PAEDIATRIC UROLOGY

ANANDA LAMAHEWAGE MS FRCS(ENG) FCSSL

CONSULTANT PAEDIATRIC SURGEON
LADY RIDGEWAY HOSPITAL FOR CHILDREN
COLOMBO
Contents
• Cryptorchidism • Trauma to the foreskin
• Acute scrotum • Hypospadias
• Hydrocele/varicocele/s • PUV
permatocele • VUR
• Testicular cancer • PUJO/Hydronephrosis
• Phimosis • Heamturia
• Paraphimosis
Cryptorchidism
• Cryptorchidism is the most common genital problem
encountered in pediatrics.
• Cryptorchidism literally means hidden or obscure testis
and generally refers to an undescended or
maldescended testis.
• Occurs on the right-50%, left-35%, bilateral-10-15%

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IN GENERAL
• Referral for cryptorchidism should occur by 6
months of age
• Imaging for cryptorchidism is not recommended
prior to referral
• Orchidopexy is the most successful therapy to
relocate the testis into the scrotum
• Hormonal therapy is not recommended
• Successful scrotal repositioning of the testis may
reduce but does not prevent the potential long-
term issues of infertility and testis cancer
• Spontaneous descent after the first year of life is
uncommon.

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INCIDENCE
• Overall, 2% of full-term male newborns
• Decreasing to 1% by 6 months to 1 year.
• The prevalence of cryptorchidism is 30% in premature
neonates

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 Predisposing factors

• Prematurity
• low birth weight
• small size for gestational age,
• twinning, and
• maternal exposure to estrogen during the first
trimester

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crypto
• Undescended testis: is arrested along its normal
path of descent
• Retractile testis: can be manipulated into scrotum
where it remains without tension
• Ectopic testis is located outside the normal path of
descent
• Ascended: previously descended, then “ascends”
spontaneously
• Gliding

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Cryptorchidism: A greek word which
means ‘hidden testis’
• Retractile- 60%
• Undescended- 35%
• Ectopic- 3%
• Ascending- <2%

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UDT
• 80% of undescended testes are palpable
• 20% are nonpalpable.
• Most intra-abdominal testes are found within a
few centimeters of the internal ring.
• Absent or vanishing testes are thought to be
due to an intrauterine or perinatal vascular
event
• Only 20-40% of nonpalpable testes are absent
upon surgical exploration.

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Ectopic testes
• testis exit the external inguinal ring and are
then misdirected along the normal course of
the testis.

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Comparison between ectopic & undescended testis
Undescended testis
• The testis is arrested in its normal
path of descent
• Usually undeveloped
• Undeveloped & empty scrotum on
the affected side
• Shorter length of spermatic cord
• Poor spermatogenesis after 6 yrs
• Usually associated with indirect
inguinal hernia
• Treatment: surgery & HT
• Associated with a number of
complications
Ectopic testis
• The testis deviates from its normal
path of descent
• Fully developed testis
• Empty but usually fully developed
scrotum
• Longer length of spermatic cord
• Spermatogenesis is perfect
• Never associated with indirect
inguinal hernia
• Treatment: basically surgical
• Complications: liability to injury

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Wyllie: Retractile testis
• Seen at different positions
• Can be brought down to scrotum and will remain
there
• Normal size

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Retractile testes
• palpated anywhere along the natural course of the
testis, although most are inguinal.
• suprascrotal secondary to an active cremasteric
reflex.
• This reflex is usually weak in infants and most
active in boys aged 5 years.
• These testes can be manipulated into the scrotum,
where they remain without tension.
• AS they can asend children with retractile testes
should be monitored regularly, at least until
puberty
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Ascending testis
• Descended testis which later goes up
• Can be iatrogenic as well

Gliding testes
• can be manipulated into a satisfactory scrotal
position but will retract quickly once released
• considered the most distal form of true
undescended testis.

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from genital ridge descent -
complex interaction of hormonal
and mechanical factors
Hormonal factors(Inguinoscrotal)
• Testosterone
• Dihydrotestosterone
• Mullerian-inhibiting factor
• HCG
• Genital branch of genitofemoral nerve which secret
CGRP (elaborated by testosterone)
• Non androgen–insulin like factor 3
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Mechanical factors

• Shortening and traction of the gubernaculum testis

• Enlargement/elongation of processus vaginalis
• Intra-abdominal pressure from increase visceral size
• Straightening of fetus
• Resolution of physiological hernia
• Enlargement of testes/growth of epididymis
• Propulsive force of the developing cremasteric muscle

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Complications of undescended testis
• Infertility
• Associated hernia: indirect inguinal hernia usually accompanies
a congenital undescended testis in about 90% cases but rarely
symptomatic.
• Testicular atrophy: due to pressure effects and histological
changes.
• Trauma
• Tumour: 10% of testicular cancer originate in cryptochid testis.
• Torsion
• Epididymo-orchitis in a chryptochid right testis can mimic
appendicitis
• Psychologic effects of an empty scrotum
• Testicular-Epididymal fusion abnormality

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Cryptorchidism is associated with
• inguinal hernia and/or patent processus vaginalis
• cerebral palsy
• mental retardation
• Down syndrome
• Wilms tumor
• Prader-Willi syndrome
• Abdominal wall defects (eg, gastroschisis,
omphalocele, prune belly syndrome)
• Hypospadias
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Clinical features
• Most in infancy and around school age. A few present after
puberty. (must at routine neonatal ex)
• Absence of one or both testes
• Swelling in the groin (may be the testis or a hernia)
• Pain in the groin due either to recurrent torsion of the
testis or strangulation of an associated hernia
• Gestational age at birth- usually preterm
• Determining if the testis was palpable in the scrotum at
any time is important
• Past history of inguinal surgery should be noted
• Family history of cryptorchidism and other associated
conditions.

