Cryptorchidism


Article Author:
Stephen Leslie
Hussain Sajjad


Article Editor:
Carlos Villanueva


Editors In Chief:
David Wood
Andrew Wilt
Hajira Basit


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
10/8/2019 10:15:44 AM

Introduction

Cryptorchidism is the absence of at least one testicle from the scrotum. It is the most common birth defect involving the male genitalia. About 3% of full-term and 30% of premature male infants are born with one or both testicles undescended. Approximately 80% of cryptorchid testes descend by the third month of life. This makes the true incidence around 1%. [1]

Cryptorchidism may occur on one or both sides, but more commonly affects the right testicle.

The testicle may be anywhere along the "path of descent," such as: [2]

  • Located high in the retroperitoneal abdomen to the inguinal ring
  • In the inguinal canal
  • Ectopic from the path of descent
  • Hypoplastic
  • Dysgenetic
  • Missing or Absent
  • Unilateral (two-thirds)

The undescended testicle can usually be palpated in the inguinal canal. In a minority of patients, the missing testicle may be located in the abdomen or be nonexistent.

Undescended testicles are associated with decreased fertility (bilateral cases), increased testicular germ cell tumors (overall risk under 1%), testicular torsion, inguinal hernias, and psychological problems.

Without surgical correction, an undescended testicle may descend during the first three months of life. To reduce risks, undescended testes may be brought into the scrotum with an orchiopexy.

Cryptorchidism, hypospadias, testicular cancer, and poor semen quality make up testicular dysgenesis syndrome (TDS).  This syndrome is thought to be due to harmful environmental factors that disrupt embryonal programming and gonadal development during fetal life.

Etiology

A normal hypothalamic-pituitary-gonadal axis is a prerequisite for normal testicular descent.

Birth weight appears to be the main risk factor for undescended testes, followed by family history.

Absence of an appendix testis has been linked to abdominal testes and cryptorchid testes especially if located proximal to the external ring.  The exact role of the appendix testis in testicular descent is unclear.

In full-term infants, the cause of cryptorchidism often cannot be determined, making this a common but sporadic, idiopathic birth defect. It is thought that genetics, combined with maternal and environmental factors, may disrupt hormones and physical changes that influence testicular development and descent. 

Possible underlying risk factors include: [1]

  • Premature infants born before the descent of the testicles
  • Small for gestational age infants
  • Smaller placental weight
  • Chemicals endocrine disruptors may interfere with normal fetal hormone balance
  • Maternal obesity
  • Maternal diabetes
  • Maternal exposure to DES
  • Pesticides
  • Alcohol consumption during pregnancy (5 or more drinks per week, 3x increase)
  • Cigarette smoking
  • Family history
  • Cosmetics use
  • Exposure to phthalate (DEHP)
  • Ibuprofen
  • Preeclampsia (The more severe the preeclampsia, the greater the risk of cryptorchidism)
  • Congenital malformation syndromes - Down syndrome, Prader–Willi syndrome, and Noonan syndrome
  • Persistent Mullerian Duct Syndrome
  • In vitro fertilization

Epidemiology

Three percent of full-term, newborn infants have cryptorchidism. This decreases to 1% in infants aged six months to 1 year. [1]

  • The prevalence of cryptorchidism is 30% in premature male neonates.
  • Seven percent of siblings of boys with undescended testes have cryptorchidism.
  • In the United States, cryptorchidism ranges from about 3% at birth to 1% from 1 year to adulthood.
  • Internationally, prevalence ranges from 4% to 5% at birth to about 1% to 1.5% at age three months and 1% to 2.5% at nine months.
  • Cryptorchidism occurs in approximately 1.5% to 4% of fathers and 6% of brothers of individuals with cryptorchidism.
  • Heritability in first-degree male relatives is estimated to be approximately 0.5% to 1%.
  • There may also be an association between cryptorchidism and autism.

Pathophysiology

One contributing mechanism for the reduced function of cryptorchid testes is temperature. It is also likely that transient hormone deficiencies may lead to a lack of testicular descent and impair the development of spermatogenic tissue. [2]

History and Physical

Signs and symptoms of undescended testicles include the following:

Infertility

Men with undescended testes may have reduced fertility, even after orchiopexy. [3]

  • The infertility rate for unilateral cases is not believed to be very different from the general population.
  • The fertility reduction after orchiopexy for bilateral cryptorchidism is about 38%. This is the basis for the universal recommendation for early surgery due to degeneration of spermatogenic tissue and reduced spermatogonia counts after the second year of life in patients with untreated undescended testes.

Psychological Consequences

Boys with undescended testicles do not tend to be effeminate, gender-disordered, or pre-homosexual. A disturbed self-image may occur when the family dynamics are destructive toward male self-esteem. When cryptorchism is surgically corrected, a healthy masculinity generally occurs. 

