Paediatric urology Flashcards
How common is congenital urogenital anomalies on prenatal ultrasounds?
up to 2%
What is the most common urogenital anomoly that is discovered on prenatal ultrasounds?
60% is hydronephrosis
How common is UPJ obstruction?
1:1500
males 2:1 females in newborn
Differential diagnosis with hydronephrosis in newborns:
UPJ problems 43% VUR (vesicourethral reflux) 24% Megaureter 12% PUV (posterior urethral valves) 11% Duplex systems 10%
What carachterises Megaurethers?
More often males
More on the left side
Can be caused by obstruction and/or reflux
Is primary or secondary (PUV, neurogenic bladder)
If a hydronephrosis is not an obstruction, what else can it be a sign of?
VUR (vesicourethral reflux)
What information is needed from the antenatal ultrasound if a hydronephrosis is discovered?
Laterality, grade of dilatiation, echogenicitiy of the kidney With or without a dilated urether Bladder volume and emptying Sex of child Amniotic fluid volume
What is a VCUG?
Voiding CystoUrethroGram
Gold standard for imaging the bladder and urethra
Can detect: Vesicoureteral reflux urethral valves ureteroceles diverticula neurogenic bladder
What is a MAG3?
Type of Dynamic Renography
shows:
blood flow to the kidney
renal clearance
drainage function
When should UPJ obstruction be operated?
Decreased renal function <40% on affected side Increasing hydronephrosis Manifesting symptoms Poor drainage grade III and IV dilatation
What are the goals in diagnosing and treating UPJ obstruction?
Perserve renal function
Less: UTI Pain Hematuria Urolithiasis
What share of megaurethers will resolve spontaneously?
85%
When should a megaurether be surgically corrected?
Progression in dilatation
Deterioration of renal function
Symptomatic
What is more common, a complete duplex system or a partial?
Partial
What other conditions are a duplex collecting system associated with?
Ectopic urether
Ureterocele
What are the most common sites for an ectopic urether?
Males: posterior urethra
Females: bladder neck, urethra, vagina
more common in girls
What is a ureterocele?
Malformation characteried by pseudo-cystic dilatation of the distal, intravesical portion of urethra
are often a source of obstruction
How common are ureteroceles?
1:4000
How common are ureteroceles in duplex systems?
80% are found in upper pole urether
20% in single systems
How common are bilateral ureteroceles?
10%
What is the ratio boys:girls in the prevalence of ureteroceles?
1:4-7
What is a Posterior Urethral Valve?
Obstructing membranous fold within the lumen of the posterior urethra
What is the most common urinary obstruction that affects newborn boys?
Posterior Urethral Valve (PUV)
1: 5000-8000 live births
is lifethreatening
35% renal insufficiency despite optimal treatment
How do you treat PUV and what is the result?
Shunting
Fetal valve ablation
bladder decompression restoration of amniotic fluid lungs can expand restoration of surfactant no benefit for renal function
At what age is the foreskin most often retractable?
90% at 3 years old
How many boys still have physiological fimosis at 17?
1%
How do you treat secondary fimosis?
Stereoid creme (class III-stereoids, twice daily 39 days)
Dorsal slit
Circumcision
What are the medical indications for circumcision?
Congenital urinary tract malformation to reduce risk for UTI
Secondary phimosis with recurrent UTI
BXO (balanitis xerotica obliterans)
What is the NNT to prevent UTI for circumcisions?
111
therefore not justified as a routine
What are the contraindications for circumcision?
acute infection
congenital anomalies
bleeding disorders
What is the complication rate for circumcision?
0,2-5%
What is the incidence of hypospadias?
1:300 newborn boys
In hypospadias, how many are distal and how many are proximal?
Distal 80%
proximal 20%
What factors are associated with hypospadias?
Mainly unknown
low birth weight young and older mothers vegeterian diet during pregnancy IVF some genetic disorders disorders of sexual differentation
What are the indication for surgical correction of hypospadias?
