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EAU Pocket On Paediatric Urology 2024

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EAU GUIDELINES ON

PAEDIATRIC UROLOGY

(Limited text update April 2024)

C. Radmayr (Chair), G. Bogaert (Vice-chair), A. Bujons, B. Burgu,


M.S. Castagnetti, F. O’Kelly, N.A Pakkasjärvi, J. Quaedackers,
Y.F.H. Rawashdeh, M.S. Silay, L.A ‘t Hoen
Guidelines Associates: U.K. Kennedy, M. Gnech, M. Skott,
A. van Uitert, A. Zachou
Guidelines Office: C. Bezuidenhout

Introduction
Due to the scope of the extended Guidelines on Paediatric
Urology, only a short introduction of the individual chapter
in combination with recommendations can be given in this
pocket version. Additionally, some algorithms and flow charts
are enclosed. For further details please refer to the full length
version.

PHIMOSIS
Phimosis is either primary (physiological), with no sign of
scarring, or secondary (pathological), resulting from scarring
due to conditions such as balanitis xerotica obliterans.

Childhood circumcision should not be recommended without


a medical reason. An absolute indication for circumcision
is secondary phimosis. Contraindications are congenital
anomalies of the penis, particularly hypospadias or buried
penis, as the foreskin may be required for a reconstructive
procedure.

Paediatric Urology 435


Paraphimosis is characterised by retracted foreskin with the
constrictive ring localised at the level of the sulcus.

Recommendations Strength rating


Offer topical corticosteroids (ointment Strong
or cream) as first-line treatment in
symptomatic phimosis.
Consider surgical intervention if patient Strong
/ caregivers prefer for symptomatic
phimosis.
Offer circumcision in case of balanitis Strong
xerotica obliterans (BXO) or phimosis
refractory to treatment.
Offer treatment for asymptomatic phimosis Strong
in infants with a risk of recurrent urinary
tract infection due to upper urinary tract
abnormalities (vesico-ureteral reflux or
posterior urethral valves).
Inform patients about the risk of meatal Strong
stenosis in BXO.
Await spontaneous resolution of Weak
asymptomatic preputial adhesions before
puberty.
Treat paraphimosis by manual reposition Strong
and proceed to surgery if this fails.
Do not perform simple circumcision if Strong
phimosis is associated with other penile
anomalies such as buried penis, congenital
penile curvature, epispadias or hypospadias.

436 Paediatric Urology


UNDESCENDED TESTIS
Cryptorchidism or undescended testis is one of the most
common congenital malformations of male neonates with
an incidence of 1.0-4.6% of full-term neonates. Boys with one
undescended testis have a lower fertility rate whereas boys
with bilateral undescended testes suffer both, lower fertility
and paternity rates. In addition, boys who are treated for an
undescended testis have an increased risk of developing
testicular malignancy. Therefore, screening and self-
examination both during and after puberty is recommended.

Figure 1: Classification of undescended testes

Undescended tess

Palpable Non-palpable

Intra-
Inguinal Inguinal Ectopic Absent
abdominal

Ectopic
Agenesis

Retracle
Vanishing
tess

Diagnosis Treatment Follow-up

Paediatric Urology 437


Figure 2: Treatment of unilateral non-palpable undescended
testes
Unilateral non-palpable
testis

Re-exam under
anaesthesia

Still
Palpable
non-palpable

Inguinal
Diagnostic exploration Standard
laparoscopy with possible orchidopexy
laparoscopy

Blind ending Spermatic


Testis close to Testis too high
spermatic vessels enter
internal ring for orchidopexy
vessels inguinal ring

Laparoscopic or Staged Fowler- Vanishing testis


Inguinal
inguinal Stephens no further
exploration
orchidopexy procedure steps

Diagnosis Treatment Follow-up

438 Paediatric Urology


Recommendations Strength rating
Do not offer medical or surgical treatment Strong
for retractile testes instead undertake close
follow-up on a yearly basis until puberty.
Perform surgical orchidolysis and Strong
orchidopexy before the age of twelve
months, and by eighteen months at the
latest.
Evaluate male neonates with bilateral non- Strong
palpable testes for possible disorders of
sex development.
Perform a diagnostic laparoscopy to locate Strong
an intra-abdominal testicle.
Hormonal therapy in unilateral undescended Strong
testes is of no benefit for future paternity.
Offer endocrine treatment in case of Weak
bilateral undescended testes.
Inform the patient/caregivers about the Weak
increased risk of a later malignancy with
an undescended testis in a post-pubertal
boy or older and discuss removal in case
of a contralateral normal testis in a scrotal
position.

TESTICULAR TUMOURS IN PREPUBERTAL BOYS


Testicular tumours account for approximately 1-2% of
all paediatric solid tumours. In prepubertal boys most
intratesticular tumours are benign and teratomas and yolk
sac tumours more common than germ cell tumours, whereas
post-puberty the tumours are most likely malignant.

Paediatric Urology 439


Recommendations Strength rating
High-resolution ultrasound (7.5 – 12.5 MHz), Strong
preferably a doppler ultrasound, should be
performed to confirm the diagnosis.
Alpha-fetoprotein should be determined in Strong
prepubertal boys with a testicular tumour
before surgery.
Surgical exploration should be done with Strong
the option for frozen section, but not as an
emergency operation.
Organ-preserving surgery should be Strong
performed in all benign tumours.
Staging (magnetic resonance imaging Strong
abdomen/computed tomography chest)
should only be performed in patients with a
malignant tumour to exclude metastases.
Magnetic resonance imaging should only Weak
be performed in patients with the potential
malignant Leydig or Sertoli-cell-tumours to
rule out lymph node enlargement.
Patients with a non-organ confined tumour Weak
should be referred to paediatric oncologists
post-operatively.

FERTILITY PRESERVATION IN CHILDREN AND


ADOLESCENTS
The continuous increase in the incidence of paediatric
cancers and post-treatment survivorship over the years
coupled with the further development of potentially
gonadotoxic therapies, has contributed to the recognition
and rapid endorsement of fertility preservation counselling
for prepubertal children and adolescents. Patients and
caregivers should be informed not only about the impact
of gonadotoxic treatments on future fertility but also about

440 Paediatric Urology


fertility-preservation options and their risk-benefit ratio. There
are also a number of non-oncological congenital anomalies
where fertility preservation can become an issue.

Figure 3: Ovarian tissue cryopreservation for girls and


adolescents:

Assessment of ferlity risk

Indicaon for ferlity preservaon

Pre-pubertal Post pubertal

Ovarian cortex Ovarian


biopsy s mula on

Ovarian ssue Oocyte


cryopreserva on cryopreserva on

Diagnosis Treatment Follow-up

Adapted from Anderson et al. 2015.

Paediatric Urology 441


Recommendations Strength rating
Inform patients and caregivers about Strong
the impact of gonadotoxic treatments
on future fertility and about fertility
preservation options and their risk-benefit
balance.
Discuss the indications and options Strong
for fertility preservation in a paediatric
multidisciplinary fertility preservation team
and consider the toxicity of the planned
therapy, the age and pubertal status as well
ethical and financial issues.

HYDROCELE
A communicating hydrocele vacillates in size, usually
relative to activity. It is diagnosed by medical history and
physical investigation, the swelling is translucent, and
transillumination of the scrotum confirms the diagnosis.
Non-communicating hydroceles are found secondary to
minor trauma, testicular torsion, epididymitis, or varicocele
operation, or may appear as a recurrence after primary repair
of a communicating hydrocele.

Recommendations Strength rating


Observe hydrocele for twelve months prior Strong
to considering surgical treatment.
Perform early surgery if there is suspicion Strong
of a concomitant inguinal hernia or
underlying testicular pathology.
Perform ultrasound in case of doubt about Strong
the character of an intrascrotal mass, or
suspicion of an abdominoscrotal hydrocele.

