Paediatric Urology

Introduction and Methodology

• The European Association of Urology (EAU) Paediatric Urology Guidelines Panel produced this document to increase care quality for children with urological conditions.
• Paediatric urology is often a distinct subspecialty from parent specialties in Europe, defined by its own body of knowledge and management diversity.
• Guidelines should not replace clinical expertise or individual circumstances; they focus on best outcomes based on available evidence but are not mandates or legal standards.
• Methodology is based on structured reviews of databases: PubMed, Ovid, EMBASE, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews.
• Due to a lack of large randomised controlled trials (RCTs) for many congenital surgical interventions, the document is largely a consensus document.
• The strength of each recommendation is determined by the balance between desirable and undesirable consequences, quality of evidence, magnitude of effect, certainty of results, and patient preferences.
• Strong recommendations indicate high evidence quality or favorable benefit/harm balance, while weak recommendations indicate lower-quality evidence or equivocal balance.

Phimosis and Penile Skin Abnormalities

• Definitions and Epidemiology:
  • Prepuce Retraction: Possible in $4\%$ of boys at birth. tip of glans hidden in $42\%$ of neonates. By year one, $50\%$ of boys can retract; by age three, this rises to $89\%$.
  • Phimosis: Inability to retract the foreskin due to a narrow ring in the prepuce. Incidence is $9-20\%$ in ages 5–13 and decreases to $1\%$ by ages 16–18.
  • Physiological Phimosis: Defined by "pouting" of the inner prepuce when retracted; usually resolves without treatment.
  • Pathological (Secondary) Phimosis: Characterized by scarring and a fibrous, white, thickened ring. Often requires treatment.
  • Preputial Adhesions: Physiological bonding of the inner prepuce to the glans. Present in $63\%$ of 6- to 7-year-olds and $3\%$ of 16- to 17-year-olds. Smegma (epithelial debris) may form nodules mistaken for cysts.
  • Paraphimosis: Retracted foreskin cannot be returned over the glans. Incidence is approximately $0.2\%$. It is a medical emergency due to the risk of impaired perfusion and glans necrosis.
  • Balanitis xerotica obliterans (BXO): Chronic inflammatory disease (genital lichen sclerosus et atrophicus) causing scarring and urethral outflow problems. BXO signs appear in $35\%-53\%$ of medical circumcisions (decreases to $17\%$ if aged < 10 years).
• Inconspicuous Penis Types:
  • Buried Penis: Shaft is normal size but appears short due to skin folding or prepubic fat.
  • Megaprepuce: Swelling occurs during voiding as urine fills the space between the shaft and inner prepuce.
  • Webbed Penis: Scrotum attaches high on the ventral side of the penile shaft.
  • Trapped Penis: Iatrogenic buried penis usually following circumcision.
• Management:
  • Hygiene: Forced retraction should be avoided to prevent scarring.
  • Medical Treatment: Topical corticosteroids ($0.05-0.1\%$) twice daily for 4–8 weeks; success rate $>80\%$. Recurrence rate up to $17\%$.
  • Surgical Treatment: Indications include recurrent balanoposthitis or symptomatic phimosis. Options include preputioplasty (preserves prepuce) or circumcision.
  • Paraphimosis Treatment: Manual compression followed by retraction; dorsal incision if manual maneuvers fail.
• Complications: Post-circumcision meatal stenosis occurs in $20\%$ of BXO cases versus $6\%$ for non-BXO phimosis.

Management of Undescended Testes (Cryptorchidism)

