Interactive Tutorial: Paediatric Urology Flashcards

1
Q

Paediatric Urology

A
  • Problems of genitalia + urinary tract in children
  • Diagnosed by antenatal USG / UTI after birth

Scope:
1. UTI

  1. Penile disorders
    - Phimosis
    - Hypospadias
    - Chordee
    - Buried penis
  2. Scrotal pathology
    - Undescended testes
    - Torsion
    - Varicocele
  3. Vesicoureteral reflux
  4. Urinary obstruction
    - PUJO
    - VUJO
    - Posterior urethral valve (PUV)
  5. Bladder function condition
    - Neurogenic bladder
    - Other voiding dysfunction problems
  6. Congenital anomalies
    - Hydronephrosis
    - Duplex kidneys
    - Cystic kidney disease
  7. Stone disease
  8. Paediatric oncology
  9. GU trauma
  10. Disorders of sex development
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

History

A
  1. ***Age
  2. ***Presenting complaint
  3. History
  4. Medications + allergy
  5. Past medical history
    - Antenatal USG
  6. Elimination / Voiding history
    - Voiding frequency
    - Holding maneuvers (may suggest neurogenic bladder)
    - Incontinence (day + night)
    - Bowel movements (hard stool / constipation put more pressure on bladder)
    - Fluid intake (type + volume)
  7. Family history
    - Recurrent UTI
    - Nocturnal enuresis (paternal side / uncles)
    - Vesicoureteral reflux
    - Cystic kidney disease / Absence of kidney
    - Hypospadias / Cryptochidism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

P/E

A
  1. Abdominal exam
    - Masses
    - Pain (tender spot)
    - Palpable bladder
    - Ballotable kidneys
  2. GU exam
    - Nappy rash (signify continuous dribbling of urine)
    - Labial adhesions (cause urinary leakage)
    - Urethral prolapse (in female can cause constipation, other symptoms: haematuria, urinary leakage)
    - Ureterocele prolapse (obstruction of urinary tract, need to check esp. in those UTI + high fever)
    - Urethral opening (location)
    - Foreskin (phimosis vs retractable)
  3. Back exam
    - Sacral dimples
    - Hairy patches
    —> Spina bifida occulta
  4. Watch peeing process
    - Urine stream (strong, any deviation of stream signify abnormal urethral opening)
  5. Post-void residual
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Phimosis, Circumcision and other prepuce disorder

A

Phimosis: a pathological condition
- secondary to Balanitis xerotica obliterans (BXO)
- chronic + progressive inflammatory condition characterised by hyperkeratosis (~ lichen sclerosis in skin) affecting glans + prepuce —> scarred + non-retractile foreskin, narrow preputial ring, meatal stenosis if glans involved

Physiological phimosis:
- physiological tightness of foreskin
- natural condition in which prepuce cannot be retracted
- natural adhesion between glans and prepuce
- with time will become retractable
- only problematic when complications arise e.g. UTI

Signs:
- Ballooning of prepuce during peeing
- Balanitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Circumcision

A

Indications:
- BXO
- Severe recurrent attacks of balanoposthitis
- Recurrent febrile UTI
- Penile malignancy
- Traumatic foreskin injury where it cannot be salvaged
- Underlying urological anomalies (to prevent UTI)
—> NO absolute medical indication for circumcision in ***neonatal period

Potential medical advantages:
- ↓ incidence of UTI in first year of life
- Prevent phimosis
- Prevent balanoposthitis (superficial infection of glans + foreskin)
- ↓ incidence of penile cancer
- may ↓ incidence of STD / HIV (safe sex practice ONLY way to prevent STD / HIV)

Method:
1. Device method
- Gomco clamp
- Plastibell clamp
- Mogen clamp
2. Surgical operation
- Free hand surgery

Complications:
- 0.2-0.5% overall
- Bleeding
- Injury to penis (e.g. amputation of glans)
- Skin issues (taking off / leaving on too much, skin bridges, inclusion cysts, penile curvature (∵ uneven skin removal), urethrocutaneous fistula)
- Long term (***meatal stenosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Foreskin care