Straighten the penis
Enable standing voiding
Enable normal sexual function
Meatal stenosis correction
cosmetic- foreskin reconstruction
What is the most prevalent procedure for correcting hypospadias?
Tubularised incised plate
if proximal, two stage procedure
with buccal or skin behind the ear
(important, skin without hair)
What are longtime complications after hypospadia surgery?
urethrocutaneous fistula 7-25%
Stricture/stenosis 5-15%
What is Epispadias?
Defect in the dorsal wall of the urethra
together with incontinence and small penis size
What is the incidence of epispadias?
1: 120 000 male births
How do you define a micropenis?
stretched penis length < 2,5 SD of age specific length
What other conditions are micropenises associated with?
Hypogonadism PAIS Chromosomal abberations -Klinefelter's syndrome -Prader- Willis syndrome Idiopathic
What other investigations should be conducted on a patient with a micropenis?
Karyotype
Hormonal analysis
hCG stimulations test
Paediatric endocrinologist
When is surgical intervention varranted for a hydrocele?
If persistent >12 months
Differential diagnoses for acute onset scrotal masses:
Torsion Trauma Abscess Orchitis Epididymnitis
Differential diagnoses for non-acute onset scrotal masses:
Hydrocele Hernia Spermatocele Varicocele Neoplasia
What is the most common age for testicular torsion?
12-20 years
What is the difference between regular testicular torsion and Neonatal testicular torsion?
Neonatal testicular torsion can be extravaginal
What are the most common viral causes for epididymo-orchitis in children?
Adenovirus
Mumps
What is Acute Idiopathic scrotal edema?
Inflammation, tenderness and swelling of scrotal skin
can extend to the perineum & abdomen
age 2-10
non-tender testis
spntanous resolution <72 hours
UL -thickening of scrotal skin
Colud be an allergic reaction
How common is cryptorchidism?
3% at birth
1% at 1 year
How often is cryptochidism bilateral?
30%
How often is the testicle palpable in cryptochidism?
80%
How often is the testicle absent in cryptochidism?
of those that are not palpable 20% are without testicle
When should cryptiochidism be corrected?
Before 1 year of age
____ is not necessary for renal scarring to occur
renal scarring
VCUG after UTI
- After 1st UTI with abnormal ____
- After ___ UTI without other abnormalities
renal u/s
2nd
Prescribe prophylactic antibiotics to patients with VUR & ____ or ____
febrile UTIS or bowel-bladder dysfunction
All newborn males with bilateral undescended testes must undergo workup for ___
DSD
Providers should not perform ____ in patients with cryptorchidism prior to urologic referral
ultrasound/imaging
Pulling down a possibly retractile testes into the scrotum for 30 second fatigues the _____ allowing for better assessment
cremasteric muscle
History of UDT is an independent predictor of ____
infertility
unilateral UDT - 10.5% infertility
bilateral UDT - 35% infertility
Prepubertal orchiopexy reduces risk of ____
testicular cancer
Bladder capacity at full term - ___ cc
50cc
Normal APD diameters on renal u/s
16-28 week: < ____ mm
28 weeks: < ___ mm
2 days+ postnatal:
4, 7, 10
DMSA binds to ____ cells
renal tubule
____ is the most common cause of neonatal hydronephrosis
UPJ obstruction
Epididymitis in prepubertal boy is concerning for ____
duplication system with ectopic ureter
What is the incidence of renal abnormalities detected on prenatal testing?
0.28% -1.4%
What is the definition of prenatal hydronephrosis?
Anteroposterior diameter greater than 5mm.
Large non communicating renal cysts on prenatal US?
Multicystic dysplastic kidney
Appearance of ARPKD on prenatal US?
Highly echogenic kidney
What does the keyhole sign mean on perinatal US?
Dilated posterior urethra. Posterior urethral valves.
Fetus is found to have antenatal hydronephrosis. What is the management?
Repeat RUS >48 hours after birth.
