Julia S. Barthold, MD, FACS
BASICS
DESCRIPTION
• Torsion of the testicle or testicular/epididymal appendages results in vascular compromise to the testicle or 1 of the appendages.
• Impaired perfusion of the testis, appendix testis, or appendix epididymis is caused by spermatic cord (testicular) torsion or appendix torsion
• Presents as acute scrotal and/or inguinal pain with or without scrotal erythema and swelling
• Occurs primarily in children
EPIDEMIOLOGY
Incidence
• Most scrotal pain occurs at age 12–18
• Bimodal age distribution
– Perinatal: Extravaginal testicular torsion
– Prepubertal:
More commonly appendix torsion
Some data suggests this may be the most common cause of acute scrotal pain in children
– Puberty: Peak incidence of intravaginal testicular torsion, but can occur at any age
Prevalence
1:4,000 males <25 yo
RISK FACTORS
• Usually none
• Cryptorchidism
• History of contralateral torsion
• Familial clustering has been reported
Genetics
• Testicular torsion reported in 10% of family members; may be autosomal or X-linked recessive (1)
• No specific genetic defects identified
PATHOPHYSIOLOGY
• Testicular torsion can be either intravaginal or extravaginal
– Intravaginal testicular torsion
Twisting of the spermatic cord within the tunica vaginalis
Due to congenital incomplete fixation of testis within the tunica vaginalis (bell-clapper deformity, see image)
Intermittent or sustained
Impaired venous outflow, impaired arterial inflow, ischemia, potential testicular necrosis
May progress to compartment syndrome
Ischemia/reperfusion injury causes impaired spermatogenesis in animal models
• Extravaginal testicular torsion
– Twisting of both the spermatic cord and tunica vaginalis
– Due to incomplete fixation of the tunica vaginalis to the scrotum in the perinatal period
• Appendix torsion: Vascular compromise may be related to pedunculated anatomy of the appendage (image)
– Appendix testis (also known as hydatid of Morgagni)
A vestigial Müllerian duct remnant present in majority of males (92%)
Typical position: The superior testicular pole in the groove between the testicle and the epididymis.
Accounts for 95% of appendage torsions
– Appendix epididymis
A vestigial Wolffian duct remnant is less commonly present
– The paradidymis (organ of Giraldes) and the vas aberrans (organ of Haller) are 2 other appendages that are not clinically important.
ASSOCIATED CONDITIONS
• Bell-clapper deformity: 10–15% of males
• Cryptorchidism
GENERAL PREVENTION
• Reduce testicular necrosis risk by:
– Early diagnosis and treatment
– Community awareness about testis pain
– Elective bilateral orchidopexy for intermittent pain or contralateral orchidopexy at surgery for an episode of acute torsion
DIAGNOSIS
HISTORY
• Intravaginal testicular torsion
– Usually severe pain, sudden onset
– Nausea/vomiting more common
– May be recurrent usually same side
• Extravaginal testicular torsion
– Usually painless and asymptomatic
• Appendix torsion
– Usually more gradual but may be acute
– Pain may be mild or severe
– Nausea/vomiting uncommon
– No prior episodes
• Inguinal or abdominal pain may be associated or may be only site of pain in younger boys
• Irritative voiding symptoms possible
• History alone is suggestive only and often not reliable in differentiating testicular torsion, appendix torsion, and other causes of scrotal pain
PHYSICAL EXAM
• Note: Phren sign (elevation of scrotum relieves pain in epididymitis but in torsion it is no longer considered reliable)
• Intravaginal testicular torsion: These are possible findings but these may be highly variable.
