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Posterior Urethral Valve


Presentation

  • OBJECTIVES: To analyze the effect of delayed presentation of posterior urethral valves in children.[ncbi.nlm.nih.gov]
  • Patients presenting with it may be mistaken for urinary tract infection (UTI) because of the presence of frequency and dysuria. To present a rare cause of urinary bladder stone in a child in the tropics.[ncbi.nlm.nih.gov]
  • Recent advancements in antenatal ultrasound and endoscopy have altered the presentation and management of PUV. Herein we describe the presentation, management and outcome of PUV patients in Eastern Ontario/Western Quebec over the last 3 decades.[ncbi.nlm.nih.gov]
  • Evidence is presented which suggests that these two conditions may coexist.[ncbi.nlm.nih.gov]
  • Less is known about presentation and outcome in older patients. We are describing a patient who presented with renal stones and was found incidentally on cystoscopy to have Type I urethral valves.[ncbi.nlm.nih.gov]
Aspiration
  • The decision to intervene prenatally is dependent on gestational age, amniotic volume, and renal function of fetal urine aspiration. Identification of the patients who may benefit from early intervention remains inconclusive.[ncbi.nlm.nih.gov]
  • Specific procedures for in utero intervention include infusions of amniotic fluid, serial bladder aspiration, and creating a connection between the amniotic sac and the fetal bladder, or vesicoamniotic shunt.There are three specific endoscopic treatments[en.wikipedia.org]
  • Sequential bladder aspiration every 48 to 72 hours should be performed because the initial urine sample may be stagnant and fresh urine more accurately reflects the function of the fetal kidneys. 11, 12 If fetal urine is hypotonic, and oligohydramnios[clinicalgate.com]
  • .  Vesicocentesis Fine-needle aspiration under ultrasound guidance 9/11/2013 38.[slideshare.net]
Failure to Thrive
  • Neonatal presentation: anuria palpable bladder rapidly developing renal failure First year of life presentation: failure to thrive vomiting poor stream, dribbling, incontinence often develop sepsis Diagnosis is made on micturating cystourethrography.[gpnotebook.co.uk]
  • Newborns may also be brought with few urinary symptoms and more non specific ones like abdominal distension, failure to thrive, signs of infection (sepsis) somewhere in the body, recurrent urinary infections with fever.[pediatriconcall.com]
  • If the obstruction is severe and remains unrecognized during the neonatal period, infants may present later in life with failure to thrive due to uremia or sepsis caused by infection in the obstructed urinary tract.[archintsurg.org]
  • The affected patients may also present with complications of the disease such as urinary tract infections, sepsis, impaired renal function, anemia, and failure to thrive. [1], [3], [4] Diagnosis of the condition may be suspected with an antenatal ultrasound[afrjpaedsurg.org]
Suggestibility
  • Evidence is presented which suggests that these two conditions may coexist.[ncbi.nlm.nih.gov]
  • However, the long-term follow-up of survivors suggests that many will require renal transplantation when they reach early adulthood.[ncbi.nlm.nih.gov]
  • A review of previous reports suggests that the clinical course of the affected fetuses is highly variable.[ncbi.nlm.nih.gov]
  • Features that suggest posterior urethral valves are bilateral hydronephrosis, a thickened bladder wall with thickened smooth muscle trabeculations, and bladder diverticula. Voiding cystourethrogram (VCUG) is more specific for the diagnosis.[en.wikipedia.org]
  • The urine electrolyte concentrations (sodium 115 mEq/L; chloride 93 mEq/L) and the osmolality (230 mOsm/L) were elevated, suggesting impaired renal function and a poor prognosis.[ncbi.nlm.nih.gov]
Kidney Failure
  • Since boys with urethral valves have an up to 25 percent chance of kidney failure over the course of their lifetime, our specialists in Boston Children's Department of Urology make maintaining optimal bladder function the primary goal of treatment.[childrenshospital.org]
  • Males with PUV have about a 20% chance of developing kidney failure over their lifetime.[choc.org]
  • Some children, however, maintain preserved kidney function into adulthood, whereas others develop kidney failure in childhood ( 8 ). Previous reports suggested that nadir creatinine predicts the risk of kidney failure in children with PUV.[cjasn.asnjournals.org]
  • Somehow his lungs formed enough for him to survive, but he was born with kidney failure. After over 4 years on dialysis and many surgeries, I was able to donate a kidney to him when he was 5. He is now almost 20 years old and doing great.[puvs.org]
Incomplete Bladder Emptying
  • Renal deterioration may also occur due to hyperfiltration injury that causes glomerulosclerosis, chronic pyelonephritis associated with vesicoureteral reflux, urinary stasis, or incomplete bladder emptying, all of which are common in boys with a posterior[emedicine.medscape.com]
  • The term ‘valve bladder syndrome’ was introduced as a concept in the 1980s to encompass the abnormal voiding patterns and symptoms of voiding dysfunction, the persistent thick walled bladder, incomplete bladder emptying and associated upper tract dilatation[pediatricurologybook.com]
Urethral Bleeding
  • Urinary extravasations occurred in 3 cases, significant hematuria from urethral bleeding occurred in 2, and obstructive anuria developed in 1 patient. Most of cases were treated conservatively.[ncbi.nlm.nih.gov]
  • Complications such as urinary retention, urinary extravasation, urethral bleeding and urethral stricture have been reported to be associated with the procedure. [6] However, these were not recorded in our patients within the period of follow-up.[afrjpaedsurg.org]

