Normal testicular development in utero begins along the mesodermal ridge of the posterior abdominal wall. By 28 weeks, the right and left testes reach their respective inguinal canals and, by 28-40 weeks, each testis has usually reached the scrotum.
An undescended testis is a testis that is absent from the scrotum. The term cryptorchidism, from the Greek kryptos (hidden) and orchis (testicle), is also used. Absence may be due to:
- Testicular agenesis (anorchia) - uncommon.
- Retractile testis.
- The ascending testis syndrome.
- Testicular maldescent.
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- Prepubertal boys can have an exaggerated cremasteric reflex.
- The testis may retract out of the scrotum in the cold, on examination, on excitement or on physical activity.
- It is normal and will descend when relaxed and warm, or it can be manipulated back into the scrotum.
- Retractile testes do not need any treatment but do need close follow-up until puberty, as they can become ascendant.
- Retractile testes have a 32% risk of becoming an ascending or acquired undescended testis.
The ascending testis syndrome
- A previously normal or a retractile testis can become high with a shortened spermatic cord that prevents the testis from staying in the scrotum.
- It is a rare condition which occurs more commonly on the left side.
- It is usually diagnosed in those aged 8-10 years.
- Some sources say that this needs corrective treatment but others suggest a 'wait-and-see' approach for spontaneous descent until puberty.
- One study found that acquired undescended testis had a 77.5% tendency to spontaneous descent in puberty. Long-term testicular growth matched that which followed pubertal orchidopexy (ie it was in the normal range).
- These are usually unilateral.
- The scrotum may be underdeveloped.
- Maldescent may be due to an anatomical abnormality or due to hormone lack or hormone resistance. The release of testosterone from the fetal testis, the release of substances from an intact genitofemoral nerve and gonadotrophin hormone have all been cited as having possible involvement in normal testicular descent.
- Descent can be:
- Arrested - where descent is along the normal path but incomplete. The testis may be located near the pubic tubercle, in the inguinal canal (80%), or, uncommonly, in the abdomen. The testis is often small and abnormal with a short spermatic cord. There may be associated inguinal hernia.
- Ectopic - where descent deviates from the normal path. The testis is most often found in the superficial inguinal pouch. Perineal, abdominal (5% of undescended testes in one study), pelvic, crural, penile and femoral positions are also all possible. The testis and spermatic cord are usually normal.
- The most common congenital anomaly of the male genitalia.
- A study looking at the birth prevalence of cryptorchidism in northern England from 1993-2000 found that it was 7.6 per 1,000 live births.
- The incidence has not changed much over a period of fifty years.
- There is a higher incidence in premature babies (up to 30%).
- It affects almost 1% of all full-term male infants at the age of 1 year.
- Unilateral cryptorchidism is four times more likely than bilateral.
- Binge drinking and mild gestational diabetes have all been proposed as potential risk factors for cryptorchidism.
- Smoking during pregnancy has been held to be a risk factor although a recent meta-analysis found that the evidence to support this assertion is weak.
- One study reported a link with maternal intake of mild analgesics, eg paracetamol, between 14-22 weeks.
- Placental abnormalities are a known link.
- This is by physical examination.
- Imaging or ultrasound does not add any benefit to differentiating between palpable and non-palpable testes.
- Examination should take place while the child is supine and in a cross-legged position and cover the following steps:
- Perform a visual examination of the scrotum.
- Inhibit the cremasteric reflex with one hand above the symphysis in the groin region before touching the scrotum.
- 'Milking' of the groin region towards the scrotum may help to move the testis into the scrotum. It can also help to differentiate between an inguinal testis and enlarged inguinal lymph nodes.
- A retractile testis can usually be moved into the scrotum and will remain there until it retracts back into the groin again with a cremasteric reflex (eg touching the inner thigh).
- Look at the femoral, penile and perineal region for ectopic testes.
- Diagnostic laparoscopy is the only way to confirm or rule out an intra-abdominal, inguinal or absent/vanishing testis (non-palpable testis). However, an examination under anaesthetic should be carried out before laparoscopy, as a previously non-palpable testis may become palpable.
- Abdominal and pelvic ultrasonography may be required if intersexuality is suspected.