• Background:
  • Incidence: Affects $1.0-4.6\%$ of full-term and $1.1-45\%$ of preterm neonates. Spontaneous descent stops after six months of life.
  • Risk at age one: Nearly $1.0\%$
  • Bilateral occurrence: Up to $30\%$
• Classification:
  • Palpable ($80\%$, Inguinal or Ectopic).
  • Nonpalpable ($20\%$, Intra-abdominal, Absent, or Rudimentary).
  • Retractile Testis: Suprascrotal due to cremasteric reflex; can be manipulated into the scrotum; $33\%$ risk of secondary ascent.
  • Ascending Testis: Acquired cryptorchidism (previosuly scrotal testis moves upward).
• Diagnostic Evaluation:
  • Physical Examination: Primary tool. Localisation imaging (Ultrasound, MRI) has limited sensitivity and is not routinely recommended except in suspected DSD cases to find Müllerian structures.
  • Indicators: Unilateral nonpalpable testis with contralateral compensatory hypertrophy often suggests agenesis or atrophy (vanishing testis).
• Treatment Timing:
  • Must start by 6 months of age and be completed by 12 months (18 months at the latest) to prevent loss of germ/Leydig cells.
• Medical Therapy:
  • Hormonal therapy (hCG or GnRH) success is limited ($20\%$). Not recommended by Nordic consensus, but EAU Panel consensus suggests GnRH analogues for bilateral cases as it may improve fertility indices (LE: 4).
• Surgical Therapy:
  • Palpable: Inguinal or scrotal orchidopexy. Scrotal approach is a good alternative for low-lying testes with success rates of $88-100\%$.
  • Nonpalpable: Diagnostic laparoscopy is the optimal approach. 40% are intra-abdominal; 10% are peeping; 10% are blind-ending (vanishing).
  • Staged Procedures: Fowler-Stephens (dividing testicular vessels) or Shehata technique (vessel traction). Systematic reviews show comparable success; FS has shorter initial operative time.
• Outcomes:
  • Malignancy risk: Orchidopexy before age 13 reduces relative risk from $5.4$ to $2.2$.
  • Fertility: Untreated bilateral cases result in $100\%$ oligospermia and $75\%$ azoospermia. Treated bilateral cases improve to $42\%$ azoospermia.

Prepubertal Testicular Tumours

• Epidemiology:
  • Account for $1-2\%$ of paediatric solid tumours. Peak incidence is between ages 0 and 4.
  • Most prepubertal tumours are benign ($60-75\%$).
• Common Entities:
  • Germ cell tumours (GCT): $71-90\%$ of intratesticular tumours. Teratomas comprise $40-50\%$
  • Epidermoid Cysts: Consistently benign; $10-15\%$ of GCTs.
  • Yolk Sac Tumours: Most common malignant GCT in prepubertal boys ($15\%$ of all prepubertal tumours); characteristically produce high Alpha-fetoprotein (AFP).
  • Testicular adrenal rest tumours (TARTs): Occur in boys with Congenital Adrenal Hyperplasia (CAH). Benign but impact fertility potential.
• Evaluation and Management:
  • Ultrasound: Detection rate nearly $100\%$. Microlithiasis incidence is higher in post-orchidopexy patients.
  • Marker: AFP must be taken before and 5 days after surgery. Beta-hCG is rarely useful pre-pubertally.
  • Surgery: Testis-sparing surgery (TSS) with intraoperative frozen sections is the goal. Clamping of vessels may improve view in organ-sparing procedures.

Fertility Preservation in Children

• Indicators: Pediatric cancers, post-treatment survivorship, and potentially gonadotoxic therapies (chemotherapy, radiation).
• Girls:
  • Ovarian tissue retrieval (laparoscopy or laparotomy) for cryopreservation. Retrieval is advised to be combined with other medically indicated surgeries to minimize anesthesia risk.
  • Ovarian reserve reduction after chemotherapy is estimated at $10-30\%$.
• Boys:
  • Gonadal shielding during irradiation is a primary measure.
  • Postpubertal: Sperm cryopreservation via masturbation.
  • Prepubertal: Immature testicular tissue cryopreservation is still experimental.

Hydrocele and Acute Scrotum

• Hydrocele:
  • Types: Communicating (processus vaginalis patent), Hydrocele of the cord (focal patency), Abdominoscrotal (ASH; hourglass extension into abdomen).
  • Management: Observation warranted until 12 months of age ($92\%$ resolve below 1 yr). Surgery involves ligation of the patent processus vaginalis.
• Acute Scrotum Emergencies:
  • Causes: Testicular torsion ($69\%$ present < 12 hrs), appendix testis torsion ($62\%$), or epididymitis ($31\%$).
  • Torsion Critical Window: Irreversible changes and necrosis occur if not treated within 4–6 hours.
  • Clinical markers: Absent cremasteric reflex (100% sensitivity for torsion). Prehn sign (elevation relief) suggests epididymitis, not torsion.
  • Surgical Salvage: Atrophy occurs if torsion turns exceed $360^{\circ}$ and duration is $>4$ hours. If duration exceeds 24 hours, atrophy is found in all cases $>360^{\circ}$.