Workup

Bladder Trabeculation
  • During cystoscopy mild bladder trabeculation and resected bladder neck were seen. While the cystoscope was taken off, PUV were obtained.[ncbi.nlm.nih.gov]
  • Bladder trabeculation and diverticula occur with any type of bladder outlet obstruction (functional or anatomic). Vesicoureteral reflux and hydronephrosis are often seen.[advancedradteaching.com]
  • trabeculation / diverticula Treatment and prognosis Antenatal treatment is possible, consisting of vesicoamniotic shunting (allowing urine to exit the bladder via the shunt, bypassing the obstructed urethra).[radiopaedia.org]
  • They are multiple and small with bladder trabeculation. While congenital diverticulae are not associated with bladder outlet obstruction[2].[jpss.eu]

Treatment

  • OBJECTIVES: The present study aims to evaluate the effect of desmopressin treatment on urine output, density and glomerular filtration rate (GFR) in patients with posterior urethral valve (PUV) and the factors affecting the response to this treatment.[ncbi.nlm.nih.gov]
  • HUN resolved in 15 RUs and improved to a lower grade in 11 in 26 of the 27 RUs (96.3%) in the 17 patients compliant with treatment. The 3 boys (5 RUs) who were noncompliant with treatment had neither decreased HUN nor improved urodynamic parameters.[ncbi.nlm.nih.gov]
  • If suspected antenatally, a consultation with a paediatric surgeon/ paediatric urologist maybe indicated to evaluate the risk and consider treatment options. Treatment is by endoscopic valve ablation.[en.wikipedia.org]
  • Abstract The treatment of patients with posterior urethral valves has improved dramatically in recent times.[ncbi.nlm.nih.gov]
  • Kaplan-Meier analysis of outcomes of the different treatments indicated no statistical difference in patient age at end stage renal disease development.[ncbi.nlm.nih.gov]

Prognosis

  • This determines the final prognosis, which is worse the younger the child at presentation.[ncbi.nlm.nih.gov]
  • The urine electrolyte concentrations (sodium 115 mEq/L; chloride 93 mEq/L) and the osmolality (230 mOsm/L) were elevated, suggesting impaired renal function and a poor prognosis.[ncbi.nlm.nih.gov]
  • Posterior urethral valve is found exclusively in males with an incidence of approximately 1 in 5000. [1] In 1919, Young et al. [4] first described PUV, and until the 1960s, it was reported to have an extremely poor prognosis.[archintsurg.org]
  • The overall prognosis is most affected by the degree and duration of obstruction. Severe cases with obstructive cystic renal dysplasia , oligohydramnios and pulmonary hypoplasia are often incompatible with life 2 .[radiopaedia.org]

Etiology

  • We have attempted to identify clearly the incidence, etiologic factors, preventive measures, and surgical treatment of complications arising in these patients.[ncbi.nlm.nih.gov]
  • EMBRYOLOGY OF POSTERIOR URETHRAL VALVES Three discrete embryologic defects are postulated in the etiology (1). The most common type (Type I in the Young classification).[fetalultrasound.com]
  • Unfortunately, there are a number of etiologies for this sonographic appearance, including urethral atresia, prune-belly syndrome, as well as PUV.[clinicalgate.com]
  • […] congenital posterior urethral segment occlusion is caused by hypertrophy of the normal prostatic urethral valve because of incomplete differentiation of the terminal part of mesonephric ducts or of persistence of the urogenital membrane, however, the etiology[degruyter.com]
  • Etiology A PUV is a congenital obstruction caused by a malformation of the posterior urethra. The significance of this obstruction depends on the secondary effects on the bladder, ureters, and kidneys.[emedicine.com]

Epidemiology

  • Population-based normative data were provided by Perinatal and Reproductive Epidemiology Research Unit.[nature.com]
  • Epidemiology In the United States, PUV is the most common cause of lower urinary tract obstruction in male neonates; the reported incidence ranges from 1 per 8000 to 1 per 25,000 live births.[emedicine.com]
  • Original Articles Epidemiology and Outcomes CJASN March 2014, 9 (3) 499-505; DOI: This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article.[cjasn.asnjournals.org]
Sex distribution
Age distribution

Pathophysiology

  • One involved the pathophysiology of posterior urethral valves (PUV), which came up in a clinical case.[renalfellow.blogspot.com]
  • Pathophysiology Posterior urethral valves can appear at the earliest stage of urinary tract development; therefore, the entire urinary tract develops in an abnormal environment of high intraluminal pressure due to the mechanical obstruction.[emedicine.medscape.com]
  • Pathophysiology During the early stages of embryogenesis, the most caudal end of the wolffian duct is absorbed into the primitive cloaca at the site of the future verumontanum in the posterior urethra.[emedicine.com]

Prevention

  • We have attempted to identify clearly the incidence, etiologic factors, preventive measures, and surgical treatment of complications arising in these patients.[ncbi.nlm.nih.gov]
  • Valve ablation was performed to prevent the recurrence of epididymo-orchitis. Improvement of urinary force was achieved and the patient has been free of recurrence during a 2-year follow up.[ncbi.nlm.nih.gov]
  • The ultimate goal of management should be to maximize renal function, maintain normal bladder function, minimize morbidity and prevent iatrogenic problems.[ncbi.nlm.nih.gov]
  • Oligohydramnios prevents normal lung development in utero. Pathologically, this process results in reduced branching of the bronchial tree and reduced numbers and size of alveoli.[clinicalgate.com]
  • This extra tissue prevents the urethra from properly carrying urine from the bladder to the tip of the penis and out of the body.[childrenshospital.org]

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