A
  • By 3 yo 90% boys will have retractable foreskin

Suggestion to parents:
- Normal cleaning
- No forceful retraction
- Teach boys to pull back foreskin to void

Treatment only necessary for foreskin causing **infection / **difficulty voiding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Paraphimosis

A
  • Painful constriction of glans penis by foreskin which has been retracted behind the corona
  • Urgent condition

Treatment:
- Manual reduction +/- Dorsal slit of foreskin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Buried / Hidden penis

A

May have problem with loss of anchoring of penis and pubic symphysis

  1. Webbed penis
    - penis enclosed by skin of scrotum which extends onto its shaft
  2. Trapped penis
    - normal-sized penis that is partially stuck in the pubic fat pad, scarring / adhesion prevent retraction of fat pad
  3. Concealed penis
    - normal size penis hidden in pubic fat pad but is retractable

Management:
- Preputioplasty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Testicular pain

A

Causes:
1. Testicular torsion
2. Torsion of testicular appendix
3. Orchitis
4. Epididymitis
5. Constipation / other abdominal conditions

Testicular torsion:
- Adolescent (not always)
- **Sudden onset
- Severe sharp pain
- **
Sometimes only abdominal pain
- **N+V
- Abnormal lie of the testicle (e.g. transverse lie / oblique lie)
- **
High lying testicle
- ***Ideally fix within 6 hours

Torsion of testicular appendix:
- Sudden onset of pain
- Pre-adolescent
- **Pinpoint tenderness
- **
Blue dot sign (not always, bluish patch discolouration on scrotum)
- Over time can cause local inflammation which looks like epididymitis / torsion of testes on USG —> may need surgical exploration of scrotum

Epididymitis / Orchitis:
- Adolescent / older
- ***Gradual onset
- Tender superior portion (esp. tip of epididymis)
- May be preceded by URTI / UTI

Investigation:
- Look like torsion —> straight to operation
- Urinalysis + culture —> if normal —> unlikely to be epididymitis
- Scrotal USG (but should not waste time in highly suspicious patients)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Undescended testicle (Cryptorchidism)

A
  • Most common birth abnormality involving male genitalia (0.8% incidence at 6 months)
  • All (except premature infants) will descend in first 3 months of life —> if undescended at 3 months —> ***Refer specialist + Plan for surgery
  • ***Retractile testicle is a normal descended testicle that is pulled out of the scrotum by an overactive cremasteric reflex (normal position during normal situation)

P/E:
- Bimanual examination (one hand on abdomen swiping testicles down to scrotum, other hand try grasp testicle from scrotum)
—> Palpable undescended testicle (i.e. within Scrotum / Inguinal canal) —> Orchidopexy
—> Retractile testicle —> Monitor —> If ascending testis syndrome —> Orchidopexy

—> Non-palpable testicle —> EUA
—> Palpable testis —> Orchidopexy
—> Non-palpable —> Laparoscopy (try to find testicle) —>
1. Intraabdominal testicles —> Primary / Staged Fowler-Stephens Orchidopexy
2. Vas / vessels entering canal (i.e. testis should be in inguinal canal but just not enter scrotum) —> Inguinal exploration + orchidopexy
3. Blind-ending vas / vessels (i.e. no testicles at all) —> Vanishing testis

Fowler-Stephens orchidopexy:
- indication: >2 cm from inguinal ring
- clip spermatic vessels >1 cm cranial to testis
- wait >=6 months then perform laparoscopic stage 2 Fowler-Stephen orchidopexy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Hypospadias

A

Disease spectrum (different level of urethral opening):
- mild: glandular / subcoronal
- moderate: mid-penile
- severe: perineal / scrotal

Clinical features:
- Ventral + proximally located urethral meatus
- Chordee (ventral curvature of penis)
- Hooded prepuce (deficient ventral prepuce)

