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Urethral Valve Formation


Presentation

  • The girl presented with a female urethra with a vaginal orificium as well as a male urethra culminating in a microphallus. Additionally, a urethral valve formation just cranial to the bifurcation of the male and female urethra was also present.[ncbi.nlm.nih.gov]
  • With this in mind, topics are presented in a clear and easily digestible format, with the most important learning points in each area clearly identified.[books.google.com]
  • Originals 49 Downloads 18 Citations Abstract A retrospective analysis of radiologically determined individual renal function was performed in five boys who presented in the neonatal period with posterior urethral valves, vesicoureteral reflux and documented[link.springer.com]
  • Anterior urethral diverticulum: A rare presentation. Indian J Plast Surg 2016;49:265-7 How to cite this URL: Gopalkrishna A, Sharma SM, Ananda B. Anterior urethral diverticulum: A rare presentation.[ijps.org]
  • In general, boys with a greater degree of urinary tract obstruction present earlier. [5] Infants who are not diagnosed prenatally may present with infection, hydronephrosis, ascites, and/or thick and distended bladder.[archintsurg.org]
Recurrent Urinary Tract Infection
  • Due to concomitant diseases, a complicated clinical course and recurrent urinary tract infections, the final operative correction resulting in a female phenotype, was performed at the age of 2 (1/2) years as a single-stage procedure.[ncbi.nlm.nih.gov]
  • We are reporting a four years old boy who presented with recurrent urinary tract infections, difficulty in micturition and dribbling of urine.[pjms.com.pk]
  • Bilateral 3rd degree reflux and recurrent urinary tract infection were prominent sequelae of obstruction.[jpma.org.pk]
  • If not recognized early, many congenital kidney and urinary tract problems can cause recurrent urinary tract infections and scarring in the kidneys, further compromising kidney function.[nyulangone.org]
  • A provisional clinical diagnosis of recurrent urinary tract infection complicating posterior urethral valves was made.[archintsurg.org]
Falling
  • Observing the stream of urine while the child is micturiting will reveal a poor stream, falling close to the body, child straining while passing urine.[pediatriconcall.com]
  • When a scab has formed, try to avoid long baths as this may cause the scab to soften and fall off too early. The wound site may be closed by Dermabond (plastic glue). The Dermabond usually peels off after about a week.[gosh.nhs.uk]
  • After one year, however, recurrence rates approach 50 to 60 percent and by five years, recurrence falls in the range of 74 to 86 percent (depending on stricture length and degree of spongiofibrosis).[urology.wustl.edu]
  • With urethral catheter 22% of cases showed improvement of renal function (fall in serum creatinine level). Mean of follow-up period was 3.4 1.2 years (range: 1.5 months to 5 years).[ncbi.nlm.nih.gov]
  • The retainer bearing 26 is screwed to affixed at a distal end portion of the valve housing 23 to prevent the magnet rotor 32 from falling out.[google.ch]
Enuresis
  • 日向 泰樹, 中井 秀郎, 川合 志奈, 中村 繁 夜尿症研究 Enuresis : 日本夜尿症学会雑誌 19 49-54 May 2014 Pediatric daytime incontinence/non-monosymptomatic nocturnal enuresis requiring referral to pediatric urologists from pediatricians 渡邊 常樹, 藤永 周一郎, 池田 裕一, 日向 泰樹, 川合 志奈, 中村 繁, 中井 秀郎[researchmap.jp]
  • Most common cause of urinary tract obstruction in newborn males Respiratory distress secondary to pulmonary hypoplasia Abdominal distention due to bladder distention If late presentation: Difficulty voiding, poor urinary stream UTI urosepsis Diurnal enuresis[amboss.com]
