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Disorders of the urethra
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Posterior Urethral Valves (PUV)
Congenital Proximal Urethral Obstruction Abnormal congenital mucosal folds in the prostatic urethra that look like a thin membrane that impairs bladder drainage
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PUV Defined Type I Type II Type III
Obstructing membrane that extends distally from each side of the verumontanum towards the membranous urethra where they fuse anteriorly Type II Described as folds extending cephalad from the verumontanum to the bladder neck Type III Represent a diaphragm or ring-like membrane with a central aperture just distal to the verumontanum Thought to represent incompelte dissolution of the urogenital membrane
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Type I PUV Obstructing membrane radiating distally from the posterior edge of the verumontanum to the membranous urethra During voiding, the fused anterior portion bulges into the urethra with a narrow posterior opening Possibly due to anomalous insertion of the mesonephric ducts into the primitive fetal cloaca
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Type I PUV
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Type III PUV Represent incomplete dissolution of the UG membrane
Distal to the verumontanum at the membranous urethra Ring-like with a central opening, “wind sock valve”
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Incidence Males only 1: 5000 – 8000 male births Type I > 95%
Type III - 5% Children with Type III PUVs have a worse prognosis as a group 50% of patients with PUV will have vesicoureteral reflux 50% unilateral, 50% bilateral
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Clinical Presentation
Varies by degree of obstruction Symptoms vary by age of presentation Antenatal Bilateral hydronephrosis Distended and thickened bladder Dilated prostatic urethra Oligohydramnios - accounts for co-presentation of pulmonary hypoplasia.
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Clinical Presentation
Newborn Palpable abdominal mass Distended bladder, hydronephrotic kidney Bladder may feel like a small walnut in the suprapubic area Ascites 40% of time due to obstructive uropathy History of Oligohydramnios Respiratory distress from pulmonary hypoplasia Severity often does not correlate with degree obstruction Primary cause of death in newborns
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Clinical Presentation
Early Infancy Dribbling / poor urinary stream Urosepsis Dehydration Electrolyte abnormalities Uremia Failure to thrive; due to renal insufficiency Toddlers Better renal function (less obstruction) Febrile UTI Voiding dysfunction – incontinence Daytime incontinence may be the only symptom in boys with less severe obstruction
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Initial Management Bladder Drainage
A 5 or 8 Fr pediatric feeding tube is ideal A Foley catheter should not be used, due to the tendency of the balloon to occlude the ureteral orifice and cause a bladder spasm. Secondary obstruction Broad spectrum antibiotic coverage Metabolic panel Assess renal function and metabolic abnormalities Acidosis, hyperkalemia common problems
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Radiologic Evaluation of the Lower Tract
VCUG Mandatory for all PUV evaluations Showing a dilated prostatic urethra, valve leaflets, detrusor hypertrophy, bladder diverticula, bladder neck hypertrophy, and narrow penile urethra stream, as well as possible incomplete emptying
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VCUG dilated prostatic urethra valve leaflets detrusor hypertrophy
cellules or bladder diverticula bladder neck hypertrophy narrow penile urethra stream possible incomplete emptying
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Radiologic Evaluation of the Lower Tract
U/S Examining the prostatic urethra for characteristic dilation and thickening of the bladder wall
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Case Report
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Management Transurethral Valve Ablation
Incise at 4, 8 & 12 o’clock positions via Pediatric resectoscope Avoid urethral sphincter Catheter drainage for 1-2 days VCUG at 2 months to ensure destruction of valves Regular U/S to evaluate resolution of hydronephrosis
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Management Transurethral Valve Ablation
Alternatively, 8F cystoscope with a Bugbee electrode adjacent Insulated crochet hook (“Whitaker hook”) When urethra too small to accommodate cystoscope/Bugbee
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Treatment Consist of destruction of the valves. In children with mild to moderate obstruction transurethral fulguration of the valves is usually successful. The more severe degree of obstruction require individualized management. Treatment of children with urosepsis & uremia associated with hydronephrosis include use of antibiotics, catheter drainage of the bladder, & correction of the fluid & electrolyte imbalance.
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Vesicostomy percutaneous loop ureterostomies may be done to preserve renal function.
After renal function is stabilized, valve ablation & reconstruction of the urinary tract can be done. The period of proximal diversion should be as short as possible, since vesical contracture can be permanent after prolong supravesical diversion.
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50% of patients with posterior urethral valves have vesicourethral reflux, after removal of obstruction reflux cease spontaneously in about one third of the patients. The remaining should be corrected surgically. Long term use of antibiotics is often required to prevent recurrent urosepsis & UTI even though the obstruction has been relieved.
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URETHRAL STRICTURES
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More common in males than females
it is either congenital or acquired, most acquired strictures are due to infection or trauma. Infection remains a major cause particularly infection from long term user of indwelling catheters. External trauma eg pelvic fracture can partially or completely sever the membranous urethra & cause severe & complex urethral stricture. Straddle injuries can produce bulbar stricture.
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Urethral strictures are fibrotic narrowing compose of dens collagen & fibroblasts.
These narrowing restrict urine flow & cause dilatation of the proximal urethra & prostatic ducts The bladder muscle may become hypertrophic, & increased residual urine may be noted. Sever prolong obstruction may lead to reflux, hydronephrosis, & renal failure. Urethral fistula & periurethral abscess commonly develop in chronic severe stricture.
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Clinical findings A decrease in urinary stream is the most common complaint Spraying or double stream is often noted, as is postvoiding dribbling Acute cystitis or symptoms of infections are seen at times Acute urinary retention seldom occurs unless infection or prostatic infection develops Urinary frequency & mild dysuria may be initial complaint.