– Early
Generalized testicular tenderness
Loss of ipsilateral cremasteric reflex
Elevated ipsilateral testis or transverse lie (look for this in intermittent testicular torsion cases)
Anterior epididymis
– Late
Any of the above; increasing scrotal swelling and erythema
Loss of scrotal rugation ± hydrocele
Inability to distinguish epididymal landmarks
• Extravaginal testicular torsion
– Firm to hard nontender testis
– Scrotal discoloration
• Appendix torsion
– Early
Localized tenderness superior to testis
Supratesticular nodule
Preserved ipsilateral cremasteric reflex
Normal testicular position and orientation
Blue dot sign: Rare, more likely in prepubertal boys; tender nodule with blue discoloration on the upper pole of the testis and more easily see in light-skinned individuals
– Late
Any of the above
Generalized tenderness
Increasing scrotal swelling and erythema
Hydrocele
ALERT
• No H&P findings are completely reliable in diagnosis of the acute scrotum.
• Urgent surgery always needed if evaluation does not rule out the possibility of spermatic cord torsion.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Urinalysis
– If pyuria suspect epididymitis/UTI
– If hematuria consider renal or ureteral source of pain (eg, stone)
• Urine culture if indicated
• Additional labs not needed
Imaging
• Scrotal US with Doppler
– Need to identify waveforms that originate in the central parenchyma
– Intravaginal testicular torsion findings:
Usually shows decreased or absent arterial flow but may be normal
Increased flow possible in intermittent testicular torsion
Increased or mixed echogenicity suggests torsion: Compare both sides
Hydrocele and/or enlarged epididymis may be present
Visible twist of cord: Requires expertise but highly specific if present
– Extravaginal testicular torsion findings:
Heterogeneous appearance typical
Doppler flow may be hard to demonstrate in neonatal testes
Calcification may be present
– Appendix torsion findings:
Normal exam most common
Supratesticular complex mass w/o vascular flow may be present
Enlarged epididymis reported as “epididymitis” often present
Doppler flow normal or increased
– Doppler flow normal
– Thickened scrotal skin and hydrocele are nonspecific findings in acute scrotum cases
• Nuclear scan: Rarely performed
Diagnostic Procedures/Surgery
Exploration for diagnosis if equivocal findings on exam and/or US, suspicion of testicular rupture, or tumor
Pathologic Findings
• Testicular necrosis (intravaginal) or subtotal loss of tubules with calcification (extravaginal) testicular torsion
• Severity of injury depends on age, duration of testicular torsion, number of twists/thickness of spermatic cord
• In cases of appendicular torsion the necrotic tissue is reabsorbed usually without any sequelae
DIFFERENTIAL DIAGNOSIS
• Acute testicular pain
– Appendix torsion most common in prepubertal boys
– Testicular torsion most common in peripubertal boys but can occur at any age; less common than appendix torsion
– Epididymitis due to UTI or STD: Rare or uncommon in pediatric age group; more likely in adult
– Communicating hydrocele ± meconium
– Incarcerated inguinal hernia
– Trauma and possible testicular rupture
Hematocele present
– Orchitis (eg, mumps)
– Henoch–Schönlein purpura
Rash usually present
– Fournier gangrene (rare in children)
– Referred pain from urolithiasis or intra-abdominal process such as appendicitis
– Orchalgia; consider voiding dysfunction
TREATMENT
GENERAL MEASURES
• Testicular torsion: Consider manual detorsion in ER
– Not routine; consider if surgical delay
– Most likely to be effective early in course
– Sedation recommended
– External rotation of testis when viewed from the feet (“opening a book”)
– Not always effective, 1/3 of cases may rotate laterally
– Does not preclude need for immediate surgery
• Appendix torsion: Rest until pain resolves
– Urgent re-evaluation if pain worsens or is recurrent
MEDICATION
First Line
• Ibuprofen to reduce inflammation in appendix torsion
