Pediatric Cryptorchidism Surgery

Embryology of testis development

The embryology of testis development is critical to understanding the most common theories formulated to explain cryptorchidism.

Shortly after 6 weeks' gestation, the testis-determining SRY gene on chromosome Y directly affects the differentiation of the indifferent gonad into a testis. Germ cells are located in the germinal ridge close to the kidney in the retroperitoneum. Around 6-7 weeks' gestation, Sertoli cells develop and secrete müllerian inhibitory substance (MIS; also known as antimüllerian hormone [AMH]), which leads to the regression of the female genital organs.

Around 9 weeks' gestation, Leydig cells start producing testosterone, which promotes development of the wolffian duct into portions of the male genital tract. Concurrently, the testis organizes as a distinct organ with its distinct seminiferous tubules surrounded by vessels and encapsulated by the tunica albuginea. Owing to the differential growth of the fetus, the testicles move into the pelvis, close to the internal ring.

The testis remains in a retroperitoneal position until 28 weeks' gestation, at which time inguinal descent of the testicle begins. Most testes have completed their descent into the scrotum by 40 weeks' gestation.

Theories of pathophysiology of cryptorchidism

Several potential explanations for the pathophysiology of cryptorchidism have been proposed, including gubernacular abnormalities, reduced intra-abdominal pressures, intrinsic testicular or epididymal abnormalities, and endocrine abnormalities, as well as anatomic anomalies (eg, fibrous bands within the inguinal canal or abnormal arrangement of the cremasteric muscle fibers).

The gubernaculum testis is a structure that attaches the lower portion of the tunica vaginalis to base of the scrotum. The gubernaculum is thought to aid in testicular descent by widening the inguinal canal and guiding the testis down to the scrotum. Accordingly, it has been suggested that anomalies in this attachment may contribute to cryptorchidism.

Cryptorchidism is common in patients with prune belly syndrome and those with gastroschisis; both are associated with decreased intra-abdominal pressures. However, the theory based on reduced pressures has not explained most cases of cryptorchidism.

Another theory of testicular maldescent has been based on intrinsic testicular or epididymal abnormalities. Several studies have shown that the germinal epithelium of the maldescended testis may be histologically abnormal. Infertility is associated with cryptorchidism, and the risk of infertility increases with the degree of maldescent.

Moreover, approximately 23-86% of maldescended testes have been associated with some form of epididymal abnormality. Studies have shown an increased degree of epididymal abnormalities in intra-abdominal testis as compared with mild cases of cryptorchidism.[3]  Sharma et al reported an 8% incidence of complete testicular dissociation in boys with cryptorchidism.[4]

Abnormalities in the hypothalamic-pituitary-gonadal axis have been postulated as a possible explanation for anomalies of testicular descent and abnormal germ cell development.[5] Animal and human endocrine studies have not fully elucidated the pathophysiology of testicular maldescent. The causative hormonal abnormality may be found at different levels. That the condition most often affects one side indicates that endocrine anomalies may be partially responsible but does not completely explain why the testis does not descend normally.

Ongoing research

The molecular mechanisms by which the newly determined testicle descends from its position in the posterior abdomen into the scrotum is a complex process that likely involves multiple genetic, hormonal, environmental, and stochastic factors.[6] Although a comprehensive explanation has not yet been elucidated, several observations have suggested that specific genetic loci play important roles in normal testicular descent and the occurrence of cryptorchidism.

Models for the study of cryptorchidism include experiments in knockout mice. Homozygous mutants for the loss of HOXA10 and HOXA11 exhibit cryptorchidism. Both genes are members of the family of homeobox genes, which are highly conserved throughout evolution and which play a critical role in anteroposterior positioning in the developing embryo. Early orchiopexy rescues HOXA11 mutants from an infertile state. HOXA10 polymorphisms have been found in human cryptorchid populations, though their functional significance has not yet been established.

In the literature, much attention has been focused on insulinlike factor 3 (INSL3) and its receptor, leucine-rich repeat-containing G protein-coupled receptor 8 (LGR8), also referred to as relaxin family peptide receptor 2 (RXFP2) or G-protein–coupled receptor affecting testes descent (GREAT).[7, 8, 9] Homozygous knockouts of either INSL3 or LGR8 lead to the phenotype of bilateral intra-abdominal testes. As in the murine HOXA11 model, early orchiopexy of INSL3-deficient mice allows for the development of fertility.

