The risk of a germ cell malignancy (Chapter 497) developing in an undescended testis is 2-4 times higher than that in the general population and is approximately 1/80 with a unilateral undescended testis and 1/40-50 for bilateral undescended testes. Testicular tumors are less common if the orchiopexy is performed before 10 yr of age, but they still occur, and adolescents should be instructed in testicular self-examination. The peak age for developing a testis tumor is 15-45 yr. The most common tumor developing in an undescended testis in an adolescent or adult is a seminoma (65%); after orchiopexy, seminomas represent only 30% of testis tumors. Orchiopexy seems to reduce the risk of seminoma. Whether early orchiopexy reduces the risk of developing cancer of the testis is controversial, but it is uncommon for testis tumors to occur after orchiopexy performed before the age of 2 yr. The contralateral scrotal testis is not at increased risk for malignancy.

An indirect inguinal hernia usually accompanies a congenital undescended testis but rarely is symptomatic. Torsion and infarction of the cryptorchid testis also are uncommon but can occur because of excessive mobility of undescended testes. Consequently, inguinal pain and/or swelling in a boy with an undescended testis should raise the suspicion of an incarcerated hernia or testicular torsion of the undescended testis.

“Acquired” or ascending undescended testes occurs when a boy has a descended testis at birth, but during childhood, usually between 4-10 yr of age, the testis does not remain in the scrotum. Such boys often have a history of a retractile testis. With testicular ascent, on physical examination the testis often can be manipulated into the scrotum, but there is obvious tension on the spermatic cord. This condition is speculated to result from incomplete involution of the processus vaginalis, restricting spermatic cord growth, resulting in the testis gradually moving out of its scrotal position.

Retractile testes may be misdiagnosed as undescended testes. Boys >1 yr often have a brisk cremasteric reflex, and if the child is anxious or ticklish during scrotal examination, the testis may be difficult to manipulate into the scrotum. Boys should be examined with their legs in a relaxed frog-leg position, and if the testis can be manipulated into the scrotum comfortably, it is probably retractile. It should be monitored every 6-12 mo with follow-up physical examinations, because it can become an acquired undescended testis. Overall, as many as one third of boys with a retractile testis develop an acquired undescended testis, and boys <7 yr of age at diagnosis of a retractile testis are at greatest risk. Most think that boys with a retractile testis are not at increased risk for infertility or malignancy.

A nonpalpable testis accounts for 10% of undescended testes. Of these, 50% are viable testes in the abdomen or high in the inguinal canal, and 50% are atrophic or absent, almost always in the scrotum, secondary to spermatic cord torsion in utero (vanishing testis). If the nonpalpable testis is abdominal, it will not descend after 3 mo of age. Although sonography often is performed to try to identify whether the testis is present, it rarely changes clinical management, because the abdominal testis and atrophic testis are not identified on sonography. CT scanning is relatively accurate in demonstrating the presence of the testis. MRI is even more accurate, but the disadvantage is that general anesthesia is necessary in most young children. Because imaging has not been proved to be 100% reliable in demonstrating whether the testis is present or absent, its routine use is discouraged.

On physical examination of the scrotum, the child should be entirely undressed, to help him relax. If the testis is nonpalpable, the “soap test” often is useful; soap is applied to the inguinal canal and the examiner’s hand, significantly reducing friction and facilitating identification of an inguinal testis. In addition, pulling on the scrotum might make an inguinal testis palpable. One soft sign that a testis is absent is contralateral testicular hypertrophy, but this finding is not 100% diagnostic.

Treatment

The congenital undescended testis should be treated surgically no later than 9-15 mo of age. With anesthesia by a pediatric anesthesiologist, surgical correction at 6 mo is appropriate, because spontaneous descent of the testis will not occur after 4 mo of age. Most testes can be brought down to the scrotum with an orchiopexy, which involves an inguinal incision, mobilization of the testis and spermatic cord, and correction of the indirect inguinal hernia. The procedure is typically performed on an outpatient basis and has a success rate of 98%. In some boys with a testis that is close to the scrotum, a prescrotal orchiopexy can be performed. In this procedure, the entire operation is performed through an incision along the edge of the scrotum. Often the associated inguinal hernia also can be corrected with this incision. Advantages of this approach over the inguinal approach include shorter operative time and less postoperative discomfort.

