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Urethral diverticula are often asymptomatic and symptoms that are present tend to be nonspecific. They can co-occur with cancer, in approximately 6-9% of cases, most commonly adenocarcinoma, but also including squamous cell carcinoma and transitional cell carcinoma. Approximately 10% of cases co-occur with kidney stones.
There are 2 types of urethral diverticulums. Congenital and acquired. In infancy usually the urethral diverticulum is congenital but in rare instances acquired urethral diverticulum can be seen in infancy specially following traumatic catheterization.
Common symptoms of urethral diverticulum include incontinence, urinary frequency and urgency, pain during sex, and pain during urination. Other symptoms include pain localized to the urethra or pelvis and frequent urinary tract infection.
When urethral diverticulum becomes severe, a painful mass can sometimes be felt inside the introitus of the vagina, which can discharge pus. If the mass is hard or bleeds, complications like cancer or kidney stones may be present.
The hallmark sign of urethral stricture is a weak urinary stream. Other symptoms include:
- Splaying of the urinary stream
- Urinary frequency
- Urinary urgency
- Straining to urinate
- Pain during urination
- Urinary tract infection
- Prostatitis
- Inability to completely empty the bladder.
Some patients with severe urethral strictures are completely unable to urinate. This is referred to as acute urinary retention, and is a medical emergency. Hydronephrosis and renal failure may also occur.
A urethral diverticulum is a condition where the urethra or the periurethral glands push into the connective tissue layers (fascia) that surround it.
Urethral strictures most commonly result from injury, urethral instrumentation, infection, non-infectious inflammatory conditions of the urethra, and after prior hypospadias surgery. Less common causes include congenital urethral strictures and those resulting from malignancy.
Urethral strictures after blunt trauma can generally be divided into two sub-types;
- Pelvic fracture-associated urethral disruption occurs in as many as 15% of severe pelvic fractures. These injuries are typically managed with suprapubic tube placement and delayed urethroplasty 3 months later. Early endoscopic realignment may be used in select cases instead of a suprapubic tube, but these patients should be monitored closely as vast majority of them will require urethroplasty.
- Blunt trauma to the perineum compresses the bulbar urethra against the pubic symphysis, causing a "crush" injury. These patients are typically treated with suprapubic tube and delayed urethroplasty.
Other specific causes of urethral stricture include:
- Instrumentation (e.g., after transurethral resection of prostate, transurethral resection of bladder tumor, or endoscopic kidney surgery)
- Infection (typically with Gonorrhea)
- Lichen sclerosus
- Surgery to address hypospadias can result in a delayed urethral stricture, even decades after the original surgery.
A prostatic stent is a stent used to keep open the male urethra and allow the passing of urine in cases of prostatic obstruction and lower urinary tract symptoms (LUTS). Prostatic obstruction is a common condition with a variety of causes. Benign prostatic hyperplasia (BPH) is the most common cause, but obstruction may also occur acutely after treatment for BPH such as transurethral needle ablation of the prostate (TUNA), transurethral resection of the prostate (TURP), transurethral microwave thermotherapy (TUMT), prostate cancer or after radiation therapy.
Most children with vesicoureteral reflux are asymptomatic. Vesicoureteral reflux may be diagnosed as a result of further evaluation of dilation of the kidney or ureters draining urine from the kidney while in utero as well as when a sibling has VUR (though routine testing in either circumstance is controversial). Reflux also increases risk of acute bladder and kidney infections, so testing for reflux may be performed after a child has one or more infections.
In infants, the signs and symptoms of a urinary tract infection may include only fever and lethargy, with poor appetite and sometimes foul-smelling urine, while older children typically present with discomfort or pain with urination and frequent urination.
Vesicoureteral reflux (VUR), also known as vesicoureteric reflux, is a condition in which urine flows retrograde, or backward, from the bladder into the ureters/kidneys. Urine normally travels in one direction (forward, or anterograde) from the kidneys to the bladder via the ureters, with a 1-way valve at the vesicoureteral (ureteral-bladder) junction preventing backflow. The valve is formed by oblique tunneling of the distal ureter through the wall of the bladder, creating a short length of ureter (1–2 cm) that can be compressed as the bladder fills. Reflux occurs if the ureter enters the bladder without sufficient tunneling, i.e., too "end-on".
A rectovaginal fistula is a medical condition where there is a fistula or abnormal connection between the rectum and the vagina.
Rectovaginal fistula may be extremely debilitating. If the opening between the rectum and vagina is wide it will allow both flatulence and feces to escape through the vagina, leading to fecal incontinence. There is an association with recurrent urinary and vaginal infections. The fistula may also connect the rectum and urethra, which is called recto-urethral fistula. Either conditions can lead to labial fusion. This type of fistula can cause pediatricians to misdiagnose imperforate anus. The severity of the symptoms will depend on the size of fistula. Most often, it appears after about one week or so after delivery.
