Testicular Disorders and Disorders of the Scrotal Contents

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[edit] Testicular Disorders and Disorders of the Scrotal Contents

Grannum R. Sant

Kenneth A. Kingsly

Diseases of the scrotum and its contents usually lead affected individuals to seek medical attention. Most scrotal conditions are benign and self-limiting, although malignant testicular tumors can be life-threatening. The clinician evaluating scrotal lesions should differentiate benign from malignant diseases, pursue an efficient cost-effective diagnostic evaluation, and initiate prompt and appropriate urologic referral.

Pain and swelling are the cardinal symptoms of scrotal disease: congenital or acquired, benign or malignant. Accurate diagnosis requires a careful history and a structured physical examination, including urinalysis. Radiologic studies such as scrotal ultrasonography, radionuclide studies, and Doppler blood flow studies are performed in selected patients with uncertain diagnosis.

[edit] ANATOMY AND PHYSICAL EXAMINATION

The scrotum is partitioned into two sacs, each containing a testis, epididymis, and the lower portion of the spermatic cord. The scrotal wall consists of skin, dartos muscle, and several fascial layers.^[1]

Examination of the scrotum is best performed with the patient standing. A warm room and warm examining hands are encouraged because cold temperatures cause contraction of the dartos and cremaster muscles and elevation of the testis toward the external inguinal ring. The left testis usually lies lower than the right, and the scrotal sac is hypoplastic when its gonad is absent. Palpation is carried out by systemically examining the testis, epididymis, and cord for size, consistency, and other factors (Fig. 155-1). The epididymis is adherent to the posterolateral aspect of the testis, and the spermatic cord is palpable in the upper portion of the scrotum. Fig. 155-2 illustrates common epididymal abnormalities.

Figure 155-1 Lateral view of the right testis, epididymis, and vas deferens. (From Krane RJ, Siroky MB, Fitzpatrick JM, editors: Clinical urology, Philadelphia, 1994, JB Lippincott.)

Figure 155-2 Various abnormalities of the epididymis, including spermatocele, epididymal tumor, and epididymitis. (From Krane RJ, Siroky MB, Fitzpatrick JM, editors: Clinical urology, Philadelphia, 1994, JB Lippincott.)

Scrotal masses should be evaluated by careful palpation (Box 155-1 and Fig. 155-3). The exact nature and location of the mass should be noted. Is it hard and firm or cystic and fluid filled? Does it arise from the testis or the other intrascrotal structures? Does it change with straining or Valsalva's maneuver? When transilluminated, fluid-filled structures (e.g., hydroceles) radiate a reddish glow. Tables 155-1 and 155-2 outline the physical signs and symptoms of mass lesions of the scrotum and the common causes of scrotal swelling.

Figure 155-3 Intratesticular abnormalities, including testis tumor, fibrous plaque, and cyst of the tunica albuginea. (From Krane RJ, Siroky MB, Fitzpatrick JM, editors: Clinical urology, Philadelphia, 1994, JB Lippincott.)

Table 155-1 Common Intrascrotal Conditions

Table 155-2 Causes of Scrotal Swelling

[edit] INFLAMMATORY DISEASES

Fungal skin diseases are treated with topical or systemic fungicides and furuncles. Minor lacerations of the scrotum are treated as they would be elsewhere in the body.

Purulent fistulous drainage in the scrotum requires prompt management. Causes include urethral stricture with formation of urethrocutaneous fistulas, tuberculosis, or syphilis of the testis or epididymitis with abscess formation and fistulization. Pus should be examined and cultured for acid-fast bacilli and spirochetes. Scrotal fistulas require surgical drainage. Fistulas from inflammatory bowel diseases (e.g., Crohn's disease) may occur in the perineoscrotal area.

[edit] Fournier's Gangrene

Fournier's gangrene, an uncommon but potentially life-threatening infection of the scrotal, perineal, and inguinal areas, can be a urologic emergency. It causes tissue and fascial necrosis (necrotizing fasciitis) and is usually secondary to perirectal, ischiorectal, or periurethral infection. Initially, erythema and edema of the scrotum and a small patch of dry gangrene develop. Gangrene may then extend rapidly along fascial planes into the lower abdominal wall, especially in diabetic and immunocompromised patients. A complete blood count, platelet count, and Gram's stain with cultures (aerobic and anaerobic) of the gangrenous area are performed.