442 Paediatric Urology


Close the processus vaginalis at the Strong
inguinal ring.
Do not use sclerosing agents in children Strong
with hydroceles, because of the risk for
chemical peritonitis.

ACUTE SCROTUM
Acute scrotum is a paediatric urological emergency, most
commonly caused by torsion of the testis or appendix testis,
or epididymitis/epididymo-orchitis.

HYPOSPADIAS
Hypospadias are usually classified according to the
anatomical location of the proximally displaced urethral
orifice.

Patients with hypospadias should be diagnosed at birth.


The diagnostic evaluation also includes an assessment
of associated anomalies, which include cryptorchidism
and open processus vaginalis or inguinal hernia. Severe
hypospadias with unilaterally or bilaterally impalpable testis,
or with ambiguous genitalia, require a complete genetic
and endocrine work-up immediately after birth to exclude
disorders of sex development, especially congenital adrenal
hyperplasia.

Paediatric Urology 443


Figure 4: Algorithm for the management of hypospadias

Diagnosis at birth Exclude DSD

No
Paediatric urologist
reconstruction

Reconstruction
required

Distal Proximal

Chordee No chordee

Urethral Urethral
plate cut plate preserved

Thiersch Duplay, TIP, Two-stage,


Mathieu, MAGPI, tube-onlay Onlay, TIP
advancement Koyanagi repair

Diagnosis Treatment Follow-up

DSD = disorders of sex development; TIP = tubularised incised


plate urethroplasty; MAGPI = meatal advancement and
glanuloplasty incorporated.

444 Paediatric Urology


Recommendations Strength rating
At birth, differentiate isolated hypospadias Strong
from disorders of sex development which
are mostly associated with cryptorchidism
or micropenis.
Counsel caregivers on functional indications Strong
for surgery, aesthetically feasible operative
procedures (psychological, cosmetic
indications) and possible complications.
In children diagnosed with proximal Weak
hypospadias and a small appearing penis,
reduced glans circumference or reduced
urethral plate, pre-operative hormonal
androgen stimulation treatment is an
option but the body of evidence to
accentuate its harms and benefits is
inadequate.
For distal hypospadias, offer Duplay- Weak
Thiersch urethroplasty, original and modified
tubularised incised plate urethroplasty;
use the onlay urethroplasty or two-stage
procedures in more severe hypospadias. A
treatment algorithm is presented (Figure 4).
Correct significant (> 30°) curvature of the
penis.
Ensure long-term follow-up to detect Strong
urethral stricture, voiding dysfunctions
and recurrent penile curvature, ejaculation
disorder, and to evaluate patient’s
satisfaction.
Use validated objective scoring systems Strong
to assist in evaluating the functional and
cosmetic outcome.

Paediatric Urology 445


CONGENITAL PENILE CURVATURE
Congenital penile curvature presents penile bending of a
normally formed penis due to corporal disproportion. Most of
the cases are ventral deviations. Curvature > 30° is considered
clinically significant; curvature > 60° may interfere with
satisfactory sexual intercourse in adulthood. The treatment is
surgical.

Recommendations Strength rating


Ensure that a thorough medical history is Strong
taken and a full clinical examination done
to rule out associated anomalies in boys
presenting with congenital curvature.
Provide photo documentation of the erect Strong
penis from different angles as a
prerequisite in the pre-operative evaluation.
Perform surgery after weighing aesthetic Weak
as well as functional implications of the
curvature.
At the beginning as well as at the end of Strong
surgery, perform artificial erection tests.

VARICOCELE IN CHILDREN AND ADOLESCENTS


Varicocele is unusual in boys under ten years of age, but
becomes more frequent at the beginning of puberty. Fertility
problems will arise in about 20% of adolescents with
varicocele. Testicular catch-up growth and improvement in
sperm parameters after varicocelectomy have been reported
in adolescents. Varicocele is mostly asymptomatic, rarely
causing pain at this age. Diagnosis and classification depend
upon the clinical finding and US investigation.

446 Paediatric Urology


Figure 5: Algorithm for the diagnosis of varicocele in chil-
dren and adolescents

Varicocele in children
and adolescents

Physical examination Ultrasound


in the investigation
upright position

Venous
reflux Size
Grade I - Valsalva positive detected of the
Grade II - Palpable on Doppler testes
Grade III - Visible ultrasound

Diagnosis Treatment Follow-up

Paediatric Urology 447


Figure 6: Algorithm for the management of varicocele in
children and adolescents

Varicocele in children
and adolescents

Surgery Conservative treatment

Indication Type Indication Type


• Small testis Microsurgical • Symmetrical • Measurement
(growth lymphatic-sparing testes of testicular
arrest) repair • Normal size (during
• Additional (microscopic spermiogram adolescence)
testicular or laparoscopic) (in older • Repeated
pathology adolescents) sperm analysis
• Bilateral (after
palpable adolescence)
varicocele
• Pathological
spermiogram
• Symptomatic
varicocele

Diagnosis Treatment Follow-up

Recommendations Strength rating


Examine varicocele in the standing position Strong
and classify into three grades.
Use scrotal ultrasound to evaluate testicular Strong
volume and to detect venous reflux in the
supine and upright position and during
Valsalva manoeuvre.

448 Paediatric Urology


In all pre-pubertal boys with a varicocele Strong
and in all isolated right varicoceles perform
standard abdominal ultrasound to rule out
a retroperitonal mass.
Inform caregivers and patients and offer Strong
surgery for varicocele associated with a
persistent small testis (size difference of
> 2 mL or 20%).
Varicocele treatment can be also Weak
considered under the following
circumstances:
• symptomatic varicocele;
• additional testicular condition affecting
fertility such as a contralateral testicular
condition;
• bilateral palpable varicocele;
• pathological sperm quality (in older
adolescents);
• cosmetic reasons related to their scrotal
swelling.
Use some form of optical magnification Strong
(microscopic or laparoscopic
magnification) for surgical ligation.
Use lymphatic-sparing varicocelectomy to Strong
prevent hydrocele formation.

Paediatric Urology 449


URINARY TRACT INFECTIONS IN CHILDREN

Figure 7: Algorithm for the management of a first febrile UTI

Febrile child with clinical symptoms

Physical examinaon

Blood sample (Blood cell count + CRP +/- Procalcitonin

Proper urine sampling & Urinalysis


(Dipsck +/- Microscopy +/- Flow-imaging analysis)

Nitrate -ve and leukocyte -ve / Nitrate +ve and/or leukocyte


Microscopy -ve / Flow +ve / Microscopy +ve / Flow
imaging -ve imaging analysis +ve

Exclude other causes Urine culture

Start AB treatment following


anbacterial stewardship

Figure 8

Diagnosis Treatment Follow-up

CRP = C-reactive protein; AB = antibiotic

450 Paediatric Urology


Figure 8: Diagnosis strategy for first febrile UTI

First febrile UTI

Renal and bladder


ultrasound

Abnormal findings (e.g. dilata on


Normal findings of upper tract/abscess
forma on/obstruc ng stone)

E. Coli Non-E. Coli Complicated UTI


infec on infec on close monitoring + i.v. an bio cs

Children Good Cri cal clinical


>12 Infants clinical status/no
months response response

Imaging Further Urinary


Exclusion
aer evalua on diversion
of VUR (by
recurrent of upper (nephrostomy/
imaging)
febrile UTI tract JJ/catheter)

Toilet trained children


Exclude BBD
Diagnosis Treatment Follow-up

BBD = bladder and bowel dysfunction; VUR = vesicoureteral


reflux; i.v. = intravenous; UTI = urinary tract infection

Recommendations Strength rating


Take a medical history, assess clinical Strong
signs and symptoms and perform a
physical examination to diagnose children
suspected of having a urinary tract
infection (UTI).