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Examination
• Examination of potential ectopic sites- penile,
femoral, & perineal areas if the testicle cannot be
felt.
• If there is hypothalamic-pituitary dysfunction, the
patient is obese and the penis small for the age.
• Technique:
• Examination under anaesthesia is also done for
impalpable testis before exploration
• Clinical distinction between retractile and
undescended testis may be difficult

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Investigation
Imaging
• Abdominal USS
• CT Scan
• MRI
• Because imaging has not been proved to be
reliable in demonstrating whether the testis is
present or absent, its routine use is discouraged

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Management
• Hormone therapy
• Orchidopexy
• Orchidectomy
• Laparoscopic surgery

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Why opexy
• To decrease the likelihood of subfertility
• Facilitation of testicular self-examination for
testicular cancer
• Males with undescended testis are 40 times as
likely to develop testicular cancer as males without
undescended testis. Ten percent of testicular
cancer cases involve patients with undescended
testis. Prepubertal orchiopexy reduces this risk
• Prevention of testicular torsion
• Prevention of injury against pubic bone
• Psychological effects of an empty scrotum

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Surgery - done 6-12 months
• Open orchidopexy
• single-stage Fowler-Stephens open procedure –clip
vessels and opexy
• 2-stage Fowler-Stephens procedure theoretically allows
improved collateral blood supply; clip vessels and do
opexy later
• primary LAO
• laparoscopic two-stage Fowler-Stephens orchiopexy
(FSO)
• Microvascular orchiopexy allows adequate scrotal
position with preservation of the spermatic artery
blood flow. However, it requires special expertise. The
success rate is 84%.
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WHY AT SIX MONTHS
• the rarity of spontaneous descent after age 6
months and
• the possible improvements in fertility that early
intervention may confer.

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Standard Orchiopexy.
• The key steps in this procedure are
• complete mobilization of the testis and spermatic cord,
• repair of the patent processus vaginalis by high ligation
of the hernia sac,
• skeletonization of the spermatic cord without sacrificing
vascular integrity to achieve tension-free placement of
the testis within the dependent position of the scrotum,
and
• creation of a superficial pouch within the hemiscrotum
to receive the testis.

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AT SURGERY
• Nonpalpable testis
• An extended inguinal incision; an abdominal incision;
or, more commonly, diagnostic laparoscopy is used to
explore for a nonpalpable testis. At the time of
exploration, one the following 3 main features is likely
to be encountered:
1. Blind-ending spermatic vessels above the internal
inguinal ring (44%)
2. Intra-abdominal testis (36%)
3. Cord structures (vessels and vas deferens) that enter
the internal ring (20%)

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Complications of orchiodopexy
• Inadequate testis position occurs in up to 10% of patients
• Testicular atrophy due to devascularization during dissection
of the cord occurs in approximately 5% of patients..
• Accidental division of the vas deferens occurs in 1-2% of
patients. Immediate or post-pubertal microvascular repair
may be used.
• Epididymoorchitis is uncommon.
• Scrotal swelling may occur and is usually secondary to
edema.
• A later presentation of swelling may be secondary to
a hydrocele, which, if large, requires trans-scrotal repair.

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Complications of laparoscopy
• Pre-peritoneal emphysema
• Hypercarbia may occur with
pneumoperitoneum..
• The puncture of a viscus with the Veress
needle
• The puncture of a major abdominal vessel is a
life-threatening complication.
• Injury to the inferior epigastric vessels.
• Bladder puncture

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Acute scrotum
• is defined as an acute painful swelling of
the scrotum or its contents accompanied by local
signs and general symptoms

• Torsion of the spermatic cord is a true surgical

emergency of the highest order

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Anatomy – Nuts and Bolts

Posterior Anterior

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Causes of Pain and Swelling
• Pain
• Testicular torsion
• Torsion of appendix testis/epidydymis
• Epididymitis
• Trauma
• Orchitis
• Swelling
• Hydrocele
• Varicocele
• Spermatocele
• Tumor

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Torsion
• Inadequate fixation of testes to tunica vagnialis at
gubernaculum
• Torsion around spermatic cord
• Venous compression to edema to ischemia

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Torsion of testes or spermatic
cord
• 1.Intravaginal scrotal
• 2.Extravaginal scrotal-Neonatal
• 3.Undescented testis torsion

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 Acute scrotum dd
• Hydatid of Morgagni
• Idiopathic scrotal edema (dermatitis, insect bite)
• Scrotal abscess/cellulitis
• Tumor
• Obstructed or incarcerated hernia
• Furuncle
• Hemangioma

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Acute scrotum dd
• Abdominal trauma with hemiscrotum
• Splenogonadal fusion
• Adrenal neuroblastoma
• Meconium peritonitis
• Antenatal Meckel diverticulum perforation
• Hernial sac torsion
• Pyocele
• Ventriculoperitoneal shunt migration

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Torsion of testis-Epidemiology
• Accounts for 30% of all acute scrotal swelling
• Bimodal ages – neonatal (in utero) and pubertal
ages
• 65% occur in ages 12-18yo
• Incidence 1 in 4000 in males <25yo
• Increased incidence in puberty due to inc weight of
testes

