Paediatric urology

Congenital Abnormalities not uncommon; 1/200 have congenital abnormalities of the GU tract 1. ANTENATAL HYDRONEPHROSIS 2. POSTERIOR URETHRAL VALVES (PUV) 3.URETEROPELVIC JUNCTION (UPJ) OBSTRUCTION 4. VESICOURETERAL REFLUX (VUR) 5. HYPOSPADIAS 6. EPISPADIAS-EXSTROPHY COMPLEX 7.CRYPTORCHIDISM/ECTOPIC TESTES 8.RENAL AND URETER ABNORMALITIES 9.MEGAURETER

1. ANTENATAL HYDRONEPHROSIS Epidemiology 1-5% fetal usg, detectable as early as first trimester Differential Diagnosis ureteropelvic junction or ureterovesicular junction obstruction multi-cystic dysplastic kidney VUR posterior urethral valve (only in boys) duplication anomalies ureterocele ectopic ureter Treatment antenatal in utero intervention rarely indicated unless evidence of posterior urethral valve with oligohydramnios

Majority of antenatal hydronephroses resolve during pregnancy or within the first year of life.

2. POSTERIOR URETHRAL VALVES (PUV) Epidemiology common congenital obstructive urological defect (affects only boys) Pathophysiology abnormal mucosal folds at the distal prostatic urethra causing varying degrees of obstruction

2. POSTERIOR URETHRAL VALVES (PUV) Clinical Presentation dependent on age: antenatal: bilateral hydronephrosis, distended bladder, oligohydramnios neonatal (recognized at birth): palpable abdominal mass (distended bladder, hydronephrosis), ascites, respiratory distress (pulmonary hypoplasia from oligohydramnios), weak urinary stream neonatal (not recognized at birth): within weeks present with urosepsis, dehydration, electrolyte abnormalities, failure to thrive toddlers: UTIs or voiding dysfunction school-aged boys: voiding dysfunction and urinary incontinence

2. POSTERIOR URETHRAL VALVES (PUV) Investigations most commonly recognized on prenatal USG - bilateral hydronephrosis, thickened bladder, dilated posterior urethra (“keyhole sign”), oligohydramnios in a male fetus voiding cystourethrogram - dilated and elongated posterior urethra, trabeculated bladder, VUR Treatment immediate catheterization to relieve obstruction, followed by cystoscopic resection of PUV when baby is stable if resection of PUV is not possible, cystocostomy is indicated

3. URETEROPELVIC JUNCTION (UPJ) OBSTRUCTION Etiology unclear: adynamic ureteral segment, stenosis, strictures, extrinsic compression, aberrant blood vessels Epidemiology the most common congenital defect of the ureter M:F = 2:1 10-40% bilateral, which may be associated with worse prognosis

3. URETEROPELVIC JUNCTION (UPJ) OBSTRUCTION Clinical Presentation symptoms depend on severity and age at diagnosis (mostly asymptomatic finding on USG) infants: abdominal mass, urinary infection children: pain, vomiting, failure to thrive some cases are diagnosed after puberty and into adulthood in adolescents and adults, the symptoms may be triggered by episodes of increased diuresis,such as following alcohol ingestion Investigations antenatal USG most common, IVU, and renal scyntygraphy ± furosemide Treatment surgical correction (pyeloplasty Hynes-Anderson), consider nephrectomy if <15% differential renal function

URETEROPELVIC JUNCTION

pyeloplasty Hynes-Anderson

4. VESICOURETERAL REFLUX (VUR) Definition retrograde passage of urine from the bladder, through the ureterovesicular junction (UVJ), into the ureter Epidemiology estimated ~1% of newborns, but not well known incidence and clinical relevance higher in children with febrile UTIs and prenatal hydronephrosis risk factors: race (white > black), female gender, age (<2 yr), genetic predisposition

4. VESICOURETERAL REFLUX (VUR) Classification primary reflux: incompetent or inadequate closure of UVJ, lateral ureteral insertion, short submucosal segment secondary reflux: abnormally high intravesical pressure resulting in failure of UVJ closure - often associated with anatomic (PUV) or functional (neurogenic) bladder obstruction

4. VESICOURETERAL REFLUX (VUR) Investigations History focused particularly of voiding dysfunction (frequency, urgency, constipation) screen for signs of infection (UTI, pyelonephritis, urosepsis) and renal failure initial evaluation of renal status, growth parameters, and blood pressure is warranted in any child with VUR due to high incidence of renal scarring height, weight, blood pressure Creatinine Urine Analysis, Urine Culture renal usg cystography, renal scyntygraphy if at high risk

4. VESICOURETERAL REFLUX (VUR)

4. VESICOURETERAL REFLUX (VUR) Treatment spontaneous resolution in 60% of primary reflux in lower grades (I-III), goal is to prevent infection or renal damage via medical treatment and monitoring medical treatment: long-term ABx prophylaxis at half the treatment dose for half the treatment time (TMP/SMX, trimethoprim, amoxicillin, or nitrofurantoin) surgical treatment: ureteral reimplantation ± ureteroplasty, or subureteral injection with bulking agents (DefluxR or MacroplastiqueR) Indications for surgery : failure of medical management, renal scarring (e.g. renal insufficiency), breakthrough UTIs, persistent high grade (IV or V) reflux

