Urology Surgeries in Children

Undescended testicle - What is a shy testicle?

How is it monitored, when should it be operated?

Undescended testis is the most common congenital condition in boys, characterized by the testicle not being palpable in the scrotum (sac). An undescended testicle is the absence of unilateral or bilateral testicles in the scrotum during examinations from the day of birth.

Frequency of occurrence

Undescended testis is seen between 0.8-1% at the age of 1 year.¹ The rate of preterm birth and/or under 2500 g is 1.1- 45.3%, 1.7-5.2% at 3 months of age and 1.9-7.3% at 1 year of age. The rate of preterm birth and/or over 2500 g is 1.0-4.6%, 0.9-1.6% at 3 months of age and 1.0-1.5% at 1 year of age.² In one third of cases, undescended testis is bilateral.¹

Definitions

Undescended testicle

An undescended testicle is a testicle that cannot be held firmly in the scrotum, cannot be lowered into the scrotum and causes pain due to the stretching of the spermatic cord when it is tried to be lowered.

Retractile testis (shy testicle)

Retractile testis is a variant of the normal testicle and is defined as a testicle that spontaneously leaves the scrotum and spontaneously returns to the scrotum and can remain in the scrotum for a certain period of time.³ In addition, testicles that are easily lowered to the lower part of the scrotum, no pain is felt during the stretching of the cord structures during lowering, and testicles that remain in the scrotum until the cremasteric reflex occurs are also considered retractile testicles. These testicles usually remain in place until puberty and retain their normal fertility (the ability to have children). The distinction between a retractile testicle and an undescended testicle can sometimes be difficult. For this, it is necessary to pay attention to 3 details in physical examination. The undescended testicle is smaller than the opposite testicle. As soon as it is lowered, it quickly escapes into the canal and if it is forced to be lowered into the scrotum, pain occurs due to tension. Some authors accept two of these three conditions as undescended testicle and perform surgery.⁴ Retractile testes have normal consistency and size. Agarwal et al. reported that 30% of retractile testes descended, 32% were marked undescended testes and 38% were still retractile during follow-up.⁵

Diagnosis

Anamnesis and physical examination are important in the diagnosis. Imaging methods to determine the localization of the testicles have no additional contribution. Early diagnosis can be made in the genital examination of the newborn boy.

Physical examination should be performed in a quiet, calm and warm environment. Examination of the inguinal region and scrotum by an experienced doctor is sufficient for diagnosis. Ultrasonography will not be of additional benefit. Ultrasonography may be ordered if the child is not cooperative during the examination and the examination is inadequate.

In unilateral undescended testis, it is also important to evaluate the other testicle. If there is a testicle that cannot be palpated on one side, the contralateral testicle should be examined in detail. The size and location of the contralateral testicle is very important. Compensating hypertrophy indicates that the undescended testicle has either atrophied or is not congenital.

Patients with bilateral non palpable testicles require genetic and endocrinologic investigation.

Imaging studies

Imaging methods do not show with certainty whether testicles are present or not.⁶ Ultrasonography and magnetic resonance imaging methods can be used for imaging. Ultrasonography and magnetic resonance imaging are non-invasive, but costly, time consuming and less accurate. In addition, magnetic resonance imaging can be performed under anesthesia. This will lead to a second anesthesia for most of the patients who will undergo surgery. Computed tomography is not ordered to avoid radiation damage.

Treatment

As soon as the diagnosis of undescended testicle is made, surgery should be performed at the earliest (from 6 months of age). The aim here is to increase the chance of adequate hormone production and to protect future fertility (the ability to have children), to prevent unnecessary imaging studies, to reduce the anxiety of the family, to repair the concomitant hernia, if any, to prevent malignancy (transformation into a malignant tumor) that may occur in the future, and to prevent psychological damage by placing it in the scrotum and keeping it in normal anatomy.

Treatment should be started from 6 months of age. The intervention to lower the testicle should be completed by 12 months, at the latest by 18 months.

