Renal & Urology: Hypospadias; Hydrocele; PUV Flashcards
Define what is meant by Hypospadias [1]
Hypospadias is a condition affecting males, where the urethral meatus (the opening of the urethra) is abnormally displaced to the ventral side (underside) of the penis, towards the scrotum.
- This might be further towards the bottom of the glans (in 90% of cases), halfway down the shaft or even at the base of the shaft.
- Epispadias is where the meatus is displaced to the dorsal side (top side) of the penis. Usually, the foreskin is abnormally formed to match the position of the meatus. There can also be an associated condition called chordee, where the head of the penis bends downwards.
What are the presenting features of hypospadias [5]
Abnormal location of urethral meatus:
- The most characteristic feature is an ectopic urethral opening that can be found anywhere from the glans penis to the perineum.
- The severity ranges from glandular (meatus near normal position), penile shaft, penoscrotal to perineal hypospadias.
Penile curvature (chordee):
- Chordee refers to abnormal ventral curvature of the penis which can be mild or severe.
- It is generally more pronounced during erection and may interfere with sexual function in adulthood if left untreated.
Foreskin abnormalities:
- Incomplete foreskin development on the underside of the penis results in a dorsal hooded prepuce with lack of foreskin ventrally, giving a **characteristic ‘hooded’ appearance. **
- This may lead to misdiagnosis as circumcision in infancy.
Inguinal hernia and cryptorchidism:
- These conditions are often associated with proximal hypospadias due to common embryological origin.
- Cryptorchidism refers to undescended testes while inguinal hernia involves protrusion of abdominal contents into inguinal canal.
Dysfunctional voiding:
- Depending on severity and location of meatal displacement, individuals may experience abnormal urine stream direction or difficulty in voiding. Urinary symptoms may include spraying, dribbling or split stream
How are hypospadias managed? [2]
Hypospadias requires referral to a paediatric specialist urologist for ongoing management. It is important to warn parents not to circumcise the infant until a urologist indicates this is ok.
* Mild cases may not require any treatment
* Surgery is usually performed after 3 – 4 months of age (ZtF), or 12months (PM)
* Surgery aims to correct the position of the meatus and straighten the penis
Hypospadias most commonly occurs as an isolated disorder. However, associated conditions include [2]
Hypospadias most commonly occurs as an isolated disorder. However, associated conditions include cryptorchidism (present in 10%) and inguinal hernia.
A child is dx with hypospadias. What is essential to instruct the parents? [1]
it is essential that the child is not circumcised prior to the surgery as the foreskin may be used in the corrective procedure
The management of hypospadias can be complex, with a variety of potential complications that may arise postoperatively or later in life. The most common include: [3]
Fistula formation:
- This is one of the most frequently encountered complications following hypospadias repair, occurring when an abnormal connection forms between the urethra and the skin. Factors such as tension on suture lines, infection, or poor tissue healing can contribute to fistula development.
Meatal stenosis:
- Narrowing at the meatus can occur due to scarring or inadequate sizing during surgery. It often presents with symptoms such as straining during urination or a deflected urinary stream.
Urethral stricture:
- This complication involves narrowing along any part of the urethra, potentially leading to obstructive uropathy if severe. Strictures may be caused by scar tissue formation after surgery.
What causes a hydrocele? [1]
Simple hydroceles are common in newborn males. They occurs where fluid is trapped in the tunica vaginalis. Usually this fluid gets reabsorbed over time and the hydrocele disappears.
Hydroceles may develop secondary to [3]
epididymo-orchitis
testicular torsion
testicular tumours
Hydroceles can be divided into communicating and non-communicating. What are the differences? [2]
communicating:
- caused by patency of the processus vaginalis allowing peritoneal fluid to drain down into the scrotum.
- Communicating hydroceles are common in newborn males (clinically apparent in 5-10%) and usually resolve within the first few months of life
non-communicating:
- caused by excessive fluid production within the tunica vaginalis
Describe the features of a hydrocele [4]
- soft, non-tender swelling of the hemi-scrotum. Usually anterior to and below the testicle
- the swelling is confined to the scrotum, you can get ‘above’ the mass on examination
- transilluminates with a pen torch
- the testis may be difficult to palpate if the hydrocele is large
Describe how you manage hydroceles:
- infantile hydroceles [1]
- adults [1]
Management
* infantile hydroceles are generally repaired if they do not resolve spontaneously by the age of 1-2 years
* in adults a conservative approach may be taken depending on the severity of the presentation. Further investigation (e.g. ultrasound) is usually warranted however to exclude any underlying cause such as a tumour
Define what is meant by a congenital undescended testis [1]
A congenital undescended testis is one that has failed to reach the bottom of the scrotum by 3 months of age.
In the vast majority of cases the cause of the maldescent of tests is unknown. A proportion may be associated with other congenital defects including: [5]
- Patent processus vaginalis
- Abnormal epididymis
- Cerebral palsy
- Mental retardation
- Wilms tumour
- Abdominal wall defects (e.g. gastroschisis, prune belly syndrome)
What are the reasons for correction of cryptochordism? [4]
- Reduce risk of infertility
- Allows the testes to be examined for testicular cancer
- Avoid testicular torsion
- Cosmetic appearance
NB: Males with undescended testis are 40 times as likely to develop testicular cancer (seminoma) as males without undescended testis
The location of the undescended testis affects the relative risk of testicular cancer (50% intra-abdominal testes)
Describe the treatment for cryptochordism [3]
Orchidopexy at 6- 18 months of age.