Syndromes associated with cryptorchidism
- Prader-Willi syndrome.
- Kallmann's syndrome.
- Laurence-Moon-Biedl syndrome.
- Intersexuality/congenital adrenal hyperplasia.
- Prune belly syndrome.
- If, by the age of 1 year, descent has not occurred, spontaneous descent is unlikely. Treatment should be initiated, as there is also potential for histological deterioration and loss of testicular quality (may affect future fertility).
- Treatment should be completed by 12-18 months of age.
- American studies showed that guidelines were not always adhered to and that the timing of surgery varied greatly from one hospital to another.
- Testicular descent is hormonally dependent.
- Treatment with human chorionic gonadotrophin (hCG) or gonadotrophin-releasing hormone (GnRH) can be used.
- Success rates are best the lower the undescended testis is located.
- Maximum success rates are 20%.
- Medical treatment may be useful before or after surgery and may have a beneficial effect on later fertility. However, this view is not universally held.
- Side-effects of hCG treatment can include enlargement of the penis, pubic hair growth, increased testicular size and aggressive behaviour during treatment.
- If the testis is palpable: an inguinal approach is usually used. Orchidopexy or orchidofuniculolysis (mobilisation of the testis and cord) can be performed. Success rates are up to 92%. Orchidopexy involves mobilisation of the testis on its essential structures (the vas, the testicular vessels and the spermatic cord) so that the testis can be brought down into the scrotum. The testis may also be fixed within the scrotum. Some authorities recommend orchidopexy as early as 6 months in order to preserve spermatogenesis. One study reports the successful use of trans-scrotal orchidopexy.
- If the testis is non-palpable: examination under anaesthetic may reveal the previously non-palpable testis. If not, inguinal surgical exploration ± laparoscopy is needed. Every attempt should be made to locate the non-palpable testis. It is likely that the testis will be found just through the inguinal incision but laparoscopy may be needed to search the abdomen. Removal, orchidolysis or orchidopexy can then be performed laparoscopically.
For postpubertal men, the current guidelines are:
- Age 32 or younger with a unilateral undescended testis and normal contralateral testis - orchidectomy.
- Older than 32 with a unilateral undescended testis - close observation and physical examination; orchidectomy or orchidopexy if physical examination is difficult.
- Increased risk of testicular torsion. This may be associated with the development of a testicular tumour. Torsion of an intra-abdominal testis may present as an acute abdomen.
- Increased risk of testicular trauma.
Effect on fertility
- Boys with one undescended testis have a lower fertility rate but the same paternity rate as boys with bilateral descended testes.
- Boys with bilateral undescended testes have lower fertility and paternity rates.
Risk of testicular malignancy
- There is a history of cryptorchidism in 5-10% of testicular cancers.
- Prepubertal orchidopexy for cryptorchidism may be associated with a lower risk of testicular cancer.
- Orchidopexy facilitates testicular self-examination.
- Surgical transfer of the testis into the scrotum produces a better cosmetic appearance.
- Prostheses may be used if the testis is removed. Prostheses should be implanted during adolescence.
Prevention of complications
The 'National Screening Committee Policy - cryptorchidism screening' agrees that screening for undescended and maldescended testes should take place in the routine physical examination of boys within 72 hours of birth and at the six- to eight-week check. Screening - during and after puberty - is also recommended for boys with a history of undescended testis because of the risk of testicular malignancy. The policy is due to be reviewed in 2014/2015.
Further reading & references
- Park K, Choi H; An evolution of orchiopexy: historical aspect. Korean J Urol. 2010 Mar;51(3):155-60. Epub 2010 Mar 19.
- Imamoglu M, Bulbul SS, Kaklikkaya N, et al; Oxidative, Inflammatory and Immunologic Status in Children with Undescended Pediatr Int. 2012 Jul 11. doi: 10.1111/j.1442-200X.2012.03695.x.
- Marchetti F, Bua J, Tornese G, et al; Management of cryptorchidism: a survey of clinical practice in Italy. BMC Pediatr. 2012 Jan 10;12:4.
- Sumfest J et al, Cryptorchidism, Medscape, Jan 2012
- Mouriquand P; The nomad testis. Arch Dis Child. 2007 Jan;92(1):3.
