Cutaneous pyelostomy◦ Renal pelvis to skin◦ Uncommon

End ureterostomy◦ Stoma from distal

ureter

Cutaneous Urinary Diversion

Loop ureterostomy◦ Double barrel◦ Proximal and distal

ureter

Intestinal diversion◦ Bowel between skin

and ureters

Cutaneous Urinary Diversion

Cutaneous Vesicostomy◦ Most common◦ Bladder to skin

Appendicovesicostomy◦ Continent◦ Intermittent cath

Cutaneous Urinary Diversion

Nephrostomy ◦ Catheter from upper urinary tract to skin

Cutaneous Urinary Diversion

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. MyelomeningoceleB. Cloacal extrophyC. Hindgut extrophyD. Prune belly syndromeE. Classic bladder extrophy

Question 4

Cloacal Extrophy◦ 1/200,000◦ Rupture of cloacal

membrane Before urorectal septum

completes descent to separate hindgut from bladder

◦ Prognosis Long-term survival 50%

Extrophic Anomalies

Cloacal Extrophy◦ Exam

Bladder 2 widely separate halves

Bowel mucosa in the middle Ileocecal segment

Imperforate anus Hypoplastic genitalia

◦ Associated findings Omphalocele Myelomeningocele Hydrocephalus

Extrophic Anomalies

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

Extrophic Anomalies

Classic Extrophy◦ Exam

Epispadias Bifid or rudimentary penis Normal or bifid scrotum Inguinal hernias Widespread pubic symphysis Hemiclitoris Duplicate vagina

Extrophic Anomalies

Classic Extrophy

◦ Treatment Keep bladder surface moist Examine upper tract Neonatal closure

Pelvic osteotomy

Extrophic Anomalies

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

Extrophic Anomalies

Epispadias◦ Girls

Rare Incontinence Wide urethra Bifid clitoris

◦ Treatment Genitoplasty Staged surgical correction Renal U/S and VCUG

Extrophic Anomalies

Acute◦ Usually voluntary◦ Associated with

Severe acute cystitis Urethritis Meatitis Vaginitis

◦ Other causes Boys

Urethral stricture Meatal stenosis with meatitis

Girls Ureterocele

Urinary Retention

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

Urinary Retention

Neurovesical Dysfunction

Congenital Acquired

Meningocele Myelomeningocele Intradural lipoma Diastematomyelia Sacral agenesis

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

Neurovesical Dysfunction

AKA Hinman-Allen syndrome Dysfunctional voiding

◦ Features Day and night incontinence Fecal soiling UTI Behavioral problems Detrusor/sphincter discoordination

◦ Consequences Incontinence Renal failure

Non-Neurogenic Vesical Dysfunction

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

Non-Neurogenic Vesical Dysfunction

The parents of a newborn male with the pictured physical exam finding inquire about circumcision. What do you tell

them?

A. With their consent, you will proceed with circumcision

B. You will call the urologist and have him do the circumcision

C. Since they are medicaid, you will not perform the circumcision since it is no longer covered

D. Circumcision should be delayed in case the skin is needed for reconstruction

E. The patient needs immediate repair

Question 5

Hypospadias◦ 1/250 males◦ Location of meatus

Glanular Coronal Subcoronal Distal shaft Midshaft Proximal shaft Penoscrotal Scrotal Perineal

◦ ? Chordee

Anomalies of the Male Genitalia

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

Anomalies of the Male Genitalia

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

Anomalies of the Male Genitalia

Penile Torsion◦ Congenital or acquired◦ Most commonly mild◦ Median raphe spirals

around shaft Counterclockwise

◦ May be seen after circumcision or hypospadias repair

Anomalies of the Male Genitalia

Webbed penis

◦ Cosmetic

◦ Transposition of scrotal skin onto the ventral penile shaft at the penoscrotal junction

Anomalies of the Male Genitalia

Buried penis◦ Most common after

circumcision◦ Result of thick

suprapubic fat pad◦ Resolves with normal

development◦ Severe cases may

require surgery

Anomalies of the Male Genitalia

Postcircumcision Concerns 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

Postcircumcision Concerns 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

Question 6 Which of the following is the most accurate

definition of micropenis in an infant?◦ A Penile stretch length <2cm◦ B Penile stretch length <1cm◦ C Penile length <2cm (not-stretched)◦ D Penile length <0.5cm (not-stretched)

Microphallus (Micropenis) 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

Diphallus Usually associated with severe deformities

of lower urinary tract and genitalia Complete evaluation of upper and lower

tract