Sexually Transmitted Diseases - I. I. Mavrov 2005

Anomalies of the Urogenital System
Anomalies of the Scrotal Organs

Developmental anomalies of the scrotal Organs (Testes, Epididymis, vas deferens) are relatively rare. They include Congenital absence of the Testis (anorchia), supernumerary testes (polyorchism), underdevelopment of the testes (hypoplasia), positional Anomalies of the testis, Torsion of the Spermatic Cord and testis, and congenital hydrocele of the tunica vaginalis (hydrocele) and spermatic cord (funiculocele).

Anorchia is the absence of testes accompanied by hormonal dysfunction and eunuchoidism, which may be associated with Aplasia of the epididymis and vas deferens. Unilateral absence of the testis (monorchia) is the most common presentation.

Patients with anorchia typically have a documented male sex assignment (in identity documents), yet their "masculine traits" are limited to a rudimentary Penis and an undeveloped, empty Scrotum. In some individuals, the scrotum is replaced by a small perineal Swelling and a scrotal raphe. Evidently, anorchia results from the destruction of the male gonad early in embryonic life. This is likely caused by a short-lived secretion of embryonic androgens, which proved potent enough to induce the fusion of the labioscrotal folds into a rudimentary scrotum and stimulate penile development.

The primary Complaints of patients include underdevelopment of the genitalia and inability to engage in sexual activity. Occasionally, patients raised as females consult a physician regarding the absence of female secondary sex characteristics and abnormal Development of the external genitalia. As a rule, these patients present with a eunuchoid habitus; height is average or slightly below average; the physique is predominantly infantile (boyish); pubic Hair is absent; the nipples are underdeveloped and widely spaced; and Muscle tissue is poorly developed. The external genitalia are underdeveloped, featuring a small or even rudimentary penis and an empty scrotum that is typically unpigmented and covered with smooth Skin. Digital rectal examination reveals a non-palpable Prostate Gland.

Treatment involves androgen replacement therapy aimed at developing male secondary sex characteristics; indicated preparations include sustained-release testosterone ester blends (sustanon), as well as testosterone propionate, methyltestosterone, and vitamin E.

Polyorchism, or the presence of supernumerary testes, is rare and typically manifests as triorchidism.

Patients sometimes complain of a painless swelling at the Base of the penis or in the inguinal region. Upon Palpation, the tumor-like mass is elastic and soft in consistency, and digital pressure causes it to retreat behind the anterior abdominal wall. The swelling moves easily sideways and downward. The scrotum is asymmetrical, with the right (or left) hemiscrotum poorly developed. Palpation of the scrotum clearly reveals two testes of normal size within one hemiscrotum. In such cases, suprapubic ectopia of the testis (right or left) may also be detected. In polyorchism, the accessory testis is usually underdeveloped, may lack a vas deferens, and frequently undergoes malignant transformation.

The treatment for polyorchism is surgical. Removal of the accessory testis is performed according to clinical indications (pain in the inguinal region or lower abdomen triggered by heavy lifting).

Testicular hypoplasia (the rudimentary testis syndrome) is characterized by extremely small testes in which the convoluted tubules are dysgenetic, undergoing very early degeneration and atrophy. It frequently occurs among members of the same family. As a rule, these testes fail to descend into the inguinal canals and remain in the Abdominal cavity. The external genitalia are either female or hermaphroditic: 1) typical female genitalia — hypertrophied Clitoris, under-formed Labia minora, sparse pubic hair, and a relatively long Vagina with a rudimentary uterine cervix located at its apex; 2) hermaphroditic genitalia — unformed penis, small and bifid empty scrotum, with the urinary meatus located on the Perineum.

Primary patient complaints: Individuals raised as females consult a physician mainly regarding primary Amenorrhea and the lack of developed female secondary sex characteristics. Those raised as males complain of an absence of male secondary sex characteristics and genital anomalies.

