery (meatotomy)
      • Urethral diverticulum
      • UTI

Circumcision is contraindicated!

References:[5][6]

Posterior urethral valves

References:[4][7][8][9][10]

Webbed penis

  • Definition: congenital malformation with growth of the scrotum extending to the tip of the penis (usually only extends to the root of the penis); urethra is typically not affected
  • Symptoms/clinical features
    • Formation of webbing between the tip of the penis and the scrotum when erect
    • Potential erectile dysfunction and ventral deviation
  • Diagnostics: clinical
  • Treatment: early surgical correction with suturing of the scrotum to the penile shaft; generally for cosmetic reasons

References:[11]