• Antibiotics for UTI, STDs
Second Line
N/A
SURGERY/OTHER PROCEDURES
• Testicular torsion
– Emergent exploration indicated if evaluation suggests intravaginal testicular torsion or diagnosis is equivocal
– Detorsion, observation for reperfusion, and bilateral orchidopexy via scrotal approach with fixation of testis extravaginally
– Consider capsulotomy; if flow improves with placement of tunica vaginalis patch
– Urgent exploration, bilateral fixation for extravaginal testicular torsion to avoid asynchronous contralateral torsion (2)
– Avoid imaging/delay if findings are classic
– Elective surgery for resolved intermittent testicular torsion (3)
– Orchiectomy in antenatal extravaginal testicular torsion or if testis appears nonviable after detorsion ± capsulotomy
– Consider delayed prosthesis placement for monorchia
• Appendix torsion
– Prolonged pain not responsive to conservative measures
– Recurrent episodes (rare) or diagnostic uncertainty
ADDITIONAL TREATMENT
Radiation Therapy
N/A
Additional Therapies
Experimental agents such as nitric oxide (NOS) inhibitors to reduce reperfusion injury in testicular torsion not used clinically
Complementary & Alternative Therapies
N/A
ONGOING CARE
PROGNOSIS
• Intravaginal testicular torsion
– Risk of postoperative atrophy increases with duration of torsion
– Testicular loss 1st seen at 4 hr, increasing after 6 hr, 80% after 12 hr and essentially universal after 24 hr
– Risk of subfertility unclear but probably higher after postpubertal torsion
• Extravaginal testicular torsion
– No salvage in cases of antenatal torsion
– Risk of contralateral torsion in the neonatal period low but present with contralateral fixation recommended
– Long-term risks unknown but fertility potential presumed normal
COMPLICATIONS
• Recurrent testicular torsion: Rare, occurs with failure of absorbable or nonabsorbable suture fixation of a testis that remains within an intact tunica vaginalis
• Testicular atrophy
FOLLOW-UP
Patient Monitoring
• Follow for at least 6 mo to determine risk of atrophy
• Monitor for recurrent testicular pain
• Scrotal protection in contact sports
• Education of healthy adolescent populations about the signs/symptoms of testicular torsion and the benefits of early evaluation and treatment
• Specific educational focus on family members of affected individuals
Patient Resources
MedlinePlus: Testicular torsion. http://www.nlm.nih.gov/medlineplus/ency/article/000517.htm
REFERENCES
1. Cubillos J, Palmer JS, Friedman SC, et al. Familial testicular torsion. J Urol. 2011;185:2469–2472.
2. Nandi B, Murphy FL. Neonatal testicular torsion: A systematic literature review. Pediatr Surg Int. 2011;27:1037–1040.
3. Eaton SH, Cendron MA, Estrada CR, et al. Intermittent testicular torsion: Diagnostic features and management outcomes. J Urol. 2005;174:1532–1535.
ADDITIONAL READING
• Mellick LB. Torsion of the testicle: It is time to stop tossing the dice. Pediatr Emerg Care. 2012;28:80–86.
• Sells H, Moretti KL, Burfield GD. Recurrent torsion after previous testicular fixation. ANZ J Surg. 2002;72:46–48.
See Also (Topic, Algorithm, Media)
• Appendix Testis and Appendix Epididymis, Torsion
• Scrotum, Tumors, Benign and Malignant
• Testis, Pain (Orchalgia)
• Testis, Tumor and Mass, Adult, General
• Testis, Tumor and Mass, Pediatric, General
• Torsion, Testis or Testicular/Epididymal Appendages Images
CODES
ICD9
• 608.20 Torsion of testis, unspecified
• 608.23 Torsion of appendix testis
• 608.24 Torsion of appendix epididymis
ICD10
• N44.00 Torsion of testis, unspecified
• N44.03 Torsion of appendix testis
• N44.04 Torsion of appendix epididymis
CLINICAL/SURGICAL PEARLS
• Diagnosis of spermatic cord torsion requires a high index of suspicion, particularly in patients with intermittent testicular pain.
• Emergent surgery is indicated for all cases of suspected spermatic cord torsion.
• The risk of testicular loss increases after 4–6 hr of untreated spermatic cord torsion.