Hormone therapy

The success rate of treatment with HCG and GnRH hormones is maximum 20%.⁷ With hormone therapy, 20% of the descended testicles go back up again. When HCG and GnRH are given together, the success rate is 28%. In addition, HCG and GnRH treatments have been reported to cause increased apoptosis in germ cells, inflammatory changes in the testicles, and decreased testicular volume, which may damage spermatogenesis in the future.⁸ In 2008, a group of physiologists, surgeons, endocrinologists, andrologists, pathologists and anesthesiologists from the Nordic countries published a consensus. Accordingly, they reported that hormone therapy is not recommended, that the results are not good and that it has a negative effect on spermatogenesis in the long term and that surgery should be preferred.⁹

Possible long-term consequences

One of the long-term consequences of undescended testis is reduced or lost fertility due to inadequate spermatogenesis and the other is the development of testicular cancer.

Decreased fertility (ability to have children)

Inadequate spermatogenesis leads to decreased fatalities. While the fertility rate is unchanged in unilateral undescended testis, it decreases to 33-65% in bilateral undescended testis.¹⁰ Although testicular location and length of stay affect leydig and germ cell loss, germ cell loss is most prominent in testes that are not lowered at two years of age and are located in the abdomen.¹¹

Although fertility in undescended testis is affected by many factors, it is attributed to inadequate development of germ cells, reduction of Leydig cells and testicular fibrosis.^12-14 Although the fertility rate is reduced in unilateral undescended testes, the paternity rate is the same as in those with normal testicles. In those with bilateral undescended testicles, both fertility and paternity rates are low.

In untreated bilateral undescended testes, 100% were oligospermic and 75% azoospermic, while in successfully reduced bilateral undescended testes, 75% were oligospermic and 42% azoospermic.¹⁴ Although it is recommended to perform undescended testicular surgery before 12 months of age, it should be performed at the latest at 18 months to preserve fertility. According to Tasian et al., in undescended testis, there is a 2% loss of germ cells and 1% loss of leydig cells for each month over the age of 1 year.¹¹

Risk of developing testicular cancer

In a study conducted in Sweden, testicular cancer was found in 56 of 17000 adults who underwent surgery for undescended testis in childhood. In this study, it was shown that patients who underwent orchiopexy for undescended testis after the age of 13 had 2 times more testicular cancer than those who underwent orchiopexy before the age of 13.¹⁵ Since 11% of adult men with testicular cancer have a history of undescended testis, there is a link between undescended testis and testicular cancer. In the last few studies, it has been shown that the relative risk of testicular cancer development in children with undescended testis is between 2.75-8%.^16,17 The rate of cancer development is 2.23% even in testes lowered in the prepubertal period and this rate is higher than in patients without undescended testes. Orchiectomy is recommended when intraabdominal testis or hypotrophic inguinal testis is detected after puberty. 3-5% of testicular germ cell tumors are in cases of undescended testis. This rate is 4-7 times higher than the rate in healthy people.¹⁸

Follow-up

In adolescents with a history of undescended testicle surgery, the individual is asked to perform a monthly self-examination. If an abnormal growth is detected in the testicle during the examination, it should be evaluated by ultrasonography and alpha-fetoprotein, beta HCG and LDH levels, which we call tumor markers, should be checked. Annual examination is recommended in retractile testes. Testicular prosthesis can be applied in testicular atrophy. In bilateral atrophy, testosterone should be given under the supervision of an endocrinologist.