Treatment for patients with a female gender identity is quite effective. Therapy with Female Sex Hormones promotes The Development of the Mammary Glands and external genitalia. In patients raised as males, treatment with Male Sex Hormones yields no therapeutic effect.

Positional anomalies of the testis include cryptorchidism, ectopia, and testicular torsion.

Cryptorchidism is the absence of one or both testes in the scrotum due to an arrest in their intrauterine descent from the retroperitoneal space. A distinction is made between abdominal cryptorchidism, where the testis is located in the retroperitoneal space, and inguinal cryptorchidism, where it resides in the Inguinal Canal. False cryptorchidism also occurs, in which a normally descended testis may temporarily or periodically reside outside the scrotum due to contractions of the cremaster muscle.

The causes of cryptorchidism may include mechanical obstructions hindering testicular descent, as well as hormonal and genetic factors. Mechanical factors comprise delayed development of the gubernaculum, short testicular vessels, fibrous barriers along the path of gonadal migration, obliteration of any segment of the inguinal canal, a narrowed inguinal ring, transverse lie of the testis, a short cremaster muscle, or a short vas deferens. Hormonal factors include deficiencies in androgens, maternal chorionic gonadotropin, and fetal luteinizing hormone. The primary factors contributing to damage in retained testes are altered thermal environment, trauma from surrounding Tissues, hypokinesia, and impaired Regional Blood Flow. Histological studies have established that degenerative changes in undescended testes begin as early as 6 months of age.

Bilateral abdominal cryptorchidism is accompanied by genital underdevelopment, infantilism, eunuchoidism, gynecomastia, feminization, hypospadias, epispadias, and Urinary Incontinence.

In unilateral cryptorchidism, the corresponding hemiscrotum is underdeveloped, while in bilateral cases, the entire scrotum is underdeveloped. The undescended testis is typically smaller and easily palpable upon digital pressure along the inguinal canal. Frequently, this maneuver allows it to be guided to the external inguinal ring and the upper scrotum. In abdominal cryptorchidism, achieving spontaneous scrotal descent of the testes is generally unlikely.

Possible complications of cryptorchidism include Hypogenitalism and Infertility, hydrocele of the tunica vaginalis, inflammatory Changes in the undescended testis, incarceration of an associated hernia, malignant degeneration of the testes, as well as torsion and strangulation of the undescended testis.

The optimal window for treating cryptorchidism is between 6 and 24 months of age. Initial management involves hormone therapy (administration of chorionic gonadotropin preparations During the first year of life). If hormone therapy fails, surgical intervention is required, which consists of mobilizing the testis into the scrotum and fixing it in place. It is advisable to operate on patients with cryptorchidism at 6–8 years of age. At this age, a testis brought down into the scrotum encounters more favorable conditions for development and the restoration of Spermatogenesis. When spontaneous scrotal descent remains a possibility, surgery may be deferred up to the latest acceptable age of 10 years.

Testicular ectopia is a condition where a descended testis deviates from its normal pathway and comes to rest subcutaneously in the inguinal region, on the medial thigh, or in the perineum. Occasionally, transverse testicular ectopia occurs, where both testes reside within the same hemiscrotum. Causes of testicular ectopia include underdevelopment of the corresponding hemiscrotum or the gubernaculum testis, and the presence of adhesions. Due to the risk of trauma to the ectopic testis, surgical relocation into the scrotum is recommended.

Torsion of the spermatic cord and testis is prompted by anatomical predispositions: a wide inguinal canal, underdevelopment of the gubernaculum testis and an excessively long ligament fixing the testis to the vaginal process of the Peritoneum, Separation of the testis and epididymis, bifurcation of the spermatic cord, and excessive testicular mobility. Precipitating causes of spermatic cord torsion include abrupt movements, excessive straining of the Abdominal Muscles, and blunt trauma. Torsion of the spermatic cord is typically accompanied by Rotation of the testis around its long axis (testicular torsion). Torsion most commonly affects a testis located in the inguinal canal (i.e., in inguinal cryptorchidism) or one that has descended late into the scrotum.