Paediatric Urology 451


Exclude bladder- and bowel dysfunction in Strong
any toilet-trained child with febrile and/or
recurrent UTI.
Clean catch urine can be used for Strong
screening for UTI. Bladder catheterisation
and suprapubic bladder aspiration to
collect urine can be used for urine cultures.
Do not use plastic bags for urine sampling Strong
in non-toilet-trained children since it has a
high risk of false-positive results.
Midstream urine is an acceptable Strong
technique for toilet-trained children.
The choice between oral and parenteral Strong
therapy should be based on patient age;
clinical suspicion of urosepsis; illness
severity; refusal of fluids, food and/or oral
medication; vomiting; diarrhoea; non-
compliance; complicated pyelonephritis.
Treat febrile UTIs with four to seven day Strong
courses of oral or parenteral therapy.
Treat complicated febrile UTI with broad- Strong
spectrum antibiotics.
Offer long-term antibacterial prophylaxis in Strong
case of high susceptibility to UTI and risk
of acquired renal damage and lower urinary
tract symptoms.
In selected cases consider dietary Strong
supplements as an alternative or add-on
preventive measure.
In infants with febrile UTI use renal and Strong
bladder ultrasound to exclude obstruction
of the upper and lower urinary tract within
24 hours.

452 Paediatric Urology


In infants, exclude vesicoureteral reflux Strong
after first episode of febrile UTI with a
non-E. Coli infection. In children more than
one year of age with an E. Coli infection,
exclude vesicoureteric reflux after the
second febrile UTI.

DAY-TIME LOWER URINARY TRACT DYSFUNCTION


Urinary incontinence in children may be caused by congenital
or neurologic abnormalities, however, many children have
functional bladder problems for which the term day-time
lower urinary tract (LUT) conditions is used. Day-time LUTD
has a high prevalence ranging between 1%-20%. Symptoms
can be classified as filling-phase (storage) dysfunctions and
voiding-phase (emptying) dysfunctions.

Table 1: Management algorithm

Children above 5 years of age applying with LUTS


DIAGNOSTIC WORK-UP
Voiding diary 2-3 full days minimum
Bristol Stool scale
Physical exam
- T o exclude neurogenic pathology or anatomic problems
(meatal stenosis, labial fusion)
Urinalysis
- To exclude presence of UTO or any other pathology (DM, DI)
Uroflowmetry and PVR determination (USG or bladder scan)
- To evaluate urine flow and emptying efficacy
Questionnaires (optional)
- T o evaluate voiding and bowel habits, wetting severity/
frequency, fluid intake, quality of life

Paediatric Urology 453


Ultrasonography (optional)
- To determine bladder wall thickness, upper tract changes,
signs of constipation
Urodynamic studies (not required unless refractory to
management)
VCUG (only required if recurrent febrile UTI is present)
MANAGEMENT
- If UTI is present, treat UTI first
- If constipated, treat bowel first with dietary changes and
laxatives
- Urotherapy is initial therapy in all cases to maintain
controlled fluid intake, regular and efficient
bladder emptying
- Medical treatment (anticholinergics); if OAB symptoms
dominate and persist despite urotherapy
- Antibiotic prophylaxis: in case of recurrent UTI
- Biofeedback is optional as first line therapy as part of
urotherapy program; otherwise it is
recommended if refractory to urotherapy
- Neural stimulation or Botulinum Toxin A injection in
detrusor is suggested if refractory to
urotherapy and medical treatment but is still experimental

Recommendations Strength rating


Use two day voiding diaries and/or Strong
structured questionnaires for objective
evaluation of symptoms, voiding drinking
habits and response to treatment.
Use a stepwise approach, starting with Weak
the least invasive treatment in managing
day-time lower urinary tract dysfunction in
children.

454 Paediatric Urology


Initially offer urotherapy involving bladder Weak
rehabilitation and bowel management.
If bladder bowel dysfunction is present, Weak
treat bowel dysfunction first, before
treating the lower urinary tract condition.
Use pharmacotherapy (mainly Strong
antispasmodics and anticholinergics) as
second-line therapy in overactive bladder.
Use antibiotic prophylaxis if there are Weak
recurrent infections.
Re-evaluate in case of treatment failure; Weak
this may consist of (video) urodynamics
magnetic resonance imaging of
lumbosacral spine and other diagnostic
modalities, guiding to off-label treatment
which should only be offered in highly
experienced centres.

MONOSYMPTOMATIC NOCTURNAL ENURESIS -


BEDWETTING
Monosymptomatic nocturnal enuresis is incontinence during
the night without daytime symptoms above the age of five
years. Due to an imbalance between night-time urine output
and night-time bladder capacity, the bladder can easily
become full at night, and the child will either wake-up to
empty the bladder or will void during sleep.

A voiding diary, registering the day-time bladder function and


the night-time urine output will help guide the treatment.

Paediatric Urology 455


Figure 9: A stepwise assessment and management options
for nocturnal enuresis

Child ≥ 5 years with nocturnal enuresis

2 weeks Upon indication


night-time • Urine
urine microscopy
Detailed production • Uroflowmetry
2 days
questions for Physical recording • Ultrasound
day-time
day-time exam and (= weight • ENT referral
voiding and • Psychologist
symptoms urinanalysis night-time
drinking diary referral
diapers +
morning first
voided
volume)

Supportive measures (not a treatment) (max. 4 weeks)


• Normal and regular drinking habits
• Regular voiding and bowel habits
• Monitor night-time production (weight diapers)

Child + caregivers seek a treatment

Nocturnal enuresis
wetting alarm
desmopressin
treatment
+/ - anticholinergics
with regular
follow-up

If no improvement (< 4 weeks) OR Lack of compliance


+
Re-evaluate

Diagnosis Treatment Follow-up

ENT = ear, nose, throat

456 Paediatric Urology


Recommendations Strength rating
Do not treat children less than five years Strong
of age in whom spontaneous cure is likely,
but inform the family about the involuntary
nature, the high incidence of spontaneous
resolution and the fact that punishment
will not help to improve the condition.
Use micturition diaries or questionnaires to Strong
exclude day-time symptoms.
Perform a urine test to exclude the Strong
presence of infection or potential causes
such as diabetes insipidus.
Offer supportive measures in conjunction Strong
with other treatment modalities, of which
pharmacological and alarm treatment are
the two most important.
Offer desmopressin in proven night-time Strong
polyuria.
Offer alarm treatment in motivated and Strong
compliant families.

MANAGEMENT OF NEUROGENIC BLADDER


Neurogenic detrusor-sphincter dysfunction may result in
different forms of lower urinary tract dysfunctions and in
incontinence, urinary tract infections, vesico-ureteral reflux,
renal scarring and renal insufficiency. The most common
cause in children is myelodysplasia. Bladder and bowel
dysfunction correlates poorly with the type and level of the
spinal cord lesion. Therefore, urodynamic and functional
classifications are required to define the extent of the
pathology and in guiding treatment planning. Children with
neurogenic bladder can also have disturbances of bowel and
sexual function. The main goals of treatment are prevention of

Paediatric Urology 457


urinary tract deterioration, achievement of continence at an
appropriate age and also improving quality of life.