Hypospadias

• Epidemiology: Prevalence is $18.6$ per 10,000 male births in Europe.
• Risk Factors: Small for gestational age (SGA), maternal hypertension/preeclampsia, and gestational diabetes.
• Classification:
  • Distal (Glandular/Distal shaft).
  • Intermediate (Mid-penile).
  • Proximal (Penoscrotal, Scrotal, Perineal).
• Management:
  • Surgery age: Recommended between 6 and 18 months.
  • Chordee: Correction is warranted if curvature is $\ge 30^{\circ}$.
  • Techniques: Tubularised incised plate (TIP) urethroplasty is common for distal forms. Proximal forms often require staged repairs (Bracka or Koayanagi).
  • Complications: Fistula rates range from $5\%$ to $50\%$. Glans width $< 14\,\text{mm}$ is an independent risk factor for complications.

Varicocele in Adolescents

• Epidemiology: Unusual under age 10; found in $14-20\%$ of adolescents. $78-93\%$ occur on the left side.
• Classification: Grade I (Valvsala only), Grade II (Palpable), Grade III (Visible).
• Treatment Criteria:
  • Persistence of a small testis (size difference $>20\%$ or $>2\,\text{mL}$ over two visits).
  • Symptomatic/Painful.
  • Bilateral palpable varicoceles.
• Surgery: Lymphatic-sparing repairs are preferred to prevent postoperative hydrocele ($40\%$ risk in non-sparing) and testicular hypertrophy.

Urinary Tract Infections (UTI) in Children

• Pathogens: Escherichia coli (E. coli) remains leading cause, but is found in $<50\%$
• Diagnosis:
  • Urine collection (non-toilet-trained): Bladder catheterization or suprapubic aspiration (SPA) are the standard for cultures.
  • Plastic bags: Contamination rate $50-60\%$. Only reliable if negative.
  • Urinalysis: Cut-off for pyuria is >10\,\text{WBC/\mu L} in uncentrifuged urine or 25\,\text{WBC/\mu L} centrifuged.
  • Significant CFU: $\ge 10^5\,\text{cfu/mL}$ typical for febrile UTI, but $10^3-10^4$ significant in monocultures.
• Management: Parental antibiotic therapy recommended for infants $< 2$ months. Short-course ($5$ days) oral antibiotics shown to be non-inferior to 10 days for uncomplicated UTI.
• Prevention: Bacterial resistance is a concern with chemoprophylaxis. Cranberry products have moderate evidence level.

Monosymptomatic Nocturnal Enuresis (NE)

• Definition: Involuntary nocturnal incontinence. $5-10\%$ prevalence at age 7.
• Pathophysiology: High arousal threshold (child does not wake), high nighttime urine production (nocturnal polyuria), and/or nighttime OAB.
• Management:
  • Do not treat children under age 5.
  • Wetting Alarm: Acoustic or tactile; relies on changing arousal thresholds. Higher complete response rate than no treatment.
  • Supportive Measures: Limit electronic devices before bed; regular drinking habits.
  • Medication: Desmopressin (tablet $200-400\,\mu\text{g}$ or sublingual $120-240\,\mu\text{g}$).

Dilatation of the Upper Urinary Tract (PUJ Obstruction)

• PUJ Obstruction: Impaired flow from pelvis to proximal ureter. Incidence $1:1,500$. Ratio male to female is $2:1$.
• Diuretic Renography: MAG3 is the radionuclide choice. Study must be standardized (hydration $15\,\text{mL/kg}$ saline over 30 mins) after 4–6 weeks of age.
• Furosemide dosage: $1\,\text{mg/kg}$ in first year; $0.5\,\text{mg/kg}$ ages 1–16.
• Surgical Indications: Split renal function (SRF) $<40\%$ or a decrease in SRF of $>10\%$.
• Gold Standard: Dismembered pyeloplasty (Hynes and Anderson) with $95\%$ success rate.

