2. Congenital anomalies of the bladder, testis, penis and male urethra; Specific infections of the genitourinary tract Flashcards
Congenital anomalies of the bladder
Peri-ureteral diverticulum
Bladder extrophy - classic, cloacal, epispadias
Patent urachus
Posterior urethral valves
Peri-ureteral diverticulum
The ureteral hiatus is too large, so the distal ureter and surrounding bladder mucosa might herniate through the detrusor in the course of emptying.
Or, just an outpouching of the ureter more proximally but this is less common.
Normally the ureter traverse the bladder between the mucosa and the dtrusor muscle for 1.5-2 cm.
Bladder extrophy
• Classic bladder extrophy (60%): The cloacal membrane ruptures before the
mesoderm has separated the anterior bladder from the abdominal wall. Present
with the bladder protruding as a red mass in the suprapubic region.
• Cloacal extrophy (10%): The cloacal membrane ruptures before the separation
of the bladder and the hindgut. Massive defect in the anterior abdominal wall
with protruding bladder, ileocecal bowel and a short blind-ending colonic
segment with imperforate anus.
• Epispadias (30%)
Patent Urachus
The urachus (cranial end of developing bladder) fails to fuse and close, leading to an connection between the bladder and the umbilicus.
Leakage containing creatinine confirms the diagnosis.
Risk for infection and adenocarcinoma. Usually close spontaneously.
Posterior Urethral Valves
In some boys remnants of the mesonephric /wolfian duct persists as flaps of that obstruct the flow of urine out of the bladder.
It can cause severe obstruction in infants with palpable bladder, bilateral hydronephrosis, infection and renal insufficiency.
List the abnormal developments of testis
Failure of testicular development
Cryptochidism
Hydrocele
Indirect inguinal hernia
Varicoele
Failure of testicular development
Failure of testicular development
No testes. Can occur all along the developmental line.
- Early failure: Absence of testis and Wolfian duct
- Late failure: Absence of testis with vas present
Cryptorchidism
Failure of migration and descent of the testis. Common in premature boys.
However, most undescended testis will spontaneously descend within the first 6 months of life.
Diagnosis is made by examination with warm hands (will make cremasteric muscle contract, pulling the testis into the inguinal canal). If the testis is not palpable along the inguinal canal and scrotum, and cannot be brought into the scrotum upon examination, the diagnosis is confirmed.
Hydrocele
Occurs when the processus vaginalis fails to close, creating a tunnel between the
scrotum and the peritoneal cavity, allowing peritoneal fluid to move into the space
around the testes.
Most common in newborns. Surgery is indicated if it persists beyond 1 year.
Indirect inguinal hernia
A result of patent processus vaginalis, allowing the bowel to herniate into the inguinal canal.
Most common in premature boys. Palpable mass in inguinal canal. Surgery is
required.
Varicocele
Dilation of the pampiniform plexus. Almost 100% of idiopathic varicocele
occurs on the left side (drain into left renal vein).
Caused by incompetent valves. Usually diagnosed at 15-25 years of age.
List the abnormal developments of the penis and male urethra
Hypospadias
Epispadias
Urethral duplex
Phimosis
Inconspicuous penis - webbed, concealed, trapped
Megalourethra
Hypospadias
Fusion of the urethral folds proximal to the tip of the glans resulting in an abnormal
opening of the urethral meatus. Usually consists of 3 anomalies:
- Abnormal opening of meatus anywhere from the ventral glans to the perineum.
- Abnormal ventral curvature of the penis called chordee.
- An abnormal distribution of foreskin that is dorsally located as a “hood”.
It can be caused by decreased testosterone production or lack of androgen converting
enzyme (5-alpha-reductase), causing an incomplete virilization (masculinization).
10% also have undescended testes and/or inguinal hernia.
Epispadias
Epispadias. Caused by abnormal positioning of the genital tubercle
Open urethra on the upper dorsum of the penis and a lesser degree of separation of
pubic symphysis.
90% will have vesicoureteral reflex and 75% will present with incontinence.
30% of bladder extrophy will also have epispadias.
Urethral duplex
Very rare.
An extra urethral opening.
If it decreases the lumen of the main urethra, or if one of the urethras constantly leaks urine, surgery is recommended
Megalourethra
Megalourethra
Dilation of the urethra not due to stenosis or obstruction.
2 types:
- Dilates both dorsally and ventrally due to local lack of corpus cavernosum
- Only dilates ventrally with intact corpus cavernosum
Phimosis
At birth there is usually a natural phimosis (inability to retract the foreskin) due to
adhesions between the prepuce and glans.
During the 3-4 first years of life
smegma accumulates under the prepuce and gradually separate the foreskin from the glans.
In older boys, application of corticosteroid cream can loosen the phimotic ring in 80% of the cases.
Inconspicuous penis
A penis that appears to be small.
• Webbed penis: Scrotal skin extends onto the penis
• Concealed aka Buried penis: Penis is embedded in and concealed by an enlarged suprapubic fat pad.
• Trapped penis: Penis becomes embedded in the suprapubic fat pad after
circumcision due to too much or too little skin being removed.
Specific infections of the genitourinary tract
Candidiasis
Schistosomiasis
Tuberculosis
Candidiasis
Vaginal candidiasis, normal ph, thick purulent white discharge.
Can cause infection by local or hematogenous spread. Hematogenous especially in neutropenic patients.
Presentation
- asymptomatic
- local itching
- pyelonephritis
- candedemia
- pneumaturia in diabetics due to fermentation of glucose in urine.
Diagnosed by positive urine culture or urinary sediment/uroscopy.
Schistosomiasis
Schistosoma hematobium.
Swimmers itch - schistosomal dermatitis at the site where the skin is penetrated.
Acute schistosomiasis
systemic reaction against migrating schistosomulae as they pass through the bloodstream through the lungs to the liver.
Rash, fever, myalgia, dyspnea and cough.
Chronic urinary schisto
Adult worm pairs are distributed throughout the pelvi and mesenteric plexuses. It
presents as hematuria and dysuria. The bladder can show ulceration and polyps.
Squamous cell cancer can develop. Can also cause obstruction with hydronephrosis.
Diagnosis is made by the presence of eggs in the urine. Give praziquantel.
Tuberculosis
Causes granulomatous infection of the Kidneys and Prostates (in the genitourinary tract)
Usually asymptomatic. Destruction of the renal parenchyma over the course of 20 years.
• Can present as cystitis that does not respond to therapy
• There can also be pus in urine without detectable bacteria
• Hematuria
• Nontender, enlarged epididymis
- Also can cause destruction/fibrosis of the pelvis and ureters, obstruction and hydronephrosis.
To diagnose you can perform a tuberculin test maximal inflammation at 48-72 hrs.
Acid-fast stain (positive ~60% of the time) and Löwenstein-Jensen culture (higher rate of positives) from the first morning urination.
Chest X-ray will show the granulomas in the lungs.
Abdominal X Ray can show calcification of the renal parenchyma and granulomas/abcesses.
