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Undescended Testicle • Special situations

Testicular Agenesis: What Happens When a Testicle Never Formed?

Testicular agenesis means the testicle never formed at all in the womb, and true agenesis is genuinely very rare; in the large majority of non-palpable testicles, the testicle either lies inside the abdomen or formed and was later lost. The distinction is made at laparoscopy; when both testicles are absent, hormonal evaluation and endocrinology support come into play.

Who this is forFamilies of children in whom no testicle or testicular vessels are found at laparoscopy
Reading≈6 min
ByProf. Dr. Ali AvanoğluUpdated
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01 · Are agenesis and vanishing testis the same thing?02 · How rare is it?03 · The approach to unilateral absence04 · Bilateral absence (anorchia): hormonal evaluation05 · Endocrine support and follow-up in anorchia
In brief5 madde
  • Are agenesis and vanishing testis the same thing?: Both end in the same finding — no testicle — but the underlying process is different, and the laparoscopy (minimally invasive) findings reveal that difference.
  • How rare is it?: Most children with a non-palpable testicle actually have a testicle; true absence accounts for only a small minority.
  • The approach to unilateral absence: In unilateral agenesis, the child has one healthy testicle, and it is usually sufficient for hormone production and fertility.
  • Bilateral absence (anorchia): hormonal evaluation: When neither testicle is palpable, the picture is different: hormonal evaluation is carried out before laparoscopy, and pediatric urology, endocrinology, and, when…
  • Endocrine support and follow-up in anorchia: Once the absence of both testicles is confirmed, pediatric endocrinology is involved in follow-up from the very start, because there is no tissue left to produce the…
01

Are agenesis and vanishing testis the same thing?

Both end in the same finding — no testicle — but the underlying process is different, and the laparoscopy (minimally invasive) findings reveal that difference.

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  • In vanishing testis, the testicle formed and was later lost to a blood supply problem; laparoscopy shows blind-ending vessels and the sperm duct (vas deferens). In true agenesis, because the testicle never formed at all, the vessels — and often the sperm duct as well — never developed either; laparoscopy finds neither a testicle, nor vessels, nor a duct on that side. Developmental problems of the kidney and urinary tract on the same side may accompany it.
  • Clinically, the two conditions are managed in a similar way: there is no testicle to bring down, no further procedure is needed, and attention turns to the remaining testicle — or, if bilateral, to the hormonal picture. A definitive diagnosis is made only by laparoscopy; ultrasound or MRI failing to show the testicle is not enough to diagnose agenesis.
02

How rare is it?

Most children with a non-palpable testicle actually have a testicle; true absence accounts for only a small minority.

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  • In the literature, approximately half of non-palpable testicles turn out to be living testicles located inside the abdomen or the inguinal canal, while the rest are reported as vanishing testis or a nubbin (atrophic remnant); true agenesis, in which no vessels or duct are found at all, makes up only a small share of these. Bilateral absence of the testicles (anorchia) is far rarer still.
  • This is why it is not appropriate to simply assume 'no testicle' and wait in a child with a non-palpable testicle; guidelines recommend examination under anesthesia and diagnostic laparoscopy. In our practice, this assessment is completed in a single session with a joint decision by two pediatric urologists, and if a testicle is found, bringing it down is planned for that same session.
03

The approach to unilateral absence

In unilateral agenesis, the child has one healthy testicle, and it is usually sufficient for hormone production and fertility.

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  • The laparoscopy findings are documented during surgery, and no further procedure is needed. Fixing the remaining testicle to the scrotum is a debated question that varies by center; in our practice, it is an option decided in discussion with the family. An ultrasound may be requested to assess kidney development on the same side.
  • In the long term, the single testicle is monitored with annual examinations; its volume and development are assessed during adolescence, and the teenager is taught self-examination. If the appearance of the empty side bothers the teenager during adolescence, a testicular prosthesis can be discussed.
04

Bilateral absence (anorchia): hormonal evaluation

When neither testicle is palpable, the picture is different: hormonal evaluation is carried out before laparoscopy, and pediatric urology, endocrinology, and, when needed, genetics work together.

