Sexually Transmitted Diseases - I. I. Mavrov 2005

Developmental Anomalies of the Urogenital System
Urethral Anomalies

They occur in the form of complete (aplasia) or partial (atresia) absence of the Urethra, as well as obliteration, congenital narrowing (stricture) and dilatation (diverticulum); urethral duplication; hypo- or epispadias; congenital cysts and fistulas; and Displacement of the urethral mucosa.

Complete absence of the urethra. This is extremely rare; with this pathology, the fetus mostly dies in utero at 6–8 months. This is due to the fact that in utero, the umbilical vessels—stretched by the distended bladder—are compressed, leading to a sharp circulatory disturbance and fetal non-viability.

This anomaly is often combined with the absence of the Penis. Its occurrence is associated with the arrest of the genital tubercle and sinus ectodermalis formation, as well as the improper Development of the urorectal fold, resulting in the cloacal membrane rupturing exclusively along the intestinal segment of the cloaca.

The Fate of the fetus in the absence of the urethra depends on the extent to which the bladder communicates with the external environment via the rectum, Vagina, or Uterus. If urine drainage is established through one of the aforementioned pathways, the fetus may be born alive; in the absence of other life-incompatible malformations, such children can grow and develop relatively well.

The symptoms of the disease in these rare cases boil down to irritation of the intestine, external genitalia (if the bladder opens into the vagina), and the abdominal Skin or Perineum (if urine is excreted through the urachus or a perineal fistula) caused by urine. Treatment for such patients is symptomatic.

Partial absence of the urethra. This belongs to anomalies in which the fetus is rarely viable. It is most frequently observed in the area of the glans penis and the membranous urethra—that is, at the junctions of sections with different origins. Clinically, it manifests as urinary retention: the infant strains and cries without urinating, while a bulging of the overfilled bladder is observed above the pubic bone. Treatment is surgical. If the external urethral meatus is absent, it is sufficient to dissect the membrane with a scalpel; in cases of more pronounced urethral hypoplasia, urethrostomy is performed.

Partial congenital obliteration of the urethra. This can develop in any part of the urethra, though most commonly in the area of the glans penis. Obliteration in the area of the Rhomboid fossa also occurs. The former is caused by the persistence of the embryonic membrane formed from epithelial remnants of the prepuce, while the latter results from delayed development of the glandular segment and its failure to join the rest of the urethra.

Multiple obliterations with preservation of the urethral lumen in the intervals between the obliterated sites are exceptionally rare. These anomalies are typically accompanied by other defects, notably atresia of the rectum, or urethroperineal/urethrorectal fistulas.

Symptoms of partial urethral obliteration include the absence of natural urination in the first days of life and urethral obstruction detected during catheterization. Treatment should begin in the first days of the child's life. Obliteration of the external urethral meatus is easily eliminated using a sharp instrument; obliteration in the area of the glans penis and the pendulous urethra up to 0.5 cm in length can be corrected by tunneling. In cases of extensive obliteration, a lip-like fistula must be created proximal to the obliterated area, because following tunneling, the canal exhibits a strong tendency to narrow. Subsequently, once the child's condition and development are satisfactory, tunneling of the glandular urethra can be performed, followed by reconstruction over a polyamide tube prosthesis.

In extensive obliterations of the pendulous urethra, urine is diverted through a perineal fistula, whereas obliteration of the bulbomembranous segment sometimes requires suprapubic cystostomy. At the age of 6–7 years, the urethra can be reconstructed using local Tissues (as in hypospadias).

Congenital urethral diverticula are sac-like outpouchings of the urethral wall that communicate with its lumen via a narrow channel. They are located on the ventral surface of the urethra, originating (mostly) from the glans and extending far posteriorly (all the way to the bulbous urethra).

It is hypothesized that urethral diverticula develop from remnants of the Müllerian ducts. Their size varies considerably (ranging from a walnut to a chicken egg). Acquired diverticula also occur (especially in children), resulting from trauma to the urethral wall. At the site of injury, elements of the Muscle layer are destroyed and the resistance of the urethral wall decreases; under the pressure of urine during urination, diverticula are formed. The integrity of the urethral mucosa can be compromised not only by direct injury, but also by the breakthrough of an existing abscess or cyst into its thickness.