Prompt treatment of suspected Fournier's gangrene is mandatory because patients require hospitalization, intravenous (IV) antibiotic therapy, close monitoring and observation, and early, aggressive, urgent surgical debridement. Combination antibiotic therapy (high-dose penicillin, gentamicin, and clindamycin) is indicated until the causative organism or organisms are identified. Fournier's gangrene is associated with significant mortality in elderly, diabetic, or immunocompromised patients.

[edit] Epididymitis

Epididymitis, a common condition in younger men, is usually unilateral and is associated with “scrotal heaviness.” Some men have urethral discharge or symptoms of urinary tract infection, and others develop pyrexia, chills, and malaise. A severely inflamed and indurated epididymis may be indistinguishable from the testis, and this can lead to difficulty in differentiating epididymitis from testicular torsion and testicular tumor.

Epididymitis is caused by infection from bladder urine, prostate infection, or an ascending urethral infection that spreads via the ejaculatory duct into the epididymis. In men under 35 years of age, epididymitis is usually chlamydial in origin and is associated with symptoms of urethritis (e.g., urethral discharge). In men over 35 years of age, infection is usually due to coliform organisms.^[2] Signs and symptoms of cystitis, prostatitis, or prostatism are common. “Sterile” epididymitis associated with vigorous physical activity can be caused by vasal reflux of sterile urine and chemical inflammation of the epididymis. In boys, epididymitis may be the presenting feature of congenital urologic abnormalities (e.g., ectopic ureter, posterior urethral valves). Evaluation with IV urography, cystourethroscopy, and voiding cystourethrography is indicated in boys with epididymitis. Careful clinical and radiologic evaluation may be needed to distinguish epididymitis from testicular torsion.

The epididymis lies posterior to the testis, and this demarcation is preserved in all but the most severe cases of epididymitis. A “reactive” hydrocele may make palpation of the intrascrotal structures difficult. Transillumination usually confirms the presence of hydroceles, although ultrasonography is more accurate and may be needed for confirmation. When in doubt, surgical exploration is required to exclude torsion and should be done within 4 to 6 hours to preserve testicular viability.

Epididymitis from syphilis, gonorrhea, or tuberculosis is uncommon (Box 155-2). “Chronic” epididymitis may follow acute epididymitis. However, this diagnosis most frequently represents mild, chronic, nonspecific (i.e., noninfectious) epididymitis associated with dull scrotal ache and mild induration of the epididymis. Tuberculosis epididymitis has reemerged as a health care problem in the increasing number of immunosuppressed men with acquired immunodeficiency syndrome.

Epididymitis is usually treated on an outpatient basis with scrotal elevation, bed rest, and appropriate antibiotics. Patients with leukocytosis and high fevers may require hospitalization for parenteral antibiotics and vigorous supportive treatment. Nonsteroidal antiinflammatory drugs (e.g., ibuprofen) and antipyretics (e.g., acetaminophen, aspirin) are usually prescribed. Severe pain may necessitate spermatic cord anesthetic block.

In younger men, epididymitis is usually caused by Neisseria gonorrhoeae or Chlamydia trachomatis. A Gram's stain of the urethral discharge may reveal the characteristic gram-negative intracellular diplococci of gonococcal urethritis. Gonococcal epididymitis is much less common, however, than chlamydial epididymitis. Nongonococcal urethritis is usually treated with doxycycline (Vibramycin), 100 mg orally twice a day, or minocycline (Minocin), 100 mg twice a day for 10 to 14 days. In older men with pyuria and bacteriuria, third-generation quinolones such as ciprofloxacin (Cipro), 500 mg twice a day, or trimethoprim-sulfamethoxazole (Septra, Bactrim), one double-strength tablet twice a day for 10 days, are recommended. The newer fluoroquinolones (e.g., ofloxacin, levofloxacin) also merit consideration. Antimicrobial therapy should be continued for 4 weeks if bacterial prostatitis is suspected. Hospitalized patients require parenteral antibiotic therapy (e.g., an aminoglycoside or a cephalosporin) before they are switched to oral antibiotics. The fluoroquinolones can obviate the need for parenteral antibiotics because of their broad-spectrum activity and the high tissue levels achieved after oral administration (see Box 155-2).