  • Older children may complain of milder symptoms like frequency, dysuria, hematuria, enuresis, diminished urinary stream and post-void dribbling, as in our patient [4].[ispub.com]
  • Nocturnal enuresis, urinary frequency, and recurrent urinary tract infection [4] may also be present in mild long standing cases, as was noted in this index case.[archintsurg.org]
  • Daytime incontinence, infrequent voiding, poor urinary stream, chronic severe urinary frequency, and complicated enuresis may suggest bladder outlet obstruction (from urethral obstruction or posterior urethral valves) (9).[hawaii.edu]
Anemia
  • The possible etiological factors are inheritance, intrauterine infections, mechanical causes, placenta previa, preeclampsia, anemia and parasitic infestations and young maternal age 8, 10.[turkjpath.org]
Failure to Thrive
  • […] common cause of urinary tract obstruction in newborn males Respiratory distress secondary to pulmonary hypoplasia Abdominal distention due to bladder distention If late presentation: Difficulty voiding, poor urinary stream UTI urosepsis Diurnal enuresis Failure[amboss.com]
  • Loss of weight, unexplained fever, failure to thrive and bouts of diarrhoea are significant findings. Palpable urinary bladder and kidneys in these cases give a clue.[jpma.org.pk]
  • 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]
  • 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]
  • They may also present at an older age with febrile UTIs, incontinence, hematuria, failure to thrive, abdominal pain, or pelvic pain.[hawaii.edu]
Failure to Thrive
  • […] common cause of urinary tract obstruction in newborn males Respiratory distress secondary to pulmonary hypoplasia Abdominal distention due to bladder distention If late presentation: Difficulty voiding, poor urinary stream UTI urosepsis Diurnal enuresis Failure[amboss.com]
  • Loss of weight, unexplained fever, failure to thrive and bouts of diarrhoea are significant findings. Palpable urinary bladder and kidneys in these cases give a clue.[jpma.org.pk]
  • 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]
  • 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]
  • They may also present at an older age with febrile UTIs, incontinence, hematuria, failure to thrive, abdominal pain, or pelvic pain.[hawaii.edu]
Nausea
  • At 2 years of age, he develops left sided abdominal pain, nausea and vomiting, without fever or chills.[hawaii.edu]
  • Nausea and vomiting sufficient to cause dehydration*.[patient.info]
  • Presenting symptoms include hematuria, UTI, abdominal mass or pain, nausea, or flank pain which worsens with diuresis because of urine production that overwhelms the ability of the UPJ to accommodate an equal volume of drainage and therefore leads to[auanet.org]
Hyperreflexia
  • Children with overactive bladder (eg, detrusor hyperreflexia, detrusor instability) generate a high intravesical pressure, which can exacerbate pre-existing VUR or cause secondary VUR.[emedicine.medscape.com]
Renal Insufficiency
  • Approximately 24–45% of children born with PUV will exhibit renal insufficiency during childhood. PUV accounts for up to 17% of the cases of children with end-stage renal failure.[unboundmedicine.com]
  • PUVs are the cause of renal insufficiency in approximately 10-15% of children undergoing renal transplant, and approximately one third of patients born with PUV progress to ESRD in their lifetimes.[emedicine.medscape.com]
  • One patient had creatinine normalization after ablation, whereas the other two patients developed chronic renal insufficiency.[frontiersin.org]
  • insufficiency or renal failure in boys References: [4] [7] [8] [9] [10] Webbed penis[amboss.com]
  • There may be mild urethral dilatation without proximal dilatation of renal system or gross urethral dilatation with bilateral hydronephrosis, hydroureter and renal insufficiency in severe cases.[pjms.com.pk]