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Predisposing factors
• Bell-clapper deformity
• Testicle lacks normal attachment at vaginalis
• Increased mobility
• Tranverse lie of testes
• Typically bilateral
• Prevalence 1/125
• UDT/horizontal lie/polyorchidism
• Peripubertal growth
• Physical activities
Clinical Presentation
• Abrupt onset of pain – usually testicular, can be
lower abdominal, inguinal
• Often < 12 hrs duration
• May follow exercise or minor trauma
• Week ends/ vacation
• May awaken from sleep
• Cremasteric contraction with nocturnal stimulation in REM
• Up to 8% report testicular pain in past
• Nausea vomiting
Examination
• Odematous, tender, swollen
• Elevated from shortened spermatic cord
• Horizontal lie common (PPV 80%)
• Reactive hydrocele may be present
• Cremasteric reflex absent in nearly all
(unreliable in <30mo old) (PPV 95%)
• Prehn’s sign elevation relieves pain in
epididymitis and not torsion is unreliable
• After several hours an acute hydrocele or
massive scrotal edema obliterates all landmarks

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Intermittent Torsion
• Intermittent pain/swelling with rapid resolution
(seconds to minutes)
• Long intervals between symptoms-nature always
warns
• PE: testes with horizontal lie, mobile testes,
bulkiness of spermatic cord (resolving edema)
• Often evaluation is normal – if suspicious need GU
followup

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Diagnosis – “Time is Testicle”
• Ideally -- prompt clinical diagnosis
• Imaging
• Color doppler – decreased intratesticular flow
• False + in large hydrocele, hematoma
• Sens 69-100% and Spec 77-100%
• Lower sensitivity in low flow pre-pubertal testes
• Nuclear Technetium-99 radioisotope scan
• Show testicular perfusion
• 30 min procedure time
• Sens and spec 97-100%

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 • Acute torsion L testis
• Dec blood flow on L

• Late torsion on R
• Inc blood flow around
but dec flow w/in testis

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Images - Torsion
• Decreased echogenicity
and size of right testicle

• Nuclear medicine scan

shows "rim sign“ =no flow
to testicle and swelling

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Management
• Detorsion within 6hr = 100% viability
• Within 12-24 hrs = 20% viability
• After 24 hrs = 0% viability

• Surgical detorsion and orchidopexy if viable

• Contralateral exploration and fixation if bell-clapper
deformity
• Orchiedctomy if non-viable testicle

• Never delay surgery on assumption of nonviability

as prolonged symptoms can represent periods of
intermittent torsion

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Golden rule
• a negative finding on surgical exploration is indeed
a better outcome than a necrotic testis due to a
missed diagnosis.

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Torsion of testis

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Manual Detorsion
• If presents before swelling
• Appropriate sedation
• In 2/3rds of cases testes
torses medially, 1/3rd lateral
• Success if pain relief, testes
lowers in scrotum
• Still need surgical fixation

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Torsion: Special Considerations
• Adolescents may be embarrassed and not seek care
until late in course

• Torsion 10x more likely in undescended testicle

• Suspicious if empty scrotum, inguinal pain/swelling

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Perinatal Torsion
• 70% prenatal, 30% post-natal
• Post-natal typically 7-10 days after birth
• Unrelated to gestation age, birth weight
• Post-natal presents in typical fashion
• Doppler U/S and radionucleotide scans less accurate
with low blood flow in neonates
• Surgical intervention if post-natal
• Prenatal torsion presents with painless testicular
swelling, rare testicular viability
• Rare intervention in prenatal torsion

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Perinatal Torsion

• Prenatal (in utero) torsion is typified by the finding

at delivery of a hard, nontender testis fixed to the
overlying scrotal skin
• The skin is commonly discolored by the underlying
hemorrhagic necrosis
• Classic teaching has held that testes found to be
hard, nontender, and fixed to the skin at birth do
not merit surgical exploration
• However, controversy has arisen regarding the need
for prompt exploration of the contralateral testis
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Torsion of Appendix Testis
• Peak age 3-13 yo (prepubertal)
• Sudden onset, pain less severe
• Classically, pain more often in abd or groin
• Non-tender testicle
• Tender mass at superior or inferior pole
• May be gangrenous, “blue-dot” (21% of cases)
• Normal cremasteric reflex, may have hydrocele
• Inc or normal flow by doppler U/S

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Torsion of Appendix Testis
• Appendix testis
• Small vestigial structure,
remnant of Mullerium duct
• Pedunculated, 0.3cm long
• Other appendix structures

• Prepubertal estrogen may

enlarge appendix and cause
torsion

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 Torsion of Appendix Testis---Blue dot of gangrenous.
appendix testis

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Torted appx

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Torted appx testis

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Torsion of Appendix Testis
• Management supportive
• analgesics, scrotal support to relieve swelling
• Surgery for persistent pain
• no need for contralateral exploration

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Epididymitis
• Inflammation of epididymis
• Subacute onset pain, swelling localized to
epididymis, duration of days
• With time swelling and pain less localized
• Testis has normal vertical lie
• Systemic signs of infection
• inc WBC and CRP, fever + in 95%
• Cremasteric reflex preserved
• Urinary complaints: discharge/dysuria PPV 80%

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Epididymitis
• Scrotum has overlying erythema, edema in 60%
• Normal vertical
lie

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Epididymitis

• Young boys, adolescents often post-infectious

(adenovirus) or anatomic
• Reflux of sterile urine through vas into epididymis
• 50-75% of prepubertal boys have anatomic cause by
imaging

