Professor of Urology in the Faculty of Medicine, University of Calabar, Paul Ekwere, tells EMMANUEL OJO about the diagnosis and management of monorchidism; a condition that leaves a male with one testicle
What is monorchidism?
Monorchidism is the situation of having one testicle. That is just the definition. The situation of having just one testicle may be as a result of several things.
What’s the normal number of testicles a male is required to have?
It is two. That’s the way the Lord made it.
Are there other possible situations where an individual can have more than two testicles?
Yes. Sometimes you have polyorchidism where a person can have a third testicle, so that you find out that in one person, one side of the scrotum (the thin external sac under the penis which houses the testicle) may have two and the other side may have one but it is a very rare condition. It is extremely rare to have it that way.
How common is the monorchisdism?
Monorchidism is even a rare condition whereby you have it only in about three to four per cent of all normal birth, which means that in about a hundred live babies that are being born, you find it in only three or four of them. So, out of every hundred boy, about three or four may have monorchidism.
What are the causes of monorchidism?
Well, the causes may be what we describe as congenital or it may be acquired. When we say congenital, it means that the child is born with that condition and when we say acquired, it means that something has happened, maybe to one testicle so that after the child has been born, he now ends up with one; so, there is a condition whereby during development, one testicle may disappear. This comes under the congenital category that I just mentioned. That is called ‘vanishing testis’ and it is caused by an intrauterine accident, injury or assault, when the foetus is still in the womb. That can happen and it is thought to be more of vascular accident and the closest example we have to that is the distortion of the testes. When the testes are distorted, you find out that the blood supply to the testes is cut off as a result of the twisting of the cord. So, when the blood supply is cut off, the testicle just dies and vanishes, so that when a child is born, you will not see any testicle there.
Then the causes can be as a result of the situation where the testis hasn’t descended. Maybe only one has descended into the scrotum during normal embryonic or foetal development. This condition is also called undescended testis or cryptorchidism.
The undescended testis may pass within the abdominal cavity, near the back while one descends into the scrotum and then you have a situation where the person has one visible testicle while the other one is still inside the abdomen or if it has tried to descend, it may be in the area described as the inguinal canal. In fact, that area is the commonest site for undescended testis. Technically, the person may have one testicle which I will now describe as one visible testicle because it’s the one that has descended whereas the other is still hidden in the abdomen or it’s track, trying to come down or what we call ‘ectopic position,’ which may be anywhere within the base of the penis, the groin or even the shaft of the penis itself. The testicle may be implanted in any of those places.
Apart from that, we have a condition in which one testicle has been surgically removed. That you may find in people who either have injury or malignancy, affecting one testicle, leading to surgical removal. On the other hand, the testicle may have suffered injury from trauma by either playing football or other games to the extent that it cannot be repaired. So, it will be removed and that will mean that the person in question will be seen with only one testicle, which is monorchidism.
Is there also a possible situation where a person will have no testicle?
Yes, like the one I just described; a situation where the testicle has not descended. That may sometimes be both sides. It can be both testicles not descended. In other words, they are trapped somewhere along the line so that they don’t enter the scrotum and how we got to know about that is that when you examine a baby, you find a scrotum that is being described as hypoplastic; that is, it has not developed beyond a certain stage which means that no testicle has descended into that scrotum. We must underline this with a situation where we have one or both testicles being retractile; we call that retractile testicle. There is something we call a ‘cremaster reflex’, that is if you stroke the side of the thigh of a baby, you will find out that the testicle reacts by going up. It just disappears. The difference between that and the undescended testis bilateral is that you find that the scrotum in this case has been well developed, meaning that testicle has entered that scrotum and it is not there only because it disappeared as a result of the cremasteric reflex that we spoke about. That is the difference. The difference is that in the other one, the testis has not entered the scrotum at all, not one. They are arrested in the abdomen or on its way out.
Are there risk factors that predispose one or a child to this situation?
Yes. We know that hormonal imbalance in the mother during development can predispose a male child to this. During development, certain parts of the developing testis produce certain hormones, one of which is testosterone; the male hormone, the other one is called the anti-müllerian hormone; those are the hormones that will develop the testis and then the testosterone will then aid it to come out. There is something we find at the lower end of a developing testicle which we call gubernaculum. Now, when that is not well developed, the testis will not descend. If it descends, it will be in an ectopic position, which is like deviating, instead of taking the normal path. That’s basically what that means.
Does low weight at birth also have a predisposing factor?
