Hyeyoung Lee, MD, MS
Harry P. Koo, MD, FAAP, FACS
BASICS
DESCRIPTION
A pediatric varicocele is defined as an abnormal dilatation of the internal spermatic veins in the pampiniform plexus of the spermatic cord in a male generally <18 yr of age. Usually asymptomatic; may cause testicular hypotrophy
EPIDEMIOLOGY
Incidence
• Reported frequency of varicoceles in adolescent boys is ∼16%, developing as a result of testicular enlargement and concomitant increased blood flow in puberty
• Actual incidence may be underestimated and detected later during evaluation of infertility in adulthood
Prevalence
• 8–16% of adolescent males but unusual in prepuberty
• 90% left sided
• 1–7% right sided
• 2% bilateral
• No racial, cultural, or geographic predilection
• More common in tall, thin males
RISK FACTORS
• Increased height, and relatively low weight and body mass index
• Congenital incompetence or absence of valves of internal spermatic vein
• Acquired incompetence of valves, extrinsic compression increasing intravascular pressure (eg, inguinal hernia repair, retroperitoneal pathologic process)
• May be associated with generalized venous abnormality
Genetics
• Varicocele prevalence as high as 67% has been reported in sons of fathers with varicocele
• Risk of varicocele in 1st-degree relatives is 4–8 times higher, suggesting genetic susceptibility (1)
PATHOPHYSIOLOGY
• Unique angle of left spermatic vein entering the left renal vein compared to right spermatic vein entering IVC
• Left spermatic vein is 8–10 cm longer than the right
• “Nutcracker” phenomenon of left renal vein passing between aorta and superior mesenteric artery
• Erect posture (no varicocele in four-legged animals)
• Different mechanisms are hypothesized to cause testicular insult
• Hyperthermia:
– Increased testicular arterial blood flow interferes with countercurrent heat exchange
– Increased testicular temperature affects enzymatic reactions
– Decreased proliferation and increased apoptosis of germ cells
– Heat shock protein A2, oxidative stress patterns, calcium channels, and DNA fragmentation affected
• Testicular hypotrophy: Significant testicular volume loss in 30–70% of adolescents with a varicocele:
– Most rapid growth of testis between ages 11 and 16 yr
– Testicular hypotrophy reversible in 90% of patients after varicocelectomy
• Venous stasis:
– Possible oxygen depletion in testis
– Human studies do not support theory
• Adrenal/renal reflux:
– Theory of toxic exposure to testis from reflux of adrenal and renal metabolites
– Data inconclusive
• Endocrine imbalance:
– Abnormal response in patients with varicocele to GnRH stimulation
– Unclear how it affects future fertility or hypotrophy
ASSOCIATED CONDITIONS
Secondary causes can include retroperitoneal tumor, renal mass with renal vein extension, renal vein thrombosis, retroperitoneal fibrosis
GENERAL PREVENTION
None
DIAGNOSIS
HISTORY
• Usually asymptomatic, associated pain reported in 2–11%
• Symptomatic dull ache or fullness in scrotum, worsened with activity
• Occasional testicular pain due to venous congestion
• Change in size with position or Valsalva
• Found after routine pediatric physical exam
PHYSICAL EXAM
• Examine patient upright and supine, with and without Valsalva
• Grading criteria:
– Grade 0: Subclinical, not visible or palpable, only detected by ultrasound (US)
– Grade I: Palpable only with Valsalva
– Grade II: Palpable but not readily visible
– Grade III: Visible through scrotal skin
• Check patient in supine position—idiopathic varicoceles may disappear, while secondary varicoceles persist if caused by tumor, especially on right side
• “Bag of worms” superior to testicle
• Negative transillumination
• Examine for bilateral varicocele and lymphedema
– If present, rule out secondary varicocele
• Testicular exam:
– Visual inspection
– Orchidometer—Prader vs. disk to determine testicular volume of each testicle and the comparison between the two testes
Prader orchidometer: Consists of 12 solid ellipsoid testis-shaped models ranging in volume from 1 to 25 mL (1–6, 8, 10, 12, 15, 20, and 25 mL), against which the testis is compared
Disk or Takihara orchidometer: Series of 15 punched-out elliptical rings; volumes ranging from 1 to 30 mL (1–6, 8, 10, 12, 14, 16, 19, 22, 26, and 30 mL)
– 2 cc or 20% size discrepancy suggests testicular hypotrophy
– Testicular hypotrophy is correlated with poor semen quality
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Semen analysis: Can be performed if Tanner stage 5 or >18 yr old
• Response to GnRH stimulation: Not useful for surgical decision making
Imaging
• Scrotal US
• Color Doppler US can detect subclinical varicoceles that are not palpable
– Decrease in volume of 2 cc or 20% size warrants intervention
– Spermatic vein diameter >2 mm in standing position with Valsalva is noted in up to 96% of boys with grade III varicocele
– Lambert formula: 0.71 (length × width × depth on US measurement)