Figure 10: Management of children with myelodysplasia with


a neurogenic bladder Flowchart - First year of life

Flowchart - First year of life


First 12 months

Birth-
discharge
6-12 weeks 6 months 9 months 1 year
from the
hospital

• Bladder-catheter • Medical history • Medical history • Medical history • Medical history


until closure of • Clinical • Clinical • Clinical • Clinical
the back has examination examination examination examination
healed • Blood pressure • Urine analysis • Urine analysis • Blood pressure
• Then start • Urine analysis, • Check and • Check and • Urine analysis
CIC + AB after • Check + optimise bowel optimise bowel • Check and
peri-operative optimise bowel manageent management optimise bowel
antibiotic is manageent management
finished

At one week: • RBUS • RBUS • RBUS • RBUS


• RBUS • Creatinine • Creatinine • Creatinine
• Creatinine

• Start CIC + teach • VUD or • CMG if first • If Reflux present


the parents CIC. • VCUG & CMG, CMG showed or febrile UTI,
• After getting if VUD is not a hostile or VUD or VCUG &
comfortable with available non-conclusive CMG if no reflux
the CIC, stop CMG or febrile UTI,
AB. CMG is ok
• Check bowel
management

• Start oxybutynin • Baseline DMSA • If no reflux or


if any sign no UTI and low
of bladder grade reflux,
overactivity stop AB if given
due to reflux and
monitor urine
with dip sticks at
• Start AB if reflux home
and hostile
bladder or
non-conclusive
Diagnosis Treatment Follow-up
VUD/CMG

458 Paediatric Urology


Flowchart - 18 months – 4 years of age

18 months - 4 years

18 months 2 years 2.5 years 3 years 4 years

• Medical history • Medical history • Medical history • Medical history • Medical history
• Clinical • Clinical • Clinical • Clinical • Clinical
examination examination examination examination examination
• Blood pressure • Blood pressure • Blood pressure • Blood pressure • Blood pressure
• Urine analysis • Urine analysis • Urine analysis • Urine analysis • Urine analysis
• Check and • Check and • Check and • Check and • Check and
optimise bowel optimise bowel optimise bowel optimise bowel optimise bowel
management management management management management
• Check anti- • Check anti- • Check anti- • Check anti- • Check anti-
cholinergic cholinergic cholinergic cholinergic cholinergic
medication + medication + medication + medication + medication +
adapt to weight adapt to weight adapt to weight adapt to weight adapt to weight

• RBUS • RBUS • RBUS • RBUS • RBUS


• Creatinine • Creatinine • Creatinine

• CMG only, if • If reflux present • CMG only, if • If Reflux present • If Reflux present
there is still a or febrile UTI, clinical status or febrile UTI, or febrile UTI,
hostile bladder VUD or VCUG & has changed VUD or VCUG & VUD or VCUG
or clinical status CMG if no reflux CMG if no reflux & CMG if no
has changed or febrile UTI, or febrile UTI, hostile bladder
CMG is ok CMG is ok and clinical no
change CMG at
5 yrs. is ok

• If no reflux or no • If no reflux or no • If no reflux or no


UTI + low-grade UTI + low grade UTI + low grade
reflux, stop AB reflux, stop AB reflux, stop AB
• If AB is given • If AB is given • If AB is given
due to reflux + due to reflux + due to reflux +
monitor urine monitor urine monitor urine
with dip sticks at with dip sticks at with dip sticks at
home home home

Diagnosis Treatment Follow-up

Paediatric Urology 459


Flowchart - 5 years to adulthood

5 years - adulthood

5 years 6 years - puberty yearly Adolescence yearly Adulthood yearly

• Medical history • Medical history • Medical history • Medical history


• Clinical examination • Clinical examination • Clinical examination • Clinical examination
• Blood pressure • Blood pressure • Blood pressure • Blood pressure
• Urine analysis • Urine analysis • Urine analysis • Urine analysis
• Check + optimise • Check + optimise • Check + optimise • Check + optimise
bowel management bowel management bowel management bowel management
• Check anti- • Check anti- • Discuss sexual • Discuss sexual
cholinergic cholinergic function/fertility function + treat
medication + adapt medication + adapt • Check anti- accordingly
to weight to weight cholinergic • Check anti-
medication + adapt cholinergic
to weight medication + adapt
to weight

• RBUS • RBUS • RBUS • RBUS


• Creatinine • Creatinine • Creatinine • Creatinine
• Cystatin C • Cystatin C • Cystatin C • Cystatin C

• If Reflux present or • If no hostile bladder • If no hostile bladder • If no hostile bladder


febrile UTI, VUD or or clinical changes or clinical changes in or clinical changes in
VCUG & CMG biannually CMG a compliant patient a compliant patient
• If no reflux or febrile biannually CMG biannually CMG
UTI, CMG is ok otherwise yearly otherwise yearly

• DMSA scan, if reflux • DMSA scan at age • DMSA scan at age • DMSA scan if
was/is present of 10, if reflux was/is of 15, if reflux was/is indicated
or febrile UTI has present or febrile UTI present or febrile UTI
occurred has occurred has occurred

• In patients with • In patients with • In patients with


bowel segments bowel segments bowel segments
incorporated into the incorporated into the incorporated into the
urinary tract urinary tract urinary tract
• Acid-base balance • Acid-base balance • Acid-base balance
• Vitamin B12 • Vitamin B12 • Vitamin B12
• If pathological - • If pathological - • If pathological -
substitution substitution substitution
• Check for secondary
malignancy

Diagnosis Treatment Follow-up

RBUS = Renal bladder ultrasound; UTI = urinary tract infection;


VUD = videourodynamic; VCUG = voiding cystourethrography;
CMG = cystometrogram; DMSA = dimercaptosuccinic acid.
460 Paediatric Urology
Figure 11: Algorithm for the management of children with
myelodysplasia with a neurogenic bladder

Time at diagnosis

Newborn Late presentation

Early CIC

Understanding the detrussor-sphincter


relationship status: history, USG,
VUD/VCU,
nuclear medicine

Detrussor overactive, Detrussor Detrussor underactive, Detrussor underactive,


Sphincter overactive, Sphincter Sphincter underactive
under/normoactive Sphincter overactive over/normoactive

Antimuscarinic Antimuscarinic CIC if residual urine CIC if residual urine


CIC if residual urine CIC CAP if VUR present CAP if VUR present
CAP if VUR present CAP if VUR present

In cases of clinical In cases of clinical Decision given Bladder neck


failure or upper failure or upper regarding the clinical procedures
urinary tract urinary tract situation +/- augmentation
deterioration: deterioration: procedures
Botulinum toxin Botulinum toxin
injection to bladder: injection to bladder
added to treatment or sphincter: added
to treatment

Augmentation Augmentation
procedures procedures

Understanding the detrusor-sphincter


relationship status:
history, USG, VUD/VCU, nuclear medicine

Diagnosis Treatment Follow-up

CAP = continuous antibiotic prophylaxis; CIC = clean


intermittent catheterisation; US = ultrasound;
VCUG = voiding cystourethrography; VUD = videourodynamic;
VUR = vesicoureteric reflux.
Paediatric Urology 461
Recommendations Strength rating
Urodynamic studies should be performed Strong
in every patient with spina bifida as well
as in every child with high suspicion of a
neurogenic bladder to estimate the risk for
the upper urinary tract and to evaluate the
function of the detrusor and the sphincter.
In all newborns, intermittent catheterisation Strong
(IC) should be started soon after birth. In
those with a clear underactive sphincter and
no overactivity, starting IC may be delayed.
If IC is delayed, closely monitor babies for
urinary tract infections, upper tract changes
(US) and the lower tract (UD).
Start early anticholinergic medication Strong
in the newborns with suspicion of an
overactive detrusor.
The use of suburothelial or intradetrusoral Strong
injection of onabotulinum toxin A
is an alternative and a less invasive
option in children who are refractory to
anticholinergics in contrast to bladder
augmentation.
Treatment of faecal incontinence is Strong
important to gain continence and
independence. Treatment should be started
with mild laxatives, rectal suppositories as
well as digital stimulation. If not sufficient
transanal irrigation is recommended, if not
practicable or feasible, a Malone antegrade
colonic enema (MACE)/Antegrade
continence enema (ACE) stoma should be
discussed.

462 Paediatric Urology


Ileal or colonic bladder augmentation is Strong
recommended in patients with therapy
resistant overactivity of the detrusor,
small capacity and poor compliance,
which may cause upper tract damage and
incontinence. The risk of surgical and non-
surgical complications and consequences
outweigh the risk of permanent damage
of the upper urinary tract +/- incontinence
due to the detrusor.
In patients with a neurogenic bladder and a Weak
weak sphincter, a bladder outlet procedure
should be offered. It should be done in
most patients together with a bladder
augmentation.
Creation of a continent cutaneous Weak
catheterisable channel should be offered to
patients who have difficulties in performing
an IC through the urethra.
A life-long follow-up of renal and reservoir Weak
function should be available and offered
to every patient. Addressing sexuality and
fertility starting before/during puberty
should be offered.
Urinary tract infections are common Weak
in children with neurogenic bladders,
however, only symptomatic UTIs should be
treated.

