Cryptorchidism
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
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
retractile testicle(s)
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]Cancian M, Ellsworth P, Caldamone A. Contralateral metachronous undescended testis: is it predictable? J Pediatr Urol. 2017 Mar 28;13(5):504.e1-5. http://www.ncbi.nlm.nih.gov/pubmed/28434636?tool=bestpractice.com
undescended testicle(s): prepubertal, no hypospadias
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]Dayanc M, Kibar Y, Irkilata H, et al. Long-term outcome of scrotal incision orchiopexy for undescended testis. Urology. 2007 Oct;70(4):786-8. http://www.ncbi.nlm.nih.gov/pubmed/17991558?tool=bestpractice.com [92]Feng S, Yang H, Li X, et al. Single scrotal incision orchiopexy versus the inguinal approach in children with palpable undescended testis: a systematic review and meta-analysis. Pediatr Surg Int. 2016 Oct;32(10):989-95. http://www.ncbi.nlm.nih.gov/pubmed/27510940?tool=bestpractice.com 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]Kozminski DJ, Kraft KH, Bloom DA. Orchiopexy without transparenchymal fixation suturing: a 29-year experience. J Urol. 2015 Dec;194(6):1743-7. http://www.ncbi.nlm.nih.gov/pubmed/26141850?tool=bestpractice.com [111]Ribeiro CT, De Souza DB, Costa WS, et al. Effects of testicular transfixation on seminiferous tubule morphology and sperm parameters of prepubertal, pubertal, and adult rats. Theriogenology. 2015 Oct 15;84(7):1142-8. http://www.ncbi.nlm.nih.gov/pubmed/26187328?tool=bestpractice.com
Surgical therapy should ideally occur prior to 12-18 months of age.[1]Ritzén EM, Bergh A, Bjerknes R, et al. Nordic consensus on treatment of undescended testes. Acta Paediatr. 2007 May;96(5):638-43. https://onlinelibrary.wiley.com/doi/full/10.1111/j.1651-2227.2006.00159.x http://www.ncbi.nlm.nih.gov/pubmed/17326760?tool=bestpractice.com [55]Kolon TF, Herndon CD, Baker LA, et al. Evaluation and treatment of cryptorchidism: AUA guideline. J Urol. 2014 Aug;192(2):337-45. https://www.auanet.org/guidelines/cryptorchidism-(2014) http://www.ncbi.nlm.nih.gov/pubmed/24857650?tool=bestpractice.com [76]Ritzén EM. Undescended testes: a consensus on management. Eur J Endocrinol. 2008 Dec;159(suppl 1):S87-90. https://eje.bioscientifica.com/view/journals/eje/159/suppl_1/S87.xml http://www.ncbi.nlm.nih.gov/pubmed/18728121?tool=bestpractice.com [77]Chan E, Wayne C, Nasr A, et al; FRCSC for Canadian Association of Pediatric Surgeon Evidence-Based Resource. Ideal timing of orchiopexy: a systematic review. Pediatr Surg Int. 2014 Jan;30(1):87-97. http://www.ncbi.nlm.nih.gov/pubmed/24232174?tool=bestpractice.com [78]Komarowska MD, Hermanowicz A, Debek W. Putting the pieces together: cryptorchidism - do we know everything? J Pediatr Endocrinol Metab. 2015 Nov 1;28(11-12):1247-56. http://www.ncbi.nlm.nih.gov/pubmed/26226123?tool=bestpractice.com [79]Kollin C, Stukenborg JB, Nurmio M, et al. Boys with undescended testes: endocrine, volumetric and morphometric studies on testicular function before and after orchidopexy at nine months or three years of age. J Clin Endocrinol Metab. 2012 Dec;97(12):4588-95. http://www.ncbi.nlm.nih.gov/pubmed/23015652?tool=bestpractice.com
Adverse effects include surgery-related complications, most commonly anaesthetic complications, re-ascent of the testicle, haematoma, wound infection, and, rarely, testicular atrophy.