Epidemiology:
- Incidence: 1/300 male births
- Associations:
—> Cryptorchidism (9.3%) (Hypospadias + Cryptorchidism —> need to consider whether there is disorder of sexual differentiation —> chromosomal abnormality)
—> Inguinal hernia (9%)

Factors (Multifactorial):
- Endocrine (disruption in synthetic biopathway of androgens, maybe a delay in maturation of HPG axis)
- Genetic (familial rate 7%)
- Environmental (endocrine disrupters in environment may be responsible for increase in incidence)
- Maternal (maternal progrestin exposure may increase likelihood of hypospadias, some studies show a marked increase in hypospadias in women undergoing IVF)

Management:
- ***Do NOT circumcise (need foreskin for reconstruction, others: buccal mucosa, tunica vaginalis)
- NO need imaging studies
- Refer to paediatric urologist within 1st year of life
- Always consider DSD (Disorder of sexual differentiation) if hypospadias associated with undescended testes

Operative strategy:
- Mathieu urethroplasty
- Snodgrass urethroplasty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

***Enuresis

A

Enuresis:
- Involuntary urinary leakage only during night time
- Apply to patients >=5 yo only

Primary:
- Always wet the bed at night since birth

Secondary:
- Was dry at night (>=6 months) and now wetting the bed again

Prevalence:
- 15-20% of 5 yo
- 1-2% adolescents (majority will outgrow enuresis)

Cause (Multifactorial):
- Genetic
- Maturational delay
- Difficulty wakening
- Psychological (e.g. ADHD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

***Causes of Urinary incontinence

A
  1. ***Primary nocturnal enuresis (most common)
  2. ***Anatomic incontinence
    - Ectopic ureter (Extravesical)
    - Obstruction (PUV, (PUJO, VUJO, Labial adhesions, Urethral prolapse, Ureterocele))
    - Anomalies (Exstrophy, (Abnormal urethral opening))
  3. ***Neurogenic incontinence
    - Congenital (Neural tube defects (Spina bifida, Myelomeningocele, Anorectal malformations))
    - Acquired (Trauma, Tumour, Anoxic brain injury, Extensive pelvic surgery)
  4. ***Functional incontinence (when no anatomical / neurological cause found)
    - Detrusor overactivity
    - Infrequent voider (Lazy bladder)
    - Dysfunctional voiding (DSD)
  5. Miscellaneous (UTI, Polyuria)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Primary Monosymptomatic Nocturnal Enuresis (PMNE)

A
  • Primary: wet bed since birth, never been dry
  • Monosymptomatic: no other symptoms
    —> no daytime enuresis
    —> no urinary frequency
    —> no urgency
    —> no pain
    —> no UTI
  • Nocturnal enuresis: only wet at night time

Diagnosis:
- ***Clinical diagnosis
- Important to differentiate from “Non-monosymptomatic” / Secondary enuresis —> need investigations

Investigations:
1. ***Bladder diary
- distinguish MNE from NMNE
- volume intake
- bladder capacity
- daytime + nocturnal urine production
- consecutive 48 hours

  1. ***Urinalysis
    - UTI
    - osmolarity issues

Others (Urinalysis and ***nothing further if only PMNE, only when refractory to treatment / daytime symptoms):
3. USG urinary system
4. XR spine (spina bifida occulta)
5. Urodynamic study

Treatment:
1. **Reassurance
2. **
Lifestyle modification (no liquids after dinner, void before sleep, no caffeinated beverages)
3. **Star chart
4. **
Bed / Enuresis alarm
5. ***DDAVP for large night time urine volume

Prognosis:
- Patient usually outgrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

***Antenatal hydronephrosis

A

Dilation of the renal collecting system during antenatal period
- incidence 1/100 pregnancy (only 1/500 are significant abnormalities)