Paediatric Urology 463


DILATATION OF THE UPPER URINARY TRACT
(UPJ AND UVJ OBSTRUCTION)
Dilatation of the upper urinary tract remains a significant
clinical challenge in deciding which patient will benefit from
treatment. Ureteropelvic junction obstruction is the most
common pathological cause of neonatal hydronephrosis.

Megaureters (obstruction at the level of the ureterovesical


junction) are the second most likely cause of pathological
neonatal hydronephrosis. The widespread use of US during
pregnancy has resulted in a higher detection rate for
antenatal hydronephrosis. The challenge in the management
of dilated upper tracts is to decide which child should be
observed, which managed medically, and which requires
surgical intervention.

Figure 12: Diagnostic algorithm for dilatation of the upper


urinary tract
Postnatal US

Dilatation (uni- or bilateral) No dilatation

Voiding cystourethrogram (VCUG)* Repeat US after 4 weeks

Diuretic renography

Diagnosis Treatment Follow-up

464 Paediatric Urology


* A diagnostic work-up including VCUG must be discussed
with the caregivers, as it is possible that, even if reflux is
detected, it may have absolutely no clinical impact. However,
it should be borne in mind that reflux has been detected in
up to 25% of prenatally detected cases.
US = ultrasound.

Recommendations Strength rating


Include serial ultrasound (US) and Strong
subsequent diuretic renogram and
sometimes voiding cystourethrography in
post-natal investigations.
Offer continuous antibiotic prophylaxis to Weak
the subgroup of children with antenatal
hydronephrosis who are at high risk of
developing urinary tract infection like
uncircumcised infants, children diagnosed
with hydroureteronephrosis and high-grade
hydronephrosis, respectively.
Decide on surgical intervention based on Weak
the time course of the hydronephrosis and
the impairment of renal function.
Offer surgical intervention in case of an Weak
impaired split renal function due to
obstruction or a decrease of split renal
function in subsequent studies and
increased anteroposterior diameter on the
US, and grade IV dilatation as defined by
the Society for Fetal Urology.

Paediatric Urology 465


Offer pyeloplasty when ureteropelvic Weak
junction obstruction has been confirmed
clinically or with serial imaging studies
proving a substantially impaired or
decrease in function.
Do not offer surgery as a standard Weak
for primary megaureters since the
spontaneous remission rates are as high
as 85%.

VESICOURETERIC REFLUX IN CHILDREN


Vesicoureteric reflux (VUR) presents with a wide range of
severities, and the majority of reflux patients will not develop
renal scars and probably will not need any intervention.
The main goal in management is the preservation of kidney
function.

The diagnostic work-up should evaluate the overall health and


development of the child including a detailed medical history
(including family history, and screening for lower urinary
tract and/or bowel dysfunction [LUTD]), physical examination
together with blood pressure measurement, urinalysis
(assessing proteinuria), urine culture, and serum creatinine
in patients with bilateral renal parenchymal abnormalities.
Voiding cystourethrography still remains the gold standard in
diagnosing VUR.

Recommendation for diagnostic evaluation Strength rating


For diagnosis of VUR apart from VCUG , Weak
ceVUS is another option.
Recommendation for screening Strength rating
Inform parents of children with Strong
vesicoureteric reflux (VUR) that siblings and
offspring have a high prevalence of VUR.

466 Paediatric Urology


Recommendations for treatment Strength rating
Initially treat all symptomatic patients Weak
diagnosed within the first year of life
with continuous antibiotic prophylaxis,
regardless of the grade of reflux or
presence of renal scars.
Offer immediate, parenteral antibiotic Strong
treatment for febrile breakthrough
infections.
Initially manage all children presenting at Strong
age one to five years conservatively.
Offer close surveillance without antibiotic Strong
prophylaxis to children presenting with
lower grades of reflux and without
symptoms.
Ensure that a detailed investigation for the Strong
presence of lower urinary tract dysfunction
(LUTD) is done in all and especially in
children after toilet-training. If LUTD is
found, the initial treatment should always
be for LUTD.
Offer reimplantation or endoscopic Weak
correction to patients with frequent
breakthrough infections.
Offer reimplantation to patients with Strong
persistent high-grade reflux and endo-
scopic correction for lower grades of reflux.
Offer surgical repair to children above the Weak
age of one presenting with high-grade
reflux and abnormal renal parenchyma.
Offer surgical correction, if parents Strong
prefer definitive therapy to conservative
management.

Paediatric Urology 467


Select the most appropriate management Weak
option based on:
• the presence of renal scars;
• clinical course;
• the grade of reflux;
• ipsilateral renal function;
• bilaterality;
• bladder/bowel function;
• associated anomalies of the urinary tract;
• age and gender;
• compliance;
• parental preference.
In high-risk patients who already have Strong
renal impairment, a more aggressive, multi-
disciplinary approach is needed.

URINARY STONE DISEASE


Paediatric stone disease is an important clinical problem in
paediatric urology practice. Due to its recurrent nature, every
effort should be made to discover the underlying metabolic
abnormality so that it can be treated appropriately.

Presentation tends to be age-dependent, with symptoms such


as flank pain and haematuria being more common in older
children. Infantile urolithiasis appears to be a seperate entity
since the aetiology and the clinical course is different than
in other age groups. Non-specific symptoms (e.g. irritability,
vomiting) are common in very young children. Adequate fluid
intake and restricting the use of salt within daily allowance
range are the general recommendations besides the
specific medical treatment against the detected metabolic
abnormalities. With the advance of technology, stone
management has changed from open surgical approaches to
endoscopic techniques that are less invasive.

468 Paediatric Urology


Figure 13: Algorithm for metabolic investigations in urinary
stone disease in children

Paediatric stone patient

Elimination of stones by spontaneous


Exclude obstructive uropathy
passage or active removal (SWL, surgery)

Stone analysis

Mg Ammonium
Calcium stones
phosphate Uric acid stone Cystine
CaOX-CaPO
(struvite)

urine pH urine pH
urine culture urine and serum urine cystine
uric acid levels level

possibly acidic urine


urease producing hyperuricosuria cystinuria
bacteria hyperuricemia

Alkali High fluid intake


Total elimination replacement - potassium citrate
of stone k citrate 3-4 mEq/kg/d
(surgery/SWL) Allopurinol mercaptopropiyonilglycine
antibiotics (10 mg/kg) 10-15 mg/kg/d
low purine diet penicillamin 30 mg/kg/day

urine - blood pH
serum PTH hypercalcaemia urine - blood Ca - uric acid levels,urine
Mg, Phosphate
pH > 5.5
urine Ca-Oxalate-Citrate-Mg-Uric A -Phosphate

Elevated First morning urine First morning urine


pH < 5.5 pH > 5.5

Hyperparathyroidism Further investigation


Surgical treatment for RTA

hypercalciuria hyperoxaluria hyperuricosuria hypocitraturia

K-citrate
Regular calcium
diet Alkali
intake Citrate
(normal calcium replacement
Diet low in ox. replacement
low sodium (K-citrate)
K-citrate K-citrate
intake) allopurinol
pyridoxine
HCTZ (diuretic)

Diagnosis Treatment Follow-up

Ca = calcium; HCTZ = hydrochlorothiazide; Mg = magnesium;


Ox = oxalate; PTH = parathyroid hormone;
SWL = extracorporeal shockwave lithotripsy;
RTA = renal tubular acidosis; Uric A = uric acid.
Paediatric Urology 469
Table 2: R
 ecommendations for interventional management in
paediatric stones

Stone Primary Secondary Comment


size and treatment treatment
localisation* option options
Infant micro- Observation Intervention Individualised
lithiasis and/or decision according
(<3mm, any medical to size progression,
location) treatment symptoms and
metabolic factors.
Staghorn PCNL Open/SWL Multiple sessions
stones and accesses
with PCNL may
be needed.
Combination with
SWL may be useful.
Pelvis SWL RIRS/PCNL
< 10 mm
Pelvis SWL/PCNL/RIRS Multiple sessions
10-20 mm with SWL may be
needed. PCNL and
RIRS have a similar
recommendation
grade.
Pelvis PCNL SWL/RIRS Multiple sessions
> 20 mm with SWL may be
needed.
Lower pole Observation PCNL/RIRS Stone clearance
calyx or SWL after SWL is
< 10mm lower than other
locations.