Hormonal treatment is used infrequently. The theory is that because testicular descent is under androgenic regulation, human chorionic gonadotropin (HCG, which stimulates Leydig cell production of testosterone) or luteinizing hormone–releasing hormone (LHRH) may stimulate testicular descent. Although hormonal treatment has been used in Europe, randomized controlled trials have not shown either of these hormonal preparations to be effective in stimulating testicular descent. There has been some preliminary evidence that an LHRH analog, buserelin, may be helpful in increasing germ cell number and normalizing testicular histologic features.

In boys with a nonpalpable testis, diagnostic laparoscopy is performed in most centers. This procedure allows safe and rapid assessment of whether the testis is intra-abdominal. In most cases, orchiopexy of the intra-abdominal testis located immediately inside the internal inguinal ring is successful, but orchiectomy should be considered in more difficult cases or when the testis appears to be atrophic. A 2-stage orchiopexy sometimes is needed in boys with a high abdominal testis. Boys with abdominal testes are managed with laparoscopic techniques at many institutions. Testicular prostheses are available for older children and adolescents when the absence of the gonad in the scrotum might have an undesirable psychologic effect. The U.S. Food and Drug Administration (FDA) has approved a saline testicular implant. Solid silicone “carving block” implants also are used (Fig. 539-1). Placement of testicular prostheses early in childhood is recommended for boys with anorchia (absence of both testes).

Figure 539-1 A, Adolescent with solitary left testis. B, Appearance following implantation of right testicular prosthesis.

Scrotal Swelling

Scrotal swelling may be acute or chronic and painful or painless. Abrupt onset of painful scrotal swelling necessitates prompt evaluation because some conditions, such as testicular torsion and incarcerated inguinal hernia, require emergency surgical management. The differential diagnosis is shown in Tables 539-1 and 539-2.

Clinical Manifestations

A detailed history is helpful in determining the cause of the swelling and includes onset of pain—with testicular torsion, the pain often is sudden in onset and may be associated with exercise or minor genital trauma; duration of pain; (3) radiation of pain—inguinal discomfort is common with testicular torsion, inguinal hernia, or epididymitis, and associated flank pain can occur with passage of a ureteral calculus; previous episodes of similar pain, which are common in boys with intermittent testicular torsion or inguinal hernia; nausea and vomiting, which are associated with testicular torsion and inguinal hernia; and irritative urinary symptoms, such as dysuria, urgency, and frequency, which indicate a urinary tract infection that can cause epididymitis. Boys with lower urinary tract pathology such as urethral stricture or neuropathic bladder may be prone to epididymitis.

Physical examination may be difficult in boys with a painful scrotum. Some have advocated performing a spermatic cord block or administering intravenous analgesia to facilitate the examination, but such measures usually are unnecessary. Scrotal wall erythema is common in testicular torsion, epididymitis, torsion of the appendix testis, and an incarcerated hernia. In boys with a normal cremasteric reflex, testicular torsion is unlikely. Absence of a cremasteric reflex is nondiagnostic.

Laboratory Findings and Diagnosis

Pertinent laboratory studies include a urinalysis and culture. A positive urinalysis suggests epididymitis. Serum studies are not helpful in establishing a diagnosis, unless a testicular malignancy is suspected. After initial evaluation, imaging studies may be helpful in establishing the diagnosis, because they assess whether testicular blood flow is normal, reduced, or increased. Imaging studies include color Doppler ultrasonography and a radionuclide testicular flow scan, although the latter is performed infrequently. If a hydrocele is present and the testis is nonpalpable, or if an abnormality of the testis is found, sonography is indicated. Imaging studies are not 100% accurate; they should not be used to decide whether a boy with testicular pain should be referred for urologic evaluation.

