Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

retractile testicle(s)

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annual follow-up examination

These patients should be observed with annual follow-up.

Any development of cryptorchidism or testicular asymmetry should be treated with orchiopexy. 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.[90]

undescended testicle(s): prepubertal, no hypospadias

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orchiopexy

Orchiopexy is 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. The surgical approach is determined by surgeon preference, generally through an inguinal or scrotal incision, with minimal difference in outcomes.[91][92] Inguinal hernia repair to close the associated patent processus vaginalis is performed in the same setting. Fixation stitches to secure the testis in place are most commonly used, although this may not be necessary and there is some evidence that transfixation could alter testis morphology.[110][111]

Surgical therapy should ideally occur prior to 12-18 months of age.[1][55][76][77][78][79]

Adverse effects include surgery-related complications, most commonly anaesthetic complications, re-ascent of the testicle, haematoma, wound infection, and, rarely, testicular atrophy.

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examination under anaesthesia + orchiopexy

Examination under anaesthesia is indicated for these patients to locate the non-palpable testis. The outcome of this examination determines further treatment. If the testis is palpable on examination under anaesthesia, orchiopexy should be performed.

The surgical approach is determined by surgeon preference, generally through an inguinal or scrotal incision, with minimal difference in outcomes.[91][92] Inguinal hernia repair to close the associated patent processus vaginalis is performed in the same setting. Surgical therapy should ideally occur prior to 12-18 months of age.[1][55][76][77][78][79]

Adverse effects include surgery-related complications, most commonly anaesthetic complications, re-ascent of the testicle, haematoma, wound infection, and, rarely, testicular atrophy.

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surgical exploration

If the testis is not palpable on examination under anaesthesia, further exploration is required; laparoscopic versus open surgical approach will depend on preference and expertise of the surgeon. 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 non-palpable unilateral testicles.[58]

Exploration for the non-palpable testis generally reveals three common findings: an inguinal testis or nubbin, confirmed by the presence of spermatic vessels and vas deferens entering the internal inguinal ring; an intra-abdominal testis; or blind-ending gonadal vessels proximal to the internal inguinal ring.

A nubbin of tissue should be removed/resected for pathological evaluation.

If a viable testis is identified, the specific location of the testis (inguinal vs. intra-abdominal), 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]

In order to ensure that no residual testicular tissue is left in situ, which puts the patient at increased risk for malignancy, the gonadal vessels and vas deferens must be identified and followed throughout their course. If an absent testis is identified, the procedure is terminated, or the surgeon may elect to perform contralateral testicular orchiopexy of the remaining testis, if undescended.

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endocrinology and/or genetic evaluation

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 non-palpable testes. Occasionally, a severely androgenised female with congenital adrenal hyperplasia may present with a phallic structure and presumed bilateral undescended testes, and may have life-threatening metabolic disturbances.

A phenotypic 46 XY male with bilateral non-palpable testes has anorchia if inhibin and Mullerian inhibiting substance (MIS) levels are undetectable, coupled with elevated follicle-stimulating hormone, making neither the human chorionic gonadotrophin stimulation test nor surgical exploration necessary in this subgroup.[59][60]

The patient should be referred for endocrine and/or genetic evaluation and management, including testosterone supplementation and fertility counselling. The family is also counselled regarding future placement of testicular prostheses, if desired.

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surgical exploration

If endocrine tests indicate Sertoli and Leydig cell function (testicular tissue is present), then surgical therapy is indicated.[59][60]

Laparoscopic versus open abdominal approach will depend on the expertise and choice of the surgeon, who may order imaging in order to assist in localization of the testes for the purpose of surgical planning. The surgeon may also elect to perform both orchiopexies at one surgical session, or to perform one orchiopexy and bring the patient back at a later stage for the second orchiopexy depending upon the specific appearance, location, and mobility of each testis. Typically, laparoscopic surgery is recommended for many bilateral cryptorchid patients.[58]

undescended testicle(s): prepubertal, with hypospadias

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endocrinology and/or genetic evaluation + urology referral

Immediate referral for endocrinology and/or genetic evaluation with karyotype and biochemical workup for a difference of sex development (DSD) is indicated in newborns with hypospadias with palpable or non-palpable 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 androgenised female with congenital adrenal hyperplasia may present with a phallic structure and presumed bilateral undescended testes, 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]

undescended testicle: postpubertal

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orchiopexy ± biopsy

Surgery with orchiopexy with or without biopsy depending upon surgeon preference, testicular position (high scrotal, inguinal, intra-abdominal), and appearance of the testis at the time of surgery is recommended.

If a mass is present in the undescended testis, tumour markers should be obtained, with subsequent radical orchiectomy through an inguinal approach.

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

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Consider – 

orchiectomy

Additional treatment recommended for SOME patients in selected patient group

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

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

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orchiopexy ± biopsy

Endocrine evaluation with testosterone, LH, follicle-stimulating hormone, and semen analysis may be considered to determine degree of testicular function.

Orchiopexy with or without testicular biopsy to evaluate dysplasia/carcinoma in situ is recommended.

If a mass is present in either undescended testis, tumour markers should be obtained, with subsequent radical orchiectomy through an inguinal approach of the testis with the mass, and either simultaneous or staged biopsy and orchiopexy of the contralateral undescended testis.

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

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Consider – 

orchiectomy

Additional treatment recommended for SOME patients in selected patient group

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

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

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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