CRYPTORCHIDISM

 

CRYPTORCHIDISM

 

DEFINITION:

A disorder characterized by the failure to find one or both testes in the scrotum after 1 year of age.

EPIDEMIOLOGY:

  • incidence: 7/1000 at 1 year of age
  • age of onset:
    • after 1 year of age (by definition)
  • risk factors:
    • prematurity

PATHOGENESIS:

1. Background

  • the processus vaginalis represents a peritoneal diverticulum in the embryonic lower anterior abdominal wall that transverses the inguinal canal during the 7th month of gestation carrying with it the testis which descends through the inguinal canal and into the scrotum
  • therefore, 100% of infants <900 grams will have undescended testes while 17% of infants between 2000-2500 grams will be affected and 3.4% of full term infants
  • undescended testis is not associated with urinary tract anomal-ies
  • anorchia (absence of functioning testes) is present in 3-4% of undescended testes and 20% of nonpalpable testes

2. Types of Cryptorchidism

1. True Undescended Testes

  • the testes are found within the normal path of descent with a patent processus vaginalis
  • testis may be found intra-abdominally or intracanicular (within the inguinal canal)

2. Maldescended Testes

  • testes have descended through the inguinal canal but are positioned in the subcutaneous tissue outside the scrotum

CLINICAL FEATURES:

1. Genitourinary Manifestations

  • testis or testes are not found in the scrotum
  • bilateral in 30% of cases

2. Complications

1. Infertility

  • with unilateral cryptorchidism, the rate of infertility is similar to that of the general population
  • almost all adults with uncorrected bilateral cryptorchidism will be infertile and even those with bilateral cryptorch-idism corrected in childhood will be infertile in 67% of cases
  • an undescended testis is histiologically normal at birth with failure of development and atrophy detected by the end of the 1st year of life; by the 2nd year of life the number of germ cells is significantly reduced

2. Tumor

  • 20-44% increase in risk of developing a malignant testicu-lar tumor in the 3rd to 4th decades of life
  • seminoma is the most common tumor developing in undes-cended testes (in 60% of cases)
  • greatest risk is in those with uncorrected intra-abdominal cryptorchidism and in those with surgical correction during or after puberty
  • there is a decreased incidence of tumors in those with surgical correction occurring before 8 years of life

3. Indirect Inguinal Hernia

  • always present with true undescended testes and common with ectopic testes

4. Torsion

  • increased risk with cryptorchid testis +/- infarction

INVESTIGATIONS:

1. Pelvic Ultrasound

  • to identify the location of a non-palpable testis

2. Endocrine

1. Human Chorionic Gonadotropin (hCG) Stimulation

  • used to confirm the absence or presence of testicular tissue in patients with bilateral nonpalpable testes - an increase in testosterone levels after hCG stimulation indicates functioning testicular tissue

2. Gonadotrophins

  • high FSH and LH indicate a congenitally anorchid patient as there is a lack of negative feedback by testesterone - low or normal FSH and LH levels may indicate functioning gonadal tissue

MANAGEMENT:

1. Medical

1. Human Chorionic Gonadotropin (hCG)

  • used to induce testicular descent
  • best response in those with bilateral cryptorchidism with the testes near the scrotum (not recommended for unilateral cryptorchidism)
  • success rate <10%

2. Surgery

1. Surgical Exploration

  • not indicated in congenitally anorchic patients where the basal FSH and LH levels are elevated and there is a lack of rise of testosterone with hCG stimulation
  • indicated if the basal FSH and LH levels are low (indica-ting negative feedback by testosterone)

2. Orchiopexy

  • indications:
    • unilateral and bilateral cryptorchidism where the testes are palpable
    • when the testis is not palpable but located intra-abdominally immediately inside the internal inguinal ring
  • best performed towards the end of the first year of life or early in the 2nd year of life
  • irreversible degenerative changes may occur without cor-rection by two years of age
  • anchors the testis to the scrotum with repair of the accompanying indirect inguinal hernia
  • does not eliminate the risk of future malignancy

3. Orchiectomy

  • indications:
    • intra-abdominal testis
    • severely atrophied testis
    • bilateral cryptorchidism where the testes are not palpable
  • testicular prostheses are available

 

 

 

 

Pediatric Database - CRYPTORCHIDISM

Pediatric Organization - Pedbase [at] Gmail.com