Diverticula of the pendulous urethra usually present as a mass that enlarges during urination and disappears after manual expression. Compression of the urethra may cause dysuria (typically in the middle or at the end of the act). Secondary infection causes diverticulitis (inflammation of the diverticulum), accompanied by hyperemia, tenderness, cloudy urine, and pus accumulation within the diverticulum.

Diverticula of the posterior wall manifest as painful, obstructed urination. Digital rectal examination reveals a doughy mass that enlarges after urination. Pressure on the mass causes urine or pus to discharge from the urethra. With the prolonged existence of a diverticulum, especially one with a narrow neck, urine constantly stagnates in its cavity, which can lead to calculus formation.

Diagnosis is based on the clinical picture. Urethrography or urethroscopy clarifies the localization, width of the neck of the diverticulum, etc. Treatment is surgical. The diverticulum is excised, and the urethral defect is closed transversely. To avoid urethral stricture, excising the neck of the diverticulum is not recommended. Urine is diverted via a suprapubic fistula.

In girls with urethral diverticula, the opening into the diverticulum is widened transurethrally, effectively incorporating the latter into the urethral lumen.

Congenital urethral strictures. These occur more frequently at the junctions of urethral segments of different origins—for example, at the border between the glans penis and the corpus cavernosum, between the bulbous and membranous parts, between the membranous and prostatic parts, near the Base of the distal slope of the seminal colliculus, and at the border between the prostatic urethra and the internal sphincter. A special group comprises stenoses of the external urethral meatus, which are frequently combined with phimosis and embryonic fusion of the two layers of the prepuce.

Clinical manifestations of congenital urethral stricture depend on its localization, degree of narrowing, and duration of the disease. In the initial stages, the main complaint is difficulty in urinating. Later, signs of atonia of the Urinary Bladder, Ureters, and renal pelves, Urinary Tract infection, and ultimately renal failure join the clinical picture.

The Nature of the voiding disorder depends on the form of the stricture. With annular strictures, urination becomes sluggish, the stream is thin and cannot be amplified by straining the Abdominal Muscles; valve-like strictures are characterized by interrupted streams and short-term complete urinary retention even in the Cytology/cytology/16.html">Early stages of the disease. The extreme "cleanliness" of infants is also noteworthy: they do not urinate during Sleep, because the passive force of the bladder during sleep is insufficient to overcome the obstruction caused by the stricture.

Diagnosis is established based on voiding disorders, physical examination findings, instrumental, and radiological investigations. Circular strictures are detected by sounding with bougies and by ascending urethrography. In valve-like strictures, free passage of instruments through the urethra is impossible; therefore, to detect them, a descending urethrogram (taken during urination) is required, which reveals not only the site of the stricture but also the dilatation of the proximal urethra. Urethroscopy allows for the precise Determination of the type of stricture, its localization, and the unaffected mucosa in the area of the stricture (unlike acquired strictures).

Treatment should begin as early as possible, before changes occur and before infection of the bladder and Kidney sets in. Circular strictures in the area of the external urethral meatus are incised downward toward the frenulum. Catheter urinary diversion is not mandatory in this case. Along the rest of the canal, internal urethrotomy must be performed to divide the strictures, accompanied by urinary diversion using a polyvinyl chloride urethral catheter, which is advisable to leave in the urethral lumen for 2–3 weeks.

Valve-like strictures can be eliminated via electrocoagulation of the Valves through a urethroscope sheath or by excision of high-positioned valves transvesically.

Urethral duplication. A relatively rare anomaly. Urethral duplication is classified into cases associated with diphallus (duplication of the penis) and those without penile duplication. The Water/144.html">Origin of the accessory urethra is associated with the Separation of a portion of the urethral groove during the Embryonic period, or a failure of the edges of the urethral fold to fuse. In A number of observations, accessory ducts develop from the excretory ducts of the Prostate Gland.

In cases of diphallus, both urethras either arise separately from the bladder or merge into a single channel in the region of the prostatic urethra. This anomaly is rare.

Partial urethral duplication is observed much more frequently. The accessory urethral channels run along the dorsal or ventral surface of the penis; their outlet opens in the corona glandis or at the site of the external urethral meatus (in the latter case, the true urethra opens more inferiorly, in the region of the frenulum). The length of these ducts ranges from 1.5 to 14.5 cm, and their width ranges from 1 mm to 1 cm. In most cases, they end blindly; over a considerable extent, they extend to the Pubic Symphysis and even further posteriorly.