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]Dayanc M, Kibar Y, Irkilata H, et al. Long-term outcome of scrotal incision orchiopexy for undescended testis. Urology. 2007 Oct;70(4):786-8. http://www.ncbi.nlm.nih.gov/pubmed/17991558?tool=bestpractice.com [92]Feng S, Yang H, Li X, et al. Single scrotal incision orchiopexy versus the inguinal approach in children with palpable undescended testis: a systematic review and meta-analysis. Pediatr Surg Int. 2016 Oct;32(10):989-95. http://www.ncbi.nlm.nih.gov/pubmed/27510940?tool=bestpractice.com 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]Ritzén EM, Bergh A, Bjerknes R, et al. Nordic consensus on treatment of undescended testes. Acta Paediatr. 2007 May;96(5):638-43. https://onlinelibrary.wiley.com/doi/full/10.1111/j.1651-2227.2006.00159.x http://www.ncbi.nlm.nih.gov/pubmed/17326760?tool=bestpractice.com [55]Kolon TF, Herndon CD, Baker LA, et al. Evaluation and treatment of cryptorchidism: AUA guideline. J Urol. 2014 Aug;192(2):337-45. https://www.auanet.org/guidelines/cryptorchidism-(2014) http://www.ncbi.nlm.nih.gov/pubmed/24857650?tool=bestpractice.com [76]Ritzén EM. Undescended testes: a consensus on management. Eur J Endocrinol. 2008 Dec;159(suppl 1):S87-90. https://eje.bioscientifica.com/view/journals/eje/159/suppl_1/S87.xml http://www.ncbi.nlm.nih.gov/pubmed/18728121?tool=bestpractice.com [77]Chan E, Wayne C, Nasr A, et al; FRCSC for Canadian Association of Pediatric Surgeon Evidence-Based Resource. Ideal timing of orchiopexy: a systematic review. Pediatr Surg Int. 2014 Jan;30(1):87-97. http://www.ncbi.nlm.nih.gov/pubmed/24232174?tool=bestpractice.com [78]Komarowska MD, Hermanowicz A, Debek W. Putting the pieces together: cryptorchidism - do we know everything? J Pediatr Endocrinol Metab. 2015 Nov 1;28(11-12):1247-56. http://www.ncbi.nlm.nih.gov/pubmed/26226123?tool=bestpractice.com [79]Kollin C, Stukenborg JB, Nurmio M, et al. Boys with undescended testes: endocrine, volumetric and morphometric studies on testicular function before and after orchidopexy at nine months or three years of age. J Clin Endocrinol Metab. 2012 Dec;97(12):4588-95. http://www.ncbi.nlm.nih.gov/pubmed/23015652?tool=bestpractice.com
Adverse effects include surgery-related complications, most commonly anaesthetic complications, re-ascent of the testicle, haematoma, wound infection, and, rarely, testicular atrophy.
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]Baker LA, Docimo SG, Surer I, et al. A multi-institutional analysis of laparoscopic orchidopexy. BJU Int. 2001 Apr;87(6):484-9. http://www.ncbi.nlm.nih.gov/pubmed/11298039?tool=bestpractice.com Typically, laparoscopic surgery is recommended for all non-palpable unilateral testicles.[58]Tasian GE, Copp HL. Diagnostic performance of ultrasound in nonpalpable cryptorchidism: a systematic review and meta-analysis. Pediatrics. 2011 Jan;127(1):119-28. http://www.ncbi.nlm.nih.gov/pubmed/21149435?tool=bestpractice.com
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]Elyas R, Guerra LA, Pike J, et al. Is staging beneficial for Fowler-Stephens orchiopexy? A systematic review. J Urol. 2010 May;183(5):2012-8. http://www.ncbi.nlm.nih.gov/pubmed/20303527?tool=bestpractice.com
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.
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]Teo AQ, Khan AR, Williams MP, et al. Is surgical exploration necessary in bilateral anorchia? J Pediatr Urol. 2013 Feb;9(1):e78-81. https://www.jpurol.com/article/S1477-5131(12)00229-X/fulltext http://www.ncbi.nlm.nih.gov/pubmed/23079081?tool=bestpractice.com [60]Brauner R, Neve M, Allali S, et al. Clinical, biological and genetic analysis of anorchia in 26 boys. PLoS One. 2011;6(8):e23292. https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0023292 http://www.ncbi.nlm.nih.gov/pubmed/21853106?tool=bestpractice.com
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.