Diagnostic Procedures/Surgery
Diagnosis confirmed by physical exam ± US
Pathologic Findings
• Leydig cell atrophy is known to be associated with high levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) and low testosterone levels
• Endothelial proliferation and basement membrane thickening in venules and capillaries
• Germ cell maturation arrest and sloughing, ultrastructural changes in Sertoli cells, Leydig cell atrophy to hyperplasia have been described in testicular biopsy specimens (2)
DIFFERENTIAL DIAGNOSIS
• Epididymal cyst (spermatocele)
• Hydrocele:
– Communicating
– Scrotal
– Spermatic cord
• Inguinal hernia
• Lipoma of cord
• Paratesticular rhabdomyosarcoma
TREATMENT
GENERAL MEASURES
• Management is either observation or surgical intervention
• Nonoperative treatment can be proposed when the patient or guardian fully understands the need for lifelong follow-up and the potential for progressive subfertility
• Surgical indications in the pediatric population:
– 2 cc or 20% size discrepancy between testicles based on US or orchidometer measurements
– Symptomatic
– Bilateral varicoceles
– Abnormal semen analysis
– Solitary testis with varicocele
MEDICATION
First Line
N/A
Second Line
N/A
SURGERY/OTHER PROCEDURES
• Surgical technique based on comfort and experience of surgeon
• Techniques described in more detail in Section I: “Varicocele, Adult”
• 50–75% of patients demonstrate catch-up growth, not necessarily meaning improved semen quality
• Testicular artery sparing:
– Doppler can help identify
– Preferred in adults because of concerns with infertility
– Should be considered in adolescents
• Laparoscopic:
– Retroperitoneal or transperitoneal
– High ligation of vessels (Palomo)
– Closer to left renal vein; usually fewer veins to ligate
– Laparoscopy offers magnification, facilitating artery/lymphatic sparing
– Single port laparoscopic approach provides excellent cosmesis
• Subinguinal/inguinal microsurgical:
– Provides facilitated artery and lymphatic sparing
– Low risk of hydrocele
– Time consuming
• Radiographic embolization:
– Limited data in children and adolescents, less successful than open or laparoscopic approach
– Significant radiation exposure
– Generally reserved for recurrent/persistent varicocele
ADDITIONAL TREATMENT
Radiation Therapy
N/A
Additional Therapies
N/A
Complementary & Alternative Therapies
N/A
ONGOING CARE
PROGNOSIS
No definitive evidence that adolescents with varicocele will have impaired fertility in future or that surgical correction will improve/prevent infertility
COMPLICATIONS
• Recurrence or persistence of varicocele:
– 1–35% depending on technique (3)
• Postoperative hydrocele (1–9%)
• Testicular atrophy
• Failure of catch-up growth
• Possible decreased fertility
FOLLOW-UP
Patient Monitoring
• If asymptomatic and no testicular size discrepancies, observe with biannual or annual exams
• If postsurgical, assess for testicular catch-up growth and hydrocele formation after 3 mo with US. Then monitor biannually or annually
Patient Resources
• Urology Care Foundation: Varicoceles
http://www.urologyhealth.org/urology/index.cfm?article=116
REFERENCES
1. Mokhtari G, Pourreza F, Falahatkar S, et al. Comparison of prevalence of varicocele in first-degree relatives of patients with varicocele and male kidney donors. Urology. 2008;71:666–668.
2. Heinz HA, Voggenthaler J, Weissbach L. Histopathological findings in testes with varicocele during childhood and their therapeutic consequences. Eur J Pediatr. 1980;133:139–146.
3. Barthold JS. Abnormalities of the testis and scrotum and their surgical management. In: Wein AJ, Kavoussi LR, Novick AC, et al., eds. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Saunders, 2011.
ADDITIONAL READING
• Ayan S, Woodhouse CRJ. The treatment of adolescents presenting with a varicocele. BJU. 2007;100:744–747.
• Diamond DA. Adolescent varicocele. Curr Opin Urol. 2007;17:163–267.
• Kass EJ. Adolescent varicocele. Pediatr Clin N Am. 2001;48:1559–1569.
• Robinson S, Hampton L, Koo HP. Adolescent varicocele. Urol Clin N Am. 2010;37:269–278.
See Also (Topic, Algorithm, Media)
• Infertility, Urologic Considerations
• Spermatic Cord Mass and Tumors
• Varicocele, Adult
CODES
ICD9
• 456.4 Scrotal varices
• 459.81 Venous (peripheral) insufficiency, unspecified
• 752.89 Other specified anomalies of genital organs
ICD10
• I86.1 Scrotal varices
• I87.8 Other specified disorders of veins
• Q64.8 Other specified congenital malformations of urinary system
CLINICAL/SURGICAL PEARLS
• Varicocele is found in 8–16% of adolescents.
• Genetic susceptibility, thin and tall body habitus, and venous abnormalities increase the risk of varicocele.
• Surgical treatment is indicated if testicular hypotrophy, bilateral varicocele, abnormal semen analysis or symptoms.
• Laparoscopic and open suprainguinal varicocelectomy are almost equally effective.
• Microsurgical approach minimizes risk of hydrocele development.