Varicocele in Adolescents 

References to dilated and tortuous veins of the spermatic cord, now referred to as a varicocele, occurred as early as 1885. Even at that time, varicoceles were known to be associated with ipsilateral testicular atrophy, which appeared to be reversible following ligation. Four years later Bennett commented on his observation of a change in the character of seminal fluid following ligation of a varicocele.

Since these reports, considerable debate regarding the etiology and effects of varicoceles has appeared in the literature. The direct relationship between varicoceles and testicular atrophy, changes in Leydig and Sertoli cell function, abnormal seminal parameters, and endocrine abnormalities have been studied. Over the past 30 years, the advent of interventional radiology and minimally invasive surgical techniques has affected the way physicians approach adolescent varicocele.

Presentation

Varicoceles usually become evident around adolescence and are rarely reported to arise in older men. Patients are commonly referred to the urologist either for a scrotal mass, classically referred to as a "bag of worms," or are referred after detection of a difference in testicle size during a well-child visit or sports physical. Most varicoceles are asymptomatic; however, testicular pain or a mass may be a presenting symptom. Although varicoceles may be bilateral, they are usually unilateral and almost always left-sided. A unilateral right-sided varicocele should prompt an investigation for a retroperitoneal process such as a mass that causes obstruction of the right internal spermatic vein. Thrombosis or occlusion of the inferior vena cava must be ruled out in all patients who present with a solitary right-sided varicocele. These patients should undergo radiographic studies (eg, CT scanning) as part of their evaluation. Situs inversus is another etiology of a right-sided varicocele.

As with the arterial supply to the testis, the venous drainage has multiple anastomoses in the scrotum and the inguinal canal, which are referred to as the pampiniform plexus. A varicocele results from an abnormal dilation of this venous network.

A varicocele is an abnormal dilation of the pampiniform plexus of the testicular veins, which drain the testicle. Initial presentation usually occurs during puberty, with incidence in 13-year-old adolescent boys equal to that of adult men (15%). Rarely, varicoceles are noted in the prepubertal period.

Frequency

Varicoceles are extremely rare in patients younger than 9 years. The prevalence of varicoceles in individuals aged 10-19 years is reported to be approximately 15% and is similar to the prevalence reported for adults. However, because most adolescent varicoceles are asymptomatic, the true incidence of adolescent varicoceles is likely higher. Varicoceles are cited as one of the leading causes of male factor infertility and are detected in 35% of adult males with primary infertility.

The etiology of this condition is unknown but likely multifactorial. Various theories have been proposed to explain the cause of a varicocele in light of the fact that 90% of all varicoceles are left-sided. These theories include the following:

• Congenital absence of the valves in the left testicular vein, which normally prevent retrograde flow of blood in the upright position: Anomalous branches may also bypass the valves.
• Abnormal variations in venous drainage of the testes: An asymmetrical pattern is usually present, with the right testicular vein draining directly into the inferior vena cava and the left testicular vein inserting at a right angle into the left renal vein. This pattern predisposes to slower drainage in the left testicular vein.
• The "nutcracker" phenomenon: The left renal vein is occasionally compressed between the superior mesenteric artery and the aorta. This creates higher pressure in the left testicular vein, which drains into the renal vein.
• Increased length of the left testicular vein: The left vein is 8-10 cm longer than the right testicular vein.

A right-sided varicocele may be observed in association with a left varicocele (bilateral varicoceles), but an isolated right varicocele is very rare and raises certain concerns. The possibility of thrombosis or occlusion of the inferior vena cava must be eliminated in all patients who present with a solitary right-sided varicocele or in older adults who present with new-onset varicoceles.

Often, in the presence of a varicocele, the ipsilateral testis is abnormally small compared with the contralateral testis. Histologic studies have revealed seminiferous tubule sclerosis, small vessel degenerative changes, and abnormalities of Leydig, Sertoli, and germ cells. These changes have been documented in patients as young as 12 years. Effects of a varicocele on semen parameters have been extensively studied in adults. Consistent findings have included decreased sperm motility, lower total sperm counts, and increased number of abnormal sperm forms. A limited number of studies in adolescents with varicoceles have also shown altered seminal parameters in this age group.

Reasons for altered sperm production, testicular size, and morphologic changes are not clearly understood. Proposed mechanisms for this pathophysiology include the following:

• Dilated veins with pooling of venous blood results in increased scrotal and testicular temperature. This is theorized to alter DNA synthesis within the testicle, leading to morphologic changes in sperm and testicular tissue.
• Renal and adrenal metabolites that reflux into dilated spermatic veins affect testicular tissue damage through undefined mechanisms. Testicular hormone function may be compromised, leading to impaired spermatogenesis.
• Low oxygen content in the dilated veins may result in local tissue hypoxia. This could affect both testicular architecture and sperm production.
• Paracrine imbalances in the testicle due to any of the above mechanisms may lead to impaired testicular function.

These findings may be reversed with corrective surgery, and catch-up growth of the adolescent testicle is observed following varicocele ligation. In adults, varicocele is felt to be the most correctable cause of infertility.