How should severe ANH be managed?
infants with severe ANH should be placed on a prophylactic antibiotic (amoxicillin, 10 to 25 mg/kg/day) and undergo VCUG.
What is the management of bilateral ANH?
RUS and VCUG promptly after birth
What is the most common cause of BL ANH in males?
Posterior urethral valves.
What is the most common cause of BL ANH in Females?
Obstructing ureterocele.
What is a type I posterior urethral valve?
A. A fold of tissue running between the bladder neck and verumontanum
B. A membrane of tissue from the verumontanum to the membarnous urethra
C. A diaphragm of tissue with a central lumen at the level of the membranous urethra
D. A membrane of tissue from the proximal verumontanum dorsally to the bladder neck
B. A membrane of tissue from the verumontanum to the membarnous urethra
What is most commonly affected by a ureteral ectopia in a duplicated system?
A. The ureters of both poles of the kidneys
B. The ureter of the upper pole of the kidney
C. The ureter of the lower pole of the kidney
D. There is no rule in this type of malformation
B. The ureter of the upper pole of the kidney
When does urine production begin in the human fetus?
A. At 2 to 3 weeks
B. At 4 to 6 weeks
C. At 10 to 12 weeks
D. At 14 to 16 weeks
C. At 10 to 12 weeks
What is the approximate incidence of hypospadias?
A. 1 to 125
B. 1 to 250
C. 1 to 500
D. 1 to 1000
B. 1 to 250
The SIOP guidelines for the treatment of a Wilms’ tumour are chronologically as follows:
A. First radiation treatment, then surgery and then chemotherapy
B. First surgery, then chemotherapy and then radiation treatment
C. First chemotherapy, then surgery and then radiation treatment
D. First chemotherapy, then surgery and then chemotherapy again
D. First chemotherapy, then surgery and then chemotherapy again
A 14-year-old wheelchair-bound girl with a myelo-meningocele has urinary and faecl incontinence. She is on clean intermittent catheterisation and anticholinergics. Bladder capacity is 150 mL, leak point pressure is 60 cm H2O, no reflux. What is the most appropriate management?
A. Bladder augmentation and cathererisable stoma and eppendicostomy (ACE)
B. Bladder augmentation
C. Bladder augmentation and bladder neck reconstruction and catheterisable stoma
D. Appendicostomy (ACE)
A. Bladder augmentation and cathererisable stoma and eppendicostomy (ACE)
Which is the most likely diagnosis in a dehydrated neonate with cyanotic heart disease, a hight red blood count, hematuria and a palpable renal mass?
A. Wilms’ tumour
B. Polycystic kidney
C. Renal vein thrombisis
D. Congenital hydronephrosis
C. Renal vein thrombisis
A newborn infant presents with episodes of vomiting, dehydration, cyanosis. He shows severe electrolyte and water imbalances. A small penis with chordee, proximal hypospadias and bilateral impalpable testicles are noted. The most probable diagnosis is:
A. Adrenogenital syndrome
B. Hypospadias with renal dysplasia
C. Hypospadias and polycystic kidneys
D. Obstructive uropathy associated with hypospadias
A. Adrenogenital syndrome
Distal hypospadias in a boy is usually:
A. Associated with renal abnormalities
B. Treated at the age of 6-18months
C. Treated by multiple stage operations
D. Diagnosed before birth by ultrasonography
B. Treated at the age of 6-18months
By which triad of findings is the prune-belly syndrome in boys characterized?
A. Reflux, hypspadias, undescended testicles
B. Vesical extrophy, undescended testicles, reflux
C. Vesical extrophy, reflux, hypoplasia of the abdominal wall
D. Reflux, undescended testicles, hypoplasia of the abdominal wall
D. Reflux, undescended testicles, hypoplasia of the abdominal wall
When should the urinary tract be screened in a boy with hypospadias?
