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
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
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