CONGENITAL ANOMALIESLOWER URINARY TRACT

Ahmad HannoProfessor and Head of Pediatric Urology

General practitionerSurgeon

Urologist

Pediatric Urology

I. Urachal anomaliesII. HypospadiasIII. Posterior urethral valvesIV. Exstrophy – epispadias

complex

URACHAL ANOMALIES

URACHAL ANOMALIES

HYPOSPADIAS

Urethral meatus is ventral in location Meatus located proximal to the tip of

glans Prepuce is hooded Ventral curvature on erection

HYPOSPADIAS REPAIR

Straightening of the penis “Orthoplasty”Extending the urethra to tip of the glans

“Urethroplasty”Fashioning the new meatus “Meatoplasty”Refashioning the glans around neo-urethra

“Glanuloplasty”Covering the neo-urethra with skin

POSTERIOR URETHRAL VALVES

POSTERIOR URETHRAL VALVES

Remnant obstructing tissues at the distal end of posterior urethra in males

Prenatal infravesical obstructionHydroureteronephrosis, distended bladder

and posterior urethraVesicoureteric refluxUrosepsisCommon cause of renal insufficiency

PRESENTATIONSPrenatal UltrasoundPostnatal– Obstructed urinary stream– Renal insufficiency– Failure to thrive– Urosepsis– Hydronephrosis– Full bladder

DIAGNOSIS US

Key-hole sign

Thick-walled distended bladder

DIAGNOSIS VCUG

MANAGEMENTStabilize the critically ill babyUrethral “feeding tube”Transurethral “fulguration” of the valvesVesicostomy if renal function is impairedDelayed managenent– Clean intermittent catheterization– Attention to reflux– Bladder augmentation– Renal Tx

TRANSURETHRAL FULGURATION

Endoscopic view

TRANSURETHRAL FULGURATION

Fulguration Incision

EXSTROPHY – EPISPADIAS COMPLEXLack of development of the mesoderm of the

infra-umbilical region

Diastasis of the symphysis pubis

Bladder is opened to the outside

Epispadias in males

Bifid clitoris in the female

Bilateral inguinal hernias are common

http://www.emedicine.com/ped/images/1014760-1014971-673.jpg

ABNORMAL BLADDER PLATE IN THE OLDER CHILD

SURGICAL REPAIRClosure of bladder plateComplete penile disassemblyRepair of epispadiasVentral re-location of the urethraRepair of the clitoris in femalesClosure of the abdominal defect• Simple closure in the first 72 hours• Bilateral iliac osteotomy thereafter

Attention to continence at age 3 – 5 years