Approach

The mainstay of treatment for cryptorchidism is surgical placement of the testicle(s) into the dependent portion of the scrotum. The primary goals of surgery are to maintain hormonal and fertility function, as well as reduce malignancy potential. Other benefits include reducing testicular torsion and trauma risk.[55] The optimal timing for surgical therapy is debated, but data suggest that there may be better preservation of spermatogenesis and hormonal production with decreased risk of testicular cancer when performed early, ideally prior to 12 to 18 months of age.[1][78][79][80] Randomized studies demonstrated higher testicular volume by ultrasound and a greater number of germ cells in boys randomized to orchiopexy at 9 months compared with 3 years of age.[63][81] The major issues arising from treatment include surgery-related complications, most commonly anesthetic complications, re-ascent of the testicle, wound infection, hematoma, reactive hydrocele, and testicular atrophy. The use of hormonal therapy (human chorionic gonadotropin [hCG] or gonadotropin-releasing hormone [GnRH]) as first-line treatment for cryptorchidism is not frequently recommended because descent rates do not exceed those seen with placebo by >10%;[61][82][83] this remains a somewhat controversial approach.[84]

A variety of studies worldwide have demonstrated that the average age of orchiopexy continues to be older than 18 months, despite the recommendations of numerous professional organizations.[55][57][76][85][86][87][88] Possible etiologies include an underappreciated risk of testicular ascent, lack of provider knowledge, or poor access to care.[89][90]

Retractile testicle(s)

Annual follow-up exam is indicated. If cryptorchidism or testicular asymmetry is found at subsequent annual exam, further surgical intervention is required. Patients with ascending testes that no longer can be brought down into the dependent portion of the scrotum and/or testicular asymmetry should proceed to orchiopexy as outlined for patients with undescended testes. If a retractile testis is present in the setting of a contralateral undescended testis requiring surgery, it may be reasonable to discuss "proactively" performing bilateral orchiopexy procedures due to the risk of metachronous undescended testis.[91]

Prepubertal with undescended testicle(s): unilateral or bilateral palpable testicle(s)

A variety of professional organizations recommend orchiopexy as the treatment of choice for a palpable testicle(s) that has not descended into the dependent portion of the scrotum by 6 months of age.[55][76][57] The surgical approach is determined by surgeon preference, generally through an inguinal or scrotal incision, with minimal difference in outcomes.[92][93] Inguinal hernia repair to close the associated patent processus vaginalis is performed in the same setting. Randomized controlled studies demonstrated no difference in success rates or postoperative complications between patients randomized to single scrotal incision surgery or a traditional (2-incision) inguinal approach.[94][95] Operative time and hospital stay appear to be shorter with a single scrotal incision approach. This is a reasonable consideration for redo orchiopexy as well.[96]

Successful treatment (scrotal position without atrophy) depends upon testicular position, patient age, and surgical approach, but has been reported as 92% to 95% for testes located beyond the external inguinal ring.[97][98]

Adverse effects include surgery-related complications, most commonly anesthetic complications, re-ascent of the testicle, hematoma, wound infection, reactive hydrocele, and, rarely, testicular atrophy.

Prepubertal with undescended testicle(s): unilateral nonpalpable testicle

Exam under anesthesia to locate the nonpalpable testicle is indicated for these patients. The outcome of this exam determines further treatment.

If the testis is palpable on exam under anesthesia, the surgeon should proceed to orchiopexy.

If the testis is not palpable on exam under anesthesia, depending upon surgeon preference, an open or laparoscopic approach is used to identify the testis or testicular remnant.[77] Approximately 50% of nonpalpable testes are identified in the abdomen or inguinal region, and 50% are absent or small remnants, referred to as testicular nubbins.[99] In order to ensure no residual testicular tissue is left in situ, which puts the patient at increased risk for testicular malignancy, the gonadal vessels and vas deferens must be identified and followed throughout their course. Some surgeons prefer to do this through a scrotal or an inguinal incision and proceed to retroperitoneal dissection if an inguinal testis or nubbin cannot be found, whereas others prefer the use of a laparoscope, which may have a higher success rate for orchiopexy than open surgery for the intra-abdominal testis.[100] Typically, laparoscopic surgery is recommended for all nonpalpable unilateral testicles.[58] If an absent testis is identified by the presence of blind-ending gonadal vessels, the procedure is terminated. As blind-ending vessels are reliably associated with absent testicular elements, further exploration is unnecessary unless the surgeon's goal is to remove all nubbins.[101] If a testicular nubbin is identified during exploration, the surgeon will often remove it for pathologic evaluation. In this situation, contralateral orchiopexy is generally not performed because the pathophysiology is most consistent with a prenatal extravaginal torsion event, and the viable testis is not at any increased risk.[102] If a viable testis is identified, the specific location of the testis, ease with which it can be brought down to the scrotum, and surgeon preference determine whether the orchiopexy can be performed in a 1- or a 2-stage setting.[103] Meta-analysis of laparoscopic versus open surgery for nonpalpable testes, including two randomized controlled trials and five observational studies, found no significant difference in operative success, testicular viability, or testicular atrophy.[104] Patients with laparoscopic surgery had a shorter length of hospitalization.