In cases where the end of the duct connects with the urethra, urination occurs in two stages, but more often the urine fails to enter the accessory canal at all or only slightly moistens it. However, if the urethral canal extends to the urinary bladder and lacks a fully competent sphincter, Urinary Incontinence is observed.

If an accessory urethra extending to the bladder is present and accompanied by urinary incontinence, excision of the accessory canal is performed. When the accessory urethra (or paravrethral duct) is separated from the main urethra by a thin layer of tissue, the septum is incised along a grooved probe or using fine-bladed scissors. The partition is divided until both segments meet. A polyvinyl catheter is inserted into the urethra for 4–5 days. If the accessory urethra is situated at a considerable distance from the main one, it is excised along a probe introduced into its lumen.

Congenital urethral cysts. These occur extremely rarely. They may develop from remnants of the Müllerian ducts, at the site of the prostatic utricle (utriculus prostaticus), or presumably As a result of a failure in the fusion of the urethral fold margins. Cysts may also arise at the site of the Bulbourethral Glands. Their size ranges from 3 to 15 mm. Such cysts have been described in newborns. These formations cause voiding difficulties due to urethral compression, necessitating frequent catheterization from early infancy. Surgical treatment involves cyst excision.

Congenital urethral fistulas. These occur when the edges of the urethral groove fail to fuse at some point during embryonic development. Congenital urethrorectal fistulas are the most common type, frequently associated with anal atresia or congenital anal stenosis. This condition is rare, occurring twice as often in boys as in girls. Urethrorectal fistulas are located in the posterior wall of the urethra and pose a hazard due to the risk of ascending infection (such as Pyelonephritis), since urine enters the rectum while gas and fecal matter pass into the urethra.

For diagnostic purposes, contrast urethrography and urethrocystoscopy are performed. In cases of anal atresia, an anus is surgically created within the first hours of the child's life. Sometimes, if the urethral fistula is not corrected immediately, it subsequently narrows and becomes less hazardous. The definitive closure of the fistula (surgical separation of the urethral and rectal lumens) can be performed somewhat later, once the child has grown stronger (typically after the first year of life).

Prolapse of the urethral mucosa. This condition is primarily characterized by the protrusion of the urethral mucosa and is observed in girls aged one year and older. The prolapse may be complete or partial, occurring around the entire circumference of the canal or involving only one of its walls. In the former case, the prolapsed mucosa forms a ring-like fold around the external urethral meatus, in the center of which lies the urethral opening. In the latter case, the prolapsed mucosal segment appears as a tumor-like mass on one of the canal walls, usually the posterior one.

Mucosal prolapse develops gradually. In some cases, a complete prolapse may occur suddenly following severe straining of the abdominal muscles. The Pathogenesis of the disorder remains not fully understood, though it is predominantly seen in debilitated children.

The initial symptoms typically include: frequent urination, burning sensation during voiding, dysuria, and urinary incontinence. Due to congestion, the prolapsed mucosa turns dark red or bluish, and erosions and ulcers appear on its surface. It is painful to the Touch.

When a large segment of the mucosa prolapses rapidly and major Blood Vessels become pinched, partial mucosal necrosis may develop. This is accompanied by pain and foul-smelling discharge.

Treatment involves surgery (when indicated) alongside conservative therapy aimed at reducing the sensitivity of the prolapsed mucosa by applying gauze pads soaked in lead lotion or periodically wiping the mucosa with a 0.5–1% silver nitrate (lapis) solution. Additionally, water treatments, medications to strengthen The Nervous system, and a high-nutrient diet are recommended.

Hypospadias. The absence of the distal part of the urethra with the external urethral meatus located in an abnormal position. It is considered the most common congenital anomaly of the urogenital Organs.

Depending on the degree of displacement of the external urethral meatus, hypospadias is classified into: scrotal hypospadias (the meatus is located in the scrotal region and the spongy part of the urethra is absent); penoscrotal hypospadias (the urethral meatus is situated at the junction of the penis and Scrotum); glandular hypospadias (the meatus opens on the ventral surface of the glans penis); penile hypospadias (the meatus is located along the corpus spongiosum of the penis); perineal hypospadias (the spongy and membranous PARTS OF THE urethra are absent, and the meatus is situated in the perineum); hypospadias without hypospadias (congenital longitudinal underdevelopment of the urethra); and female hypospadias (a defect in the posterior urethral wall and anterior vaginal wall where the urethral meatus opens into the vaginal cavity).