Most patients feel better within 48 hours, but swelling and discomfort may persist for weeks or months. Persistent fever despite suitable antimicrobial therapy suggests abscess formation and is an indication for ultrasound evaluation. Surgical drainage, epididymal orchiectomy, or both, treatments are required for abscesses. Epididymitis can be complicated by testicular necrosis, testicular atrophy, or infertility. Chronic inflammation and fibrosis can block the ductal cord structures and impair sperm production, especially in severe bilateral epididymitis. Swelling and edema may compromise blood flow and cause testicular atrophy. A urologist should be consulted when there is concern regarding the diagnosis of testicular torsion vs. epididymitis or testicular cancer (Box 155-3).

[edit] BENIGN SCROTAL MASSES

Most scrotal masses are benign and due to inflammation, trauma, and congenital defects. Testicular tumors, however, must be included in the differential diagnosis of all scrotal masses.^[3]

[edit] Hydrocele and Spermatocele

Hydroceles and spermatoceles are common paratesticular lesions caused by fluid accumulation. A hydrocele is an abnormal collection of fluid between the tunical layers and is congenital or acquired. Congenital or pediatric hydroceles are found in approximately 10% of boys and result from nonclosure of the processus vaginalis during embryologic testicular descent. Congenital hydroceles vary in size during the day, becoming larger with fluid accumulation, and most resolve by 1 year of age. Surgical treatment is indicated for persistent hydroceles beyond this age. Acquired “reactive” hydroceles are secondary to intrascrotal infection, regional or systemic disease, inguinal or scrotal surgery, trauma, or neoplasm.

Adult hydroceles are usually asymptomatic and enlarge slowly or remain unchanged. Occasionally, a pulling or dragging sensation is associated with large hydroceles. The hydrocele is a nontender, cystic, transilluminating mass that lies anterior to the testis. If the underlying testis cannot be palpated because of the size of the fluid-filled mass, a scrotal ultrasound should be performed to exclude a testicular tumor.

A spermatocele is a cystic dilation of the epididymis that frequently involves the upper pole or epididymal head. It is usually small and painless and may follow an episode of epididymitis. Physical examination reveals a transilluminating cystic mass located above and separate from the testis. The cyst fluid contains dead spermatozoa in retention cysts. Ultrasonography easily differentiates spermatoceles from hydroceles and other testicular lesions.

Surgical treatment of hydroceles and spermatoceles is reserved for men with pain and discomfort or lesions that are cosmetically embarrassing. Aspiration and sclerotherapy are useful nonsurgical treatment modalities in small to moderate- sized lesions. Sclerotherapy is successful in 80% to 90% of patients, but multiple treatments may be necessary. Surgery is indicated for large hydroceles and spermatoceles, and a variety of techniques (e.g., excision, exertion) are available.

[edit] Varicocele

A varicocele can be easily recognized by the presence of spermatic cord or scrotal enlargement caused by dilation of the pampiniform venous plexus. The exact etiology of varicoceles is unknown, although valvular incompetence is common. Clinically obvious varicoceles present as a “bag of worms” in the spermatic cord and are more prominent when the patient stands. The incidence of varicoceles in healthy males is approximately 20%, and the left side is involved in more than 80% (Box 155-4).

Varicoceles occasionally cause scrotal pain and heaviness, especially if large. Varicoceles are the most common surgically correctable cause of male infertility, occurring in 30% of infertile men. Infertile men with varicoceles are oligospermic (have low sperm counts) with “a stress pattern” of reduced sperm motility and abnormal morphology.

Ipsilateral testicular atrophy may occur with varicoceles. Size is best assessed by ultrasound or calibrated orchidometers. The exact role of the varicocele in male subfertility has not been clearly ascertained, although an elevated scrotal temperature and reflux of adrenal metabolites (e.g., steroids) may contribute to the pathogenesis.

Asymptomatic men without a history of infertility should be observed. For patients with concomitant testicular atrophy (especially young boys), infertility, or both conditions, various treatment modalities are available.^[4] Several techniques, including retroperitoneal, inguinal, laparoscopic, or scrotal, may be used for surgical ligation of the spermatic vein. Nonsurgical treatment can be achieved by percutaneous spermatic vein embolization. However, a skilled interventional radiologist is needed, and a small radiation risk exists. Improvement in semen parameters (improved motility and number of sperm) occurs in 50% to 60% of patients, with successful pregnancy in 30% to 40% of patients' significant others.