Endoscopic treatment of vur

Cohen's uretero-vesical reimplantation

5. HYPOSPADIAS Definition a condition in which the urethral meatus opens on the ventral side of the penis, proximal to the normal location in the glans penis depending on severity, may result in difficulty directing urinary stream or infertility Epidemiology very common; 1/300 live male births white >> black may be associated with ventral penile curvature, disorders of sexual differentiation,undescended testicles or inguinal hernia Treatment early surgical correction; optimal repair before 2 yr

6. EPISPADIAS-EXSTROPHY COMPLEX Definition a spectrum of defects depending on the timing of the rupture of the cloacal membrane bladder exstrophy: congenital absence of a portion of lower abdominal and anterior bladder wall, with exposure of the bladder lumen cloacal exstrophy: exposed bladder and bowel with imperforate anus, associated with spina bifida in >50% epispadias (least severe) urethra opens on dorsal aspect of the penis Etiology represents failure of closure of the cloacal membrane, resulting in the bladder and urethra opening directly through the abdominal wall Epidemiology rare: incidence 1/30,000, 3:1 male to female predominance high morbidity - incontinence, infertility, reflux Treatment surgical correction at birth later corrections for incontinence, VUR, and bladder capacity may be needed

bladder exstrophy

bladder exstrophy

Cryptorchidism/Ectopic Testes Definition abnormal location of testes somewhere along the normal path of descent (external inguinal ring> inguinal canal > abdominal) ectopic testis (testis found outside its normal path of descent) is most commonly located within a superficial pouch between the external oblique fascia and Scarpa’s fascia (Denis Browne pouch) Epidemiology • 2.7% of full term newborns • 0.7%-0.8% at 1 yr old

7.Cryptorchidism/Ectopic Testes Treatment orchiopexy at age of 6 months-12 months hormonal therapy not proven to be of benefit over standard surgical treatment Prognosis reduction in fertility - untreated bilateral cryptorchidism: ~100% infertility paternity rates: 1)53%, 2)90%, and 3)93% in 1)formerly bilateral cryptorchid, 2)formerly unilateral cryptorchid, and 3)normal men increased malignancy risk intraabdominal > inguinal surgical correction facilitates testicular monitoring and may reduce malignancy risk increased risk of testicular torsion (reduced by surgical correction)

8.Renal and ureter abnormalities Agenesis – complete absence of one kidney, ureter and ipsilateral part of bladder triangle Aplasia – no transformation between kidney forming tissues into proper kidney Hypoplasia – improper growth of the kidney Improper location (ex. Pelvical, other side) Renal fusion (most common is horseshoe kidney 1/400 newborns)

8.Renal and ureter abnormalities Duplicated ureter - two ureters draining a single kidney, occurring in approximately 1% of the population. Can be partial (usually asymptomatic) or complete (usually the upper ureter has it orifice is in the lower part of the bladder with the risk of hydronephrosis due to UVJ obstruction, while the lower ureter is in the upper part of the bladder with the risk VUR) Ectopic ureter – ureter terminates in inproper site

8.Renal and ureter abnormalities Ureterocele - the distal ureter balloons at its opening into the bladder, forming a sac-like pouch. It is most often associated with a duplicated collection system, where two ureters drain their respective kidney instead of one. Simple ureteroceles, where the condition involves only a single ureter, represents only twenty percent of cases. Ureteroceles affects one in 4,000 individuals, at least four-fifths of whom are female. Patients are frequently Caucasian. Initial management is usually endoscopic incision of the ureterocele, which can be followed by surgical ureteric re-implantation to preserve renal function and prevent reflux. In some cases: upper pole nephrectomy with ureterectomy (poor renal function), ureteropyelostomy (good renal function)

9.Megaureter ureter is abnormally dilated Usualy congenital, more common in males, may be bilateral, and is often associated with other congenital anomalies. IVU/CT

9.Megaureter Primary – around the distal part of the ureter there is too much smooth muscles, which causes functional obstruction. The ureteric orifice appears normal and a ureteric catheter passes easily. Secondary VUR Neurogenic bladder Ureterocele PUV Ectopic ureter

9.Megaureter

10.Urogynecology 10.1 STRESS INCONTINENCE 10.2 URGE INCONTINENCE

10.1 STRESS INCONTINENCE Involuntary loss of urine with increased intra-abdominal pressure (coughing, laughing, sneezing, walking, running). The gold standard diagnostic test for urinary incontinence is multichannel urodynamics. A large proportion of cases are correctly diagnosed from clinical history alone

10.1 STRESS INCONTINENCE Risk Factors for Stress Incontinence in Women pelvic prolapse pelvic surgery vaginal delivery hypoestrogenic state (post-menopause) age smoking neurological/pulmonary disease

10.1 STRESS INCONTINENCE Treatment Kegel exercises local vaginal estrogen therapy surgical - tension-free vaginal tape (TVT), tension-free obturator tape (TOT)

10.2 URGE INCONTINENCE Definition urine loss associated with an abrupt, sudden urge to void “overactive bladder” diagnosed based on symptoms Etiology idiopathic (90%) detrusor muscle overactivity (“detrusor instability”) Rule Out Neurological Causes of Urge Incontinence Multiple sclerosis Herniated disc Diabetes mellitus

10.2 URGE INCONTINENCE Treatment behaviour modification (reduce caffeine/liquid, smoking cessation, regular voiding schedule) Kegel exercises anticholinergics: oxybutinin, tolterodine, solifenacin New drug – beta-mimetic: mirabegron tricyclic antidepressants: imipramine