470 Paediatric Urology


Lower pole PCNL RIRS/SWL Anatomical
calyx variations are
> 10mm important for
complete clearance
after SWL.
Upper SWL URS Flexible scopes may
ureteric be needed in case
stones of retropulsion.
Lower URS SWL
ureteric
stones
Bladder Endoscopic SWL/Open Open is easier and
stones (transure- with less operative
thral or per- time with large
cutaneous) stones.
* Cystine and uric acid stones excluded.
PCNL = percutaneous nephrolithotomy; SWL = shockwave
lithotripsy; RIRS = retrograde intrarenal surgery;
URS = ureteroscopy.

Recommendations Strength rating


Use plain abdominal X-ray and ultrasound Strong
as the primary imaging techniques for the
diagnosis and follow-up of stones.
Use low-dose non-contrast computed Strong
tomography in cases with a doubtful
diagnosis, especially of ureteral stones or
complex cases requiring surgery.

Paediatric Urology 471


Perform a metabolic evaluation in any Strong
child with urinary stone disease. Any
kind of interventional treatment should
be supported with medical treatment for
the underlying metabolic abnormality, if
detected.
Limit open surgery under circumstances Strong
in which the child is very young with large
stones, in association with congenital
problems requiring surgical correction and/
or with severe orthopaedic deformities that
limit positioning for endoscopic procedures.
Observe infant microlithiasis, unless Strong
symptoms occur or size increases
significantly.

OBSTRUCTIVE PATHOLOGY OF RENAL DUPLICATION:


URETEROCELE AND ECTOPIC URETER
Ureterocele and ectopic ureter are the two main anomalies
associated with complete renal duplication. Antenatal US
detects both conditions in the majority of cases if associated
with obstruction, and diagnosis is confirmed after birth. Later
in life, these anomalies are revealed by clinical symptoms:
UTI, pain, calculus formation, disturbances of micturition, and
urinary incontinence. There is a wide variation of symptoms
in patients with ureterocele (from the asymptomatic patient
to urosepsis, urinary retention and upper tract dilatation after
birth). Ectopic ureter is less frequent than ureterocele and
more common in females with some remaining asymptomatic.

472 Paediatric Urology


Figure 14: Algorithm for the management of duplex system
ureteroceles after the first 3-6 months of life

DSU

Asymptomatic Symptomatic or severe HUN or


No severe HUN or obstruction obstruction

No VUR VUR VUR No VUR

Low grade High grade or Good No/poor


multiple function function
infections

Observation Bladder surgery or Ectopic: upper Intravesical Ectopic


endoscopic to lower tract endoscopic UPPN
management anastomosis decompression

Diagnosis Treatment Follow-up

DSU = duplex system ureterocele; HUN = hydroureteronephrosis;


UPPN = upper pole partial nephrectomy; VUR = vesicoureteric
reflux to the lower pole.

Paediatric Urology 473


Recommendations Strength
rating
Ureterocele
Diagnosis Use ultrasound (US), radionuclide Weak
studies (mercaptoacetyltriglycine
(MAG3)/ dimercaptosuccinic acid
(DMSA)), voiding cystourethrography
(VCUG), magnetic resonance
urography, high-resolution magnetic
resonance imaging (MRI), and
cystoscopy to assess function, to
detect reflux and rule out ipsilateral
compression of the lower pole and
urethral obstruction.
Treatment Select treatment based on symptoms, Weak
function and reflux as well on surgical
and parenteral choices: observation,
Endoscopic decompression,
ureteral re-implantation, partial
nephroureterectomy, complete
primary reconstruction. Offer
early endoscopic decompression
to patients with an obstructing
ureterocele.
Ectopic ureter
Diagnosis Use US, DMSA scan, VCUG or MRI for Weak
a definitive diagnosis.

474 Paediatric Urology


Treatment In non-functioning moieties with Weak
recurrent infections, heminephro-
ureterectomy is a definitive solution.
Ureteral reconstruction (ureteral
re-implantation/ureteroureterostomy/
ureteropyelostomy and upper-pole
ureterectomy) are other therapeutic
option especially in cases in which the
upper pole has function worth preserving.

DISORDERS/DIFFERENCES OF SEX DEVELOPMENT


The term ‘disorders of sex development’ is proposed to
indicate congenital conditions with atypical development
of chromosomal, gonadal or anatomical sex. Dealing with
neonates with DSD requires a multi-disciplinary approach,
which should include geneticists, neonatologists, paediatric
and adult endocrinologists, gynaecologists, psychologists,
ethicists and social workers with each team member
specialised in DSD.

Table 3: Findings in a newborn suggesting the possibility


of DSD (adapted from the American Academy of
Pediatrics)

Apparent male
Severe hypospadias associated with bifid scrotum
Undescended testis/testes with hypospadias
Bilateral non-palpable testes in a full-term apparently male
infant
Apparent female
Clitoral hypertrophy of any degree, non-palpable gonads
Vulva with single opening
Indeterminate
Ambiguous genitalia
Paediatric Urology 475
Table 4: D
 iagnostic work-up of neonates with disorders of
sex development

History (family, maternal, neonatal)


Parental consanguinity
Previous DSD or genital anomalies
Previous neonatal deaths
Primary amenorrhoea or infertility in other family members
Maternal exposure to androgens
Failure to thrive, vomiting, diarrhoea of the neonate
Physical examination
Pigmentation of genital and areolar area
Hypospadias or urogenital sinus
Size of phallus
Palpable and/or symmetrical gonads
Blood pressure
Investigations
Blood analysis: 17-hydroxyprogesterone, electrolytes, LH,
FSH, TST, cortisol, ACTH
Urine: adrenal steroids
Genetics: karyotype, next-generation sequencing-based
molecular diagnostics, WES
Ultrasound
Genitogram
hCG stimulation test to confirm presence of testicular tissue
Androgen-binding studies
Endoscopy

ACTH = adrenocorticotropic hormone; FSH = follicle-stimulating


hormone; hCG = human chorionic gonadotropin;
LH = luteinising hormone; TST = testosterone.

476 Paediatric Urology


Recommendations Strength rating
Do not delay diagnosis and treatment of Strong
any neonate presenting with ambiguous
genitalia since salt-loss in a 46XX CAH girl
can be fatal.
Refer children to experienced centres where Strong
neonatology, endocrinology, (paediatric)
urology, psychology and transition to adult
care are guaranteed.
Utilise a multi-disciplinary approach and Strong
a shared decision model in patients with
DSD conditions including:
a. Gender assigment
b. G enital surgery (in accordance with
national regulations)
c. Gonadectomy.
Do not underestimate the significant Strong
effects on psychological and psychiatric
health, quality of life, personal
relationships, and sexual function in
individuals with DSD.
Ensure full disclosure to patients and Strong
caregivers that the presence of a
Y-chromosome in dysgenetic gonads
results in a higher malignancy risk

CONGENITAL LOWER URINARY TRACT OBSTRUCTION


(CLUTO)
The term CLUTO is used for a foetus, which during
intrauterine US screening shows a dilatation of the upper
and lower urinary tract. During pregnancy the diagnosis is
usually based on ultrasound examinations only. There is a
broad spectrum of conditions that could cause an intra-
uterine dilation of the urinary tract. Postpartum diagnosis

Paediatric Urology 477


comprises any number of anatomical and functional
disorders/anomalies/malformations causing dilatation e.g.
anterior urethral valves, urethral atresia/ stenosis, prune belly
syndrome, dilating VUR , cloacal malformation, prolapsing
ureterocele, megacystis-microcolon-intestinal hypoperistalsis
or megacystis-megaureter syndrome.