**Causes:
1. **
Normal (most common)
2. **Vesicoureteral reflux (VUR)
3. **
Multicystic dysplastic kidney (MCDK)
4. **Obstructive uropathies
- Posterior urethral valve (PUV)
- Pelvi-ureteric junction obstruction (PUJO)
- Vesicoureteric junction obstruction (VUJO / megaureter)
5. **
Prune Belly syndrome

Grading:
1. Quantitative Antero-Posterior diameter (APD):
- AP diameter between ***renal pelvis
- easy to understand
- inter + intra-observer error
- some important parameters are missing e.g. ureteral dilatation, calyceal dilatation, bladder abnormalities

  1. SFU grading (Qualitative):
    - description of dilatation of renal pelvis and calyces —> see whether there is parenchymal involvement
    - parenchymal thickness
    - grade 1-4
    - APD >4mm (2nd trimester) / >7mm (3rd trimester)
  2. UT dilatation (Combined Quantitative + Qualitative classification):
    - ADP
    - Calyceal dilation
    - Parenchymal thickness
    - Appearance
    - Bladder abnormalities
    - Urethral abnormalities
    - Based on gestational age
    —> used pre / post-natally

Investigations:
1. Mild-Moderate Unilateral hydronephrosis
- USG at **2-6 weeks
- +/- **
MCUG / ***MAG3 radionuclei scan

  1. Bilateral / Severe hydronephrosis / Dilated posterior urethra
    - ***Day 2 USG
    - +/- MCUG / MAG3 radionuclei scan

MCUG:
- only show VUR, PUV
- cannot show PUJO / VUJO (∵ contrast normally cannot flow back up anyway)

MAG3 scan:
- show drainage function
- prolonged diuretic t1/2 signify obstructive component

(3. RFT
- Too early for baby (only reflect mother’s renal function))

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Indications of MCUG

A
  1. SFU and Canadian urological association
    - SFU grade 4
    - SFU grade 3 individualised approach
    - SFU grade 0-2 deferred
  2. Bilateral disease
  3. Dilated ureter
  4. Duplex kidney
  5. Abnormal bladder
  6. Ureterocele
  7. Abnormal renal cortex
17
Q

***UTI

A

Presentation:
Young children (non-specific):
- Febrile
- Decreased appetite
- Lethargy

Older children (more specific):
- Febrile (implies pyelonephritis)
- Dysuria
- Frequency
- Abdominal / Loin pain

History:
1. Fever

  1. Voiding history
    - Voiding frequency
    - Holding maneuvers (may suggest neurogenic bladder)
    - Incontinence (day + night)
    - Bowel movements (hard stool / constipation put more pressure on bladder)
    - Fluid intake (type + volume)
  2. Family history
    - Recurrent UTI

Investigations:
1. Urinalysis
- Cath specimen ideally
- Bag specimens (but can be contaminated easily)

  1. Radiology (for Febrile UTI)
    - USG
    - MCUG
    - DMSA —> document that it is a pyelonephritis

Diagnosis:
- ***Clean catch urine sample preferred
- if not successful:
—> Bag urine (easy but not a good choice)
—> Catheterised sample (transurethral / suprapubic)

Treatment:
1. Lower tract infection
- short course antibiotic

  1. Upper tract (fever, back pain, N+V)
    - 2 week course antibiotic
    - admission if very ill
    - quick treatment decreases chance of scarring
18
Q

***Vesicoureteral reflux (VUR)

A
  • Common cause of UTI in children
  • Abnormal retrograde flow of bladder urine back into upper urinary tract through ***incompetent VUJ
  • Sibling predisposition (40% of siblings)
  • 25-30% of antenatal hydronephrosis + 1% of newborn

Presentation:
1. Hydronephrosis in Antenatal USG
2. UTI
- up to 30% with other GU anomalies
- males present earlier due to shorter ureters but girls 2x more likely to have reflux
3. Renal scarring