Color Doppler ultrasonography is performed most commonly and allows assessment of testicular blood flow and testicular morphologic features. Accuracy is 95% if the ultrasonographer is experienced. A false-negative study (demonstrates normal testicular blood flow) can occur in a boy with testicular torsion if the degree of torsion is <360 degrees and the duration of torsion is short, because there may be continued testicular perfusion. In prepubertal boys, blood flow may be difficult to demonstrate in 15% of normal testes.

Testicular (Spermatic Cord) Torsion

Etiology

Testicular torsion requires prompt diagnosis and treatment to salvage the testis. Torsion is the most common cause of testicular pain in boys 12 yr and older and is uncommon before age 10 yr. It is caused by inadequate fixation of the testis within the scrotum, resulting from a redundant tunica vaginalis, allowing excessive mobility of the testis. The abnormal attachment is termed a bell clapper deformity and often is bilateral. Shortly after torsion occurs, venous congestion begins and subsequently arterial flow is interrupted. The likelihood of testis survival depends on the duration and severity of torsion. Within 4-6 hr of absent blood flow to the testis, irreversible loss of spermatogenesis can occur.

Diagnosis

Testicular torsion produces acute pain and swelling of the scrotum. On examination, the scrotum is swollen, and the testis is exquisitely tender and often difficult to examine. The cremasteric reflex nearly always is absent. The condition can be differentiated from an incarcerated hernia because swelling in the inguinal area often is absent with torsion. If the pain duration is <4-6 hr, manual detorsion may be attempted. In 65% of cases the torsed testis rotates inward, so detorsion should be attempted in the opposite direction (e.g., the left testis is rotated clockwise). Successful manual detorsion results in dramatic pain relief.

Some adolescents experience intermittent testicular torsion. These boys report episodes of severe unilateral testicular pain that resolves spontaneously after 30-60 min. Treatment is elective bilateral scrotal orchiopexy (see later).

Treatment

Treatment is prompt surgical exploration and detorsion. If the testis is explored within 6 hr of torsion, up to 90% of the gonads survive. Testicular salvage decreases rapidly with a delay of >6 hr. If the degree of torsion is 360 degrees or less, the testis might have sufficient arterial flow to allow the gonad to survive, even after 24-48 hr. Following detorsion the testis is fixed in the scrotum with nonabsorbable sutures, termed scrotal orchiopexy, to prevent torsion in the future. The contralateral testis also should be fixed in the scrotum because the predisposing anatomic condition often is bilateral. If the testis appears nonviable, orchiectomy is performed (Fig. 539-2A). Some adolescents do not undergo prompt evaluation and treatment and present with “late phase testicular torsion,” in which the spermatic cord contracts and the testis is high in the scrotum and nontender (see Fig. 539-2B). Fertility is reduced in men with a history of spermatic cord torsion in adolescence, irrespective of whether detorsion or orchiectomy is performed.

Figure 539-2 A, Left testicular torsion in adolescent with acute scrotum; the testis is necrotic. B, “Late phase torsion” in an adolescent with severe testicular pain 1 mo previously. Note absence of inflammation and high position of testis in scrotum.

Spermatic cord torsion also can occur in the fetus or neonate. This condition results from incomplete attachment of the tunica vaginalis to the scrotal wall and is “extravaginal.” When torsion occurs in utero, the baby usually is born with a large, firm, nontender testis. Usually the ipsilateral hemiscrotum is ecchymotic (Fig. 539-3). In these cases, the testis rarely is viable because torsion was a remote event. However, the contralateral testis is at increased risk for torsion until 1 mo beyond term. Most pediatric urologists recommend exploration to establish the diagnosis, remove the necrotic testis, or rarely salvage a viable testis, and anchor the contralateral testis.

Figure 539-3 A and B, Right testicular torsion in a newborn. The right hemiscrotum is darker, and the testis was indurated and enlarged.