  • Karyotype (chromosome analysis)
  • AMH and inhibin B
  • FSH, LH, and baseline testosterone
  • hCG stimulation test if needed
  • Confirmation with diagnostic laparoscopy
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  • Blood tests give an important clue as to whether testicular tissue is present. Levels of AMH (anti-Müllerian hormone) and inhibin B, both secreted by the Sertoli cells of the testicle, are assessed together with FSH and LH from the pituitary gland; when needed, an hCG stimulation test measures the testosterone response. If no testicular tissue is present at all, AMH and inhibin B are expected to be too low to measure, FSH and LH are expected to be elevated, and there is expected to be no testosterone response to hCG. Although these findings strongly suggest absence, most centers still confirm the diagnosis with laparoscopy.
  • In an infant with bilateral non-palpable testicles, especially when an additional finding such as hypospadias is present, karyotype and hormone evaluation for differences of sex development (DSD) are completed before surgery. This is done not to alarm the family, but to ensure that planning is built on a solid foundation.
05

Endocrine support and follow-up in anorchia

Once the absence of both testicles is confirmed, pediatric endocrinology is involved in follow-up from the very start, because there is no tissue left to produce the testosterone needed for pubertal development.

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  • In infancy and childhood, additional treatment is usually not needed; as the age of puberty approaches, endocrinology starts testosterone replacement and gradually increases it to the adult dose. This treatment supports pubertal development, bone health, and overall well-being, and continues for life. A testicular prosthesis, to preserve scrotal development and for appearance, can be discussed during adolescence or before.
  • In terms of fertility, sperm production is not possible in anorchia; this fact is shared with the family at the right time, openly and supportively, and future assisted-reproduction options are addressed later together with adult urology. Follow-up continues with the same team from childhood into adulthood, in coordination with endocrinology.
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Frequently asked questions
Ultrasound didn't show either testicle — are they definitely absent?
No. Ultrasound not showing the testicles does not mean they are absent; it frequently misses testicles located inside the abdomen. For bilateral non-palpable testicles, hormonal evaluation is performed first, and the situation is then confirmed with examination under anesthesia and laparoscopy; if a testicle is found, bringing it down is planned for the same session.
Is absence of the testicles genetic — will it recur in future children?
In most cases, no identifiable genetic cause is found, and recurrence is not expected. Genetic counseling is recommended when the absence is bilateral or when there are accompanying findings; a familial predisposition is rare.
Does puberty proceed normally with one testicle?
Yes, in most cases. A single healthy testicle produces the testosterone needed for puberty, and it usually grows somewhat larger to take over the function. Volume and development are still checked during adolescence, and a hormonal evaluation is performed if any concern arises.
When is a testicular prosthesis placed?
It is usually placed during or after adolescence, at adult size, in a single procedure. In bilateral absence, it may be considered earlier to help preserve scrotal development. It is not a required procedure; the decision is made together with the child and family.
When does hormone treatment start in bilateral absence?
Treatment is generally not needed in infancy. As the age of puberty approaches — usually around 11 to 12 years — pediatric endocrinology starts testosterone replacement, and the dose is increased to the adult level over the following years. Treatment continues for life and is monitored together with endocrinology.
←Previous • Special situationsAtrophic Testicular Remnant (Nubbin)Next • Special situationsUndescended Testicle and Hypospadias Together→
Related pagesFull index →
Special situationsVanishing TestisVanishing testis refers to a testicle that formed in the womb but was lost at some point in development when its blood supply was disrupted. It is one of the most common causes of a non-palpable testicle; the diagnosis is made at laparoscopy when blind-ending vessels are seen, meaning there is no testicle left to bring down.Diagnosis and evaluationBilateral Non-Palpable TesticleBoth testicles being non-palpable is a different topic from unilateral undescended testicle. Here the first question isn't 'where are the testicles' but 'is there testicular tissue, and is it functioning' — so hormonal, and if needed genetic, evaluation is carried out before surgery. This page explains why and how, in plain language.Diagnosis and evaluationDiagnostic LaparoscopyDiagnostic laparoscopy (a minimally invasive procedure) is direct visualization of the inside of the abdomen through a thin camera introduced at the navel, and it's the only method that definitively answers both 'is there a testicle' and 'where' in a non-palpable testicle. This page explains how the procedure is performed and how the plan proceeds for every possible finding.Follow-upLong-Term Follow-UpUndescended testicle treatment doesn't end with surgery. Whether the testicle stays in place in the scrotum, grows normally, and develops as expected through puberty is monitored over years; follow-up continues seamlessly from pediatric urology into adult urology, and this page walks through that timeline step by step.
Undescended Testicle topic clustersMain treatment page →
Basics 7Diagnosis and evaluation 6Surgery in children 10Special situations 4After surgery 6Adolescents and adults 6Follow-up 3
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