surgical exploration
If endocrine tests indicate Sertoli and Leydig cell function (testicular tissue is present), then surgical therapy is indicated.[59]Teo AQ, Khan AR, Williams MP, et al. Is surgical exploration necessary in bilateral anorchia? J Pediatr Urol. 2013 Feb;9(1):e78-81. https://www.jpurol.com/article/S1477-5131(12)00229-X/fulltext http://www.ncbi.nlm.nih.gov/pubmed/23079081?tool=bestpractice.com [60]Brauner R, Neve M, Allali S, et al. Clinical, biological and genetic analysis of anorchia in 26 boys. PLoS One. 2011;6(8):e23292. https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0023292 http://www.ncbi.nlm.nih.gov/pubmed/21853106?tool=bestpractice.com
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]Tasian GE, Copp HL. Diagnostic performance of ultrasound in nonpalpable cryptorchidism: a systematic review and meta-analysis. Pediatrics. 2011 Jan;127(1):119-28. http://www.ncbi.nlm.nih.gov/pubmed/21149435?tool=bestpractice.com
undescended testicle(s): prepubertal, with hypospadias
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]Brucker-Davis F, Pointis G, Chevallier D, et al. Update on cryptorchidism: endocrine, environmental and therapeutic aspects. J Endocrinol Invest. 2003 Jun;26(6):575-87. http://www.ncbi.nlm.nih.gov/pubmed/12952375?tool=bestpractice.com
undescended testicle: postpubertal
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]Oh J, Landman J, Evers A, et al. Management of the postpubertal patient with cryptorchidism: an updated analysis. J Urol. 2002 Mar;167(3):1329-33. http://www.ncbi.nlm.nih.gov/pubmed/11832725?tool=bestpractice.com
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]Pettersson A, Richiardi L, Nordenskjold A, et al. Age at surgery for undescended testis and risk of testicular cancer. N Engl J Med. 2007 May 3;356(18):1835-41. https://www.nejm.org/doi/full/10.1056/NEJMoa067588 http://www.ncbi.nlm.nih.gov/pubmed/17476009?tool=bestpractice.com For this reason, some have advocated proceeding directly to orchidectomy of the unilateral cryptorchid testis in adults.[105]Wood HM, Elder JS. Cryptorchidism and testicular cancer: separating fact from fiction. J Urol. 2009 Feb;181(2):452-61. http://www.ncbi.nlm.nih.gov/pubmed/19084853?tool=bestpractice.com [106]Koh KB. Beware the undescended testis and abdominal mass. Aust N Z J Surg. 1996 Dec;66(12):851-3. http://www.ncbi.nlm.nih.gov/pubmed/8996073?tool=bestpractice.com [107]Ryang SH, Jung JH, Eom M, et al. The incidence and histological characteristics of intratubular germ cell neoplasia in postpubertal cryptorchid testis. Korean J Urol. 2015 Jul;56(7):515-8. https://www.icurology.org/DOIx.php?id=10.4111/kju.2015.56.7.515 http://www.ncbi.nlm.nih.gov/pubmed/26175870?tool=bestpractice.com
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]Kang KJ, Kim HS, Oh TH. The clinical experience on orchiectomy in the postpubertal unilateral cryptorchidism. Korean J Urol. 1999 Oct;40(10):1371-6. http://urology.inforang.com/journal/viewJournal.html?year=1999&vol=40&page=1371
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]Oh J, Landman J, Evers A, et al. Management of the postpubertal patient with cryptorchidism: an updated analysis. J Urol. 2002 Mar;167(3):1329-33. http://www.ncbi.nlm.nih.gov/pubmed/11832725?tool=bestpractice.com
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]Pettersson A, Richiardi L, Nordenskjold A, et al. Age at surgery for undescended testis and risk of testicular cancer. N Engl J Med. 2007 May 3;356(18):1835-41. https://www.nejm.org/doi/full/10.1056/NEJMoa067588 http://www.ncbi.nlm.nih.gov/pubmed/17476009?tool=bestpractice.com For this reason, some have advocated proceeding directly to orchidectomy of the cryptorchid testis in adults.[106]Koh KB. Beware the undescended testis and abdominal mass. Aust N Z J Surg. 1996 Dec;66(12):851-3. http://www.ncbi.nlm.nih.gov/pubmed/8996073?tool=bestpractice.com
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]Kang KJ, Kim HS, Oh TH. The clinical experience on orchiectomy in the postpubertal unilateral cryptorchidism. Korean J Urol. 1999 Oct;40(10):1371-6. http://urology.inforang.com/journal/viewJournal.html?year=1999&vol=40&page=1371
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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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