Cryptorchidism



What is Cryptorchidism?

The term comes from ancient Greek. Crypt = hidden, Orchid = testicle.

Most baby boys are born with two testicles already present in their scrotum. But in some boys, one or both seem to be missing. There are several possible reasons for this:
The testicle failed to descend - hence the layman’s expression "undescended testicles" - and it remains stuck inside the abdomen. When only one is out of place, the case is described as Unilateral. If both are apparently absent, the case is described as Bilateral. (More about testicles forming within the abdomen later.)

Descent took place, but the testicle took a wrong turning. They can end up in peculiar places when this happens, such as part-way down the inner thigh! This is sometimes described as Maldescent and the testicle is said to be Ectopic.

Anorchia. In truth this isn’t Cryptorchidism at all, but is mentioned here for the sake of giving a complete explanation of why a boy’s "balls" may be missing. Anorchia is a congenital absence of testicles; in other words they never formed in the first place. Such a boy is inevitably sterile and will not go through puberty unless given artificial hormones.

The following artist’s impression illustrates an undescended left testicle. During the pregnancy it should have migrated into the scrotum, but failed to do so. Late natural descent is unlikely because the gap in the abdominal wall closes in the early months of life.


Unilateral (left) cryptorchidism

Undecended left testicle (10444 bytes)

Image © Unknown.




Incidence of Cryptorchidism

Like hypospadias, cryptorchidism is on the increase. The definitive work on this subject is probably Thonneau, Candia and Mieusset (2003). Their literature search suggests that the incidence of cryptorchidism amongst full-term male babies in some parts of Western Europe and North America has risen from under 1.5% in 1952 to around 3% a mere 50 years (approximately two generations) later. About 30% of cases are bilateral.



What are testicles doing in the abdomen in the first place?

When a baby is conceived, its chromosomal gender is determined by the father’s sperm. Mum always contributes an X-chromosome. If Dad also contributes an X-chromosome then the result is XX and the child will be a girl. If Dad contributes a Y-chromosome then the result is XY and the child will be a boy. However, despite gender being determined at conception, the foetus spends the first eight or so weeks of the pregnancy trying to grow into both a boy and a girl. Only once the ability to produce the hormone Testosterone has developed does an XY foetus begin 'changing' into a boy. Note that the female state is the default condition; if the production of testosterone is inadequate then incomplete masculinisation (in effect partial feminisation) of the baby will result.

The undifferentiated early development gives rise to some vestigial evidence. It’s why boys have nipples. Likewise, a woman’s clitoris is what would have grown into a penis had she been male. But that’s not quite the end of the story. Some organs have already partially developed but go on to become totally different body parts in males and females. Key to understanding cryptorchidism is to realise that ovaries and testicles start off as the same thing, the proto-gonads. If the child is to be a girl, these immature organs stay put and become ovaries. If the child is to be a boy they will grow into testicles, in consequence needing to migrate in order to reach their proper place in the scrotum. Cryptorchidism occurs when that process of migration goes wrong, for whatever reason.



Consequences of Cryptorchidism

There are two principal consequences of cryptorchidism - infertility and cancer. The reason why testicles hang outside the body is that they need to keep just a little cooler than body temperature in order to do their job of producing sperm; inside the abdomen it’s too hot. Observe how, on a hot day, a male’s testicles hang lower. Conversely, after taking a cold bath, they snuggle up close to the body for extra warmth. An undescended testicle will always be too hot and in consequence its fertility will decline. It also becomes prone to developing testicular cancer, even if subsequently repositioned in the scrotum.



Corrective surgery  ("Orchidopexy")

The decline in fertility and the tendency for the undescended testicle to become malignant both increase the longer the condition remains uncorrected. Thus early intervention is preferable. Circa 1960 the norm was to operate just pre-puberty but nowadays intervention is usually much earlier, typically before the age of 4 years. The invention of ultrasound scanning has been of major importance here; prior to that technical innovation it was often difficult to locate the missing organ until it started to swell with the onset of secondary sexual development.



Failures of orchidopexy surgery

In this brief overview of Cryptorchidism, we need consider only two scenarios, the problems of 'short plumbing' and 'twisted plumbing'.