There are two types of prostatic stent: temporary and permanent.
Although a permanent prostatic stent is not a medical treatment, it falls under the classification of a surgical procedure. Placement of a permanent prostatic stent is carried out as an outpatient treatment under local, topical or spinal anesthesia and usually takes about 15–30 minutes.
A temporary prostatic stent can be inserted in a similar manner to a Foley catheter, requiring only topical anesthesia.
A Gartner's duct cyst (sometimes incorrectly referred to as "vaginal inclusion cyst") is a benign vaginal cystic lesion that arises from the Gartner's duct, which is a vestigial remnant of the mesonephric duct (wolffian duct) in females. They are typically small asymptomatic cysts that occur along the lateral walls of the vagina, following the course of the duct. They can present in adolescence with painful menstruation (Dysmenorrhea) or difficulty inserting a tampon. They can also enlarge to substantial proportions and be mistaken for urethral diverticulum or other structures.
There is a small association between Gartner's duct cysts and metanephric urinary anomalies, such as ectopic ureter & ipsilateral renal hypoplasia. Because of this, imaging is recommended before excision.
Rectovaginal fistulas are often the result of trauma during childbirth (in which case it is known as obstetric fistula) where improper medical interventions are used, such as episiotomy with forceps/vacuum extraction or in situations where there is inadequate health care, such as in some developing countries. Rectovaginal fistula is said to be known as the leading cause in maternal death in developing countries. Risk factors include prolonged labour, difficult instrumental delivery and paramedian episiotomy. Rates in Eritrea are estimated as high as 350 per 100,000 vaginal births. Fistulas can also develop as a result of physical trauma to either the vagina or anus, including from rape. Women with rectovaginal fistulae are often stigmatized in developing countries, and become outcasts.
Rectovaginal fistula can also be a symptom of various diseases, including infection by lymphogranuloma venereum, or the unintended result of surgery, such as episiotomy or sexual reassignment surgery. They may present as a complication of vaginal surgery, including vaginal hysterectomy. They are a recognized presentation of rectal carcinoma or rarely diverticular disease of the bowel or Crohn's disease. They are seen rarely after radiotherapy treatment for cervical cancer.
Bulbar urethral necrosis is a problem that can occur after a pelvic fracture associated urethral distraction defect (PFUDD).
The signs and symptoms of hydronephrosis depend upon whether the obstruction is acute or chronic, partial or complete, unilateral or bilateral. Hydronephrosis that occurs acutely with sudden onset (as caused by a kidney stone) can cause intense pain in the flank area (between the hips and ribs). Historically, this type of pain has been described as "Dietl's crisis". Conversely, hydronephrosis that develops gradually will generally cause either attacks of a dull discomfort or no pain. Nausea and vomiting may also occur. An obstruction that occurs at the urethra or bladder outlet can cause pain and pressure resulting from distension of the bladder. Blocking the flow of urine will commonly result in urinary tract infections which can lead to the development of additional stones, fever, and blood or pus in the urine. If complete obstruction occurs, kidney failure may follow.
Blood tests may show impaired kidney function (elevated urea or creatinine) or electrolyte imbalances such as hyponatremia or hyperchloremic metabolic acidosis. Urinalysis may indicate an elevated pH due to the secondary destruction of nephrons within the affected kidney. Physical examination may detect a palpable abdominal or flank mass caused by the enlarged kidney.
Urethral meatal stenosis or urethral stricture is a narrowing (stenosis) of the opening of the urethra at the external meatus , thus constricting the opening through which urine leaves the body from the urinary bladder.
Diverticula are described as being true or false depending upon the layers involved:
- True diverticula involve all layers of the structure, including muscularis propria and adventitia, such as Meckel's diverticulum.
- False diverticula (also known as "pseudodiverticula") do not involve muscular layers or adventitia. False diverticula, in the GI tract for instance, involve only the submucosa and mucosa.
Posterior urethral valve (PUV) disorder is an obstructive developmental anomaly in the urethra and genitourinary system of male newborns. A posterior urethral valve is an obstructing membrane in the posterior male urethra as a result of abnormal "in utero" development. It is the most common cause of bladder outlet obstruction in male newborns. The disorder varies in degree, with mild cases presenting late due to milder symptoms. More severe cases can have renal and respiratory failure from lung underdevelopment as result of low amniotic fluid volumes, requiring intensive care and close monitoring. It occurs in about one in 8000 babies.