From the perspective of urethral and penile Embryogenesis, The Development of hypospadias can be viewed as a failure of the urethral groove to close during the 10th to 14th weeks of embryonic development—specifically, as a Pathology of the segment derived from the ectodermal urogenital sinus. Factors causing delayed development of the urethra and penis include abnormal formation of internal reproductive organs, most likely due to either acute endocrine/hormonal shifts in the mother's body (such as administration of Female Sex Hormones or excessive hormone production during short-term yet severe emotional stress) or chronic ones (ovarian dysfunction, adrenal hyperplasia, etc.). Recently, intrauterine fetal infection, along with other infections and intoxications During the first trimester of Pregnancy, has been attributed great significance in the Etiology of hypospadias.

Scrotal hypospadias. In this rare perineal form of hypospadias, the scrotum is divided by a deep cleft into two entirely separate halves. At the bottom of this groove, 4–5 cm from the anal opening, lies the urethral meatus. The anterior portion of the urethra may be absent or represented merely by a groove. The penis is typically underdeveloped, ventrally curved, and tethered.

In severe forms of scrotal hypospadias, determining the child's sex at birth is difficult, especially when the Testes are located in the inguinal canals. Consequently, the two halves of the scrotum may be mistaken for Labia Majora, the underdeveloped phallus resembles a Clitoris, and the wide urethral opening mimics a rudimentary vagina. The literature cites instances where such children were raised as girls, and their true sex (pseudohermaphroditism) was discovered only later during Puberty.

With this form of hypospadias, urine spreads over the thighs and perineum, causing skin irritation. However, patients maintain good urinary continence because the bladder sphincter remains intact. Urination is only possible in a sitting position.

Glandular hypospadias. The most common anomaly, in which the external meatus is located at the site where the frenulum should attach. The foreskin covers only the dorsal aspect of the glans, which is slightly deflected downward. A dimple marks the site of the normally positioned urethral meatus, beneath which lies the narrow opening of the anomalous urethra.

Glandular hypospadias does not require surgical intervention unless the urethral opening is severely stenotic or the penis is markedly curved. In penile hypospadias, the urethral opening is located more proximally anywhere along the ventral surface of the penis, right down to the base of the scrotum. The urethral segment distal to the opening typically appears as a flat groove extending all the way to the tip of the glans. In this form of hypospadias, the penis is almost invariably ventrally curved.

Regardless of its severity, hypospadias may be accompanied by cryptorchidism. Less frequently, it is associated with Congenital Heart defects, anomalies of other Internal Organs, and limb or skeletal malformations. These factors are taken into account when planning treatment, which must address several goals: straightening the penis while creating a skin reserve for subsequent urethroplasty; performing urethroplasty while preserving the straightening effect; removing anatomically formed FEMALE REPRODUCTIVE ORGANS (Ovaries, uterus, Mammary Glands); and treating cryptorchidism.

Treatment should begin at 1–2 years of age so that penile straightening and urethroplasty can be fully completed by the age of 6–7.

In glandular hypospadias without penile curvature, the indication for surgery is severe stenosis of the external urethral meatus, which can sometimes lead to serious consequences such as dilation and atonia of the bladder, ureters, and renal pelves. Therefore, meatotomy should be performed as early as possible (during the first months of the infant's life). At an older age, once scar tissue develops around the narrowed urethral meatus, a simple incision is no longer sufficient, and meatal plastic surgery becomes necessary.

Methods of urethroplasty include: tunneling and formation of a neourethra; urethroplasty using local tissues (skin of the penis, scrotum, or prepuce); urethroplasty utilizing pedicled Filatov flaps harvested from adjacent areas; and urethroplasty employing free auto- and homotransplants (skin, blood vessels, ureters, bladder mucosa, cadaveric urethra, Appendix, etc.).

The basic requirements that must be strictly observed during urethroplasty include: preserving the growth and distensibility potential of the newly created urethra, and constructing a canal with a stable lumen that has no tendency toward stricture formation (the surgical technique is described in the monograph by N. E. Savchenko, "Hypospadias and Its Treatment", 1962).

Hypospadias without hypospadias. An anomaly characterized by longitudinal underdevelopment of the urethra and a discrepancy between normal corpora cavernosa and a non-growing urethra. In this anomaly, the external urethral meatus is located in the normal position, and urination is unimpaired. Consequently, of the two signs of hypospadias, only penile curvature is present, which precludes normal sexual intercourse and serves as the primary complaint of adult patients. Instrumental examination of the urethra reveals that it invariably lacks a corpus cavernosum in its distal third.