478 Paediatric Urology


Figure 15: An algorithm on the assessment, management and
follow-up of new-borns with possible PUV
New-born with suspicion of PUV

UT ultrasound

Bladder drainage
No stabilisation
+
Antibiotics

Nephrologist evaluation
+/-
ICU care if required

Assessment of RF +
Electrolyte disturbance

Voiding cystourethrogram

Confirmation of diagnosis

Valve ablation +/- circumcision

No improvement and
Improvement in No improvement, unstable or clinical
UT dilatation and RF but stable deterioration

Check residual PUV


Close surveillance for UTI
CIC if not emptying
Monitor RF and electrolytes
Consider alpha blockade
Monitor nocturnal polyuria
Consider anticholinergics

Stabilisation
Progressive loss of RF
Recurrent UTI
Poor bladder emptying

Stable and improved UT dilatation and RF Consider Urinary diversion

Long term surveillance Stable but no improvement in UT dilatation and RF

Stable Intensify monitoring + management

Diagnosis Treatment Follow-up

CIC = clean intermittent catheterisation; OAB = overactive


bladder; PUV = posterior urethral valve; RF = renal function;
UT = urinary tract; UUT = upper urinary tract; VCUG = voiding
cystourethrogram.

Paediatric Urology 479


Recommendations Strength rating
Drain the bladder in new-borns with Strong
a suspected diagnosis of infravesical
obstruction and place on antibiotic
prophylaxis.
Perform a voiding cystourethrogram (VCUG) Strong
in patients in whom a diagnosis of posterior
urethral valve (PUV) is suspected.
Attempt endoscopic valve ablation after Strong
bladder drainage and stabilisation of the
child.
Consider neonatal circumcision as an Strong
adjunct to antibiotic prophylaxis to decrease
the risk of UTI in those with a PUV, especially
in the presence of high grade vesicoureteral
reflux.
Offer prolonged urinary diversion Strong
(suprapubic/transurethral) for bladder
drainage if the child is too small for valve
ablation.
Use serum creatinine nadir as a prognostic Strong
marker.
Assess split renal function by Strong
dimercaptosuccinic acid (DMSA) scan or
mercaptoacetyltriglycine (MAG3) clearance.
Consider high urinary diversion if bladder Strong
drainage is insufficient to drain the upper
urinary tract, or in the absence of clinico-
biochemical improvement.
Monitor and manage bladder and renal Strong
function lifelong.

480 Paediatric Urology


RARE CONDITIONS:
Urachal remnants
Urachal remnants originate from failure of the obliteration of
the allantois, resulting in a urachal anomaly such as urachal
sinus, urachal cyst, vesico-urachal diverticulum, and patent
urachus, respectively. Most often the urachal anomaly is
asymptomatic, but it occasionally may become infected, may
cause urinary symptoms, or may develop a urachal carcinoma
in later life.

Recommendations Strength rating


Urachal remnants with no epithelial tissue Strong
carry little risk of malignant transformation.
Asymptomatic and non-specific atretic Strong
urachal remnants can safely be managed
non-operatively.
Urachal remnants incidentally identified Strong
during diagnostic imaging for non-specific
symptoms should also be observed non-
operatively since they tend to resolve
spontaneously.
A small urachal remnant, especially at Strong
birth, may be viewed as physiological.
Urachal remnants in patients younger than Strong
six months are likely to resolve with non-
operative management.
Follow-up is necessary only when Strong
symptomatic for six to twelve months.
Surgical excision of urachal remnants Strong
solely as a preventive measure against
later malignancy appears to have minimal
support in the literature.

Paediatric Urology 481


Only symptomatic urachal remnants Strong
should be safely removed by open or
laparoscopic approach.
A voiding cystourethogram is only Strong
recommended when presenting with
febrile urinary tract infections.

Papillary tumours of the bladder


Papillary tumours of the bladder in children and adolescents
are extremely rare and are different from papillary tumours in
adults.

Recommendations Strength rating


Ultrasound is the first investigation of Strong
choice for the diagnosis of paediatric
bladder tumours.
Cystoscopy should be reserved if a bladder Strong
tumour is suspected on imaging for
diagnosis and treatment.
After histological confirmation, inflammatory Weak
myofibroblastic bladder tumours should be
resected locally.
Follow-up should be every 3-6 months in the Weak
first year, and thereafter at least annually
with urinanalysis and an ultrasound for at
least 5 years.
Have a high index of suspicion of Strong
eosinophilic cystitis (EC) in protracted
urinary tract symptoms unresponsive to
regular treatment.
Remove any possible allergens as the Strong
obvious first step in managing EC.

482 Paediatric Urology


Eosinophilic cystitis can be managed Weak
medically with corticosteroids, antibiotics,
anticholinergics, and antihistamines, in
addition to cyclosporine A.
Manage nephrogenic adenoma (NA) by Strong
resection either transuretherally or by open
excision.
Regular endoscopic follow-up especially for Weak
augmented patients with NA is justified.

Penile lesions
Paediatric lesions of the penis are uncommon but an
important part of the paediatric urological practice. The most
common of these lesions are cystic penile lesions followed by
vascular malformations and neurogenic lesions. Soft tissue
tumours of the male external genitalia are uncommon, but
have been described in the paediatric age group and can be
malignant.

Recommendations Strength rating


Treatment of penile cystic lesions is by Weak
total surgical excision, it is mainly indicated
for cosmetic or symptomatic (e.g. infection)
reasons.
Propranolol is currently first-line treatment Strong
for infantile haemangiomas.

Penile lymphedema
Paediatric lymphedema is usually primary and generally very
rare. Inefficient lymphatic drainage leads to accumulation of
subcutaneous lymph causing tissue swelling and inflammation
and subsequently stimulates adipose deposition and fibrosis
further exacerbating enlargement. With time the edematous

Paediatric Urology 483


tissue becomes vulnerable to infection, chronic cutaneous
changes and disfigurement. Complications may ensue such
as phimosis, haematuria, bleeding, bladder outlet obstruction,
pain, dysuria, lymphorrhea and severe psychological distress
due to resultant deformity.

Recommendations Strength rating


Conservative management is the first-line Strong
treatment for penile lymphedema.
In symptomatic cases or in patients with Weak
functional impairment, surgical intervention
may become necessary for penile
lymphedema.

PAEDIATRIC UROLOGICAL TRAUMA/EMERGENCIES


In about 3% of children seen at paediatric hospital trauma
centres, there is significant involvement of the genitourinary
tract. This is caused by either blunt injuries from falls, car
accidents, sports injuries, physical assault, and sexual abuse, or
penetrating injuries, usually due to falls onto sharp objects or
from gunshot or knife wounds.