Cancer

Overall, the risk of testicular cancer if orchiopexy is done before puberty is around 2 to 3 times that of the general population. It is 5 to 6 times higher when orchiopexy is done after puberty. The risk of cancer does not seem to be different when orchiopexy is done early in infancy compared to later in childhood. [3]

  • The most common type of testicular cancer in untreated undescended testes is seminoma. 
  • The peak age range for this tumor is 15 to 45 years.
  • In contrast, after orchiopexy, seminomas represent only 30% of testicular tumors in previously undescended testes.
  • It is treatable if caught early, so boys who had an orchiopexy as infants should be taught testicular self-examination.

Evaluation

From the AUA Guidelines: “In the hands of an experienced provider, more than 70% of cryptorchid testes are palpable by physical examination and need no imaging. In the remaining 30% of cases with a nonpalpable testis, the challenge is to confirm absence or presence of the testis and to identify the location of the viable nonpalpable testis." 

Ultrasound is non-contributory in routine use, with sensitivity and specificity to localize nonpalpable testes at 45% and 78%, respectively. The cost and ionizing radiation exposure associated with CT scanning preclude its use. MRI with or without angiography has been more widely used with greater sensitivity and specificity but is discouraged due to its cost, low availability, and the need for anesthesia. At this time, there is no radiological test that can conclude with 100% accuracy that a testis is absent; "...regardless of preoperative radiological findings, these studies rarely assist in the decision making and may at times yield misleading information (such as absence when actually present or vice versa).” [4]

A karyotype can confirm or exclude dysgenetic primary hypogonadism. Hormone levels such as gonadotropins and Anti-Mullerian Hormone (AMH) may confirm hormonally functional testicles worth salvation, as can stimulation with human chorionic gonadotropin to elicit a rise in the testosterone level. In some cases, further testing is crucial and has a high likelihood of detecting intersex conditions. [3]

Treatment / Management

Medical Treatment

The AUA Guidelines state: "Providers should not use hormonal therapy to induce testicular descent as evidence shows low response rates and lack of evidence for long-term efficacy."

The American Pediatric Association Guidelines do recommend the use of hormones for cases of undescended testis associated with Prader-Willi Syndrome. Their reasoning is that a therapeutic trial of human chorionic gonadotropin (HCG) is indicated for treatment of undescended testes before surgery, because avoidance of general anesthesia is desirable for infants with low muscle tone and at high risk for underlying respiratory compromise.

  • The most commonly used hormone is human chorionic gonadotropin (HCG). A series of HCG injections is given, and the status of the undescended testicle is reassessed. The success rate is reported as 5% to 50%. 
  • Hormone treatment also will confirm Leydig cell responsiveness and induce additional growth of a small penis due to a rise in testosterone levels. 

The cost of hormone treatment is less than surgery, and the chance of complications are minimal. However, a recent meta-analysis of seven randomized clinical trials concluded that hormonal therapy was no more effective than placebo. [5]

Surgery

Surgery is recommended for congenital undescended testes between the ages of 6 and 18 months (AUA Guidelines). For premature babies, corrected age is used to determine surgery timing. Fertility is improved if the orchidopexy is performed early.  Patients with bilateral undescended testes who receive orchidopexies as adults are almost always infertile and azoospermic; but there are now a few anecdotal reports of pregnancies achieved through assisted reproduction in this group. [6]

For acquired (testis documented normal before diagnosis) and entrapped (after hernia repair) undescended testes, surgery is recommended shortly after diagnosis. 

For retractile testes, a yearly physical examination is recommended because of the 2% to 50% reported risk of a retractile testis becoming an acquired undescended testis. 

Technique of Orchiopexy

For palpable undescended testes, an inguinal or scrotal orchiopexy is recommended. [6]

  1. An incision is made in the high scrotum, median scrotal raphe, high edge of the scrotum, or groin. Many different type of retractors can be used depending of the size of the incision. Inguinal incisions can be as small as 1 cm. Scrotal incisions can be larger as they tend to heal concealed specially when in the median raphe. 
  2. The testis can be approached first or the cord first; for scrotal cases, the testis is found first. For an inguinal approach, the testis can be approached first or the external oblique fascia opened proximal to the external ring and the cord approached first. 
  3. When approaching the testis first, all the cremasteric muscles are divided as well as everything not going into the external ring.
  4. The more difficult part of the case is separating the hernia sac from the vas and testicular vessels. This can be approached anteriorly or posteriorly. The posterior approach is much easier to teach and learn.
  5. How the testis is positioned and secured in the scrotum varies. Most would agree that a sub-dartos pouch is desirable. Some surgeons do not suture the testis in place, others use absorbable sutures, others non-absorbable, and others just close the passage into the groin. 