Prepubertal with undescended testicle(s): bilateral nonpalpable testicles

Immediate referral for endocrinology and/or genetic evaluation with karyotype and biochemical workup for a difference of sex development (DSD) is indicated in term newborn boys with bilateral nonpalpable testes. A phenotypic 46 XY male with bilateral nonpalpable testes has anorchia if inhibin and Mullerian inhibiting substance (MIS) levels are undetectable, coupled with elevated follicle-stimulating hormone (FSH), making neither the hCG stimulation test nor surgical exploration necessary in this subgroup. If endocrine tests indicate Sertoli and Leydig cell function, testicular tissue is present, and surgical therapy is indicated.[59][60] Typically, laparoscopic surgery is recommended for many bilateral cryptorchid patients.[58]

  • Testes absent according to serum hormonal evaluation: the patient is referred for endocrine and/or genetic evaluation and management, including possible testosterone supplementation and future fertility counseling. The family is also counseled regarding future placement of testicular prostheses, if desired.

  • Testes present according to serum hormonal evaluation: proceed to surgery. If one or both of the nonpalpable testes can be palpated on exam under anesthesia, the patient will undergo orchiopexy as described for the palpable testes. If one of the testes is found in the inguinal region and the other is located intra-abdominally, the surgeon may choose a staged approach, performing orchiopexy on the inguinal side and later returning for orchiopexy on the intra-abdominal testis, or may perform bilateral orchiopexy in the same setting. If neither testis is palpable under anesthesia, the surgeon may choose open or laparoscopic surgery for identification and concomitant orchiopexy (or orchiectomy of testicular nubbin) on one or both sides, depending upon the exact location of the testes and the ease with which the testes can be brought down into the scrotum. For high bilateral intra-abdominal testes, some surgeons prefer a staged approach with unilateral orchiopexy in one setting, allowing healing and ensuring no testicular atrophy, and then a staged orchiopexy on the contralateral testis.

Prepubertal with undescended testicle(s): unilateral or bilateral cryptorchid testicle(s) with hypospadias

Immediate referral for endocrinology and/or genetic evaluation with karyotype and biochemical workup for a DSD is indicated in newborns with hypospadias with palpable or nonpalpable testicle(s). Endocrinology and/or genetic evaluation with karyotype should be considered electively in patients with hypospadias and either unilateral or bilateral palpable cryptorchid testicle(s). Occasionally, a severely androgenized female with congenital adrenal hyperplasia may present with a phallic structure and presumed bilateral undescended testicles, and may have life-threatening metabolic disturbances. Urology referral is also indicated for further evaluation and therapy for hypospadias and cryptorchidism. These patients have been shown to have 27% to 55% risk of a DSD.[5]

Late diagnosis in a postpubertal patient

In rare instances, diagnosis of cryptorchidism is made in a postpubertal patient. Further treatment will depend on the exam findings and age of the patient:

  • Unilateral undescended testis: surgery with orchiopexy, with or without testicular biopsy, is done depending upon surgeon preference, testicular position (high scrotal, inguinal, intra-abdominal), and appearance of the testis at the time of surgery. If a mass is present in the undescended testis, tumor markers should be obtained, with subsequent radical orchiectomy through an inguinal approach.

  • Bilateral undescended testes: surgery with orchiopexy, with or without testicular biopsy, is done to evaluate dysplasia/carcinoma in situ. Endocrine evaluation with testosterone, LH, FSH, and semen analysis may be considered to determine degree of testicular function.

The risk of malignancy increases significantly in the cryptorchid testis treated after puberty.[75][105] For this reason, some have advocated proceeding directly to orchiectomy of the cryptorchid testis in adults.[106][107]

Others have demonstrated the presence of spermatogenesis in a small percentage of postpubertal cryptorchid testes that were removed, particularly those located in the prepubic area, and thus advocate testis biopsy, orchiopexy, and periodic follow-up.[108]

Patients older than 50 years and/or those with significant comorbidities may be at greater risk of death from anesthetic complications than germ cell neoplasia. Thus, continued observation is recommended in these patients.[109]

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