Torsion of the Appendix Testis

Torsion of the appendix testis is the most common cause of testicular pain in boys 2-10 yr but is rare in adolescents. The appendix testis is a stalk-like structure that is a vestigial embryonic remnant of the müllerian (paramesonephric) ductal system that is attached to the upper pole of the testis. When it undergoes torsion, progressive inflammation and swelling of the testis and epididymis occurs, resulting in testicular pain and scrotal erythema. The onset of pain usually is gradual. Palpation of the testis usually reveals a 3- to 5-mm tender indurated mass on the upper pole (Fig. 539-4A). In some cases, the appendage that has undergone torsion may be visible through the scrotal skin, in the “blue dot” sign. In some boys, distinguishing torsion of the appendix from testicular torsion is difficult. In such cases, a testicular flow scan or color Doppler ultrasonography is useful because testicular blood flow should be normal or increased. In such cases, the radiologist often recognizes epididymal enlargement and makes the diagnosis of epididymitis, reflecting the inflammatory reaction (see Fig. 539-4B).

Figure 539-4 A, Torsion of the appendix testis; the appendix testis is necrotic (arrow). B, Color Doppler scrotal sonogram showing hyperemia to the testis and absent flow to the appendix testis (right side). Symptoms resolved with medical therapy.

The natural history of torsion of the appendix testis is for the inflammation to resolve in 3-10 days. Nonoperative treatment is recommended, including bed rest and analgesia with nonsteroidal anti-inflammatory medication for 5 days. If the diagnosis is uncertain, scrotal exploration is recommended.

Epididymitis

Acute inflammation of the epididymis is an ascending retrograde infection from the urethra, through the vas into the epididymis. This condition causes acute scrotal pain, erythema, and swelling. It is rare before puberty and should raise the question of a congenital abnormality of the wolffian duct, such as an ectopic ureter entering the vas. In younger boys, the responsible organism often is Escherichia coli (Chapter 192). After puberty, bacterial epididymitis becomes progressively more common and is the principal cause of acute painful scrotal swelling in young sexually active men. Urinalysis usually reveals pyuria. Epididymitis can be infectious (usually gonococcus or chlamydia; Chapters 185 and 218), but often the organism remains undetermined. Additional etiologies include familial Mediterranean fever, enterovirus, and adenoviruses. Treatment consists of bed rest and antibiotics (Chapter 192). Differentiation from torsion can be difficult, and surgical exploration usually is required in children.

Henoch-Schönlein purpura (HSP; Chapter 478) is a systemic vasculitis that involves multiple organ systems and that can involve the kidney and spermatic cord. When the spermatic cord is involved, typically there is bilateral painful scrotal swelling with purpuric lesions involving the scrotum. Scrotal sonography should show normal testicular blood flow. Treatment is directed toward systemic treatment of the HSP.

Varicocele

A varicocele is a congenital condition in which there is abnormal dilation of the pampiniform plexus in the scrotum (Fig. 539-5). Dilation of the pampiniform venous plexus results from valvular incompetence of the internal spermatic vein. Approximately 15% of men have a varicocele; of these men, approximately 15% are subfertile. Varicocele is the most common (and virtually the only) surgically correctable cause of subfertility in men. A varicocele is found in 5-15% of adolescent boys, but it rarely is diagnosed in boys <10 yr old, because the varicocele becomes distended only after the increased blood flow associated with puberty occurs. Varicoceles occur predominantly on the left side, are bilateral in 2% of cases, and rarely involve the right side only. A varicocele in a boy <10 yr or one on the right side might indicate an abdominal or retroperitoneal mass; an abdominal sonogram or CT scan should be performed in such cases.

Figure 539-5 Left varicocele in an adolescent boy.

A varicocele typically is a painless paratesticular mass, often described as a “bag of worms.” Occasionally patients describe a dull ache in the affected testis. Usually the varicocele is not apparent when the patient is supine because it is decompressed; in contrast, the varicocele becomes prominent when the patient is standing and enlarges with a Valsalva maneuver. Many pediatricians do not routinely screen adolescents for a varicocele. Varicoceles typically are graded from 1 to 3 with the boy standing: grade 1 is palpable only with Valsalva; grade 2 is palpable without Valsalva but is not visible on inspection; and grade 3 is visible with inspection. Boys with a grade 3 varicocele are at greatest risk for testicular growth arrest. Testicular size should be documented with calipers, an orchiometer, or scrotal sonography, because if the affected left testis is significantly smaller than the right testis, spermatogenesis probably has been adversely affected.