Dysuria
  • Classic symptoms of cystitis include urinary frequency, urgency, dysuria, hematuria, suprapubic pain, sensation of incomplete emptying, and often, even incontinence.[auanet.org]
  • Older children may complain of milder symptoms like frequency, dysuria, hematuria, enuresis, diminished urinary stream and post-void dribbling, as in our patient [4].[ispub.com]
  • In this case, obstructive voiding symptoms, dysuria, urinary retention and perineal pain can be present • Diagnosis is made by VCUG, retrograde urethrography or urethrocystoscopy. 75. Syringocele.[slideshare.net]
  • Case Report I.F. is a 9-year-old boy who presented with a chronic history of difficulty with micturition, dysuria, feeling of incomplete voiding, right loin pain, and recurrent fever, which was being managed in a peripheral clinic.[archintsurg.org]
  • Children may present with clinical pyelonephritis, fever, abdominal/flank pain, malaise, nausea, vomiting, cystitis with dysuria, frequency, urgency, and urge incontinence.[hawaii.edu]
Bladder Neck Obstruction
  • neck obstruction Q64.32 Congenital stricture of urethra Q64.33 Congenital stricture of urinary meatus Q64.39 Other atresia and stenosis of urethra and bladder neck Q64.4 Malformation of urachus Q64.5 Congenital absence of bladder and urethra Q64.6 Congenital[icd10data.com]
  • The pediatric implications of bladder neck obstruction. Pediatrics 1961; 28: 816–821. PubMed Google Scholar 7. Cass AS, Stephens FD. Posterior urethral valves: Diagnosis and management. J Urol 1974; 112: 519–525. PubMed Google Scholar 8. Odita JC.[link.springer.com]
  • neck obstruction Inclusion term(s): Congenital obstruction of vesicourethral orifice Q64.32 Congenital stricture of urethra Q64.33 Congenital stricture of urinary meatus Q64.39 Other atresia and stenosis of urethra and bladder neck Inclusion term(s):[icd10coded.com]
  • Bladder neck obstruction. Congenital urethral valves. Pinhole meatus. Neurogenic bladder. Functional failure due to denervation (eg, following spinal trauma, multiple sclerosis).[patient.info]
  • Androulakakis PA, Karamanolakis DK, Tsahouridis G, Stefanidis AA, Palaeodimos I (2005) Myogenic bladder decompensation in boys with a history of posterior urethral valves is caused by secondary bladder neck obstruction? BJU Int 96(1):140–143.[journals.plos.org]
Renal Impairment
  • When to refer patients with BPH to secondary care [ 11 ] Suspected complications (haematuria, renal impairment, hydronephrosis, recurrent UTIs). Suspected prostate cancer. Large residual volumes of urine ( 200 ml). An unclear diagnosis.[patient.info]
  • Renal impairment in children with posterior urethral valves. Pediatr Nephrol 2013;28:927-31. 2. Yu JS, Kim KW, Lee HJ, Lee YJ, Yoon CS, Kim MJ. Urachal remnant diseases: Spectrum of CT and US findings. Radiographics 2001;21:451-61. 3.[urologyannals.com]
  • impairment over the follow-up period.[pediatricurologybook.com]
  • Discussion: Posterior urethral valves result in variable degrees of renal impairment and bladder dysfunction. The anomaly severely affects some boys but is virtually innocuous in others for reasons that remain unclear[3].[jpss.eu]
Polyuria
  • Such overdistention is exacerbated by polyuria, and can be a cause of secondary hydronephrosis.[nature.com]
  • Damage to the distal nephron impairs the concentrating ability of the kidney resulting in polyuria and polydipsia (nephrogenic diabetes insipidus), which can worsen bladder function and thereby further risk upper tract function.[pediatricurologybook.com]
  • Polyuria may be a feature. Acute lower tract obstruction Often follows a history of symptoms of obstruction of bladder outflow (see below). Usually severe suprapubic pain (but not if superimposed on chronic retention or underlying neuropathy).[patient.info]