Grading:
- Grade 1, 2: mild
- Grade 3: moderate
- Grade 4, 5: severe

(SpC Paed:
Grade 1: Fills ureter without dilation
Grade 2: Fills ureter + collecting system without dilation
Grade 3: Mildly dilates ureter + collecting system with mild blunting of calyces
Grade 4: Grossly dilates ureter + collecting system with blunting of calyces
Grade 5: Gross dilates collecting system: ALL calyces blunted with loss of papillary impression +/- Intrarenal reflux + Significant ureteral dilation + tortuosity)

Classification:
1. Primary
- deficiency in formation of VUJ
- majority resolve with time (75% will outgrow)

  1. Secondary
    - a result of urinary tract ***dysfunction
    —> dysfunctional voiding
    —> neuropathic bladder
    —> obstruction
    —> ?infection
    - must correct the predisposing factor

Investigation:
1. **MCUG (gold standard, involves catheterisation + radiation)
2. **
Voiding USG (involve catheterisation but no radiation)

Treatment:
1. ***Antibiotic prophylaxis + observation
- Amoxicillin (for <2 months)
- TMP-SMX (co-trimoxazole / septrin) / Nitrofurantoin QHS
- Surveillance USG
- MCUG
- Protect kidney growth until it stops at 5 yo (then can stop antibiotics) (SpC Revision)

  1. Surgical intervention
    - Indications: breakthrough infections, poor compliance with medications, renal scarring
    - **Endoscopic: “Deflux” bulking agent injection
    - **
    Operative ureteric reimplantation: Open vs Laparoscopic vs Robotic
  2. Circumcision
    - Boys under 6 months of age: Risk of UTI ↓ by 10x
    - ***Grade 4/5 boys under 2 yo
  3. General advice
    - Primary VUR presenting with UTI
    —> treat constipation +/- bladder bowel dysfunction
    —> adequate fluid intake
    —> appropriate + prompt evaluation of febrile illness
    —> consider circumcision for boys
19
Q

Paediatric cystic kidney disease: MCDK, PKD

A
  1. Multicystic dysplastic kidney (MCDK) (多囊性發育不良腎)
    - Most severe form of renal dysplasia
    - Multiple large cysts replacing normal kidney / Ureteral atresia
    - **More common than PKD
    - **
    Sporadic (+/- VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities))
    - **Unilateral
    - **
    Presentation: Unilateral mass
    - Involute over time —> Follow with serial USG + remove if doesn’t involute
    - Urological testing on healthy kidney
    - **Check urinary tract (90% with other GU anomalies)
    - DMSA show **
    non-functioning kidney
  2. Polycystic kidney disease (多囊腎)
    - Most commonly **inherited kidney disease
    - **
    Genetic, with variable expression
    - **Bilateral
    - **
    Presentation: HT, Renal insufficiency, Oliguria, Family history
    - ***Cysts may also be in liver, spleen, pancreas
    - Results in hypertension
    - “Snowstorm” appearance of infantile polycystic disease
20
Q

***Obstructive uropathies

A
  1. Posterior urethral valves (PUV)
  2. Pelvi-ureteric junction obstruction (PUJO)
  3. Vesicoureteric junction obstruction (VUJO / megaureter)
21
Q

***1. Posterior urethral valves (PUV)

A
  • ***Congenital membrane (between anterior and posterior urethra) which (partially) obstruct urethra
  • can cause problem at bladder, ureter, kidney
  • most common cause of neonatal obstructive uropathy in **males (*NOT affect female)
  • incidence 1/5000-1/8000

Presentation:
- Antenatal
—> Severe cases can present with ***Potter’s sequence (oligohydraminios, pulmonary hypoplasia, uterine molding)
- Bilateral hydronephrosis
- Distended bladder
- Poor urinary stream (+/- dribbling)

Macroscopic appearance:
- Dilated proximal urethra
- Thickened trabeculated bladder
- Dilated ureters
- Hydronephrosis

Diagnosis:
- MCUG (dilated posterior urethra + narrow anterior urethra)