[edit] BENIGN TUMORS OF THE TESTIS AND EPIDIDYMIS

Benign tumors of the testis are rare (Box 155-5) and can be diagnosed by ultrasound evaluation. Scrotal exploration, when indicated, ascertains the diagnosis. Paratesticular masses account for 7% of all intrascrotal tumors, with 60% to 70% originating in the spermatic cord and the remainder in the epididymis. The most common paratesticular mass is a benign cord lipoma, which accounts for approximately 25% of all cord tumors. Cord lipomas arise from preperitoneal fat remnants in the spermatic cord.

Intratesticular epidermoid cysts can be confused with malignant testicular lesions on physical examination. These rare lesions can be confidently diagnosed by scrotal ultrasound. A common finding on physical examination or testicular self-examination (TSE) is tunical cysts or calcifications on the surface of the tunica albuginea of the testis. These lesions are nonmalignant, and their etiology is uncertain. Ultrasound is diagnostic, and surgical exploration can usually be avoided.

[edit] MALIGNANT TUMORS OF THE TESTIS

Testicular cancer is the most frequent cancer in young men (15 to 35 years of age) and is rare in African-Americans. Cryptorchidism is a well-known risk factor even after orchiopexy. Testicular tumors usually present as painless lumps, but some men (20% to 25%) develop scrotal pain as a result of bleeding caused by rapid tumor growth and necrosis. Most tumors are discovered as hard testicular lumps during TSE. Gynecomastia is an unusual manifestation associated with chorionic gonadotropin or estrogen production. Occasionally, patients may present with metastatic disease (e.g., retroperitoneal, mediastinal, and supraclavicular lymphadenectomy or pulmonary metastases). Unfortunately, a painful testicular tumor can be misdiagnosed as epididymitis, leading to delayed diagnosis with the risk of advanced disease and a poor prognosis.

[edit] Diagnosis and Staging

Physical examination usually reveals a hard testicular lump. “Reactive” hydrocele may make palpation of the lesion difficult. The testicular tumor may be small (e.g., a choriocarcinoma) and not easily palpable through the thick, overlying tunica albuginea. Scrotal ultrasound is an excellent imaging modality for the assessment of testicular masses. Testicular microcalcifications are associated with a high propensity for developing seminomas, and patients should be screened by ultrasound every 6 months. Testicular cancers are typically nonhomogenous with hypoechoic areas on ultrasound (Fig. 155-4).

Figure 155-4 A, Microcalcifications visualized on an ultrasound of the testis. This condition is often a premalignant sign of seminoma and requires ultrasound screening at 6-month to 1-year intervals. B, Seminoma of testis noted on ultrasound and by color Doppler.

Lymphatic spread of testicular tumors is predictable. The “first-echelon” nodes are the paraaortic and paracaval nodes at the level of the renal vessels. The primary drainage area of right-sided tumors is the interaortocaval nodes, and left-sided cancers spread initially to the left paraaortic nodal area. This is explained by the embryologic descent of the testis and vascular and lymphatic vessels from the abdomen into the scrotum. Avoidance of scrotal skin violation is mandatory when treating testicular tumors. The scrotal skin lymphatics drain into the inguinal nodes, and violation leads to nonretroperitoneal lymphatic tumor dissemination. Transscrotal needle biopsy or orchiectomy is therefore contraindicated. Suspected testicular tumors should be explored via an inguinal incision with early control of the spermatic cord to prevent vascular or lymphatic dissemination of tumor cells.

Tumor markers are helpful in the diagnosis, staging, and management of malignant testicular tumors. α-Fetoprotein (α-FP) and the β-subunit of human chorionic gonadotropin (β-hCG) are the clinically useful markers. α-FP is often associated with embryonal carcinoma, whereas β-hCG elevations occur with choriocarcinoma. However, many testicular cancers are of mixed germ cell origin (e.g., seminoma, embryonal cell carcinoma, choriocarcinoma, teratoma), and tumor marker elevation is variable. Persistent tumor marker elevation after “radical” inguinal orchiectomy suggests the presence of metastatic disease. However, there is a 25% false-negative marker elevation rate. Thus serum tumor markers are not completely reliable for staging, especially when their levels are normal.