Although some have suggested that mutations in the INSL3 gene might not play a substantial role in human cryptorchidism, a missense mutation in INSL3 has been found in a patient with cryptorchidism; this mutation causes a nonconservative amino acid substitution. A proof-of-principle study has not yet been conducted to determine whether this INSL3 mutation leads to cryptorchidism.

Ayers et al, reporting on a family in which four boys had isolated bilateral cryptorchidism, identified a homozygous missense variant in RXFP2 in all four affected boys, with heterozygosity in the parents.[10] No other variant with a link to testis biology was found. Functional analysis demonstrated that the variant protein had poor cell surface expression and failed to bind INSL3. The results supported the view that recessive variants in RXFP2 underlie familial cryptorchidism. 

LGR8 polymorphisms have been identified in both cryptorchid and healthy human populations. One of the receptor mutations found in a cryptorchid patient precluded a response to ligand stimulation in vitro.

In the search for a genetic cause of cryptorchidism, other areas of focus include Y-chromosome microdeletions, increased aromatase activity, and abnormalities in the Wilms tumor gene (WT1).

A palpable undescended testis is found in 3-5% of newborns, and bilateral undescended testis is found in 15% of newborns with cryptorchidism. Most undescended palpable testes later spontaneously descend within the first 4 months of life; only 0.7-1% of 1-year-old infants have a persistent undescended testis. Studies have shown that spontaneous descent does not occur after age 9 months. The incidence does not change between age 1 year and adulthood. However, some testes that were descended in early childhood may ascend later in life.

Nonpalpable testes account for approximately 20% of all undescended testes. Approximately 40% of the nonpalpable testes are intra-abdominal, 40% are inguinal, and 20% are atrophic or absent (vanishing testis syndrome).

Cryptorchidism is found in 30% of babies born prematurely. Other predisposing factors include low birth weight, small size for gestational age, twin pregnancy, and maternal estrogen exposure. Cryptorchidism is found in 7% of siblings and in about 2% of fathers of babies with this condition.

Cryptorchidism affects only males and has no reported racial predilection.

Success of surgical treatment

Orchiopexy for palpable testis (scrotal, inguinal, and suprainguinal) has a success rate in the range of 80-90%, as measured by the testicle being in a normal anatomic position.

Orchiopexy for nonpalpable testis has different reported success rates (as measured by testis in normal position and felt to be viable) in different surgical scenarios, as follows:

• Inguinal approach - 60-88%
• Suprainguinal approach - Up to 95%
• One-stage Fowler-Stephens procedure - 67-96%
• Two-stage Fowler-Stephens procedure - 77-95%
• Microvascular transplantation - 83-96%
• Laparoscopic orchiopexy - 80-95%
• Laparoscopic Fowler-Stephens procedure - Up to 96%

The so-called Shehata technique, or staged laparoscopic traction orchiopexy (SLTO), may be an effective alternative to a staged Fowler-Stephens procedure for high-level intra-abdominal testis; a study by Tian et al found that SLTO yielded better overall success and atrophy rates than staged FSO, along with comparable retraction rate and operating time.[11]

Mortality and morbidity

Cryptorchidism has not been associated with any factors for mortality. However, testicular maldescent has been associated with a slight increase in the risk of testicular cancer,[12, 13]  infertility, trauma, and testicular torsion. If not treated, testicular maldescent may also affect the psychological well-being of young men in whom negative self-esteem issues may arise.

Testicular cancer

In patients with cryptorchidism, the risk of testicular cancer has been reported to be 3-5%, a four- to sevenfold greater risk than the 0.3-0.7% reported in the healthy population. Early orchiopexy (at age < 1 y) appears to reduce this risk.[14]

The most common tumor in an undescended testis is a seminoma, whereas the most common tumor after successful orchiopexy is nonseminomatous germ cell tumor. Approximately 20% of these tumors occur in a contralateral descended testis.