Vesicoureteric Reflux (VUR)

• Prevalence: $0.4-1.8\%$ in nonsymptomatic children; $~1\%$ incident overall. $30-50\%$ in children with UTI.
• Grading (International Reflux Study Committee):
  • Grade I: Reflux limited to ureter.
  • Grade II: Reaches renal pelvis; no dilation.
  • Grade III: Mild/moderate dilation of ureter and collecting system.
  • Grade IV: Moderate dilation; blunt fornices.
  • Grade V: Gross dilation; papillary impressions no longer visible.
• Spontaneous Resolution: $80\%$ in VUR grades I–II; $30-50\%$ in grades III–V within 4–5 years.
• Management: Selective antibiotic prophylaxis (CAP) based on risk (RIVUR trial data). Endoscopic bulking agents (e.g., Deflux™) resolution rates: Grades I-II $78.5\%$, Grade IV $63\%$, Grade V $51\%$.

Urinary Stone Disease

• Composition: $>70\%$ contain calcium oxalate.
• Hypercalciuria Definition: 24-hour urine collection excretion of $>4\,\text{mg/kg/day}$.
• Treatment Modalities:
  • Extracorporeal shockwave lithotripsy (SWL): Number of shockwaves $1,800-2,000$ (up to 4,000). Best for stones $< 10\,\text{mm}$ in renal pelvis (clearance $90\%$).
  • PCNL: MINI-PCNL ($13-14\text{F}$ sheath), UMP ($12\text{F}$), and Microperc ($4.85\text{F}$ needle).
  • Medical Expulsive Therapy (MET): Silodosin ($89.3\%$ expulsion) compared to tamsulosin ($74.5\%$.

Disorders of Sex Development (DSD)

• Terminology: Replaces "intersex." Common form: $46\text{XX}$ CAH (80% of DSD cases; neonatal emergency due to salt-loss).
• Phallus Measurement: Stretched length measured from pubic symphysis to tip of glans; Micropenis definition is stretched length $< 2.5$ standard deviations below mean.
• Diagnostic Tests: Androgen-binding studies, hCG stimulation test, whole exome sequencing (WES).
• Malignancy Risk: Highest in Gonadal dysgenesis with Y chromosome ($15-35\%$

Congenital Lower Urinary Tract Obstruction (CLUTO)

• Leading Cause: Posterior urethral valves (PUV) ($60\%$ of cases).
• Foetal Diagnosis: Megacystis longitudinal diameter $>7\,\text{mm}$ in 1st trimester or failing to empty during 40-minute scan in 2nd trimester.
• PUV Types: Type I (ridge on floor of urethra) and Type III (membranous obstruction with small opening).
• Management: Postnatal endoscopic valve ablation. Vesicoamniotic shunt (VAS) carries complication rate of $21-59\%$

Trauma Management

• Renal Trauma Grading:
  • Grade I: Subcapsular haematoma/contusion.
  • Grade II: Perirenal haematoma; laceration $\le 1\,\text{cm}$.
  • Grade III: Laceration $> 1\,\text{cm}$.
  • Grade IV: Laceration extending into collecting system/urinary extravasation.
  • Grade V: Shattered kidney.
• Conservative Care: Standard for stable blunt renal trauma. Grade V or instability warrants surgery or angioembolisation (successful in $92\%$ of Grade III–IV stable patients).
• Bladder Injury: Intraperitoneal (dome) requires repair; Extraperitoneal treated with catheter drainage ($7–10$ days).

Perioperative Management Recommendations

• Fasting Times:
  • Clear liquids: 1 hour
  • Breast milk: 3 hours
  • Formula milk: 4 hours
  • Light meal: 6 hours
• Pain Management: WHO ladder strategy starting with Paracetamol + NSAID, progressing to weak then strong opioids. Regional blocks (DPNB/Caudal) reduce need for postoperative opioids.
• Fluids: ERAS (Enhanced Recovery After Surgery) protocols prioritize early oral intake and mobilization.
• Thromboprophylaxis: Low absolute risk in children; recommend only for post-pubertal adolescents ($>13$ yrs) with malignancy or central venous catheters (CVL).