The vast majority of adolescent patients with varicoceles are asymptomatic. The diagnosis is made by careful palpation of the scrotum during a thorough upright physical examination. The patient should be examined in the standing position, and the scrotum should be visually inspected for distended veins, which can usually be seen on the lateral aspect of the scrotum. The testes, spermatic cord, and scrotum are palpated, and testicular size is assessed with an orchidometer. A small varicocele may feel like a thickened spermatic cord. A larger varicocele has been said to feel like a bag of worms. The physician should then ask the patient to perform a Valsalva maneuver, which distends the veins of the pampiniform plexus and accentuates physical findings.

Next, examine the patient in a supine position. The venous dilation of the varicocele should diminish. Consider an obstructive etiology if this does not occur.

The testes of a normal patient should be symmetrical in size and consistency. A size difference of more than 3 cm³ is considered significant. The average volume of the male testis is 23 ±3 cm³, and standardized tables show the reference ranges for appropriate testis volume at different stages of development (see the table below). Most investigators use a standard orchidometer (ie, Prader orchidometer) to assess volume. Others suggest ultrasonography is a more accurate method of measurement. However, the added cost may be difficult to justify.

Table 1. Average Male Testis Volume at Different Stages of Development, as Determined by Orchidometer^[1] (Open Table in a new window)

Other presentations of varicoceles include symptoms of acute or chronic scrotal discomfort, differing testicular sizes without a palpable variocele on recumbent physical examination, and incidental finding on scrotal ultrasonography.

Varicoceles are graded based on physical examination findings and are classified as follows:

• Grade 0 - Subclinical varicocele; cannot be detected during physical examination; generally identified with ultrasonographic study or venography
• Grade 1 - Detected with palpation with difficulty (< 1 cm); increase in size with Valsalva maneuver
• Grade 2 - Easily detected without Valsalva maneuver (1-2 cm)
• Grade 3 - Detected visually at a distance (>2 cm)

Multiple investigators have directly correlated the degree of testicular atrophy with varicocele grade. Steeno noted that testis volume was reduced by 81% in patients with grade 3 varicoceles and by 34% in patients with grade 2 varicoceles.^[2] No patients with grade 1 varicoceles were noted to have testicular atrophy. Lyon et al reported that 77% of adolescent boys with easily palpable varicoceles had testis growth arrest, further suggesting that larger varicoceles are more likely to be associated with growth arrest.^[3] Some evidence shows that larger (grade 3) varicoceles may also place the contralateral right testis at risk for atrophy.

To date, no strict criteria indicate the physical findings or diagnostic criteria that dictate surgical intervention in adolescents. Controversies and opinions regarding when to operate and on whom to operate abound. Each case is handled individually, with a discussion among the patient, parents, and physician regarding the risks of intervention and potential impact on future fertility. Ipsilateral testicular growth retardation is the most frequent relative indication for varicocele repair in adolescents; the concern is that patients with varicocele and ipsilateral testicular growth retardation at that age may manifest impaired fertility in adulthood.

Although controversial, general guidelines used by the pediatric urologist to determine if surgery is indicated typically include the presence of one or more of the following:

• Varicocele associated with decreased ipsilateral testicular size: Generally accepted indication for correction is an orchidometer or ultrasonography measurement revealing a 20% volume deficit in the involved testis.
• Bilateral varicoceles
• Symptomatic painful varicocele
• Abnormal findings on semen analysis

Other disconcerting factors include grade 2 or 3 varicocele or significant difference in testicular consistency, with a softer ipsilateral testis.

If the decision is made to defer surgery, the patient should be monitored every 6-12 months to detect any deleterious effects as early as possible. The question of surgical intervention is readdressed at that time.

A varicocele is situated in the upper scrotum, above the testis. The spermatic cord extends upwards into the inguinal region, above the scrotum. The spermatic cord contains the spermatic veins, the vas deferens, and the testicular arteries, including the internal spermatic artery (may be multiple branches), the vasal artery, and the external spermatic artery. Above the inguinal region, the vas, with its arterial supply, diverges from the internal spermatic artery and veins, which course through the retroperitoneum, along the psoas muscle.

Corrective surgery involves interrupting the refluxing spermatic veins. This may be performed at various levels, usually above the varicocele. Surgery on the varicocele itself is generally avoided because of the many venous branches and the increased risk of bleeding. Surgery may be performed at the level of the uppermost scrotum, the inguinal area, or the retroperitoneum. When surgery is performed in the retroperitoneum, some authors advocate dividing both the testicular artery and the veins to avoid missing any venous branches. This latter technique relies on the vasal artery as the only remaining blood supply to the testis. These patients should be warned of the potential for testicular atrophy resulting from future vasectomy.

Varicocele ligation in a healthy patient has no specific contraindications, but various surgical approaches offer different advantages, and certain procedures should be avoided in specific settings. For example, a history of previous surgery may influence venous disruption within the site. With previous abdominal or retroperitoneal surgery, laparoscopic surgery is less desirable.

A history of inguinal surgery makes a second inguinal approach more difficult and potentially hazardous to the spermatic cord structures. Previous inguinal surgery may have also compromised the arterial supply of the testis. For this reason, when an adolescent with prior inguinal hernia surgery develops a varicocele, the best technique involves an inguinal approach with microscopic magnification to optimally identify and preserve the testicular artery. A retroperitoneal approach with testicular artery ligation is contraindicated because the initial hernia surgery could have inadvertently injured the vasal artery, and high ligation of the internal spermatic artery may cause testis atrophy due to arterial insufficiency.