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Etiologies of Epididymitis

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Epididymitis Diagnosis
• Leukocytosis on urine in ~40% of patients
• PCR Chlamydia + in 50%, GC + in 20% of
sexually active
• 95% febrile at presentation
• Doppler and Nuclear imaging show increased
flow
• If hx consistent with STD, CDC recommends:
• Cx of urethral discharge, PCR for C and G
• Urine culture and UA
• Syphilis and HIV testing

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Epididymitis Treatment
• Pre-pubertal boys
• Treat for co-existing UTI if present
• Symptomatic tx with NASIDs, rest
• Referral all to GU for studies to rule out VUR, post
urethral valves, duplications
• Negative culture has 100% NPV for anomaly

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Orchitis
• Inflammation/infection of testicle
• Swelling pain tenderness, erythema and shininess to
overlying skin

• Spread from epididymitis,

hematogenous, post-viral
• Viral: Mumps, coxsackie,
echovirus, parvovirus
• Bacterial: Brucellosis

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Mumps Orchitis
• Extremely rare if vaccinated
• 20-30% of pts with mumps, 70% unilateral, rare
before puberty
• Presents 4-6 days after mumps parotitis
• Impaired fertility in 15%, inc risk if bilateral

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Trauma
• Result of testicular compression against the pubis
bone, from direct blow, or straddle injuries
• Extent depends on location of rupture
• Tunica albuginea ruptures (inner layer of tuncia
vaginalis) allows intratesticular hematoma to rupture
into hematocele
• Rupture of tunica vaginalis allow blood to collect under
scrotal wall causing scrotal hematoma
• Doppler often sufficient to assess extent
• Surgery for uncertain dx, tunica albuginea rupture,
compromised doppler flow

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Testicular Hematoma
• Blood as a filling
defect in testis

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Other Causes of Pain
• Incarcerated inguinal hernia
• Henoch-Schonlein Purpura
• Vasculitis of testicular vessels
• Rarely presents with only scrotal pain
• Referred pain
• Retrocecal appendix, urolithiasis, lumbar/sacral nerve injury
• Non specific scrotal pain
• Minimal pain, nl exam – return immediately for inc symptoms

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Scrotal Swelling
• Hydrocele
• Varicocele
• Spermatocele
• Testicular Cancer

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Hydrocele
• Fluid accumulation
in potential space of
tunica vaginalis
• May be primary from
patent PV or secondary
to torsion/epididymitis

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Hydrocele
• Transilluminating
anterior cystic
mass

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Hydrocele
• Mass increases in size during day or with crying and
decreases at night if communicating
• If non-communicating and <1 yo follow
• If communicating (enlarging), scrotum tense (may
impair blood flow) requires repair
• Unlikely to close spontaneously and predisposes to
hernia

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Varicocele
• Collection dilated veins in
pampiniform plexus
surrounding spermatic cord
• More common on left side
• R vein direct to IVC
• L vein acute angle to renal vein
• ~20% of all adolescent males

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Varicocele
• Often asymptomatic or c/o dull ache/fullness upon
standing
• Spermatic cord has ‘bag of worms’ appearance that
increased with standing/valsalva
• If prepubertal, rapidly enlarging, or persists in
supine position rule out IVC obstruction
• Most management conservatively
• Surgery if affected testis < unaffected testis volume

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Spermatocele
• Painless sperm containing
cyst of testis, epipdidymis
• Distinct mass from testis
on exam
• Transilluminates
• Do not affect fertility
• Surgery for pain relief only

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Testicular Cancer
• Most common solid tumor in 15-30 yo males
• 20% of all cancers in this group
• Painless mass
• Rapidly growing germ cell tumors may cause
hemorrhage and infarction
• Present as firm mass
• Typically do not transilluminate
• Diagnostic imaging with U/S initially

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Acute Idiopathic Scrotal Edema
• Scrotal skin red and tender
• underlying testis normal
• no hydrocele
• Erythema extends off
scrotum onto perineum
• Empiric tx, cause unknown
• Antihistamine, steroids
• Resolves w/in 48-72hrs

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 Conclusions
• Clinical history and careful exam are key factors
in formulating accurate differential
• Imaging and labs useful adjuncts in unclear cases
• U/S superior to nuclear imaging if time essential
• TIME IS TESTICLE
• Early surgical intervention and GU involvement
• Swelling without pain, usually less time sensitive
diagnostically

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Phimosis
• refers to the inability to retract the distal foreskin over
the glans penis.
• Physiologic phimosis results from adhesions between
the epithelial layers of the inner prepuce and glans.
These adhesions spontaneously dissolve with
intermittent foreskin retraction and erections, so that
as males grow, physiologic phimosis resolves with age

• Physiologic phimosis occurs naturally in newborn

males.
• Natural history-----------------
Pathologic phimosis
• Pathologic phimosis defines an inability to retract
the foreskin after it was previously retractable or
after puberty, usually secondary to distal scarring of
the foreskin

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Phimosis presentation
• physiologic phimosis- Non-retractile/ballooning
• Pathologic phimosis -painful erections, hematuria,
recurrent urinary tract infections, preputial pain, or
a weakened urinary stream.
• The foreskin is retracted behind the glans penis and
cannot be replaced to its normal position.
• The foreskin forms a tight, constricting ring around
the glans.
• Glans - edema erythema necrosis

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Physiological phimosis-flowering

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Phimosis-ISSUES
• Parents worry - paraphimosis/renal failure due to
back pressure

• We don’t know natural history

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Phimosis-RX
• 1. 0.1 -0.05% betamethasone dipropionate applied to
the preputial orifice twice a day for 4-6 weeks.
• 2.CIRCUMCISION
• 3.DORSAL SLIT

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Circumcision
• Circumcision is a common procedure in which the skin
covering the tip of the penis is surgically removed
• 1 in 6 males in the world ends up being circumcised
• Indications to circumcise prepubertal boys are rare
• Circumcision is the commonest surgical procedure in
males
• It originated over 15 000 years ago, being performed
for religious, ritualistic, and cultural reasons,
• 19th century that the procedure was “medicalised.”

