
Cutaneous
pyelostomy
◦ Renal pelvis to skin
◦ Uncommon

End ureterostomy
◦ Stoma from distal
ureter

Loop ureterostomy
◦ Double barrel
◦ Proximal and distal
ureter

Intestinal diversion
◦ Bowel between skin
and ureters

Cutaneous
Vesicostomy
◦ Most common
◦ Bladder to skin

Appendicovesicostomy
◦ Continent
◦ Intermittent cath

Nephrostomy
◦ Catheter from upper urinary tract to skin
You are called to the delivery
room to evaluate a female baby
with this defect which is
located on the lower anterior
abdominal wall. What is the
most likely diagnosis?
A.
B.
C.
D.
E.
Myelomeningocele
Cloacal extrophy
Hindgut extrophy
Prune belly syndrome
Classic bladder extrophy

Cloacal Extrophy
◦ 1/200,000
◦ Rupture of cloacal
membrane
 Before urorectal septum
completes descent to
separate hindgut from
bladder
◦ Prognosis
 Long-term survival 50%

Cloacal Extrophy
◦ Exam
 Bladder
 2 widely separate halves
 Bowel mucosa in the
middle
 Ileocecal segment
 Imperforate anus
 Hypoplastic genitalia
◦ Associated findings
 Omphalocele
 Myelomeningocele
 Hydrocephalus

Classic Extrophy
◦ 1/40,000
◦ More common in boys
◦ Premature rupture of the
cloacal membrane
◦ Exam
 Red mucosal surface
 Infraumbilical abdominal
wall
 Bladder as an open book
 Inferior aspect ureteral
orifices

Classic Extrophy
◦ Exam







Epispadias
Bifid or rudimentary penis
Normal or bifid scrotum
Inguinal hernias
Widespread pubic symphysis
Hemiclitoris
Duplicate vagina

Classic Extrophy
◦ Treatment
 Keep bladder surface moist
 Examine upper tract
 Neonatal closure
 Pelvic osteotomy

Epispadias
◦ 55% boys
 Penopubic
 Widened pubic symphysis
 Broad spade-like penis
 Urethra opened fully on dorsal
surface to bladder neck
 Dorsally tethered penis
 Incontinent
◦ Other boys
 Penile or balanitic epispadias
 Normal continence

Epispadias
◦ Girls




Rare
Incontinence
Wide urethra
Bifid clitoris
◦ Treatment
 Genitoplasty
 Staged surgical correction
 Renal U/S and VCUG

Acute
◦ Usually voluntary
◦ Associated with




Severe acute cystitis
Urethritis
Meatitis
Vaginitis
◦ Other causes
 Boys
 Urethral stricture
 Meatal stenosis with meatitis
 Girls
 Ureterocele

Other causes
◦ Both
 Bladder or urethral calculi
 Masses - compression
 Pelvic masses
 Rhabdomyosarcoma
 Uterine or ovarian masses
 Hydro or hydrometrocolpos
 Sacrococcygeal tumors
 Constipation
 Involvement of nerve roots
 Spinal cord injury, tumor or
transverse myelitis
Congenital





Meningocele
Myelomeningocele
Intradural lipoma
Diastematomyelia
Sacral agenesis
Acquired



Trauma
Transverse myelitis
Spinal cord tumor

Goals of management
◦ Preserve renal function
◦ Prevent renal damage from infection
◦ Provide social continence

Evaluation
◦ Radiographic or urodynamics
◦ Several times in first year
 Yearly thereafter

Warning signs
◦ Infection
◦ Fever
◦ Change in continence


AKA Hinman-Allen syndrome
Dysfunctional voiding
◦ Features





Day and night incontinence
Fecal soiling
UTI
Behavioral problems
Detrusor/sphincter discoordination
◦ Consequences
 Incontinence
 Renal failure

Dysfunctional voiding
◦ Diagnosis of exclusion
◦ Must rule out
 Tethered spinal cord
 Infravesical obstruction
◦ Treatment
 Bladder retraining
 Timed regimen
 Biofeedback
 Intermittent cath
 Reverse complications
 Temporary diversion
 Renal function
 Behavioral or psychological therapy
The parents of a newborn male with the
pictured physical exam finding inquire
about circumcision. What do you tell
them?
A.
B.
C.
D.
E.
With their consent, you will proceed with
circumcision
You will call the urologist and have him
do the circumcision
Since they are medicaid, you will not
perform the circumcision since it is no
longer covered
Circumcision should be delayed in case
the skin is needed for reconstruction
The patient needs immediate repair

Hypospadias
◦ 1/250 males
◦ Location of meatus









Glanular
Coronal
Subcoronal
Distal shaft
Midshaft
Proximal shaft
Penoscrotal
Scrotal
Perineal
◦ ? Chordee

Hypospadias
◦ Further work up
 Cryptorchidism also?
 Karyotype
 VCUG?
 Only with severe lesions or UTI
 Renal U/S?
 Proximal lesions
◦ Treatment
 Delay circumcision
 Repair at 6 months

Chordee
◦ Ventral penile curvature
◦ Uncommon without
hypospadias
◦ 3 possibilities
 Skin tethering
 Abnormal development of
urethra and ventral penile
structures
 Congenitally short urethra
◦ Treatment
 Depends on cause
 Delay circumcision

Penile Torsion
◦ Congenital or acquired
◦ Most commonly mild
◦ Median raphe spirals
around shaft
 Counterclockwise
◦ May be seen after
circumcision or
hypospadias repair

Webbed penis
◦ Cosmetic
◦ Transposition of
scrotal skin onto the
ventral penile shaft at
the penoscrotal
junction

Buried penis
◦ Most common after
circumcision
◦ Result of thick
suprapubic fat pad
◦ Resolves with normal
development
◦ Severe cases may
require surgery

Meatal Stenosis
◦ Dysuria, strangury, deflected stream
◦ Need to observe stream
 Examination insufficient
◦ Meatotomy under local anesthesia is curative

Meatal Bridge
◦ Results from meatal stenosis in which ventral
aspect recanalizes

Preputial adhesions and skin
bridges
◦ Fibrinous adhesions
 Incomplete retraction of prepuce
in normal development
 Post-Circumcision
◦ Complications
 Disfiguring
 Recurrent inflammation and
infection
 Trapped smegma
◦ Surgical correction is
complicated

Which of the following is the most accurate
definition of micropenis in an infant?
◦
◦
◦
◦
A Penile
B Penile
C Penile
D Penile
stretch length <2cm
stretch length <1cm
length <2cm (not-stretched)
length <0.5cm (not-stretched)


Stretch length <2cm (2 S.D. below mean)
2 Causes
◦ Hypogonadotropic hypogonadism
 (Failure of hypothalamus to produce GnRH)
◦ Primary testicular failure
 Deficient testosterone production

Requires extensive workup
◦ Include karyotype, ?MRI of brain?

3 month trial of testosterone


Usually associated with severe deformities of
lower urinary tract and genitalia
Complete evaluation of upper and lower tract