Posterior Urethral Valve
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Posterior Urethral Valve (PUV) and it's management.
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Posterior Urethral Valve
- 1. POSTERIOR URETHRAL VALVES PRESENTED BY: ANISH DHAKAL (ARYAN)
- 3. Pathogenesis Normal embryologic development of the male urethra between weeks 9 to 14 of gestation is disrupted Probable causes for PUV formation: Persistence of the urogenital membrane with abnormal canalization of the urethra Overgrowth of urethrovaginal folds Abnormal integration of the Wolffian duct into the posterior urethra
- 4. Introduction Congenital membranes that cause obstruction to antegrade urinary flow in baby boys In the posterior urethra-distal to verumontanum Flap valve Most common cause of severe obstructive uropathy in male children Affecting 1 in 5000-8000 boys
- 5. Introduction Approximately 30% patients experience end stage renal disease or chronic renal insufficiency Vesicoureteral reflux occurs in 50% of patients Renal changes range from mild hydronephrosis to severe renal dysplasia Oligohydramnios Pulmonary hypoplasia Urinary tract infection, urinary retention and uraemia
- 6. Classification Type 1 folds going inferiorly from verumontanum to membranous urethra (most common) Type 2 Valves radiating from verumontanum to bladder neck Type 3 Valves are concentric diaphragms within the prostatic urethra
- 7. Clinical Features Affected boys often discovered prenatally : Maternal Ultrasonography Bilateral Hydronephrosis, Distended bladder Oligohydraminous, if obstruction severe Male neonate: Palpably distended bladder, Urinary stream weak Infant: Failure to thrive because of renal failure,uremia or sepsis Children: Difficulty in achieving diurnal urinary continence or with UTI
- 8. Diagnosis Ultrasonography ◦Antenatal and Postnatal ◦Bilateral Hydronephrosis ◦Marked distension and hypertrophy of bladder ◦Oligohydraminous in severe cases
- 9. Diagnosis Voiding Cystourethrogram (VCUG) Best imaging techniques for diagnosis Dilatation and elongation of posterior urethra Vesicoureteral reflux
- 10. Keyhole Sign ultrasonographic sign seen in boys with posterior urethral valves. proximal urethra (which is dilated) and associated thick walled distended bladder
- 11. Management Prenatal bladder decompression By percutaneous vesicoamniotic shunt or open fetal surgery Significant renal and pulmonary morbidity exist
- 12. Management Healthy Neonate ◦ Small polyethylene feeding tube (No. 5 or No. 8 French) is inserted in the bladder and left for several days ◦ Serum creatinine level remains normal or near normal: Transurethral ablation of valve leaflets ◦ If urethra to small: Temporary vesicostomy, when child become older; valve ablation and vesicostomy closed
- 13. Management If serum creatinine level remains high or increase despite bladder drainage: ◦ Suspect secondary ureteral obstruction, irreversible renal damage or renal dysplasia ◦ Cutaneous vesicostomy or Cutaneous pyelostomy
- 14. Management In septic and uremic infant: ◦ Life saving measures: correction of electrolyte balance, Infection control ◦ Drainage of upper tract by percuatneous nephrostomy, haemodialysis ◦ After conditions become stable consider valve ablation
- 15. Prognosis Favorable prognostic factors: ◦ Normal prenatal USG between 18 and 24 wk of gestation ◦ Serum creatinine level: <0.8-1.0 mg/dL after bladder decompression ◦ Visulaization of corticomedullary junction on renal sonography
- 16. Unfavorable prognostic factors: ◦ Presence of oligohydraminous in utero ◦ Identification of hydronephrosis before 24 weeks of gestation ◦ Serum creatinine level: >1.0 mg/dL after bladder decompression ◦ Persistence of diurnal incontinence beyond 5 yr of age
- 17. VURD Syndrome Valve, Unilateral reflux, dysplasia “Popoff valve” Preserves integrity of 1 or both kidneys 15% of boys with posterior urethral valve have unilateral reflux into a nonfunctioning dysplastic kidney: VURD syndrome
- 18. Post Operative Care Anti-microbial prophylaxis: Preventing UTI Evaluated annually with: renal ultrasonogram, physical examination including assessment of somatic growth and blood pressure, urinalysis, electrolytes
- 19. Some boys with: polyuria with vomiting and/or diarrhea, urine output cannot be used to assess their hydration status. They can become dehydrated quickly Some of these patients have renal tubular acidosis, requiring oral bicarbonate therapy. When vesicoureteral reflux is present,:expectant treatment and prophylactic doses of antibacterial drugs If breakthrough UTI occurs, surgical correction should be undertaken.
