Presentation
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]
Gastrointestinal
-
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]
Urogenital
-
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]
There is up to a 20% chance that kidney failure can develop over time. It is also common for boys to have problems with bladder storage and emptying such as leakage of urine or inability to completely empty the bladder. [urology.ucsf.edu]
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]
-
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.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]
Treatment
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]
Prognosis
This determines the final prognosis, which is worse the younger the child at presentation. [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]
A meta-analysis on the utility of vesicoamniotic drainage showed an improved overall perinatal survival versus the non-drainage group, especially in poor prognosis fetuses [8]. [degruyter.com]
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]
Discussion: CAUSES • The etiology is unknown • Genetics. • Developmental anomaly in the urethra • Abnormal embryology and persistent of a fold which remains and forms flaps or valves in the urethral passage which Normally forms in the 4th months of gestation [slideshare.net]
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]
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.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]
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]