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Male infant circumcision
• Indications
• Surgical
• Phimosis (90%)
• Paraphimosis
• Balanitis xerotica obliterans
• Recurrent balanitis
• (Hypospadias ?)
• Proposed health benefits – prevent HIV transmission
• Social
• Religious/cultural
• Absolute Indication
• Balanitis xerotica obliterans 1.5% boys
• Relative indications
• Recurrent balanoposthitis, 1% of boys
• Paraphimosis
• Recurrent UTI
• Not indicated
• Preputial “pearls” and redundant foreskin
• Balloning
• Long prepuce
• Itching
• Pulling
Benefits of male circumcision?
• Proposed medical benefits;
• Reduction in STI’s including HIV*, genital herpes, genital
warts, syphilis, chancroid
• Reduction in penile cancer
• Reduced UTI’s
• Reduced risk of bacterial vaginosis & Trichomoniasis in
women
• Reduced risk of cervical cancer in women (due to
reduction in HPV in men)
Male circumcision as a religious
rite
Only the Jewish and Islamic faiths
require this religious rite that
recalls Abraham’s willingness to
sacrifice his son to God. The
sacrifice of the male foreskin is a
blood covenant between the
people of these faiths and their
God. This rite is not a part of
Christian practice, although many
Christians choose circumcision for
cultural or health reasons.
Balanitis xerotica obliterans (BXO)
• Lichen sclerosus et atrophicus
• >10% association with future Ca. Penis
• White patch on glans and prepuce,
may affect meatus or navicular fossa;
koebner phenomenon (BXO recur on
split skin graft)
• Treatment
• Steroid cream 4-6/52 for mild scarring and
retractable foreskin
• Circumcision and reconstruction (don’t use
genital skin for reconstruction due to
recurrence)
• No response > biopsy to exclude other
causes (erythroplasia of Queyrat)
Circumcision techniques
• Gomco Clamp
• Mogen Clamp
• Plastibell Technique
• Surgical sleeve/guillotine

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Complications of circumcision
• Bleeding/Haematoma
• Pain
• Infection
• Swelling
• Removal of insufficient or excess tissue
• Decrease in sensation during intercourse
• Urethral stricture
• Psychosocial problems
• Accidental amputation of the head of the penis (very
rare)!
Paraphimosis

• Urological emergency
• Reduce asap
• Penile block GA
Sedation
• Manual
• Puncture Method
• Dorsal slit
• Emergency
circumcision

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Treatment
• Reduction
• Manual - with or without needle puncture/dorsal slit

• Definitive - circumcision

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Manual reduction

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Dorsal slit-for paraphimosis

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Trauma to the foreskin
• Isolated trauma to the foreskin is unusual and in
young boys should always raise the possibility of
non-accidental injury.
• Tears, zipper injuries, or crush injuries usually heal
leaving a scarred foreskin,

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Hypospadias
• Most common congenital anomaly of the penis
• Abnormal penile configuration in which the
urethral meatus is located on the ventral surface of
the penis, proximal to the end of the glans, and
anywhere from the ventral gland to the perineum.
• Penile chordee (ventral bending of the penile shaft)
is often associated with hypospadias, and may be
due to tethering or dysplasia of the ventral penile
shaft skin. A dorsal hood of incomplete prepuce
may also be present.
Hypospadias causes and
associated factors
• multifactorial mode of inheritance
• Hypospadias is more common in first degree male relatives.
Fathers of affected boys have an 8 percent incidence of
hypospadias; and male siblings, 14 percent.
• Undescended testes and inguinal hernia occur in about 9
percent of children with hypospadias. There is a significantly
increased incidence of intersexuality when both conditions
coexist, and a karyotype should be considered
• Any history of maternal ingestion of hormonal medication
during pregnancy should be noted as reduced stimulation
by testosterone leads to hypospadias
 Classification