- 20. Boys with urethral valve often do not achieve diurnal urinary continence as early as other boys Incontinence: Uninhibited bladder contraction, bladder atonia, bladder neck dyssynergia or polyuria Polyuria: Overnight catheter drainage Urinary incontinence: Improves with age particularly after puberty
- 21. Summary Congenital membranes that cause obstruction to the urinary tract in baby boys Antenatal ultrasound typically shows urinary tract dilatation Treatment is valve destruction accompanied by treatment of urinary infection and renal impairment
- 22. References 1. Norman S. Williams et al ,Bailey and Love’s Short Practice of Surgery, 26th edition 2. Kliegman et al, Nelson Textbook of Pediatrics, 20th Edition 3. Langman’s Medical Embryology, 12th Edition
Editor's Notes
- Prostatic urethra dilates Bbladder muscle undergoes hpertrophy
- A Foley (balloon) catheter should not be used, because the balloon can cause severe bladder spasm, which can produce severe ureteral obstruction. transurethral ablation of the valve leaflets, which is performed endoscopically under general anesthesia. If the urethra is too small for transurethral ablation, temporary vesicostomy is preferred, in which the dome of the bladder is exteriorized on the lower abdominal wall. When the child is older, the valves may be ablated and the vesicostomy closed.
- Cutaneous pyelostomy rarely affords better drainage when compared with cutaneous vesicostomy, and the latter also allows continued bladder growth and gradual improvement in bladder wall compliance.
- In the septic and uremic infant, lifesaving measures must include prompt correction of the electrolyte imbalance and control of the infection by appropriate antibiotics. Drainage of the upper tracts by percutaneous nephrostomy and hemodialysis may be necessary. After the patient’s condition becomes stable, evaluation and treatment may be undertaken. Posterior valves are diagnosed in some older boys because of a poor stream, diurnal incontinence, or a UTI; these boys generally are treated by primary valve ablation.
- The prognosis in the newborn is related to the child’s degree of pulmonary hypoplasia and potential for recovery of renal function. Severely affected infants often are stillborn. Of those who survive the neonatal period, approximately 30% eventually require kidney transplantation and 15% have renal insufficiency. In some series, kidney transplantation in children with posterior urethral valves has a lower success rate than does transplantation in children with normal bladders, presumably because of the adverse influence of altered bladder function on graft function and survival.
- High bladder pressure dissipated into the nonfunctioning kidney, allowing normal development of the contralateral kidney In several situations, a “popoff valve” can occur during urinary tract development, which preserves the integrity of 1 or both kidneys. For example, 15% of boys with posterior urethral valves have unilateral reflux into a nonfunctioning dysplastic kidney, termed the VURD syndrome (valves, unilateral reflux, dysplasia). In these boys, the high bladder pressure is dissipated into the nonfunctioning kidney, allowing normal development of the contralateral kidney. In newborn boys with urinary ascites, the urine generally leaks out from the obstructed collecting system through the renal fornices, allowing normal development of the kidneys.
- After valve ablation, antimicrobial prophylaxis is beneficial in preventing UTI, because hydronephrosis to some degree often persists for many years. These boys should be evaluated annually with a renal ultrasonogram, physical examination including assessment of somatic growth and blood pressure, urinalysis, and determination of serum levels of electrolytes. Many boys have significant polyuria resulting from a concentrating defect secondary to prolonged obstructive uropathy. If these children acquire a systemic illness with vomiting and/or diarrhea, urine output cannot be used to assess their hydration status. They can become dehydrated quickly, and there should be a low threshold for hospital admission for intravenous rehydration. Some of these patients have renal tubular acidosis, requiring oral bicarbonate therapy. If there is any significant degree of renal dysfunction, growth impairment, or hypertension, the child should be followed closely by a pediatric nephrologist. When vesicoureteral reflux is present, expectant treatment and prophylactic doses of antibacterial drugs are advisable. If breakthrough UTI occurs, surgical correction should be undertaken. After treatment, boys with urethral valves often do not achieve diurnal urinary continence as early as other boys. Incontinence can result from a combination of factors, including uninhibited bladder contractions, poor bladder compliance, bladder atonia, bladder neck dyssynergia, or polyuria. Often these boys require urodynamic evaluation with urodynamics or videourodynamics to plan therapy. Boys with noncompliance are at significant risk for ongoing renal damage, even in the absence of infection. Overnight catheter drainage has been shown to be beneficial in boys with polyuria and can help preserve renal function. Urinary incontinence usually improves with age, particularly after puberty. Meticulous attention to bladder compliance, emptying, and infection can improve results in the future.