Diverticula may occur in one of the three areas of the esophagus - the pharyngoesophageal, the midesophageal area or the epiphrenic area of esophagus. Zenker's diverticulum is found three times more frequently in men than in women. It occurs posteriorly through the cricopharyngeal muscle in the midline of the neck. Usually seen in people older than 60 years of age.
- Gastric diverticula - "Although usually asymptomatic, they may cause vague epigastric pain. These lesions may be confused radiologically for gastric ulcers or cancers. Endoscopically, they may be confused for paraesophageal hernias."
- Killian-Jamieson diverticulum
- Meckel's diverticulum: a persistent portion of the omphalomesenteric duct present in 2% of the population
- Rokitansky-Aschoff sinuses: in the gallbladder due to chronic cholecystitis
- Traction esophageal diverticulum: due to scarring from mediastinal or pulmonary tuberculosis
- Urethral diverticulum: congenital in males, post-infectious in females
- Zenker's diverticulum: a diverticulum of the mucosa of the pharynx affecting adults
Most of these pathological types of diverticulum are capable of harboring an enterolith. If the enterolith stays in place, it may cause no problems, but a large enterolith expelled from a diverticulum into the lumen can cause obstruction.
Patients with erectile dysfunction (ED) and PFUDD or patients with PFUDD and traumatic disruption of the dorsal arteries are susceptible to bulbar urethral necrosis. These patients need tubularized substitution urethroplasty, which is replacement of the bulbar urethra with a various number of tubularized flaps ranging from scrotal skin to sigmoid colon (and others).
Abdominal ultrasound is of some benefit, but not diagnostic. Features that suggest posterior urethral valves are bilateral hydronephrosis, a thickened bladder wall with thickened smooth muscle trabeculations, and bladder diverticula.
Voiding cystourethrogram (VCUG) is more specific for the diagnosis. Normal "plicae circularis" are variable in appearance and often not seen on normal VCUGs. PUV on voiding cystourethrogram is characterized by an abrupt tapering of urethral caliber near the verumontanum, with the specific level depending on the developmental variant. Vesicoureteral reflux is also seen in over 50% of cases. Very often the posterior urethra maybe dilated thus making the abrupt narrowing more obvious. the bladder wall may show trabeculations or sacculations or even diverticuli.
Diagnosis can also be made by cystoscopy, where a small camera is inserted into the urethra for direct visualization of the posteriorly positioned valve. A limitation of this technique is that posterior valve tissue is translucent and can be pushed against the wall of the urethra by inflowing irrigation fluid, making it difficult to visualize. Cystoscopy may also demonstrate the bladder changes.
Centers in Europe and Japan have also had excellent results with cystosonography, although it has not been approved for use in the United States yet.
Obstructive uropathy is a structural or functional hindrance of normal urine flow, sometimes leading to renal dysfunction (obstructive nephropathy).
It is a very broad term, and does not imply a location or cause.
Rupture of the urethra is an uncommon result of penile injury, incorrect catheter insertion, straddle injury, or pelvic girdle fracture. The urethra, the muscular tube that allows for urination, may be damaged by trauma. When urethral rupture occurs, urine may extravasate (escape) into the surrounding tissues. The membranous urethra is most likely to be injured in pelvic fractures, allowing urine and blood to enter the deep perineal space and subperitoneal spaces via the genital hiatus. The spongy urethra is most likely to be injured with a catheter or in a straddle injury, allowing urine and blood to escape into the scrotum, the penis, and the superficial peritoneal space. Urethral rupture may be diagnosed with a cystourethrogram. Due to the tight adherence of the fascia lata, urine from a urethral rupture cannot spread into the thighs.
Hydronephrosis—literally "water inside the kidney"—refers to distension and dilation of the renal pelvis and calyces, usually caused by urinary retention due to obstruction of the free flow of urine from the kidney. Untreated, it leads to progressive atrophy of the kidney. One or both kidneys may be affected. In cases of hydroureteronephrosis, there is distention of both the ureter and the renal pelvis and calices.
It can be caused by a lesion at any point in the urinary tract.
Causes include urolithiasis, posterior urethral valves and ureteral herniation.
Extravasation of urine refers to the condition where an interruption of the urethra leads to a collection of urine in other cavities, such as the scrotum or the penis in males. It can be associated with a calculus.
The protection provided by the foreskin for the glans penis and meatus has been recognized since 1915. In the absence of the foreskin the meatus is exposed to mechanical and chemical irritation from ammoniacal diaper (nappie) that produces blister formation and ulceration of the urethral opening, which eventually gives rise to meatal stenosis (a narrowing of the opening). Meatal stenosis may also be caused by ischemia resulting from damage to the frenular artery during circumcision.