• Classified based on the anatomical

location of the urethral meatus
• Glanular (meatus is located on the
glans)
• Coronal
• Distal shaft
• Midshaft
• Penoscrotal
• Scrotal
• Perineal
• Associated chordee should be
described in terms of severity (mild,
moderate, or severe)
Classification-contd.
• Anterior hypospadias (glanular and coronal types)
account for 50% of all hypospadias.
• Middle hypospadias (distal, midshaft, and proximal
penile types) account for 30% of hypospadias cases.
• Posterior hypospadias (penoscrotal, scrotal, and
perineal types) account for 20% of cases
Examination
• Foreskin completeness circumferentially.
• Thinned ventral foreskin (a "hooded" penis) is associated commonly
with hypospadias.
• Meatal position should be noted if abnormal
• Presence or absence of penile chordee
• The stretched penile length in the newborn is 3.5 cm normally (range
2.8 cm to 4.2 cm), and should be noted if abnormal
• The gonads should be palpated and any cryptorchidism
• Any scrotal abnormalities should also be noted, such as a bifid scrotum
(a deep cleft between the scrotal sacs) or penoscrotal transposition (the
penis lying in or beneath the scrotum)
• Any inguinal hernia should be noted
• Other congenital anomalies should also be noted (e.g., anorectal
anomalies), if present
Management principles
• No circumcision should be done in the newborn with
hypospadias or any other penile anomaly, as the
foreskin may be necessary to create a neourethra,
and/or provide penile shaft skin coverage.
• Urological consultation or referral should be obtained
during or shortly after the neonatal period.
• Gonads nonpalpable + proximal hypospadias
(penoscrotal or scrotal → high risk of intersex state;
emergent urologic consultation is indicated, as well as
observation for salt wasting congenital adrenal
hyperplasia conditions (the most common cause of
intersex states).
Corrective surgery/urethropalsty
• Goals of corrective surgery
• provide the child with a normally appearing circumcised penis with the
urethral meatus well placed at the tip of the glans.
• The child should be able to stand to void and have a straight penis
when erect.
• This will allow both normal voiding as well as reproductive functionality
of the penis after repair.
• The hypospadias repair is best performed when the patient is
between 6 and 18 months of age. At this age,
• babies are amnestic of the procedure
• post operative management while the patients are still in diapers is
easier
• allows the procedure to be performed as outpatient surgery
• anesthetic risk is lower after 6 months of age if a good pediatric
anesthesiologist is used
• Waiting for 18 months increases the amount of foreskin available for
the surgery
Surgical procedure
• There are over 200 named surgical procedures to correct
hypospadias
• General concepts in the approach to hypospadias repair
• Ventral penile chordee must be corrected first, as the urethral
meatus may move proximally as the penis is straightened.
• Next, the urethroplasty (urethral advancement) is performed to
allow the placement of the neourethra well into the glans (to the
glans tip). The neourethra is formed from either local skin flaps, or
from foreskin flaps (the reason circumcision is not performed).
• A glanuloplasty to create a normal appearing rounded glans penis
may also be performed, if necessary.
• Penile shaft skin coverage is then accomplished by bringing penile
shaft skin, or foreskin flaps ventrally.
• A short, small caliber silastic urethral catheter that drains
directly into the diaper may be used to direct the urine away
from the repair, which is removed 7 to 14 days later.
Surgical procedure – contd.
• Most hypospadias repairs can be done with a single
stage repair.
• Sometimes a 2-stage repair is necessary, especially
for very long urethral defects.
• The chordee is corrected first, and the prepuce spread
along the ventral shaft.
• Six months later, the neourethra is completed in a
second stage repair.
Complications
• Fistulae
• Strictures
• recurrent chordee
• Occur approximately 10 percent of the time
Labial Fusion

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Posterior Urethral Valves
• BOO is produced by a membrane within the posterior
urethra.
• Incidence - 1 in 5,000 to 8,000 males
• Etiology unknown -multifactorial gene mediated
embryopathy.
• Commonest cause of bladder outlet obstruction in
children. One of the most devastating anomalies to
occur in the urinary tract.
• A spectrum of severity, ranging from disease
incompatible with postnatal life to that which is minimal.
• Clinical challenge requiring active Management from
infancy into adulthood to prevent progressive
dysfunction and deterioration of both the upper and
lower urinary tracts.
Pathology
• Type I
• Most common (95%)
• Abnormally high insertion and fusion plicae colliculi
(primitive folds)
• Two folds extend anteroinferiorly from the caudal aspect of
verumontanum often fusing anteriorly at a lower level
• Type II
• No longer considered a valve
• Hypertrophic band of muscle running frim ureteric orifice
to verumontanum along posterolateral urethral wall
• Type III
• incomplete dissolution of the urogenital membrane
• Circular diaphragm with central or eccentric narrow
aperture in membranous urethra
• Dewan et al
• single diaphragm with a central defect
• different appearances due to either an antenatal rupture or
postnatal instrumentation.
• congenital obstructing posterior urethral membrane (COPUM)
Present at
• Fetus
• Neonate
• Infant
• Older child
Fetus

• The fetus -Currently more than

80%
• PUV occurs in the fetus at a
crucial time of organogenesis
causing serious consequences
• Ix – uss abdomen of a
pregnant lady
• Oligohydramnios
• Male fetus
• Bilateral upper tract dilatation
• Persistently distended full bladder
• Keyhole sign’
Neonate
• Symptoms usually related to BOO -
2/3 poor stream
• Effects of impaired renal function
(less often)
• Complications of oligohydramnios
(Potter sequence)
• Potter facies– wide-set eyes with
epicanthic folds; low set ears; broad,
flattened nose; retrognathia
• Limb abnormalities – club/rocker
bottom feet, upper limb deformities
• Pulmonary hypoplasia
Infant
• UTI
• Poor stream

Older child
• Renal failure, growth retardation, urinary infections
or voiding symptoms (typically prolonged voiding)
• Overflow Incontinence

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Postnatal-Ix
• Ultrasonography-
• Upper tract dilatation
• Perinephric urinoma (occasionally)
• Thickening of the bladder wall, with or without residual
urine
• Dilated Post Urethra

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MCUG-Gold Standard

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PUV MCUG-PUV ablation

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Initial management
• Initial management of all patients with PUV requires immediate bladder
drainage
• This should be performed even if the diagnosis has not been confirmed by
MCUG
• Neonates can be catheterized with a 3.5 or 5 Fr pediatric feeding tube
• Foley catheters have been used with success, but there have also been reports
that the balloon causes irritation and resultant bladder spasms
• After successful initial bladder drainage and when the patient's medical
condition has stabilized, the next step is to permanently destroy the valves PU
valves – CUTANEOUS VESICOTOMY
• If the infant is too small for safe instrumentation for valve ablation, a cutaneous
vesicostomy can be performed as a temporary measure. The vesicostomy
provides adequate drainage of the upper tracts in more than 90% of cases
• There has been concern that vesicostomy would cause permanent loss of
bladder volume, but this has not proved to be true, and vesicostomy does not
significantly affect bladder capacity