Carcinoma in situ occurs in approximately 0.4% of patients undergoing orchiopexy. Orchiopexy is not protective against subsequent testis cancer but does place the testis in a favorable position for routine self-examination, which is important in the early recognition of testicular cancer. The patient and family must be educated about the risk of future testicular cancer.

Infertility

Approximately 6% of infertile men have a history of orchiopexy or untreated cryptorchidism. The rate of infertility is higher in patients with bilateral cryptorchidism than in those with unilateral cryptorchidism or in the general male population. The paternity rate for patients with bilateral cryptorchidism is around 60% versus 90% for patients with unilateral cryptorchidism. The rate in those with unilateral cryptorchidism is slightly less than the 94% in the general population.

The location of the undescended testis may play a role in fertility potential. Worsening testicular biopsy findings are correlated with high locations (eg, intra-abdominal testis).

Normal spermatogram findings are found in 20% of patients with bilateral undescended testis compared with 75% of patients with unilateral cryptorchidism.

The decision to perform orchiopexy in patients younger than 24 months might be made because testicular biopsy shows that the rate of germ cell aplasia substantially increases after age 2 years. Long-term studies are needed to determine the true effect of early orchiopexy on fertility.

One evaluation of the referral practices of local pediatricians showed that physicians tended to refer patients for treatment at a mean age of around 4 years. This finding shows the importance of educating primary physicians about the timing of surgery (before age 1 y) and the benefits of early surgical intervention.

The patient and his family should be informed about the risks of infertility and malignancy. Self-examination after the onset of puberty should be discussed as very important for the early diagnosis and successful treatment of testicular cancer.

Determining if the testis was palpable in the scrotum at any time is important. The patient's antenatal history should include the following:

• Gestational age at birth
• Any need for assisted reproduction
• Maternal hormonal treatment
• Mother's number of gestations

Any previous history of inguinal surgery should be noted, as should a family history of cryptorchidism and other associated conditions.

Cryptorchidism is associated with inguinal hernia and/or patent processus vaginalis, hypospadias, cerebral palsy, mental retardation, Down syndrome, Wilms tumor, prune belly syndrome, and Prader-Willi syndrome.

Physical examination is the most important tool in the diagnostic evaluation of cryptorchidism. The patient must be examined in a warm, relaxed environment. Closely observing the scrotum before manipulation is important. The frog-leg or catcher position may be used to facilitate palpation of the testis.

Determining if the testis is palpable is essential. If the testis is palpable, it is vital to ascertain whether it can be retracted. The retractile testis should stay in the dependent portion of the scrotum after manipulation.

The best method of evaluating for an undescended testis is to start palpating at level of the inguinal canal and perform a milking motion down toward the scrotum. The examiner should look for hemiscrotal asymmetry and for contralateral testicular hypertrophy; both are partial indicators of an absent testis.

Hypoplastic right hemiscrotum in a patient with an undescended right testis.

Examination of potential ectopic sites (eg, penile, femoral, and perineal areas) is important if the testicle cannot be felt in the inguinal area. Patients with hypospadias and cryptorchidism have a higher incidence of differences (disorders) of sex development (DSDs), and a workup should be considered. If any doubt remains after the initial examination, reevaluation of the patient is mandatory before surgical management is recommended.

Ectopic testis.

No staging system is reported; the physical finding of a palpable testis versus a nonpalpable testis is the most reliable and easy way of grouping cryptorchidism cases.

Routine laboratory workup is not indicated with unilateral cryptorchidism.

Patients with bilateral nonpalpable testis and those with unilateral or bilateral undescended testis associated with hypospadias should undergo evaluation to rule out a difference (disorder) of sex development (DSD). The evaluation should include chromosomal analysis and measurement of 17-hydroxylase progesterone, testosterone, luteinizing hormone (LH), and follicle-stimulating hormone (FSH). For bilateral nonpalpable testis, abdominal-pelvic ultrasonography (US) is advisable, mainly to determine if any müllerian structures (eg, a uterus) are present.