- Yoshida T, Ohno K, Morotomi Y, et al; Clinical and pathological features of ascending testis. Osaka City Med J. 2009 Dec;55(2):81-7.
- Eijsbouts SW, de Muinck Keizer-Schrama SM, Hazebroek FW; Further evidence for spontaneous descent of acquired undescended testes. J Urol. 2007 Oct;178(4 Pt 2):1726-9. Epub 2007 Aug 17.
- Hack WW, van der Voort-Doedens LM, Goede J, et al; Natural history and long-term testicular growth of acquired undescended testis BJU Int. 2010 Oct;106(7):1052-9. doi: 10.1111/j.1464-410X.2010.09226.x. Epub 2010
- Dawson C, Whitfield H; ABC of urology. Common paediatric problems. BMJ. 1996 May 18;312(7041):1291-4.
- Abouzeid AA, Mousa MH, Soliman HA, et al; Intra-abdominal Testis: Histological Alterations and Significance of Biopsy. J Urol. 2010 Nov 13.
- Abdullah NA, Pearce MS, Parker L, et al; Birth prevalence of cryptorchidism and hypospadias in northern England, 1993-2000. Arch Dis Child. 2007 Jul;92(7):576-9. Epub 2006 Dec 1.
- Thorup J, McLachlan R, Cortes D, et al; What is new in cryptorchidism and hypospadias--a critical review on the J Pediatr Surg. 2010 Oct;45(10):2074-86.
- Jensen MS, Bonde JP, Olsen J; Prenatal alcohol exposure and cryptorchidism. Acta Paediatr. 2007 Nov;96(11):1681-5. Epub 2007 Sep 19.
- Virtanen HE, Tapanainen AE, Kaleva MM, et al; Mild gestational diabetes as a risk factor for congenital cryptorchidism. J Clin Endocrinol Metab. 2006 Dec;91(12):4862-5. Epub 2006 Oct 10.
- Thorup J, Cortes D, Petersen BL; The incidence of bilateral cryptorchidism is increased and the fertility potential is reduced in sons born to mothers who have smoked during pregnancy. J Urol. 2006 Aug;176(2):734-7.
- Virtanen HE, Sadov S, Toppari J; Prenatal exposure to smoking and male reproductive health. Curr Opin Endocrinol Diabetes Obes. 2012 Jun;19(3):228-32.
- Snijder CA, Kortenkamp A, Steegers EA, et al; Intrauterine exposure to mild analgesics during pregnancy and the occurrence of Hum Reprod. 2012 Apr;27(4):1191-201. Epub 2012 Feb 2.
- Guidelines on Paediatric Urology, European Association of Urology (2011)
- Perez-Brayfield M et al, Pediatric Cryptorchidism Surgery, Medscape, Nov 2011
- Bayne AP, Alonzo DG, Hsieh MH, et al; Impact of anatomical and socioeconomic factors on timing of urological J Urol. 2011 Oct;186(4 Suppl):1601-5. Epub 2011 Aug 19.
- Kokorowski PJ, Routh JC, Graham DA, et al; Variations in timing of surgery among boys who underwent orchidopexy for Pediatrics. 2010 Sep;126(3):e576-82. Epub 2010 Aug 23.
- Ludwikowski B, Gonzalez R; The controversy regarding the need for hormonal treatment in boys with unilateral Eur J Pediatr. 2012 Mar 7.
- Hutson JM, Balic A, Nation T, et al; Cryptorchidism. Semin Pediatr Surg. 2010 Aug;19(3):215-24.
- Gordon M, Cervellione RM, Morabito A, et al; 20 years of transcrotal orchidopexy for undescended testis: results and outcomes. J Pediatr Urol. 2010 Oct;6(5):506-12. Epub 2009 Nov 26.
- Guidelines on Male Infertility, European Association of Urology (2012)
- Cryptorchidism: The UK NSC policy on Cryptorchidism screening in newborn boys, UK National Screening Committee Policy Database, 2012
|Original Author: Dr Michelle Wright||Current Version: Dr Laurence Knott||Peer Reviewer: Prof Cathy Jackson|
|Last Checked: 14/08/2012||Document ID: 2899 Version: 22||© EMIS|
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