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PUV minor

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Treatment
FETUS-intrauterine decompression
NEONATE
1. Urethral or suprapubic bladder drainage
• 3.5-5F Feeding tube
• Foley catheter- balloon causes irritation and resulting in
bladder spasms and obstruction of ureters
• Proper placement—flush /drainage volume /scan or even
cystogram
2. Endoscopic valve ablation (cystoscopy)
• miniaturised endoscopes available for small premature
neonates
3. Supravesical diversion
• Bilateral end ureterostomies or nephrostomies

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 The Hooks-cold knife or
pure cutting Diathermy

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CYSTOSCOPY-

A delicate procedure
• Must be quick-Sect at 5 7 12
• Avoid damage to corpus spongiosum to
prevent strictures
• Give up if it is bloody
• The depth of the sulcus adjacent to the
verumontanum and the high bladder neck
emphasize the dilatation of the prostatic
urethra and hypertrophy of the bladder neck.
• Changes in Bladder

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PUV SECTION-0 degree scope
7.5-9F

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 PUV SECTION

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Follow-up protocol
• Routine at every visit
• Height and weight
• Blood pressure
• Urinary tract ultrasonography
• Urine dipstick
• Serum creatinine and electrolytes
• As indicated
• Isotope renography (DTPA ,MAG3 or DMSA)
• Flow rate
• Urodynamics
• Formal estimation of GFR

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Prognosis
• Approximately one-third of individuals have
impaired renal function in the long term

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 SOME LONGTERM ISSUES
• Impairment of Renal function
• Upper Tract Dilatation
• VBS
• Effects of Tubular Damage
• Impaired Sexual function
• Incontinence
• VUR

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Conclusion
Posterior urethral valves (PUVs) have a variable effect on the anatomy and
physiology of the bladder and the upper urinary tract.
Many variables play their role in the ultimate prognosis.
As the ability to prenatally diagnose PUV has developed over time, so has the
temptation of in utero intervention to relieve urinary obstruction secondary to
valves. However, the long-term benefits of in utero intervention are currently
unclear, and they must be weighed against the significant risks to the mother
and fetus.
Early endoscopic valve fulguration is the best initial modality of management in
the majority of children with PUVs Dysplasia of renal units, preexisting VUR
and its effect on renal parenchyma are irreversible and not amenable to the
surgical skill.
Up to 50% of valve patients have developed renal impairment later in life. In
general a large number of them have long term issues requiring a regular
follow up.

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Vesicoureteric reflux (VUR)
• Retrograde flow of urine from bladder into upper
urinary tract due to incompetent VU Junction
• Incidence General pediatric population 1 to 2% only
• In children with UTI 30 to 50%
• 30 to 60% of children with VUR have renal scarring
• Female: Male ratio= 5:1
Why is it important ?
• Recurrent UTI , Cystitis , Pyelonephritis .
• Hydroureteronephrosis.
• Renal scarring.
• hypertension.
• Renal failure.
Incidence of Reflux in Patients with
Urinary Tract Infections
• AGE (yr) INCIDENCE (%)
<1 70
4 25
12 15
Adults 5.2
Causes of VUR
• Primary Reflux:
• fundamental deficiency in the function of the UVJ
• bladder and ureter remain normal or non
contributory.
• reflux occurs despite an adequately low-pressure
urine storage profile in the bladder
• length-diameter ratio is almost always less than
that described by Paquin (i.e. 5:1)
• Secondary reflux
• normal function of the UVJ being overwhelmed
• bladder dysfunction: congenital, acquired, or
behavioural.
• considered secondary if absence was documented
at some point before its detection
Presentation
• Asymptomatic, discovered accidently
• LUTS, Lower UTI symptoms
• Fever , chills , loin and abdominal pain, nausea and
vomiting
• Loin pain associated with a full bladder or
immediately after micturition
• Recurrent UTI or Loin pain for years
• Antenatal: Hydronephrosis
• Renal Failure, Hypertension
Renal Scintigraphy/radioisotope
renography
• DMSA:
- detection of reflux-associated renal damage
- acute pyelonephritic changes
- follow-up of reflux
Management
• Principles
• Spontaneous resolution of reflux is very common
• High-grade reflux is less likely to resolve spontaneously.
• Extended use of prophylactic antibiotics & “Watchful
waiting”
• The success rate with surgical correction is very high.
• Sterile reflux is benign.
Medical management
• “Watchful waiting” while maintaining urinary sterility
through the judicious use of single daily low-dose
antimicrobial prophylaxis.
• Often antibiotics are given as oral suspensions once per
day and preferably at night.
• Night time dosing allows for high antibiotic
concentration in the bladder urine over the longest
period .
• Breakthrough febrile UTIs or pyelonephritis while on
antibiotic prophylaxis are generally considered an
indication for termination of watchful waiting and
correcting the reflux
Surgery
• Endoscopic sting procedure
• Cohen’s transtrigonal reimplantation
Follow up
• Discharged on uro-prophylaxis
• Monitoring of pt’s
- BP
- renal function
- urine analysis
• Follow up USG and urine c/s after 6-12 weeks.
• VCUG after 3 months
• Discontinuation of uro-prophylaxis on resolution of
reflux
• DMSA after 1 yr (not mandatory)
Pelviureteric Junction Obstruction
(PUJO)
• Commonest cause of paediatric hydronephrosis.
• Detected in antenatal ultrasonography

In adults, repair is recommended if obstruction is

demonstrated on DTPA MAG3 OR (IVP).