Anorchia can be confirmed by means of hormonal stimulation with human chorionic gonadotropin (hCG), with baseline and poststimulation measurement of LH, FSH, and testosterone hormone levels. Many hCG stimulation protocols are described. The authors favor a single injection of hCG 2940 IU/m2 body surface area, with hormonal levels assessed at 72 hours. Anorchia is found in patients with elevated baseline LH and FSH levels and low testosterone levels without an increase in testosterone after stimulation.

Another marker of testicular function is müllerian-inhibiting substance (MIS; also known as antimüllerian hormone [AMH]).[15] MIS levels that exceed 5 ng/mL suggest the presence of testicular tissue and are an indication for exploration. However, this study is not yet in widespread use, and its applicability to older children remains to be defined.

Imaging studies have little or no role in the diagnosis of cryptorchidism.

US, computed tomography (CT), magnetic resonance imaging (MRI), and angiography have been used to detect undescended testes. However, these studies have unacceptable false-positive and false-negative rates. CT exposes the patient to high levels of radiation, and MRI requires sedation or anesthesia; both are costly.[16]

Diagnostic laparoscopy (see the image below) is the most reliable technique for localizing the nonpalpable testis.[17] It is performed in conjunction with definitive therapy (laparoscopic orchiopexy or open orchiopexy). Laparoscopic findings can be helpful in determining the need for inguinal exploration, for deciding between one-stage and two-stage repair, and for assessing gonadal viability. They can also help clarify the anatomy in complex DSD cases.

Diagnostic laparoscopy of a crossed ectopic testis.

The histologic findings of an undescended testis range from normal histology to acquired germ cell hypoplasia with Leydig cell hyperplasia. The severity of the histologic findings is correlated with an intra-abdominal testis, delayed orchiopexy, or both.

Carcinoma in situ is present in as many as 8% of infertile patients undergoing testicular biopsy with a history of orchiopexy. In children with undescended testis, the overall incidence of carcinoma in situ is approximately 0.4%. The clinical significance of these two findings is unclear.

Engle proposed hormonal manipulation as a treatment for cryptorchidism in the 1930s. Whether treatment is hormonal or surgical, the main goals are as follows:

• To allow the testicle to occupy a normal anatomic position
• To preserve fertility and hormonal production
• To diagnose potential testicular malignancies

Other putative benefits include correction of associated hernias and prevention of testicular torsion.

The risk of trauma and possible psychological effects of having a missing testis must be taken into account. Orchiopexy should be considered after 4 months of life; the rate of descent diminishes considerably after this point.

For postpubertal adolescents and men younger than 32 years who underwent unilateral orchiopexy, orchiectomy should be considered. For postpubertal men older than 32 years, close observation and routine physical examination should be considered. Any man with bilateral undescended testes should undergo bilateral testicular biopsy and orchiopexy.

Guidelines for management of pediatric cryptorchidism have been developed by the American Urological Association (AUA)[18]  and the Canadian Urological Association (CUA)–Pediatric Urologists of Canada (PUC)[19] (see Guidelines).

Hormonal therapy should be considered for patients in whom the diagnosis of retractile testis is not certain. For patients who are not candidates for surgical interventions, hormonal therapy might be appropriate.[20, 21]

Hormonal therapy has been employed in Europe for many years as a primary therapy for cryptorchidism; the main hormones used are human chorionic gonadotropin (hCG) and luteinizing hormone (LH)-releasing hormone (LHRH). In Europe, these two hormones have been given in combination, with initial success rates of 14-65%; however, some long-term studies have shown lower success rates.

Like LH, hCG acts on Leydig cells to stimulate the production of gonadal steroid hormones; however, its effects on testicular descent are not fully understood. In most patients with retractile testis, the condition responds to hCG. Studies have shown short-term success rates as high as 70%. Controlled studies have shown less impressive results (~14%).

Multiple dosage schedules have been proposed. The authors' current protocol is 1000 IU/wk for children who weigh less than 10 kg, 1500 IU/wk for children who weigh 10-20 kg, and 2500 IU/wk for children who weigh more than 20 kg. The duration of therapy is 4 weeks.

LHRH acts indirectly in the pituitary by stimulating the release of LH and follicle-stimulating hormone (FSH). LHRH may be more efficient in increasing testosterone than hCG is. LHRH is currently available only in Europe for use in cryptorchidism. Success rates are similar to those of hCG and are in the range of 10-15%.