The goal of varicocelectomy is to maximize chances for fertility. Surgical treatment of varicoceles is indicated in boys with a significant disparity in testicular size or pain in the affected testis or if the contralateral testis is diseased or absent. Typically the involved testis enlarges and catches up with the normal testis over the following 1-2 yr. Varicocelectomy should also be considered in boys with a large grade 3 varicocele, even if there is not a disparity in testicular size. Surgical repair is accomplished with a variety of techniques by ligation of the veins of the pampiniform plexus through an inguinal or subinguinal incision (with or without an operating microscope) or by ligating the internal spermatic vein in the retroperitoneum. Laparoscopic repair is becoming more popular. The operation is performed on an ambulatory basis.

Spermatocele

A spermatocele is a cystic lesion that contains sperm and is attached to the upper pole of the sexually mature testis. Spermatoceles usually are painless and are incidental findings on physical examination. Enlargement of the spermatocele or pain is an indication for removal.

Hydrocele

Etiology

A hydrocele is an accumulation of fluid in the tunica vaginalis (Fig. 539-6). From 1-2% of neonates have a hydrocele. In most cases, the hydrocele is noncommunicating (the processus vaginalis was obliterated during development). In such cases, the hydrocele fluid disappears by 1 yr of age. If there is a persistently patent processus, the hydrocele persists and becomes progressively larger during the day and is small in the morning. A rare variant of a hydrocele is the abdominoscrotal hydrocele, in which there is a large, tense hydrocele that extends into the lower abdominal cavity. In some older boys, a noncommunicating hydrocele can result from an inflammatory condition within the scrotum, such as testicular torsion, torsion of the appendix testis, epididymitis, or testicular tumor. The long-term risk of a communicating hydrocele is the development of an inguinal hernia. Some older boys and adolescents also develop a hydrocele. In some cases hydrocele develop acutely after an episode of scrotal trauma or epididymo-orchitis, whereas others develop more insidiously.

Figure 539-6 Newborn with large right hydrocele.

Diagnosis

On examination, hydroceles are smooth and nontender. Transillumination of the scrotum confirms the fluid-filled nature of the mass. It is important to palpate the testis, because some young men develop a hydrocele in association with a testis tumor. If compression of the fluid-filled mass completely reduces the hydrocele, an inguinal hernia/hydrocele is the likely diagnosis.

Treatment

Most congenital hydroceles resolve by 12 mo of age following reabsorption of the hydrocele fluid. If the hydrocele is large and tense, however, early surgical correction should be considered, because it is difficult to verify that the child does not have a hernia, and large hydroceles rarely disappear spontaneously. Hydroceles persisting beyond 12-18 mo usually are communicating and should be repaired. Surgical correction is similar to a herniorrhaphy (Chapter 338). Through an inguinal incision, the spermatic cord is identified, the hydrocele fluid is drained, and a high ligation of the processus vaginalis is performed. If an older boy has a large hydrocele, often diagnostic laparoscopy can be performed to determine whether there is a patent processus vaginalis, and if the internal ring is closed, then the hydrocele may be corrected with a scrotal incision.

Inguinal Hernia

Inguinal hernia is discussed in Chapter 338.

Testicular Tumor

Testicular and paratesticular tumors can occur at any age, even in the newborn. Approximately 35% of prepubertal testis tumors are malignant; most commonly they are yolk sac tumors, although rhabdomyosarcoma and leukemia also can occur in this age group. In adolescents, 98% of painless solid testicular masses are malignant (Chapter 497). Most manifest as a painless, hard testicular mass that does not transilluminate. Scrotal ultrasonography should be performed to confirm the finding of a testicular mass and it can help to delineate the type of testis tumor. Serum tumor markers, including α-fetoprotein and β-HCG, should be drawn. Definitive therapy includes surgical exploration through an inguinal incision. In most cases, a radical orchiectomy, consisting of removal of the entire testis and spermatic cord, is performed. In a prepubertal boy, if the ultrasonographic study or surgical exploration suggests that the tumor is localized and benign, such as a teratoma or epidermoid cyst, testis-sparing surgery with removal only of the mass may be appropriate.

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