Polyorchidism

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Polyorchidism
Tritestes ultrasound.jpg
Ultrasound scan showing a side view of Type A3 polyorchidism, with annotations showing the superior and inferior testes and the head and tail of the epididymis

Polyorchidism is the incidence of more than two testicles. It is a very rare congenital disorder, with fewer than 200 cases reported in medical literature [1] and six cases (two horses, two dogs and two cats) in veterinary literature. [2]

Contents

Polyorchidism is generally diagnosed via an ultrasound examination of the testicles. However, the diagnosis of polyorchidism should include histological confirmation. The most common form is triorchidism, or tritestes, where three testicles are present. The condition is usually asymptomatic. A man who has polyorchidism is known as a polyorchid.

Classification

Polyorchidism occurs in two primary forms: Type A and Type B. [1]

Type A3 is the most common form of polyorchidism, and types A2 and A3 together account for more than 90% of cases. [3] In 65% of cases, the supernumerary testicle is found in the left scrotal sac. [1]

Numeric system

An older system of classification structures polyorchidism into similar types, but with no subdivision [3] between connected and disconnected testicles:

Cause

Complications

Most cases of polyorchidism are asymptomatic, and are discovered incidentally, in the course of treating another condition. In the majority of cases, the supernumerary testicle is found in the scrotum. [1]

However, polyorchidism can occur in conjunction with cryptorchidism, where the supernumerary testicle is undescended or found elsewhere in the body. These cases are associated with a significant increase in the incidence of testicular cancer: 0.004% for the general population vs 5.7% for a supernumerary testicle not found in the scrotum. [1]

Polyorchidism can also occur in conjunction with infertility, inguinal hernia, testicular torsion, epididymitis, hydrocele testis and varicocele. [4] However, it is not clear whether polyorchidism causes or aggravates these conditions, or whether the existence of these conditions leads sufferers to seek medical attention and thus become diagnosed with a previously undetected supernumerary testicle.[ citation needed ]

Management

Because polyorchidism is very uncommon, there is no standard treatment for the condition. Prior to advances in ultrasound technology, it was common practice to remove the supernumerary testicle. [3] Several cases have been described where routine follow-up examinations conducted over a period of years showed that the supernumerary testicle was stable. [1]

A meta-analysis in 2009 suggested removing non-scrotal supernumerary testicles because of the increased risk of cancer, and regular follow-up in the remaining cases to ensure that the supernumerary testicle remains stable. [1]

Related Research Articles

<span class="mw-page-title-main">Testicle</span> Internal organ in the male reproductive system

A testicle or testis is the male reproductive gland or gonad in all bilaterians, including humans. It is homologous to the female ovary. The functions of the testes are to produce both sperm and androgens, primarily testosterone. Testosterone release is controlled by the anterior pituitary luteinizing hormone, whereas sperm production is controlled both by the anterior pituitary follicle-stimulating hormone and gonadal testosterone.

<span class="mw-page-title-main">Vasectomy</span> Surgical procedure for male sterilization

Vasectomy, is an elective surgical procedure for male sterilization or permanent contraception. During the procedure, the male vasa deferentia are cut and tied or sealed so as to prevent sperm from entering into the urethra and thereby prevent fertilization of a female through sexual intercourse. Vasectomies are usually performed in a physician's office, medical clinic, or, when performed on an animal, in a veterinary clinic. Hospitalization is not normally required as the procedure is not complicated, the incisions are small, and the necessary equipment routine.

<span class="mw-page-title-main">Epididymis</span> Tube that connects a testicle to a vas deferens

The epididymis is an elongated tubular structure attached to the posterior side of each one of the two male reproductive glands, the testes. It is a single, narrow, tightly coiled tube in adult humans, 6 to 7 meters in length. It connects the testicle to the vas deferens in the male reproductive system. The epididymis serves as an interconnection between the multiple efferent ducts at the rear of a testicle (proximally), and the vas deferens (distally). Its primary function is the storage, maturation and transport of sperm cells.

<span class="mw-page-title-main">Vas deferens</span> Part of the male reproductive system of many vertebrates

The vas deferens, with the more modern name ductus deferens, is part of the male reproductive system of many vertebrates. The ducts transport sperm from the epididymis to the ejaculatory ducts in anticipation of ejaculation. The vas deferens is a partially coiled tube which exits the abdominal cavity through the inguinal canal.

<span class="mw-page-title-main">Cryptorchidism</span> Medical condition

Cryptorchidism, also known as undescended testis, is the failure of one or both testes to descend into the scrotum. The word is from Greek κρυπτός 'hidden' and ὄρχις 'testicle'. It is the most common birth defect of the male genital tract. About 3% of full-term and 30% of premature infant boys are born with at least one undescended testis. However, about 80% of cryptorchid testes descend by the first year of life, making the true incidence of cryptorchidism around 1% overall. Cryptorchidism may develop after infancy, sometimes as late as young adulthood, but that is exceptional.