A. In every case
B. In case of an esophageal atresia
C. With unilateral undescended testis
D. In case of midshaft hypospadias
B. In case of an esophageal atresia
What is needed for the development of the Wolffian duct deriviatives in the 46XY fetus?
A. Testosterone
B. 5-α-reductase
C. Dihydrotestosterone
D. Müllerian inhibiting factor
A. Testosterone
Which condition has been reported to occur in association with posterior urethral valves?
A. Exomphalos
B. Hypospadiass
C. Cryptorchidism
D. Orthotopic ureterocele
C. Cryptorchidism
In a young boy with acute scrotum which is more suggestive for testicular torsion?
A. Presence of hydrocele
B. Elevation of the scrotum may reduce complaints
C. Presence of fever
D. Loss of cremaster reflex
D. Loss of cremaster reflex
Which is the most successful method of varicocele treatment in adolescents?
A. Antegrade sclerosation
B. Microsurgical inguinal ligation
C. Laparoscopic ligation
D. Lymphatic sparing suprainguinal ligation
B. Microsurgical inguinal ligation
In an 8-month-old severely ill girl suffering from a proven first febrile urinary tract infection, what is the first advisable diagnostic modality?
A. Renal and bladder ultrasound
B. Voiding cystourethrography (VCUG)
C. Mercaptoacetytriglycin (MAG3) scan
D. Dimercaptosuccinic acid (DMSA) scan
A. Renal and bladder ultrasound
When should intermittent catheterization and antimuscarinic treatment be started in children with neurogenic bladder and bowel emptying problems?
A. Soon after birth
B. In infancy
C. Before puberty
D. If upper tract deterioration is present
A. Soon after birth
According to the EAU guidelines congenital penile curvature is:
A. Commonly associated with urethra malformation
B. Most commonly dorsally deviated
C. Always ventrally deviated
D. Sometimes laterally deviated
D. Sometimes laterally deviated
What is the incidence of posterior urethral valves?
A. 1 in 3 000
B. 1 in 7 000
C. 1 in 10 000
D. 1 in 50 000
B. 1 in 7 000
Which is an indication to recommend adolescent varicocelectomy?
A. Grade 2 right-sided varicocele
B. Varicocele associated with hydrocele
C. Ipsilateral testicular hypoplasia
D. Grade 3 varicocele with normal sperm parameters
C. Ipsilateral testicular hypoplasia
Which of the following DSD conditions has the highest risk for the development of a malignant germ cell tumour?
A. 45X0 Turners syndrome
B. 47XXY Klinefelters syndrome
C. 46XY complete gonadal dysgenesis
D. 46XX congenital adrenal hyperplasia
C. 46XY complete gonadal dysgenesis
Vesico-amniotic shunting during pregnancy:
A. Has no effect on lung function
B. Is associated with significant morbidity
C. Is offered routinely in cases of posterior urethral valves
D. Has improved long-term outcomes in boys with posterior urethral valves
B. Is associated with significant morbidity
During pregnancy an US at 20 weeks gestation shows normal amniotic fluid and a significant hydronephrosis on the left side. At 32 seeks the US shows the same hydronephrosis:
A. The parents have to be counseled and offered drainage of the left kidney
B. If the pulmonary situation allows, early delivery should be promoted around week 36-38
C. No specific measures are necessary and the baby should be delivered at term
D. After delivery an US study of the kidneys should be done on day 1 or 2
C. No specific measures are necessary and the baby should be delivered at term
The age and the appearance of a child with a Wilms’ tumour is most frequently as follows:
A. A 3-year old who looks very ill
B. A 7-year old who looks very ill
C. A 3-year old who does not look ill
D. A 7-year old who does not look ill
C. A 3-year old who does not look ill
What is the most common cause of bladder outflow obstrucion in males during the neonaltal and infancy period?
A. Vesico-ureteral reflux
B. Posterior urethral valves
C. Urethral congenital stricture
D. Hypospadis
B. Posterior urethral valves
The urachus involutes to become:
A. The appendix testes
B. The veru montanum
C. The lateral umbilical folds
D. The median umbilical ligament
D. The median umbilical ligament
At what age should surgery be carried out for boys with undescended testes?