Well, if the child is premature, a large number of premature male child are born with the testicle not well descended. Descent of the testicle happens in the seventh month of gestation. If there has been any delay and that child is born prematurely, you can imagine that there won’t be any descent. The good thing is that most of the children, at least 50 per cent, who are born that way, within the first few months, the testicle will now descend, the rest will have to wait for an intervention from the doctors.
What kind of intervention comes from the doctors?
Those days, in the earlier days, they used to be given some hormones; developmental hormones, which will aid the descent, but these days, we do it surgically, a condition we call orchidopexy. We bring the testis down; we get it from where it is after searching for it. We get it down into the scrotum and fix it there and it stays there. That’s the surgical procedure we do.
Are there side effects that can come up from the intervention by the doctors surgically or hormonally?
Of course, every surgery has its possible complications and all that, which in modern times, are really insignificant and if an inexperienced person handles it, it can end up fixing the testicle and it is too tight and when that happens, you may actually destroy the testicle if it is handled by an inexperienced person because he implants it in the scrotum and make the cord very tight. When that happens, it cuts off the blood supply and what you are trying to secure now becomes a disaster for the patient. So, it is always good for this to be handled by experienced people; urology or paediatric surgeons.
Are there other complications with monorchidism?
Yes. What I will say there is that a testis that is not descended is more prone to developing cancer. So, if it turns out that one testis is down in the scrotum and the other one is not there, and it’s due to the conditions that I have earlier enumerated, then that is prone to become cancerous and if that happens, then, we may not see it in time to save the situation. If it has been brought down, it may still develop that malignancy, but it is now in a position that it can easily be seen and dealt with by the specialist doctor, so that it does not become complicated.
What impact does monorchidism have on fertility?
Well, if you have only one testicle; which is why the good Lord gave us two, so that if something happens to one, the other one can still function. The function of the testicle is not only to produce sperm but it produces hormones for the male. The male hormones depend on it. If a person has one testicle, he can still be fertile, unless there are other issues involved, in which case those issues will have to be defined and taken care of. The mere fact that one has one testicle does not mean that one will automatically become infertile, unless in the process whereby only one descended and the other one in the abdomen or ectopic position is left there.
The trouble is that that testicle may become injured due to its position and it may develop into something we call ‘anti-sperm antibodies’, which is the body developing a system, which recognises its own tissues as if it is a foreign tissue and it goes ahead to attack it. If that happens, even the normal testis that is descended into the scrotum now becomes a victim of that and the body will now produce antibodies against that testicular tissues, thus, making it incapable of producing sperms or quality sperms to cause fertility. Those are possibilities.
Does it also affect the sexual drive in such a man?
Yes. If the testis is not there, then of course, it means the hormone is not there and it’s as good as being a Eunuch. One testis can perform the function of two. If there’s a normal one testis that’s not affected, that testis will function normally and give one the sexual drive that he needs and the potency that is needed will be there because it will produce the hormones require to drive such functions.
How is the condition of monorchidism and undecended testicle diagnosed?
When we have a patient, we usually ask questions from the mother; ‘who is the closest person to the baby?’ Some of them may tell you that ever since he was born, they didn’t see any testicle and that’s why we usually encourage mothers to examine their babies right from the start. Some will tell you that they didn’t see it and some will say that they only saw one. Some will even say that they didn’t see anything, but when you examine, you will find the testis somewhere; either in the inguinal canal or somewhere.
How do you feel the testis?
It is done by palpation, using your hand. Every doctor that is trained is trained to use his or her hands to palpate tissues and you will feel the testis that is not in the scrotum going along the lines that you expect the testis to have descended through. You may feel or check the areas you expect the ectopic testicle to be there and when you do, you know that that is what it is. If it’s a retractile testicle, you know that that is easier to detect and when you detect it, you try to persuade it to come down and if it comes down, it means that it was truly a retractile testicle.
On the other hand, you may examine the patient and still not find it. So, we may resort to what we call imaging techniques. One of them is using ultrasound machine, what a layman refers to as scanning. It is an ultrasound machine that we use to see if we can locate the testicle anywhere in the body, including on the inside of the abdomen, towards the back; so, that’s the thing. If we cannot find it on ordinary ultrasound, then we go up higher to what we call CITI scan or MRI. Those are all imaging techniques that we can use in order to locate where an undescended testicle is. Those are ways that we diagnose the condition and the treatment is what we have already talked about.
How does the scrotal sac look like when the testicles are yet to descend into it?
The fact that the scrotum is hypoplastic means that no testicle has descended into it. So, that’s the first sign you see. There is also a condition where the penis and the scrotum are not developed at all. That is even a more sinister condition that you can find, but when we say hypoplastic, we mean that the scrotum has not developed properly to its optimum. That’s the description of that.