Adverse effects from both hormonal therapies include increase in scrotal rugae, pigmentation, growth of pubic hair, increased penile size, and erections. LHRH has fewer adverse effects than hCG does.

A meta-analysis by Chua et al suggested that in a subset of children with cryptorchidism, gonadotropin-releasing hormone (GnRH) might be beneficial as an adjunct to orchidopexy in improving the fertility index; however, additional study would be required to identify specific characteristics of patient subgroups that would clearly benefit from such treatment.[22]

Several surgical approaches to the undescended testis have been described. The approach chosen is determined by the position of the testis and the surgeon's expertise.

The palpable testis can be approached via a scrotal, subinguinal, inguinal, or suprainguinal approach. The nonpalpable testis can be approached via an inguinal, suprainguinal, or laparoscopic approach.

During the period between January 2002 and July 2009, one study showed a shift in surgical approach toward more scrotal-approach procedures for palpable testes and fewer transinguinal abdominal orchidopexies for nonpalpable testes.[23]

In a prospective study of 200 patients with palpable undescended testis who underwent orchidopexy via either a two-incision inguinal approach or a single-incision scrotal approach between January 2011 and December 2013, Ben Dhaou et al found the latter approach to be safe and effective for undescended testicles palpable in the inguinal canal or in a high scrotal position.[24]

In a randomized study by Nazem et al, single-incision transscrotal orchiopexy (group I) was compared with traditional two-incision inguinal-scrotal orchiopexy (group II) in 100 children who required surgical treatment for undescended testis.[25] ​ Success rates were 98% in group I and 94% in group II, and the two groups did not differ significantly with respect to wound infection, testicular atrophy, testicular hypotrophy, and relapse. However, the single-incision transscrotal approach was judged more successful in terms of cosmetic results, patient satisfaction, and duration of hospitalization.

In a randomized controlled trial evaluating the effects of scrotal (n = 50) versus inguinal orchiopexy (n = 50) on the testicular function of children with clinically palpable inguinal undescended testis, Huang et al found no apparent differences between groups with respect to testicular volume, serum testosterone, or anti-müllerian hormone (AMH) and inhibin B (InhB) levels at 6 and 12 months after operation.[26] The groups also did not differ with respect to operating time and intraoperative blood loss. Although the overall complication rate was slightly lower in the scrotal group than in the inguinal group (2.0% vs 8.0%), the difference was not statistically significant.

Several studies reported successful treatment of cryptorchidism with laparoscopic techniques.[27, 28] A systematic review and meta-analysis of five studies (N = 705) by Anand et al concluded that laparoscopic orchiopexy was safe and effective in children with palpable cryptorchidism and was comparable to conventional open orchiopexy with respect to the rates of redo orchiopexy, early complications, and testicular atrophy.[29]

Routine testicular biopsy during orchiopexy is not recommended and should be considered only in cases involving prune belly syndrome, ambiguous genitalia, abnormal karyotypes, or postpubertal adolescents or men. Some authors have recommended that if the biopsy reveals carcinoma in situ, repeat exploration and unilateral orchiectomy should be performed. In bilateral cases, radiation therapy may be useful.

Palpable testis

Look for the testis after incising the Scarpa fascia to avoid injuring a testis and its cord found outside of the external inguinal ring (ectopic testis in the superficial inguinal pouch).

Divide all attachments, including the gubernaculum, the cremasteric fibers, and the lateral spermatic fascia.

Identify the patent processus vaginalis in the anteromedial surface of the cord, separate it from the cord structures, and perform a high ligation; be careful not to trap the vas or vessels.

Place the testis in a subdartos pouch.

Nonpalpable testis

Diagnostic laparoscopy should be preferred to inguinal exploration.

Blind-ending vas and vessels confirm the diagnosis of a vanishing testis and do not warrant further therapy. Consideration should be given to exploring the contralateral scrotum and placing some anchoring stitches to prevent possible testis torsion on the other side.

Vessels entering the internal inguinal ring require further inguinal or scrotal exploration to identify the undescended testis or testicular nubbin.