A. At birth
B. Around 6 months of age
C. Around 12 months of age
D At 2 years of age
C. Around 12 months of age
Complete double urethers are seen when:
A. Two metanephric blastema develop
B. Two ureteric buds arise from the mesonephric duct
C. A single ureteric bud divides into two parts
D. Two mesonephric ducts arise from urogenital sinus
B. Two ureteric buds arise from the mesonephric duct
Which of the following possible abnormalities is most commonly found in a complete duplex system?
A. VUR in both ureters
B. Obstruction in both ureters
C. VUR in the lower pole moiety
D. Veisco-ureteral reflux (VUR) in the upper pole moiety
C. VUR in the lower pole moiety
Which radiopharmaceutical is the agent of choice for demonstrating renal scarring in childhood?
A. 99m Tc-DPTA
B. 99m Tc-DMSA
C. 99m Tc-MAG3
D. 99m Tc Gluconate
B. 99m Tc-DMSA
A 10-month-old otherwise normal boy underwent a pyeloplasty. In the immediate postoperative phase the amount of fluid for intravenous maintenance is:
A. 25 ml/kg/24hr
B. 50 ml/kg/24hr
C. 100 ml/kg/24hr
D. 200 ml/kg/24hr
C. 100 ml/kg/24hr
After having one child with hypospadias, what is the relative risk for a couple of giving birth to another child with hypospadias?
A. Approxiamately 1%
B. Approxiamately 17%
C. Approxiamately 42%
D. Approxiamately 62%
B. Approxiamately 17%
Which gene does NOT play a role in the development of hypospadias?
A. Wilm’s tumor gene 1 (WT1)
B. Sonic hedgehehog gene (SHH)
C. Homeox gene a13 (HOXa13)
D. Fibroblast growth factor 8 (FGF8)
A. Wilm’s tumor gene 1 (WT1)
In a 13-year-old girl with urinary incontinence since birth the diaper test shows a continuous leakage of urine with low volume. What is the most likely diagnosis?
A. Lazy bladder
B. Ectopic ureter
C. Vaginal pooling
D. Urge syndrome
B. Ectopic ureter
The most accurate diagnosis in acute scrotum in children is provided by:
A. CT-scan
B. Doppler-ultrasound
C. operative exploration
D. Testicular nuclear scan
C. operative exploration
What is the main cause of nocturnal enuresis in children?
A. An imbalance of urine production and bladder capacity
B. An insufficient nocturnal ADH secretion
C. A problem of high arousal combined with either a high night-time urine production or a night-time OAB (or both)
D. A delay in maturity of the pontine micturition center in combination with an insufficient nocturnal ADH secretion
C. A problem of high arousal combined with either a high night-time urine production or a night-time OAB (or both)
Which patients should be categorised as “high”-risk for vesicoureteral reflux (VUR) and be advised open surgery (ureteral reimplantation) rather than endoscopic surgery or antibiotic prophylaxis?
A. Toilet-trained boys and girls with low-grade VUR and lower urinary tract and bowel symptoms
B. Small, not toilet-trained boys and girls with bilateral high-grade (4-5) VUR and unilateral renal damage
C. Small, not toilet-trained boys and girls with bilateral high-grade (4-5) VUR and bilateral renal damage
D. Toilet-trained boys and girls with high-grade reflux, recurrent UTI, unilateral renal damage and unilateral high-grade (4-5) VUR
D. Toilet-trained boys and girls with high-grade reflux, recurrent UTI, unilateral renal damage and unilateral high-grade (4-5) VUR
A patient experienced an anaphylactic reaction to chlorhexidine. What may be unsafe to use during a future cystoscopy?
A. Antibiotics
B. Sterile water
C. Local /topical anaesthetic
D. Water soluble iodine based skin preparation
C. Local /topical anaesthetic