For nonpalpable testes under anesthesia, exploratory laparoscopy is recommended. If a testis is found during exploratory laparoscopy, the options are: [6]

  1. Laparoscopic orchiopexy preserving the vessels: the testis is dissected off a triangular pedicle containing the gonadal vessels and the vas.
  2. Laparoscopic one stage Fowler Stevens (FS) orchiopexy: gonadal vessels are divided and the testis is dissected off a pedicle of the vas and brought down in one stage.
  3. Laparoscopic two stage Fowler Stevens orchiopexy: vessels are divided with clips but dissection of the testis is postponed for 6 months to allow for optimal development of collaterals. 

If no testis is found during exploratory laparoscopy, one has to determine the presence of either blind ending vessels or a testicular nubbin to completely rule out a missing testis. The vas can be dissociated from the testis and thus is not always a good guide to find the gonad. [6]

If the internal ring is closed but vessels are going into it, a scrotal exploration usually will find a testicular nubbin. Look for a small structure with a brown spot.

If vessels are going into an open inguinal ring, one can usually push the testis into the abdomen but if not, an inguinal or scrotal exploration would be warranted.

Differential Diagnosis

A common diagnostic challenge is distinguishing a retractile testicle from a testicle that is not spontaneously descending into the scrotum. Retractile testes are more common than undescended testes and do not require surgical correction. In normal males, as the cremaster muscle contracts, the testicles retract into the upper scrotum and inguinal canal. This reflex is more active in infants. 

A retractile testicle high in the scrotum is difficult to distinguish from one positioned in the lower inguinal canal. Maneuvers used to assist identification include the cross-legged position, soaping the clinician's fingers, and examining the patient in a warm bath. 

The American Urological Association (AUA) Guidelines define a retractile testis as "...one that is initially extrascrotal on examination or moves easily out of the scrotal position, (often associated with a vigorous cremasteric reflex), but that can be manually replaced in a stable, dependent scrotal position and remain there without tension at least temporarily."

Prognosis

When properly diagnosed and treated, the prognosis is excellent. Testicular cancer risk and infertility remains somewhat increased compared to the general population.[7]

Complications

Orchiopexy is associated with two major testicular complications: atrophy and testicular ascent. For palpable testes, these occur less than 5% of the time. For laparoscopic orchiopexies, the rate is also around 5%. For Fowler-Stephens orchiopexies (dividing the vessels) in one or two stages, the testicular atrophy rate is around 20% to 30% (worse for the one stage procedure).

Pearls and Other Issues

There may be a slight decrease in penis size in cryptorchid patients. 

It has been suggested that adjuvant LHRH treatment be offered to cryptorchid patients with a high risk for infertility due to their insufficient gonadotropin stimulation at the time of surgery. Sperm cryopreservation is another prophylactic option in case of resultant infertility in spite of the adjuvant LHRH.

Enhancing Healthcare Team Outcomes

An undescended testicle is the most prevalent male congenital defect affecting the male genitalia. Most (about 80%) will descend by the third month of life.  It is more frequently found in premature infants.  Risks for male infertility and cancer are increased compared to the general population but still relatively small. Optimal timing of surgical orchidopexy (<18 months of age) is not always followed. Close collaboration and communication between nurse practitioners, physician assistants, pediatricians, and urologic surgeons will result in better outcomes and avoid unnecessary procedures while increasing the incidence of timely surgery when indicated.


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Cryptorchidism - Questions

Take a quiz of the questions on this article.