Result

İnmemiş testis tanısında radyolojik incelemelerin yeri yoktur ve  tanı fizik muayene ile konulur. İnmemiş testisler en geç 18 aylık olana kadar ameliyat edilmelidir. İnmemiş testisin hormonla tedavisi günümüzde kabul görmemektedir. Retraktil testislerin üçte biri inmemiş testis haline dönüşmesi nedeni ile yakın takip edilmelidir. Tedavisi gecikmiş çift taraflı inmemiş testis olan hastalarda fertilite oranı düşmektedir. Puberte sonrası inmemiş testis ameliyatı olan hastalarda kanser gelişme ihtimali oldukça yüksektir. İnmemiş testis ameliyatı olan hastalar periyodik fizik muayeneler, ultrasonografi ile değerlendirilme ve kan tahlillerinde kanser belirteçlerine  bakılarak takip edilebilir.

Referanslar

1. Berkowitz GS, Lapinski RH, Dolgin SE, Gazella JG, Bodian CA, Holzman IR. Prevalence and natural history of cryptorchidism. Pediatrics. 1993 Jul;92(1): 44-9.
2. Sijstermans K, Hack WW, Meijer RW, van der Voort-Doedens LM. The frequency of undescended testis from birth to adulthood: a review. Int J Androl. 2008 Feb;31(1):1-11.
3. Barthold JS, González R. The epidemiology of congenital cryptorchidism, testicular ascent and orchiopexy. J Urol. 2003 Dec;170(6 Pt 1):2396-401.
4. Wyllie GG. The retractile testis. Med J Aust. 1984 Mar 31;140(7):403-5.
5. Agarwal PK, Diaz M, Elder JS. Retractile testis–is it really a normal variant? J Urol. 2006 Apr;175(4):1496-9.

6. Hrebinko RL,Bellinger MF. The limited role of imaging techniques in managing children with undescended testes. J Urol. 1993 Aug;150(2 Pt 1):458-60.
7. Pyörälä S,Huttunen NP, Uhari M. A review and meta-analysis of hormonal treatment of cryptorchidism. J Clin Endocrinol Metab. 1995 Sep;80(9):2795-9.
8. Cortes D,Thorup J, Visfeldt J. Hormonal treatment may harm the germ cells in 1 to 3-year-old boys with cryptorchidism. J Urol. 2000 Apr;163(4):1290-2.
9. Ritzén EM. Undescended testes: a consensus on management. Eur J Endocrinol. 2008 Dec;159 Suppl 1:S87-90.
10. Lee PA. Fertilityafter cryptorchidism: epidemiology and other outcome studies. Urology. 2005 Aug;66(2):427-31.
11. Tasian GE, Hittelman AB, Kim GE, DiSandro MJ, Baskin LS. Age at orchiopexy and testis palpability predict germ and Leydig cell loss: clinical predictors of adverse histological features of cryptorchidism. J Urol. 2009 Aug;182(2):704-9.
12. Trussell JC, Lee PA. The relationship of cryptorchidism to fertility. Curr Urol Rep. 2004 Apr;5(2):142-8.
13. Hadziselimovic F,Herzog B. The importance of both an early orchidopexy and germ cell maturation for fertility. Lancet. 2001 Oct 6;358(9288):1156-7.
14. Lee PA. Fertility after cryptorchidism: epidemiology and other outcome studies. Urology. 2005 Aug;66(2):427-31.
15. Pettersson A,Richiardi L, Nordenskjold A, Kaijser M, Akre O. Age at surgery for undescended testis and risk of testicular cancer. N Engl J Med. 2007 May 3;356(18):1835-41
16. Wood HM,Elder  Cryptorchidism and testicular cancer: separating fact from fiction. J Urol. 2009 Feb;181(2):452-61.
17. Cook MB,Akre O, Forman D, Madigan MP, Richiardi L, McGlynn KA.  A systematic review and meta-analysis of perinatal variables in relation to the risk of testicular cancer–experiences of the son. Int J Epidemiol. 2010 Dec;39(6):1605-18.
18. Hack WW, van der Voort-Doedens LM, Goede J, van Dijk JM, Meijer RW,Sijstermans  Natural history and long-term testicular growth of acquired undescended testis after spontaneous descent or pubertal orchidopexy. BJU Int. 2010 Oct;106(7):1052-9.