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HYDRONEPHROSIS
• 1 in 100 pregnancies
• 1 in 500 neonates- adults?

• PUJO 40-50% - common cause of paed HN

• 75% of PUJO RESOLVE
• B/L—10-40%

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DIFFICULTY-

• IS THERE A PUJO????
• CAN IT CAUSE DETERIORATION IN THE FUNCTION
???? 75% of PUJO RESOLVE

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Pathophysiology
• Primary
• Intrinsic - commonest, aperistaltic
segment
• Extrinsic - abnormal vessel/bands/kinks
• Secondary
• Due to severe VUR, VUJO

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EFFECTS OF OBS
• Early and complete obstruction
• rare
• total nonfunctioning- renal dysplasia
• Late and partial prenatal obstruction
• varying degrees of pyelocaliectasis
• functional renal impairment

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Pathophysiology - initial
pathologic response
• elevation in renal pelvic pressures.
• compensatory reduction in renal blood flow and
glomerular filtration rate (GFR)
• pelvic pressures back into the normal range.
• mode of injury -ischemic injury due to this chronic
compensatory reduction in renal blood flow --not
elevated pressures GRAPH Koff

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Clinical presentation
• prenatal screening sonography/Infants
asymptomatic

• older children
• episodic flank or abdominal pain (50%)
• a palpable flank mass (50%)
• recurrent UTIs (30%)
• nausea and/or vomiting.
• feeding difficulties
• failure to thrive secondary to nausea and/or vomiting, haematuria,
pain, or urosepsis.
• gross haematuria following mild abdominal trauma,

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CLINICAL PRESENTATION
• Hypertension
• incidentally on radiologic studies.
• ass ectopic or horseshoe kidneys; duplication of the
collecting system, contralateral renal dysplasia, multicystic
dysplastic kidney (MCDK), or renal agenesis
• Contralateral PUJO (10-40%)
• VUR Abnormalities in other organ systems-.

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A/N HN P/N Mx
• Antibiotic Prophylaxis-- Controversial- In cases of
moderate-to-severe dilatations because any UTI,
especially in the neonatal period.

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 A/N Hydronephrosis postnatal Mx
P/N US SCAN—48 hrs

U/L---B/L HN NORMAL

MCUG
REPEAT in 4 Months

Diuretic Renography
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Ultrasonography

• Screening tool & follow up

• Help in Differential Diagnosis–
MCDK/VUJO/PUV/TUMOURS

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 Ultrasonography

• When— ?48 hrs later

• How often? Higher grade every 3-4 months
• Diagnosis-AP diameter-progressive
>20mm
Cortical ThicknessUS SCAN-False Positives

• large extrarenal pelvis-- BAGGY

• peripelvic renal cyst
• nonobstructive hydronephrosis
• VUR

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PUJO US SCAN

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IVP- Swick in 1929
• Primary study in evaluating hydronephrosis in older
children / adults
• Contraindicated in neonates and in renal failure
• Ultrasonography and nuclear renography has
replaced this in children

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Ureterocoele

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IVP - Drooping Lilly

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PUJO - Retrograde pyelogram

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Nuclear medicine
• USS AND DIURETIC RENOGRAPHY has replaced IVU
in the assessment of PUJO in Kids

• GFR , Vascular resistance and plasma flow are low

in neonates and Renal maturity improves with
time ---Need to wait 1-3 m for DTPA in Neonates???

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Whitaker Test

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Pre-op pyeloplasty
Pre-op pyeloplasty
POST OP PYELOPLASTY

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182
Post-op pyeloplasty
B/L OBS 1 ST GRAPH-2M

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SURGERY
• to improve renal drainage
• to maintain or improve renal function

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 Observation
• Avoid risks associated with surgery and anaesthesia
BUT-
• A definite percentage of patients sustain
irreversible renal damage -could have been
prevented by early pyeloplasty.
• After a short learning period, pyeloplasty in
infants is not demanding and is associated with
little perioperative morbidity.

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Indications for surgery in PUJO
1. Symptomatic- pain-Dietls crisis,mass.uti etc
2.Progressive OR Persistent Obstruction
USS—APD increasing

3.DTPA Obstructed curve/ Images

Split Function <40%( if first DTPA?)
Drop >10%
<10% Nephrectomy (Infants???)
T1/2 -- >20 min
-- 15-20min
-- <15min

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PUJO-NGram -neonate

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SURGERY
The principles of surgical repair (Foley) :
• Formation of a funnel
• Dependent drainage
• Watertight anastomosis
• Tension-free anastomosis
Procedure of choice is an Anderson-Hynes
dismembered pyeloplasty via anterior
extraperitoneal approach

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SURGERY
• Laparoscopic Pyeloplasty

• Endoscopic Treatment alternatives include an

antegrade or retrograde endopyelotomy, which is
an endoscopic incision with laser, electrocautery, or
endoscopic scalpel

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pujo

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Anderson hynes pyeloplasty

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 INTERVENTION
• Surgical intevention is highly successful in PUJO.
but finding out when to do and when not to do is
the biggest problem in PAEDIATRIC PUJO

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U/SSCAN- vital MCDK--
• Shows multiple diffuse hypoechoic renal
cysts,Noncommunicating without significant
normal parenchyma

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Haematuria-Needs early referral
• Calculi
• UTI
• Trauma
• Tumours
• Obstruction
• Cystitis(RT Or CT)
• Glomerulonephritis

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THANK YOU

Ananda Lamahewage 2017 197