Take Quiz
A 12-month old child presents with an empty left hemiscrotum. The right testicle is in the scrotum and normally he is healthy. You decide to treat him with gonadotrophin-releasing hormone six times per day for four weeks to enhance descent of the testes. How should the hormone be administered to the child?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 17-year-old patient presents with an empty right hemiscrotum. The left testicle is normal. He is healthy, and a right testicle is seen in the right paracolic gutter. Which of the following would be most appropriate for the management of this patient?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 17-year-old male presents with complaints of abdominal discomfort and bloating sensation for one year. He also started noticing weight loss for the past three months. On physical examination, there is a palpable well-defined mass in the left lower quadrat of the abdomen, measuring around 9x9 cm. The clinician also noticed the absence of the left testicle in the scrotum. Upon inquiry, the patient revealed that it was missing from the scrotum since birth. Ultrasound of abdomen and pelvis reported a large homogeneous echotexture mass lesion in the left lumbar region. What type of malignancy is commonly associated with undescended testes?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A male patient presents to a clinic with dull aching pain in the lower abdomen for two years. He also reports that he has abdominal distension and discomfort for the past one month. Physical examination reveals bilateral undescended testis, but he is well built and has attained all the secondary sexual characters. He told the clinician that both the testis were missing from the scrotum since birth. Detailed medical history reveals that he is heterosexual and has no children. Neither is there any history of loss of weight and appetite nor any history of surgery in the past. Ultrasound of abdomen and pelvis demonstrated two large homogeneous echogenic masses in the lumbar region. Scrotal ultrasound shows an absence of testes in the scrotum. What is the primary reason an undescended testis is removed?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 23-month-old male is found to have an empty left hemiscrotum by his mother. On physical examination, his right testicle is in the scrotum, whereas the left testicle is nowhere to be palpated. Ultrasound of abdomen and pelvis was unable to locate the left testicle along the path of descent of the testis. He otherwise appears healthy. The clinician advised a laparoscopic exploration surgery. At laparoscopy, the testis is found to be in the left paracolic gutter. What is the next step in management?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 14-month-old male was brought to a clinic by a mother. She complaints that while bathing her child, she noticed that his testes were not present in his scrotal sac. On physical examination, the testes can be palpated in this inguinal canal. At what age is it necessary to perform orchiopexy in a child affected with cryptorchism?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 2-year-old boy was brought to a clinic with complaints of the absence of testes in the scrotum. Her mother told the clinician that she noticed it when she was bathing her child. Physical examination reveals bilateral undescended testis. What is the next best step to determine the location of the undescended testis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A mother brings her 24-month-old male child to a clinic with a complaint of an empty left hemiscrotum. On physical examination, the right testicle is papable in the scrotum, whereas the left testicle is papable in the left inguinal canal region. On examination of the patient in warm water, the left testicle becomes papable in the scrotum. What is the next best step in the management of this patient?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 12-month-old male was brought to a pediatric clinic by his mother. She complaints that while bathing her child, she noticed he had an empty scrotal sac. Detailed medical history revealed that the patient was born immature at seven months of pregnancy. On physical examination, neither the right nor the left testis can be palpated in the scrotal sac. The mother reports that her brother also had undescended testes after birth. What is the most common risk factor for undescended testes?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 3-year-old male is brought to a pediatric clinic by his mother with complaints of the empty scrotal sac. Detailed medical history reveals that the patient has Prade-Willi syndrome. His mother is concerned about the undescended testes. On physical examination, the left testicle can be palpated in the left inguinal region, whereas the right testicle is nowhere to be found. What is the initial best step in the management of this patient?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 5-year-old male is found to have an empty left hemiscrotum. On physical examination, his right testicle is in the scrotum, whereas the left testicle is nowhere to be palpated. Ultrasound of abdomen and pelvis was unable to locate the left testicle along the path of descent of the testis. The clinician advised an exploratory laparoscopic surgery. On laparoscopic exploration, the surgeon is unable to find the missing testis. What is the next best step during the laparoscopic exploration?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Cryptorchidism - References

References

Canadian Urological Association-Pediatric Urologists of Canada (CUA-PUC) guideline for the diagnosis, management, and followup of cryptorchidism., Braga LH,Lorenzo AJ,Romao RLP,, Canadian Urological Association journal = Journal de l'Association des urologues du Canada, 2017 Jul     [PubMed]
Nonpalpable testes: Ultrasound and contralateral testicular hypertrophy predict the surgical access, avoiding unnecessary laparoscopy., Berger C,Haid B,Becker T,Koen M,Roesch J,Oswald J,, Journal of pediatric urology, 2017 Nov 30     [PubMed]
Khatwa UA,Menon PS, Management of undescended testis. Indian journal of pediatrics. 2000 Jun;     [PubMed]
Cheng L,Albers P,Berney DM,Feldman DR,Daugaard G,Gilligan T,Looijenga LHJ, Testicular cancer. Nature reviews. Disease primers. 2018 Oct 5;     [PubMed]
Thorup J,Cortes D, Surgical Management of Undescended Testis - Timetable and Outcome: A Debate. Sexual development : genetics, molecular biology, evolution, endocrinology, embryology, and pathology of sex determination and differentiation. 2019 Jan 26;     [PubMed]
Wei Y,Wang Y,Tang X,Liu B,Shen L,Long C,Lin T,He D,Wu S,Wei G, Efficacy and safety of human chorionic gonadotropin for treatment of cryptorchidism: A meta-analysis of randomised controlled trials. Journal of paediatrics and child health. 2018 Aug;     [PubMed]
Hadziselimovic F, On the descent of the epididymo-testicular unit, cryptorchidism, and prevention of infertility. Basic and clinical andrology. 2017;     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Pediatrics-Medical Student. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Pediatrics-Medical Student, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Pediatrics-Medical Student, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Pediatrics-Medical Student. When it is time for the Pediatrics-Medical Student board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Pediatrics-Medical Student.