Computed tomography (CT) scanning and chest x-ray studies are used for staging (Box 155-6). A false-negative rate of 20% to 25% occurs in the presence of nonenlarged (smaller than 1.5 cm) but microscopically involved retroperitoneal nodes. Stage A disease is confined to the testis, whereas stage B disease is associated with retroperitoneal lymphatic metastases below the diaphragm but an absence of visceral metastases. Stage B is subdivided into B1, B2, and B3 stages according to tumor extent (B1 smaller than 5 cm, B2 larger than 5 cm, B3 smaller than 10 cm). Stage C disease indicates metastases (usually visceral) beyond the retroperitoneum.

[edit] Histology

Most testicular tumors are of germ cell origin—seminoma, embryonal cell carcinoma, teratoma, and choriocarcinoma— but 40% are “mixed,” containing more than one cell type. Choriocarcinoma is the most aggressive testicular cancer, with a propensity for rapid growth and early hematogenous spread.

Non–germ cell testicular tumors are rare and usually benign. Interstitial cell (Leydig's cell) tumors in prepubertal male patients may secrete androgens and estrogens and cause gynecomastia. Sertoli's cell tumors are extremely rare. The testis can be the site of metastatic spread from other primary tumors (e.g., lymphoma, leukemia, and lung, prostate, and gastrointestinal cancers).

[edit] Treatment

Whenever a testicular tumor is suspected on physical examination, the next step is confirmation of the diagnosis by orchiectomy. Inguinal exploration with ligation of the spermatic cord at the level of the internal inguinal ring is recommended. Serum tumor markers (α-FP, β-hCG) and a chest x-ray study are obtained before surgical exploration.

Further treatment is based on histologic assessment of the tumor and staging (Box 155-7). Microscopic examination identifies tumor type (pure or mixed), tumor extent (involvement of epididymis or spermatic cord), and vascular space (lymphatic and venous) tumor invasion (Box 155-8).

[edit] Nonseminomatous Germ Cell Tumors

Stage A nonseminomatous germ cell tumors are treated by modified, unilateral lymphadenectomy, which spares the sympathetic chain and preserves ejaculation (see Box 155-7). If the nodes are negative, further therapy is unnecessary, and cure rates approach 95%. Recent improvements in chemotherapy for metastatic disease have led to the emergence of surveillance protocols for selected patients with stage A disease (i.e., those with negative tumor markers, normal abdominal CT scans, and lack of vascular space invasion). However, strict selection and meticulous clinical and radiologic follow-up are needed. The overall tumor relapse rate with surveillance protocols is 20% to 30%.

Microscopic lymph node involvement or tumor marker elevation after lymphadenectomy are indications for multidrug combination chemotherapy with vinblastine, bleomycin, and cisplatin. Cure rates for stage B and C testicular tumors exceed those for any other human solid-organ neoplasm. Five-year survival rates in excess of 90% are now achievable. The keys to the optimistic outlook in nonseminomatous testis cancer are early diagnosis, prompt inguinal orchiectomy, modified retroperitoneal lymphadenectomy, and combination chemotherapy.

[edit] Seminomas

The usual treatment for testicular seminoma is “radical” inguinal orchiectomy and external beam radiation to the retroperitoneum (see Box 155-6). Pure seminomas are exquisitely radiosensitive. Patients with stage A seminoma undergo postorchiectomy radiation to the paraaortic and ipsilateral iliac nodes. Patients with stage B disease receive additional “boosts” to involved areas and prophylactic radiation to the mediastinal and supraclavicular nodes. Radiation therapy is less effective in “bulky” stage B or C disease. The success of combination chemotherapy for nonseminomatous germ cell tumors has led to its use for the treatment of “bulky” seminomas (i.e., stages B and C).

[edit] ACUTE SCROTUM

Testicular pain should always be considered an emergency.^[5] Evaluation should not be delayed if testicular damage from torsion of the spermatic cord and subsequent ischemia are to be avoided.^[6]Box 155-9 outlines the differential diagnosis of scrotal pain. Differentiation between testicular torsion and epididymitis is usually possible on the basis of the presentation and physical findings (see Box 155-1).