Particularly in cases where the undescended testicle is high in the abdomen, close to the correct position for a girl’s ovary, it can often be the case that the Vas Deferens (the spermatic cord) and/or the blood vessels supplying the testicle are not long enough to reach the scrotum. Such cases may be amenable to a 'two-stage orchidopexy'; full details of that procedure are outside the scope of this article. A CIRCLIST member with experience of it at age 11 reports it as being somewhat painful and restrictive in the months between the two operations.

If the 'short plumbing' problem is insuperable, the surgeon may perform an orchidectomy even though the testicle itself is viable. That’s in effect a castration, done in order to remove the potential source of cancer that would result if the testicle was left within the abdomen.

'Twisted plumbing' is properly termed Testicular Torsion. It is possible that the testicle may already have died ("become necrotic") before surgery commences. If necrotic tissue is found it is imperative to remove it immediately in order to prevent infection resulting from exposure of the dead tissue to the air. Identification of a necrotic testicle is fairly certain, it will be black in colour.

Loss of one testicle, for whatever reason, does not of itself bring about a total loss of fertility. Neither does it mean that all children fathered will be of the same sex - the story that girls come from the right testicle and boys from the left is superstitious nonsense!

Cosmetic implants are available to counter any embarrassment arising from the absence of one or both testicles. The quality of such prosthetic devices has improved hugely in recent years.



Implications regarding circumcision

Unilateral cryptorchidism is not a contra-indication to neonatal circumcision. Unlike hypospadias and epispadias repairs, no donor tissue is required for orchidopexy. If only one testicle is undescended, parents favouring neonatal circumcision need not hold back on their intent. However they might consider the possibility of deferring the circumcision until the orchidopexy is performed, then combining the two surgeries under one general anaesthetic.

Bilateral cryptorchidism is regarded as a manifestation of ambiguous genitalia and for that reason is considered to be a contra-indication to neonatal circumcision. If and when it has been established that the boy requires only an orchidopexy and is otherwise normal, circumcision might then proceed under the guidance of the paediatrician dealing with the Intersex assessment.



Female equivalents

There is no direct female equivalent of an undescended testicle; if the proto-gonad is destined to become an ovary it’s meant to stay put. However, ovaries can wander out of position, suffer torsion problems and become necrotic as a result. This necessitates removal, the proper name for the procedure being oophorectomy.



Acknowledgements

The following resources were used in the preparation of this web page:
Globe (2229 bytes) Yahoo! logo (1999 bytes) Archives of the Yahoo! Group 'Cryptorchidism'.
Spanish flag (1357 bytes) Vall d’Hebron Hospital logo (1890 bytes) Aso, C. et al. (2005) Gray-Scale and Color Doppler Sonography of Scrotal Disorders in Children: An Update. RadioGraphics 2005;25:1197-1214.
UK flag (1443 bytes) St.George’s Hospital (London) Medical School logo (4534 bytes) Nussey, S.S. and Whitehead, S.A., Endocrinology - An integrated approach. London : Taylor & Francis, 2001. Their excellent explanation of testicular development and migration is available online here, starting at the second paragraph of the section headlined "Sexual differentiation of the gonads and internal reproductive tracts".
French flag (1767 bytes) La Grave Hospital, Toulouse logo (2582 bytes) Thonneau, P.F., Candia, P. and Mieusset, R. Cryptorchidism: Incidence, Risk Factors and Potential Role of Environment; An Update. Journal of Andrology, Vol. 24, No. 2, March/April 2003, pp.155-162.
UK flag (1443 bytes) Birmingham Children’s Hospital logo (3579 bytes) Medscape logo (5542 bytes) Medscape article number 1017689
Testicular Torsion : Govindarajan, K.K. et al.
USA flag (1336 bytes) Stanford School of Medicine logo (2730 bytes) Website of Stanford School of Medicine.
Thai flag (351 bytes) Chula Faculty of Medicine logo (18627 bytes) The Online Learning facilities of Chulalongkorn University Faculty of Medicine, Bangkok.
Globe (2229 bytes) Circlist Website logo (6480 bytes) Circlist Group logo (8847 bytes) Personal testimony of members of the CIRCLIST discussion group.




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