<span class="mw-page-title-main">Hydrocele testis</span> Medical condition

A hydrocele testis is an accumulation of clear fluid within the cavum vaginale, the potential space between the layers of the tunica vaginalis of the testicle. It is the most common form of hydrocele and is often referred to simply as a "hydrocele". A primary hydrocele testis causes a painless enlargement in the scrotum on the affected side and is thought to be due to the defective absorption of fluid secreted between the two layers of the tunica vaginalis. A secondary hydrocele is secondary to either inflammation or a neoplasm in the testis.

<span class="mw-page-title-main">Epididymitis</span> Medical condition

Epididymitis is a medical condition characterized by inflammation of the epididymis, a curved structure at the back of the testicle. Onset of pain is typically over a day or two. The pain may improve with raising the testicle. Other symptoms may include swelling of the testicle, burning with urination, or frequent urination. Inflammation of the testicle is commonly also present.

<span class="mw-page-title-main">Persistent Müllerian duct syndrome</span> Medical condition

Persistent Müllerian duct syndrome (PMDS) is the presence of Müllerian duct derivatives in what would be considered a genetically and otherwise physically normal male animal by typical human based standards. In humans, PMDS typically is due to an autosomal recessive congenital disorder and is considered by some to be a form of pseudohermaphroditism due to the presence of Müllerian derivatives.

<span class="mw-page-title-main">Spermatocele</span> Medical condition

Spermatocele is a fluid-filled cyst that develops in the epididymis. The fluid is usually a clear or milky white color and may contain sperm. Spermatoceles are typically filled with spermatozoa and they can vary in size from several millimeters to many centimeters. Small spermatoceles are relatively common, occurring in an estimated 30 percent of males. They are generally not painful. However, some people may experience discomfort such as a dull pain in the scrotum from larger spermatoceles. They are not cancerous, nor do they cause an increased risk of testicular cancer. Additionally, unlike varicoceles, they do not reduce fertility.

<span class="mw-page-title-main">Hematocele</span> Medical condition

A hematocele is a collections of blood in a body cavity or potential space. The term most commonly refers to the collection of blood in the tunica vaginalis around the testes, known as a scrotal hematocele. Hematoceles can also occur in the abdominal cavity and other body cavities. Hematoceles are rare, making them harder to diagnose and treat. They are very common especially as slowly growing masses in the scrotum usually in men older than 50 years.

<span class="mw-page-title-main">Testicular pain</span> Medical condition

Testicular pain, also known as scrotal pain, occurs when part or all of either one or both testicles hurt. Pain in the scrotum is also often included. Testicular pain may be of sudden onset or of long duration.

Congenital absence of the vas deferens (CAVD) is a condition in which the vasa deferentia reproductive organs fail to form properly prior to birth. It may either be unilateral (CUAVD) or bilateral (CBAVD).

Post-vasectomy pain syndrome (PVPS) is a chronic and sometimes debilitating genital pain condition that may develop immediately or several years after vasectomy. Because this condition is a syndrome, there is no single treatment method, therefore efforts focus on mitigating/relieving the individual patient's specific pain. When pain in the epididymides is the primary symptom, post-vasectomy pain syndrome is often described as congestive epididymitis.

<span class="mw-page-title-main">Sperm granuloma</span> Lump of extravasated sperm found in some vasectomized men

A sperm granuloma is a lump of leaked sperm that appears along the vasa deferentia or epididymides in vasectomized individuals. While the majority of sperm granulomas are present along the vas deferens, the rest of them form at the epididymis. Sperm granulomas range in size, from one millimeter to one centimeter. They consist of a central mass of degenerating sperm surrounded by tissue containing blood vessels and immune system cells. Sperm granulomas may also have a yellow, white, or cream colored center when cut open. While some sperm granulomas can be painful, most of them are painless and asymptomatic. Sperm granulomas can appear as a result of surgery, trauma, or an infection. They can appear as early as four days after surgery and fully formed ones can appear as late as 208 days later.

<span class="mw-page-title-main">Scrotum</span> Sac of skin that protects the testicles

The scrotum or scrotal sac is an anatomical male reproductive structure located at the base of the penis that consists of a suspended dual-chambered sac of skin and smooth muscle. It is present in most terrestrial male mammals. The scrotum contains the external spermatic fascia, testicles, epididymis, and ductus deferens. It is a distention of the perineum and carries some abdominal tissues into its cavity including the testicular artery, testicular vein, and pampiniform plexus. The perineal raphe is a small, vertical, slightly raised ridge of scrotal skin under which is found the scrotal septum. It appears as a thin longitudinal line that runs front to back over the entire scrotum. In humans and some other mammals the scrotum becomes covered with pubic hair at puberty. The scrotum will usually tighten during penile erection and when exposed to cold temperatures. One testis is typically lower than the other to avoid compression in the event of an impact.