Therapeutic goal:
- Relieve obstruction
- Preserve renal function + Avoid renal failure (30% at risk for progressive renal insufficiency)

Treatment:
1. Immediate: Foley to decompress bladder
2. Surgical
- Ablation of valves
- Urinary diversion
- Fetal therapy with vesicoamniotic shunt (no good evidence)

Monitoring:
- Blood test monitoring
- Post-obstructive diuresis

Prognosis:
- 1/3 can still progress to renal failure despite surgical treatment due to damage done antenatally

22
Q

***2. Pelvi-ureteric junction obstruction (PUJO)

A
  • Most common cause of congenital hydronephrosis
  • Abnormal muscle development at PUJ
  • M>F
  • Can be associated with other congenital abnormalities (e.g. VATER)

Presentation:
- Prenatal: Hydronephrosis (***Unilateral)
- Postnatal: Renal mass (Hydronephrosis) / workup after UTI

Imaging:
- USG (see dilatation of renal pelvis)
- MAG3 / DTPA scan (***obstructive pattern on diuretic enhanced radionucleotide scan)
- CT / MR urogram (Not standard)

Treatment:
1. Pyeloplasty (Anderson-Hynes)
- removing blockage + reconnecting ureter to renal pelvis
- stent (double J) may be left across the pyeloplasty anastomosis / a nephrostomy may be left above the repair to decompress the kidney

23
Q

Collecting system abnormalities

A
  1. Duplication
    - Partial (only 1 ureteric opening into bladder)
    - Complete (2 ureteric openings into bladder)
    —> associated with Ureterocele
  2. Ureterocele
    - Intravesicle
    - Ectopic
    —> terminal cystic dilatation of ureter, associated with duplication anomalies
    —> both can cause obstruction to upper urinary tract
24
Q

Paediatric renal tumours

A

Neonates:
Renal:
1. Mesoblastic nephroma
2. Wilms tumour (Nephroblastoma) (less common)
3. Clear cell sarcoma (less common)
4. Rhabdoid tumour (less common)

Adrenal:
1. Phaeochromocytoma
2. Neuroblastoma

25
Q
  1. Mesoblastic nephroma
A
  • Most common renal neoplasm in 1st year of life
  • 60% diagnosed before 6 months
  • Neonatal tumours: 1:27000 (7% are renal tumours)
  • Account for 3-6% of renal tumours in childhood
  • Most frequent benign renal tumour

Treatment:
1. Radical nephrectomy
- generally curative

  1. Chemotherapy
    - for incomplete resection, infrequent local recurrences and rarely metastases
26
Q
  1. Wilm’s tumour
A
  • Most common paediatric renal tumour, but rarely diagnosed in 1st month of life
    —> 80% diagnosed between 1-5 yo
  • 15% associated with other syndromes
    —> WAGR syndrome
    —> Hemihypertrophy
    —> Beckwith-Wiedemann
  • Most have good prognosis and cured with Primary nephrectomy (some may need Chemotherapy)
27
Q

Disorder of sexual differentiation (DSD)

A

Patient is born but difficult to decide boy / girl

Causes:
1. Congenital adrenal hyperplasia (CAH)
- most common diagnosis in **virilised XX infants (look like male from external appearance)
- most common type: **
21-hydroxylase deficiency
- presentation: **hypoglycaemia, vomiting, diarrhoea, hypovolaemia, **HypoNa with HyperK, **shock
- **
paediatric emergency (SpC Paed)
(vs Androgen insensitivity syndrome (AIS): XY infants who look like female due to androgen insensitivity (self notes))

Investigations:
- Monitor electrolytes, glucose
- 17-OHP high (newborn screen / lab studies)

Treatment:
- Correct electrolytes, glucose
- Glucocorticoids (Hydrocortisone)
- Mineralocorticoid (may be required in salt wasters)

28
Q

Genitourinary malformations

A
  1. Bladder exstrophy
  2. Cloacal exstrophy
  3. Epispadias
  4. Urachal anomalies