Paediatric renal trauma

Table 5: R
 enal injury classified according to the kidney injury
scale of the American Association for the Surgery of
Trauma

Grade Type of injury Description


I Heamatoma Subcapsular haematoma and/or
and/or parenchymal contusion without
contusion laceration

484 Paediatric Urology


II Haematoma Perirenal haematoma confined to
Gerota fascia
Laceration Renal parenchymal laceration ≤1 cm
depth without urinary extravasation
III Laceration Renal parenchymal laceration >1
cm depth without collecting system
rupture or urinary extravasation
Vascular Any injury in the presence of a
kidney vascular injury or active
bleeding contained within Gerota
fascia
IV Laceration - Parenchymal laceration extending
into urinary collecting system with
urinary extravasation
- Renal pelvis laceration and/or
complete ureteropelvic disruption
Vascular - Segmental renal vein or artery
injury
- Active bleeding beyond Gerota
fascia into the retroperitoneum or
peritoneum
- Segmental or complete kidney
infarction(s) due to vessel
thrombosis without active
bleeding
V Laceration Shattered kidney with loss of
identifiable parenchymal renal
anatomy
Vascular - Main renal artery or vein laceration
or avulsion of hilum
- Devascularized kidney with active
bleeding

Paediatric Urology 485


Vascular injury is defined as a pseudoaneurysm or
arteriovenous fistula and appears as a focal collection of
vascular contrast that decreases in attenuation with delayed
imaging. Active bleeding from a vascular injury presents
as vascular contrast, focal or diffuse, that increases in size
or attenuation in delayed phase. Vascular thrombosis can
lead to organ infarction. Grade based on highest grade
assessment made on imaging, at operation or on pathologic
specimen. More than one grade of kidney injury may be
present and should be classified by the higher grade of
injury. Advance one grade for bilateral injuries up to Grade III.

Recommendations Strength rating


Use imaging in all children who have Strong
sustained a blunt or penetrating trauma with
any level of haematuria, especially when the
history reveals a deceleration trauma, direct
flank trauma or a fall from a height.
Use contrast enhanced CT-scanning with Strong
delayed images for diagnostic and staging
purposes.
Manage most injured kidneys conservatively. Strong
Offer surgical intervention in case of Strong
haemodynamic instability and a Grade V
renal injury.

Paediatric ureteral trauma

Recommendations Strength rating


Diagnose suspected ureteral injuries by Strong
retrograde pyelogram.

486 Paediatric Urology


Manage ureteral injuries endoscopically, Weak
using internal stenting or drainage of an
urinoma, either percutaneously or via a
nephrostomy tube.

Paediatric bladder injuries

Recommendations Strength rating


Use retrograde cystography to diagnose Strong
suspected bladder injuries.
Ensure that the bladder has been filled to its Strong
full capacity and an additional film is taken
after drainage.
Manage extra-peritoneal bladder ruptures Strong
conservatively with a transurethral catheter
left in place for seven to ten days.
Perform surgical exploration in cases of Strong
intra-peritoneal bladder ruptures.

Paediatric urethral injuries

Recommendations Strength rating


Assess the urethra by retrograde Strong
urethrogram in case of suspected urethral
trauma.
Perform a rectal examination to determine Strong
the position of the prostate.
Manage urethral injuries conservatively Strong
initially if a transurethral catheter can be
placed.

Paediatric Urology 487


Manage posterior urethral injuries by either: Weak
• primary drainage with a suprapubic
catheter alone and delayed repair
• primary re-alignment with a transurethral
catheter

Priapism
Priapism is a prolonged full or partial erection of the penis
unrelated to sexual stimuli lasting ≥4 hours. Although the
prevalence of priapism in children is not well reported in
literature, it is considered a rare disease. The most common
cause of priapism in children is sickle cell disease (SCD),
which accounts for about 65% of all cases, followed by
leukemia (10%), trauma (10%), idiopathic (10%) and drugs
(5%). In patients with SCD, the mean age of the first episode
of priapism has been shown to be 15 years old, with 25%
presenting prepubertally.

Recommendations Strength rating


Perform a doppler ultrasonography in all Strong
patients presenting with priapism.
In children with ischaemic (low-flow) Strong
priapism, perform a full blood count and
haemoglobinopathy screen to exclude
sickle cell disease or other haematological
disorders.
Adopt a multidisciplinary approach when Strong
managing patients with SCD-associated
priapism.
Use a step-wise approach starting with Strong
the least invasive therapy in patients with
ischaemic (low-flow) priapism.

488 Paediatric Urology


Manage neonatal and non-ischaemic (high- Strong
flow) priapism conservatively in the initial
management period.

PERI-OPERATIVE FLUID MANAGEMENT


Children have a different total body fluid distribution, renal
physiology and electrolyte requirements, as well as weaker
cardiovascular compensation mechanisms, compared
to adults. Therefore, special child specific requirements
regarding preoperative fasting and intra- as well as post-
operative fluid have to be considered and close monitoring is
essential. This is especially true for interventions relieving any
kind of obstruction as this may result in substantial polyuria.

Table 6: Pre-operative fasting times for elective surgery

Ingested material Minimum fasting period (hours)


Clear liquids 1
Breast milk 3
Formula milk-based 4
products
Light meal 6

Recommendations Strength rating


Ensure shorter pre-operative fasting Strong
periods for elective surgeries (one hour for
clear liquids, three hours for breast milk,
four hours for formula milk-based products
and six hours for a light meal).
Start early postoperative oral fluid intake Strong
in all patients scheduled for minor surgical
procedures.

Paediatric Urology 489


Use enhanced recovery after surgery Strong
protocols for abdominal surgery in children
with pre-existing normal bowel function.

POST-OPERATIVE PAIN MANAGEMENT


The provision of adequate pain control requires proper pain
evaluation, accurate choice of drug and route of administration,
and consideration of age, physical condition and type of surgery
and anaesthesia.

A proposed strategy for post-operative analgesia may be as


follows:
1. Intra-operative regional block and/or local wound
infiltration.
2. Paracetamol + NSAID.
3. Paracetamol + NSAID + weak opioid (e.g. tramadol or
codeine).
4. Paracetamol + NSAID + strong opioid (e.g. morphine,
fentanyl, oxycodone or pethidine).

Recommendations Strength rating


Prevent/treat pain in children of all ages. Strong
Evaluate pain using age-compatible Strong
assessment tools.
Use pre-emptive and balanced analgesia in Strong
order to decrease opioids requirements.

Thromboprophylaxis management: general information


Thromboprophylaxis in children involves preventive
measures aimed at reducing the risk of blood clot formation.
Unlike adults, the majority of children do not require
thromboprophylaxis after surgery. It is only considered in
certain high-risk situations such as underlying medical
conditions like malignancies, congenital heart disease etc.

490 Paediatric Urology


Recommendations Strength rating
Use physical methods for venous thrombo- Strong
embolism (VTE) risk reduction in older
children and adolescents who are at
increased risk of VTE.
Consider low molecular weight heparin Strong
VTE prophylaxis in children, particularly
adolescents, with additional risk factors.

Premedication management: general information


The majority of children undergoing anaesthesia and surgery
develop anxiety that could lead to adverse reactions. Many
factors may influence preoperative anxiety. Anxiety and distress
can be prevented or relieved combining: premedication,
distraction techniques and parental or caregivers presence.
Non-pharmacological age-appropriate methods such as play
therapy, toys, storybooks, videos, tablet, mobile phone, can all
be useful. A successful plan must therefore take into account
the age and temperament of the child.

Recommendations Strength rating


Use non-pharmacological age-appropriate Weak
premedication methods to decrease
anxiety levels in children before surgery.
Use pharmacological premedication to Strong
decrease anxiety levels in children and
monitor for potential side effects.

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BASIC PRINCIPLES OF LAPAROSCOPIC SURGERY IN
CHILDREN
Laparoscopy in children requires specific anaesthetic
precautions. Physiological effects of CO2 pneumoperitoneum,
positioning of the patient and operative time need to be
considered by the anaesthesiology team.

Recommendations Strength rating


Use lower intra-abdominal pressure (6-8 Strong
mmHg) during laparoscopic surgery in
infants and smaller children.
Use open access for laparoscopy in infants Strong
and smaller children.
Monitor for laparoscopy-related cardiac, Strong
pulmonary and diuretic responses.

This short booklet text is based on the more comprehensive


EAU Paediatric Urology Guidelines (978-94-92671-23-3), available
at their website, http://www.uroweb.org/guidelines.

492 Paediatric Urology

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