- The operation usually consists of inguinal exploration, mobilisation of the testis and implantation into a dartos pouch.
Intra-abdominal testis should be evaluated laparoscopically and mobilised. Whether this is a single stage or two stage procedure depends upon the exact location.
After the age of 2 years in untreated individuals the Sertoli cells will degrade and those presenting late in teenage years may be better served by orchidectomy than to try and salvage a non functioning testis with an increased risk of malignancy.
Describe what is meant by a posterior urethral valve, what it causes a risk of [2]
A posterior urethral valve is where there is tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output.
- It occurs in newborn boys.
- The obstruction to the outflow of urine creates a back pressure into the bladder, ureters and up to the kidneys, causing hydronephrosis.
- A restriction in the outflow of urine prevents the bladder from fully emptying, leading to a reservoir of urine that increases the risk of urinary tract infections.
Describe the presentation of posterior urethral valves [+]
- Difficulty urinating
- Weak urinary stream
- Chronic urinary retention
- Palpable bladder
- Recurrent urinary tract infections
- Impaired kidney function
Severe cases can cause obstruction to urine outflow in the developing fetus resulting in bilateral hydronephrosis and oligohydramnios (low amniotic fluid volume). The oligohydramnios leads to underdeveloped fetal lungs (pulmonary hypoplasia) with respiratory failure shortly after birth.
Severe cases of posterior urethral valves may be picked up antenatally as [2]
How should you investigate cases presenting after birth?
Severe cases may be picked up on antenatal scans as oligohydramnios and hydronephrosis.
To investigate cases presenting after birth, for example young boys presenting with urinary tract infections:
* Abdominal ultrasound may show an enlarged, thickened bladder and bilateral hydronephrosis
* Micturating cystourethrogram (MCUG) shows the location of the extra urethral tissue and reflux of urine back into the bladder
* Cystoscopy involves a camera inserted into the urethra to get a detailed view of the extra tissue. Cystoscopy can be used to ablate or remove the extra tissue.
How do you manage PUV? [2]
Mild cases may simply be observed and monitored
- If required a temporary urinary catheter can be inserted to bypass the valve whilst awaiting definitive management.
Definitive management is by ablation or removal of the extra urethral tissue, usually during cystoscopy.
Consider [] in a child under the age of 5 years presenting with a mass in the abdomen.
Consider a Wilms tumour in a child under the age of 5 years presenting with a mass in the abdomen.
Consider a Wilms tumour in a child under the age of 5 years presenting with a mass in the abdomen. The parents may have noticed the mass, or they may present with signs and symptoms of:
[5]
Consider a Wilms tumour in a child under the age of 5 years presenting with a mass in the abdomen. The parents may have noticed the mass, or they may present with signs and symptoms of:
Abdominal pain
Abdominal mass
- Unilateral in 95% of cases
Haematuria - painless
Lethargy
Fever
Hypertension
Weight loss
Dx [3] and Mx of Wilms tumour?
Diagnosis:
* The initial investigation is an ultrasound of the abdomen to visualise the kidneys.
* A CT or MRI scan can be used to stage the tumour
* Biopsy to identify the histology is required to make a definitive diagnosis.
Management
- Treatment involves surgical excision of the tumour along with the affected kidney (nephrectomy).
- Adjuvant treatment refers to treatment that is given after the initial management with surgery. This depends on the stage of the disease, the histology and whether it has spread. The main options are:
Adjuvant chemotherapy
Adjuvant radiotherapy
NICE cancer referral guidelines for Wilms’ tumour suggest the following [3]
- a palpable abdominal mass
- an unexplained enlarged abdominal organ
- unexplained visible haematuria.
How do you distinguish between a hydrocele and a hernia? [+]
Lecture
How do you investigate and manage unilateral undescended testes? [2]
Unilateral UDT:
- Without hypospadias - no further investigations required
Mx:
- Orchidopexy from 6 months
How do you investigate and manage biilateral undescended testes / unilateral w/ hypospadias? [2]
Rule out disorder of sexual development
USS
Lecture
What are the 4 main reasons for acute scrotal pain in children? [4]
Testicular torsion
Torsion of testicular / epididymal appendages
Trauma
Epididymo-orchitis
Lecture
How do you investigate for acute scrotal pain in children? [2]
MSU for epididymo-orchitis
Urgent scrotal exploration
- unless torsion can be confidently excluded
What is important to note about the presentation of neonatal torsion ?[1]
Woody-hard testis - can be painless
Dx? [1]
Idiopathic scrotal oedema
Define: [2]
- Balanitis
- Phimosis
Phimosis
* Irretractile foreskin
* Mostly physiological - ie normal < 5yr
* Does not require circumcision
* Retropreputial secretions “pearls”, “cysts”
Balanitis
* Inflammation of foreskin
* Red, swollen, (pus)
What is paraphimosis? [1]
What is the early [1] and late [1] mx?
Tight prepuce stuck behind corona of glans - causes oedema of glans / forskin
Early: manual reduction
Late: dorsal slit / circumcision
CI for circumcision [2]
Hypospadias
Buried penis
Indications for circumcision? [2]
BXO
Recurrent balanitis with scarred foreskin