Testicular torsion and spermatic cord torsion account for 1.9% of all cases of undescended testes. In 20% of cases, this anomaly occurs within the first 10 years of life, and in approximately 50% of cases, before Puberty. Torsion of the spermatic cord and testis is also observed in infancy.

The Clinical presentation is driven by impaired testicular blood supply, venous congestion, Thrombosis of the spermatic cord Veins, and multiple hemorrhages within the Tissues of the testis and epididymis. The testis and epididymis become edematous and enlarged. Within the first hours following torsion—or sometimes after 24–48 hours, depending on the degree of twist—phenomena of partial or total testicular necrosis develop. The condition typically has an acute onset, accompanied by severe pain, occasionally leading to syncope, alongside nausea and vomiting. The pain is not confined to the swollen testis and spermatic cord but radiates to the abdominal cavity, leaving the abdominal muscles rigid and tender to pressure.

In mild torsion, the symptoms gradually subside. In severe cases, Gangrene of the testis sets in 1–3 days post-torsion. Acute symptoms persist until the necrotic testicular tissue is resorbed or sloughs off through a draining sinus tract. It should be noted that even following spontaneous detorsion 24 hours after onset, the testis will subsequently undergo atrophy.

Diagnosing testicular torsion and spermatic cord torsion is challenging. The condition can be mistaken for orchitis, epididymitis, or an incarcerated hernia. Abdominal testicular torsion mimics acute surgical abdomen and can simulate intestinal obstruction or appendicitis. Only the absence of the testis in the scrotum suggests the possibility of its torsion.

Treatment is strictly surgical: the spermatic cord is untwisted, and any necrotic testis is removed. In the absence of gangrenous signs, the testis and cord are fixed in their normal anatomical position (funiculopexy) by suturing them to the rings of the inguinal canal.

Congenital hydrocele of the testis and spermatic cord occurs due to the non-closure of the processus vaginalis peritonei. As a result, the cavity containing the testis communicates with the peritoneal cavity, leading to an accumulation of serous fluid within the cavity of the tunica vaginalis of the testis or the spermatic cord.

If the processus vaginalis remains open along the entire length of the spermatic cord and the testis, a hydrocele of both the testis and the spermatic cord is diagnosed. If it obliterates entirely down to the testis and fluid accumulates between the two layers of the tunica vaginalis, a testicular hydrocele is present. When the processus remains patent along the entire length of the spermatic cord and fluid collects between its layers, it is termed a communicating hydrocele of the spermatic cord. In most cases, the latter develops gradually and imperceptibly. Partial obliteration of the process along the spermatic cord results in The formation of one or several cysts.

In such cases, under METABOLISM/18.html">The Influence of increased intra-abdominal pressure—typically induced by coughing—a painful or tense swelling suddenly appears in one or the other groin. Concurrently, the abdomen becomes slightly distended and tender to palpation, accompanied by vomiting and constipation. This clinical presentation closely mimics an incarcerated hernia. A condition manifesting with such Clinical Features is known as an incarcerated communicating hydrocele of the spermatic cord.

Hydrocele of the testis and spermatic cord is characterized by enlargement of one or, occasionally (in bilateral cases), both hemiscrota, with the swelling extending superiorly toward the inguinal canal. In testicular hydrocele, the swelling is spherical, soft, and elastic to the Touch, with the testis located at its lower aspect. A hallmark of hydrocele is its translucency; therefore, diaphanoscopy is utilized as a diagnostic tool.

Fluid accumulation within the cavity of the tunica vaginalis may manifest immediately after birth or later in life. In the latter scenario, it can be assumed that the hydrocele developed due to elevated intra-abdominal pressure (e.g., persistent coughing, etc.) in a child whose processus vaginalis peritonei failed to undergo complete obliteration.

Occasionally, spontaneous resolution of a communicating hydrocele is observed during infancy. Consequently, small hydroceles that show no tendency to enlarge do not require surgical intervention.