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
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