Testicular torsion is a surgical emergency (Box 155-10). Testicular loss can be avoided only by prompt diagnosis and early treatment (either manual or surgical detorsion). Torsion, a disease mainly of adolescents and young adults, can be confused with epididymitis, especially in postpubertal boys. A teenager with a sudden onset of scrotal pain and a normal urinalysis most likely has testicular torsion. Unrelieved torsion (longer than 6 hours) leads to testicular ischemia and atrophy, and early treatment is therefore imperative.^[7] Some 90% of testicular torsions occur in patients younger than 30 years of age.

A delay in the diagnosis of testicular torsion beyond 12 hours causes progressive testicular ischemia and irreversible damage. A technique for early manual detorsion of the spermatic cord is available. Under IV sedation, detorsion can be performed in the emergency department by rotating the testis toward the respective thigh for approximately 1½ turns. Relief of pain can be dramatic, and return of testicular blood flow can be documented by color Doppler ultrasound.

Testicular torsion can be confirmed by Doppler ultrasound (greater than 90% sensitive, 70% specific) and nuclear scanning (greater than 90% sensitive, 80% specific). How ever, if any doubt exists, scrotal exploration and detorsion should be performed. A management algorithm for the acute scrotum is outlined in Fig. 155-5. The common anatomic defect in testes that undergo torsion is high “investment” (or attachment) of the tunica vaginalis—the so-called bell-clapper deformity (Fig. 155-6). This inherited defect is usually bilateral, and detorsion should always be followed by testicular fixation (orchiopexy) of the affected gonad as well as its contralateral mate.

Figure 155-5 Acute scrotum: management algorithm.

Figure 155-6 Attachment of the testicular mesentery to the testis. A, Normal peritoneal disposition around the testis. B, Capacious tunica vaginalis surrounding the cord; the testis lies horizontally. C, Inverted testis, which most easily twists because of its narrow attachment, the bell-clapper deformity. (From Krane RJ, Siroky MB, Fitzpatrick JM, editors: Clinical urology, Philadelphia, 1994, JB Lippincott.) ABC

If, after intraoperative detorsion, the testis remains dusky and cyanotic, irreversible ischemia is probable, and an orchiectomy is indicated. Torsion of an appendix testis or an acutely incarcerated scrotal hernia may mimic the symptoms of testicular torsion. Torsion of the appendix testis can be diagnosed by the presence of localized pain in the upper pole of the testis and the presence of a “blue dot” on transillumination; this blue dot is caused by the ischemic appendage seen through the skin. An incarcerated hernia causes thickening of the spermatic cord and is associated with peritoneal signs of intestinal obstruction (Box 155-11).

[edit] SCROTAL TRAUMA

Penetrating gunshot or knife wounds can involve the scrotum. Emergency surgical debridement and exploration are required for hemostasis and testicular repair, if indicated. Severe blunt injuries of the scrotum may cause testicular dislocation or rupture. Palpation and examination of the scrotum are extremely painful, and ultrasound imaging can be misleading. Surgical exploration with repair or orchiectomy is indicated.

Minor trauma can cause scrotal pain or hematoma formation. Testicular tumors are more likely to bleed after minor trauma compared with normal testes. Occasionally, a testicular tumor may present in this manner, with the degree of trauma not being commensurate with the degree of pain or hematoma formation.

[edit] CRYPTORCHIDISM

The absence of the testis in the scrotum warrants a careful search for the missing gonad by palpation of the upper scrotum and groin. Testicular maldescent or cryptorchidism is common, affecting 0.8% of all males. Surgical repositioning of a cryptorchid testis is recommended in the pediatric age group. However, patients who have not undergone such a procedure and still have a malpositioned testis should undergo removal of that gonad because of the high incidence of testicular tumors (usually seminomas) in cryptorchid testes (8% to 10%). If no testis is found on physical examination, surgical exploration is indicated. Transabdominal laparoscopic evaluation of patients with nonpalpable testes is now the recommended modality for localization of cryptorchid testis. Testicular absence can be confirmed and first stage orchidopexy performed if intraabdominal testes are discovered.

Atrophy of the testis may result from neonatal torsion or prior injury. Surgical removal of the testis is not mandatory. However, associated masses or abnormalities should be excluded by scrotal ultrasound. A number of patients have compensatory, contralateral testicular hypertrophy.

[edit] REFERENCES