Sarcoidosis is a systemic disease of unknown cause that results in the formation of non-caseating granulomas in multiple organs. The prevalence is higher among black males than white males by a ratio of 20:1. Usually the disease is localized to the chest, but urogenital involvement is found in 0.2% of clinically diagnosed cases and 5% of those diagnosed at necropsy. The kidney is the most frequently affected urogenital organ, followed in men by the epididymis. Testicular sarcoidosis can present as a diffuse painless scrotal mass or can mimic acute epididymo-orchitis. Usually it appears with systemic manifestations of the disease. Since it causes occlusion and fibrosis of the ductus epididymis, fertility may be affected. On ultrasound, the hypoechogenicity and ‘infiltrative’ pattern seen in the present case are recognized features. Opinions differ on the need for histological proof, with reports of limited biopsy and frozen section, radical orchiectomy in unilateral disease and unilateral orchiectomy in bilateral disease. The peak incidence of sarcoidosis and testicular neoplasia coincide at 20–40 years and this is why most patients end up having an orchiectomy. However, testicular tumours are much more common in white men, less than 3.5% of all testicular tumours being found in black men. These racial variations justify a more conservative approach in patients of Afro-Caribbean descent with proven sarcoidosis elsewhere. Careful follow-up and ultrasonic surveillance may be preferable in certain clinical settings to biopsy and surgery, especially in patients with bilateral testicular disease.

Vasectomy reversal is a term used for surgical procedures that reconnect the male reproductive tract after interruption by a vasectomy. Two procedures are possible at the time of vasectomy reversal: vasovasostomy and vasoepididymostomy. Although vasectomy is considered a permanent form of contraception, advances in microsurgery have improved the success of vasectomy reversal procedures. The procedures remain technically demanding and may not restore the pre-vasectomy condition.

<span class="mw-page-title-main">Scrotal ultrasound</span> Medical ultrasound examination of the scrotum.

Scrotalultrasound is a medical ultrasound examination of the scrotum. It is used in the evaluation of testicular pain, and can help identify solid masses.

Male genital examination is a physical examination of the genital in males to detect ailments and to assess sexual development and is normally included in an annual physical examination. The examination includes checking the penis, scrotum, and urethral meatus. A comprehensive assessment of the male genitals assesses the pubic hair based on Sexual Maturity Rating and the size of the testicles and penis. The exam is conducted to detect various ailments, measure sexual development, or verify a person's age and biological sex. The genitourinary system can also be assessed as part of the male genital examination. During a genital examination, the doctor can detect any of the following: structural abnormalities, urethral opening abnormalities, problems related to uncircumcision, lumps, tumors, redness, excoriation, edema, lesions, swelling, cancer, hair-related issues and many others. In some instances where a physical examination of the male genitals is not sufficient to diagnose an individual than an internal genital examination using imaging or ultrasounds will be needed for further evaluation.

The epididymis, which is a tube that connects a testicle to a vas deferens in the male reproductive system, evolved by retention of the mesonephric duct during regression and replacement of the mesonephros with the metanephric kidney. Similarly, during embryological involution of the paired mesonephric kidneys, each mesonephric duct is retained to become the epididymis, vas deferens, seminal vesicle and ejaculatory duct. In reptiles and birds both the testes and excurrent ducts occur in an intra-abdominal location (testicond). Primitive mammals, such as the monotremes (prototheria), also are testicond. Marsupial (metatheria) and placental (eutheria) mammals exhibit differing degrees of testicular descent into an extra-abdominal scrotum. In scrotal mammals the epididymis is attached to the testes in an extra-abdominal position where the cauda epididymis extends beyond the lowest extremity of the testis. Hence, the cauda epididymis is exposed to the coolest of temperatures compared to all other reproductive structures.

References

  1. 1 2 3 4 5 6 7 Bergholz, R.; Wenke, K. (2009). "Polyorchidism: A Meta-Analysis". The Journal of Urology. 182 (5): 2422–2427. doi:10.1016/j.juro.2009.07.063. PMID   19765760.
  2. Roca-Ferrer, J.; et al. (2015). "A Rare Case of Polyorchidism in a Cat with Four Intra-abdominal Testes". Reprod Dom Anim. 50 (1): 172–176. doi:10.1111/rda.12461. PMID   25472870.
  3. 1 2 3 Leung, A. K. (1988). "Polyorchidism". American Family Physician. 38 (3): 153–156. PMID   3046267.
  4. Kundu, A; et al. "Triorchidism : An Incidental Finding And Review Of Literature" (PDF). Retrieved 21 March 2012.{{cite journal}}: Cite journal requires |journal= (help)