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On examination Indurations in the area of the stricture may be palpable Tender enlarged masses along the urethra usually represent periurethral abscess Urethrocutaneous fistula may be present The bladder may be palpable due to retention of urine.
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Laboratory findings. With stricture creating significant problem the urine flow rate will be less than 10 ml/sec. In urinalysis urine may be infected. X-Ray findings. Urethrogram or voiding cystourethrogram or both will demonstrate the location & extent of the stricture. Urethral fistula & diverticula are some times noted. Vesical stones, trabeculations, or diverticula may also be seen.
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Instrumental examination.
Urethroscopy allows visualization of the stricture. The stricture diameter can be calibrated. Complications. Chronic prostatitis, cystitis, chronic UTI, diverticula, urethrocutaneous fistula, periurethral abscess, & urethral carcinoma. Vesical calculi may develop from chronic urinary stasis & infection.
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Treatment. 1- dilatation of the urethra is not usually curative, it fracture the scar tissue as the healing occur, the scar tissue reform. 2- endoscopic urethrotomy. 3- surgical reconstruction.
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Hypospadias The condition in which the urethral meatus opens on the ventral side of the penis proximal to the tip of the glans penis Urethral development begin in utero at approximately 8 weeks & are completed by 15 weeks.
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The urethra is formed by fusion of the urethral folds along the ventral surface of the penis, which extends to the corona on the distal shaft. The glandular urethra is formed by canalization of an ectodermal cord. Hypospadias result when the fusion of urethral folds is incomplete. Incidence one in every 300 male children. Estrogen & progestin given during pregnancy are known to increase incidence
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Classification According to location: 1- glandular, opening on the proximal glans penis coronal, opening on the coronal sulcus. 3- penile shaft. 4- penoscrotal. 5- perineal.
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Hypospadias in the male is evidence of feminization.
Patients with penoscrotal & perineal should be considered to have potential intersex problems requiring appropriate evaluation. Hypospadiac new born should not be circumcised, because the preputial skin may be useful for future reconstruction.
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Clinical findings. Although new born & young children seldom have symptoms related to Hypospadias, older children & adult may complain of difficulty directing the urinary stream & stream spraying Chrodee (curvature of the penis) causes ventral pending of the penile shaft. Proximal hypospadias may cause infertility.
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Also complaining of abnormal hooded appearance of the penis caused by absent or deficient ventral foreskin. The hypospadiac meatus may be stenotic & should be carefully examined & calibrated. A meatatomy should done when stenosis exist. There is increase incidence of undescended testicles in children with hypospadias, so scrotum should be examined.
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Laboratory, X-Ray,& Endoscopic finding.
Since children with penoscrotal & perineal hypospadias often have a bifid scrotum & ambiguous genitalia, a buccal smear & karyotyping are indicated to establish the genetic sex. Urethroscopy & cystoscopy are of value to determine whether internal male sexual organs are normally developed. Excretory urography is also indicated in these patients to detect additional congenital anomalies of the kidneys & the ureters.
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Treatment For psychological reasons hypospadias should be repaired before the patient reach the school age; in most cases this can be done before the age 2. > 150 methods of corrective surgery for hypospadias have been described.
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Epispadias The urethra is displaced dorsally, & classification is based on its position in males. Glandular epispadias, the urethra opens on the dorsal aspect of the glans, which is broad & flattened. Penile type, the urethral meatus is located between the pubic symphysis & the coronal sulcus. Penopubic type.
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Pt with glandular epispadias seldom have urinary incontinence. However,
penopubic & penile type, incontinence is present in % & 75% of cases respectively. Most females with epispadias are incontinent. The pubic bone are separated as in exstrophy of the urinary bladder. Epispadias is a mild form of exstrophy & in severe cases both are coexist. Treatment, by surgery.
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Phimosis Is a condition in which contracted foreskin cannot retracted over the glans. Chronic infection from poor local hygiene is its most common cause. Calculi & squamous cell ca may developed under the foreskin. Phimosis can occur at any age.
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In diabetic older men, chronic balanoposthitis may lead to Phimosis & may be the initial presenting complaint. Children under 2 yr of age seldom have true phimosis, their relatively narrow preputial opening gradually widens & allows for normal retraction of the foreskin over the glans. Edema, erythema, & tenderness of the prepuce & the presence of purulent discharge are usual presentation.
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Treatment The initial infection should be treated with broad spectrum antimicrobial drugs. The dorsal foreskin can be slit if improved drainage is necessary. Circumcision should be done after the infection is controlled.
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Paraphimosis Is the condition in which the foreskin once retracted over the glans cannot replaced in its normal position. This is due to chronic inflammation under the redundant foreskin, which leads to contracture of the preputial opening (phimosis) & formation of tight ring of skin when the foreskin retracted behind the glans. The skin ring cause venous congestion leading to edema & engorgement of the glans, which make the condition worse. As the condition progresses, arterial occlusion & necrosis of the glans may ensues.
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Treatment Paraphimosis one of urologic emergencies can be treated by firmly squeezing the glans for 5 min to reduce the tissue edema & decrease the size of the glans. Occasionally the constricting ring require incision under local anesthesia. Antibiotic should be administrated & circumcision should be done after the infection has subside.
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Circumcision routinely performed in some countries for religious or cultural reasons. There is higher incidence of penile carcinoma in uncircumcised males, but chronic infection & poor hygiene are usually underlying factors in such instances. Circumcision is indicated in patients with -infection, -phimosis -Paraphimosis.
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