In patients with findings of a vanishing testis or a testicular nubbin, fixation of the contralateral testis should be considered but is controversial.[30]

A small intra-abdominal testis or an abnormal testis requires orchiectomy.

Length-increasing maneuvers

The Prentiss maneuver involves rerouting the cord under the epigastric vessels or the division of epigastric vessels.[31]

The internal inguinal ring can be opened to perform more complete retroperitoneal mobilization.

The Fowler-Stephens principle involves dividing the testicular vessels to allow the blood supply to the vas deferens to keep the undescended testis viable. The testicular vessels should be divided away from the testis.

Testicular autotransplantation can be performed by transecting the testicular vessels and by performing a microvascular anastomosis to the inferior epigastric vessels.

The surgical procedure is done on an outpatient basis. Pain control medications should be prescribed as needed; minimal pain medication is required in the first 24-48 hours. The surgical incision site should be kept dry for 48 hours. If surgical buttons were used, consider removing them 7-10 days after the operation.

Children should avoid playing on straddle toys and participating in physical education for 1-2 weeks after surgery.

Complications related to the surgical correction of the maldescended testis include the following:

• Testicular atrophy (5%)
• Injury to vas deferens (1-2%)
• Reascent of the testicle or abnormal anatomic position (< 10%) - This can often be corrected by means of redo orchiopexy ^[32]
• Epididymo-orchitis
• Hydrocele

No changes in diet are required after treatment. After surgery, patients should be advised to limit their activities for a week and refrain from straddling.

Early orchiopexy performed before age 2 years may prevent possible damage to the testis and may improve spermatogenetic viability. The data from one study indicated that orchiopexy, when performed on patients younger than 2 years, resulted in significant recovery of testicular volume at follow-up.[33] These results suggested that the timing of surgery is a significant factor for recovery of delayed cryptorchid testicular growth.

Patients with bilateral anorchia or a difference (disorder) of sex development (DSD) may benefit from a consultation with a pediatric endocrinologist.

Office visits should be scheduled postoperatively and at 1 year to evaluate the location, size, and viability of the testis. Consideration should be given to seeing the patient again at the time of puberty. Discussions of fertility issues and the need for self-examination to detect cancer should be revisited.

In 2014, the American Urological Association (AUA) issued the following statements regarding evaluation and treatment of cryptorchidism, which were reviewed and reaffirmed in 2018.[18]

Diagnosis

AUA diagnostic recommendations are as follows:

• Providers should obtain a gestational history during the initial evaluation of boys with suspected cryptorchidism (standard; grade B evidence)
• Primary care providers should palpate testes for quality and position at each recommended well-child visit (standard; grade B evidence)
• Providers should refer infants with a history of cryptorchidism (detected at birth) who do not have spontaneous testicular descent by 6 months (corrected for gestational age) to an appropriate surgical specialist for timely evaluation (standard; grade B evidence)
• Providers should refer boys with the possibility of newly diagnosed (acquired) cryptorchidism after 6 months (corrected for gestational age) to an appropriate surgical specialist (standard; grade B evidence)
• Providers must immediately consult an appropriate specialist for all phenotypic male newborns with bilateral, nonpalpable testes for evaluation of a possible disorder of sex development (DSD) (standard; grade A evidence)
• Providers should not perform ultrasonography (US) or other imaging modalities to evaluate boys with cryptorchidism prior to referral, in that these studies rarely assist in decision-making (standard; grade B evidence)
• Providers should assess the possibility of a DSD when there is increasing severity of hypospadias with cryptorchidism (recommendation; grade C evidence)
• In boys with bilateral, nonpalpable testes who do not have congenital adrenal hyperplasia (CAH), providers should measure müllerian-inhibiting substance (MIS; anti-müllerian hormone [AMH]) and consider additional hormone testing to evaluate for anorchia (option; grade C evidence)
• In boys with retractile testes, providers should assess the position of the testes at least annually to monitor for secondary ascent (standard; grade B evidence)

Treatment

AUA therapeutic recommendations are as follows:

• Providers should not use hormonal therapy to induce testicular descent; evidence shows low response rates and lack of evidence for long-term efficacy (standard; grade B evidence)
• In the absence of spontaneous testicular descent by 6 months (corrected for gestational age), specialists should perform surgery within the next year (standard; grade B evidence)
• In prepubertal boys with palpable, cryptorchid testes, surgical specialists should perform scrotal or inguinal orchidopexy (standard; grade B evidence)
• In prepubertal boys with nonpalpable testes, surgical specialists should perform examination under anesthesia to reassess for palpability of testes; if testes are nonpalpable, surgical exploration and, if indicated, abdominal orchidopexy should be performed (standard; grade B evidence)
• At the time of exploration for a nonpalpable testis in boys, surgical specialists should identify the status of the testicular vessels to help determine the next course of action (clinical principle)
• In boys with a normal contralateral testis, surgical specialists may perform an orchiectomy (removal of the undescended testis) if a boy has a normal contralateral testis and either very short testicular vessels and vas deferens, dysmorphic or very hypoplastic testis, or postpubertal age (clinical principle)
• Providers should counsel boys with a history of cryptorchidism, monorchidism, or both, as well as their parents, regarding potential long-term risks and should provide education on infertility and cancer risk (clinical principle)

In 2017, the Canadian Urological Association (CUA) and the Pediatric Urologists of Canada (PUC) formulated a guideline for the diagnosis, management, and follow-up of cryptorchidism.[19]  Recommendations included the following:

• Imaging in cryptorchidism is not cost-effective, may delay referral and surgical treatment, and thus cannot be recommended as a standard adjunct to preoperative assessment of these children (level 3 evidence; grade B recommendation)
• Routine karyotype or genetic workup of patients with undescended testis (UDT) is not recommended (level 4 evidence; grade D recommendation)
• A karyotype should be performed in patients with at least one UDT and proximal hypospadias, especially in the setting of nonpalpable gonads (level 4 evidence; grade D recommendation)
• Consideration should be given to include targeted WT1 genetic testing in patients with proximal hypospadias and at least one UDT (level 3 evidence; grade C recommendation)
• When müllerian remnants are found incidentally during an inguinal orchidopexy, the proximal aspect of the fallopian tube can be transected and removed with the uterus, with the distal component left attached to the vas deferens, allowing the testis to be brought to a scrotal position (level 4 evidence; grade D recommendation)
• Hormone therapy has a limited role in the management of cryptorchidism and should not be recommended as first-line therapy (level 2 evidence; grade B recommendation)
• Orchidopexy is best performed between 6 and 18 months of age (level 2 evidence; grade B recommendation)
• For palpable UDT undergoing surgery, both inguinal and prescrotal techniques are acceptable, depending on the surgeon’s preference and experience (level 2 evidence; grade B recommendation)
• In uncertain cases or when tissue analysis is not consistent with atrophic testicular tissue, laparoscopic exploration should be strongly considered (level 4 evidence; grade C recommendation)
• In the absence of literature strongly supporting or discouraging prophylactic orchidopexy, the decision should be made on the basis of informed discussion of options with the parents or legal guardian (level 5 evidence; grade D recommendation)
• Given the reports of testicular cancer ^[13] (sometimes early) in these patients, orchidopexy is recommended when they are clinically fit for anesthesia for the purpose of surveillance (level 4 evidence; grade D recommendation)
• Redo orchidopexy should be offered for cases where inadequate position is detected postoperatively (level 5 evidence; grade D recommendation)
• Orchiectomy should be considered for postpubertal patients with hypotrophic/atrophic undescended testicles up to the age of 50 (level 4 evidence; grade D recommendation)

Class Summary

This treatment should be considered when the diagnosis of retractile testis is uncertain. This is also used for cases of nonpalpable testis in which Fowler-Stephens orchiopexy is considered or in patients who are poor candidates for surgical intervention. Hormonal therapy has been used in Europe for many years as a primary therapy for cryptorchidism.

Human chorionic gonadotropin (hCG, Choron, Pregnyl)

Acts on Leydig cells similar to pituitary LH by stimulating production of gonadal steroid hormones, including testosterone. Effect on testicular descent not fully understood. Success rates 14-70%.