Treatment of congenital urethral hypoplasia is a highly complex task because it involves an intermediate stage of artificial hypospadias (where the normally positioned urethral meatus is transected and displaced to the scrotum or even the perineum). Another factor complicating treatment is the absence of the urethral corpus cavernosum over a significant length, making the anastomosis of thin-walled segments after penile straightening extremely challenging.

Surgical treatment for severe penile curvature (as well as for hypospadias) consists of two stages: penile straightening accompanied by urethral incision, followed by the anastomosis of the urethral segments.

Female hypospadias. In girls, hypospadias is much less common than in boys; however, unlike the latter, it may be accompanied by urinary incontinence of varying severity. In some cases, the distal urethra and vagina merge into a single common channel with a shared external orifice. Some authors (N. E. Savchenko, 1970; A. S. Pereverzev, 2000) suggest that the condition described as female hypospadias should be interpreted as a defect of the urethrovaginal septum.

Prevention of hypospadias should focus on improving the mother's general health and eliminating any factors that may cause sharp fluctuations in blood hormone levels. Women who have previously given birth to children with Congenital Malformations should ideally be placed under dispensary surveillance during the first 4 months of pregnancy, with appropriate preventive treatment administered as indicated.

Epispadias. A congenital anomaly characterized by complete or partial failure of fusion (splitting) of the anterior wall of the urethra. In epispadias, the dorsal wall of the urethra is absent to a greater or lesser extent, while the ventral wall forms a flat groove.

The pathogenesis of the disorder remains unclear to date. It is believed that the causes of epispadias include delayed urethral development during its formation, obliteration of the external urethral meatus followed by secondary rupture of its dorsal wall, delayed fusion of the paired anlagen of the genital tubercle, and their more caudal development.

In boys, three forms of epispadias are distinguished: glandular, penile-pubic (subtotal), and total, or complete. According to statistical data (based on autopsy results), this anomaly occurs in one out of every 6,000 cases. It is significantly rarer than hypospadias.

Patient symptoms and Complaints depend on the severity of the defect. Glandular epispadias presents as a cleft within the glans penis; subjective functional disorders are absent, and treatment is not required. Penile epispadias (with a cleft extending to the penopubic angle) features a shortened penis drawn up toward the abdominal wall, while the urinary sphincter remains intact. Patients with these two forms of epispadias experience inconveniences of a rather aesthetic nature, associated with the spraying of the urine stream and its contact with undergarments and adjacent skin areas. Penopubic epispadias presents with some degree of urinary sphincter clefting and associated partial urinary incontinence. Due to the shortening and deformation of the penis in this form of epispadias, sexual intercourse is difficult and sometimes impossible.

Total epispadias is characterized by the clefting of the urethra, bladder neck, and anterior bladder wall, resulting in complete urinary incontinence. The penis is poorly developed, shortened, curved, and drawn up toward the pubic bone. When pulled downward, a flattened glans is visible, from which a strip of urethral mucosa extends along the dorsal surface of the organ. At the base of the penile ROOT, There is a funnel-shaped depression leading into the bladder, from which urine leaks. The foreskin is split and hangs down like an apron over the glans; the prostate gland is absent; testicular atrophy, cryptorchidism, congenital hernias, rectal sphincter atony, and other anomalies are observed. In total epispadias, sexual life is often impossible.

In all forms of epispadias, upward curvature of the penis is noted. However, unlike hypospadias—where the curvature is caused by scarring—the curvature in epispadias results from a mismatch in the length and abnormal positioning of the crura of the corpora cavernosa, related to anatomical incongruity of the pubic and ischial BONES OF THE pelvis.

Epispadias in girls is less common than in boys. Clitoric, subpubic, and total forms are distinguished. In female epispadias, the urethra courses above the clitoris, with the anterior portion or the entire urethral channel converted into an open groove. The superior commissure of the labia majora and minora is absent, and the clitoris is typically split. Epispadias does not affect female sexual function, pregnancy, or childbirth.

Mild forms of epispadias do not require treatment. Severe forms are an indication for surgery, the MAIN OBJECTIVES OF which are urethral reconstruction and the creation of a voluntarily controlled urinary sphincter. In cases of urinary incontinence, primary attention is directed toward reconstructing the bladder sphincter. It is advisable to operate on children between the ages of 5 and 8 years.