Workup

Neonatal Urinary Ascites
  • Development of renal function after neonatal urinary ascites due to obstructive uropathy. J Urol. 2002; 168 (2):675–8. [ PubMed ] [ Google Scholar ] 20. Choudhury SR, Mitra SK, Jhon P.[ncbi.nlm.nih.gov]

Treatment

  • , one death due to sepsis prior to treatment).[bradyurology.blogspot.com]
  • Key points Transurethral valve ablation is the initial treatment for obstruction caused by PUV, and the vast majority of patients have resolution of hydroureteronephrosis following treatment; however, a small proportion of patients experience persistent[nature.com]
  • Treatment [ edit ] 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]
  • Before treatment, VUR was present in 43% of boys and after EVR, VUR was present in 22%. Post treatment, 19% (0–70%) was reported to suffer from urinary incontinence.[journals.plos.org]
  • Endoscopic treatment is the best treatment but some newborns may have to wait for this procedure until they have grown.[chw.org]

Prognosis

  • 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]
  • What is my baby's long-term prognosis? Long-term prognosis for the baby who is diagnosed with bladder outlet obstruction before birth and who has adequate lung development, is improving every day.[chw.org]
  • Young reviews the "Symptomatology" or clinical presentation of patients, the diagnostic algorithm including differential diagnosis, treatment strategies including the prepatory treatment, and prognosis.[bradyurology.blogspot.com]
  • This determines the final prognosis, which is worse the younger the child at presentation. Key words Urethral obstruction Congenital valve This is a preview of subscription content, log in to check access. Preview Unable to display preview.[link.springer.com]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • The etiology of PUV was unknown with a number of hypotheses put forward including: persistence of the urogenital membrane, or anomalous development of the Wolffian and Muellerian ducts.[bradyurology.blogspot.com]
  • More studies are required on the inheritance, etiology and pathogenesis of the prune belly syndrome.[turkjpath.org]
  • Congenital urethral diverticulum has been postulated to occur due to distal obstruction such as meatal stenosis. [2] Various etiological theories, which have been proposed for the AUV and diverticulae, are developmental derangement of the corpus spongiosum[jiaps.com]
  • This review discusses the etiology of valve–bladder syndrome and examines each of the studies which have investigated nocturnal bladder drainage in its treatment.[nature.com]

Epidemiology

  • Bladder exstrophy Definition : embryological defect of ventral abdominal wall due to an overdeveloped cloacal membrane Epidemiology : incidence is 1/30,000 live births; more common in males Symptoms/clinical features Bladder and urethra herniated through[amboss.com]
  • Epidemiology Most common cause of lower urinary tract obstruction in males The incidence is estimated to be between 1:3,000 and 1:8,000 live male births.[unboundmedicine.com]
  • A.: Urinary tract infections in children younger than 5 years of age: epidemiology, diagnosis, treatment, outcomes and prevention.[auanet.org]
  • 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.medscape.com]
  • Epidemiology Age Urinary tract obstruction occurs most commonly in the young and the old: In older men, this is a relatively common condition due to prostatic enlargement: Incidence of lower urinary tract symptoms (LUTs)/BPH averages 15 per 1,000 man-years[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Commonly associated pathophysiologic findings in children with PUV include (1) hydroureteronephrosis, (2) dilated bladder with trabeculations and/or diverticulum, and (3) vesicoureteral reflux.[unboundmedicine.com]
  • Koff SA et al . (2002) The valve bladder syndrome: pathophysiology and treatment with nocturnal bladder emptying . J Urol 167 : 291–297 15.[nature.com]
  • CrossRef PubMed Google Scholar Koff, S.A. (1990) Pathophysiology of ureteropelvic junction obstructions. Urol. Clin. North. Am. 17, 263–272.[link.springer.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.medscape.com]
  • Certainly, behavior is crucial to the pathophysiology of most types of dysfunctional voiding.[auanet.org]

Prevention

  • Valve Tissue in the passageways between the heart's upper and lower chambers that controls passage of blood and prevents regurgitation. valve fold/or flap in lining of a tubular structure; prevents content reflux valve ( valv ) [TA] 1.[medical-dictionary.thefreedictionary.com]
  • Surgery may be required to prevent kidney damage.[ummchealth.com]
  • Early intervention with antibiotics may prevent kidney damage since antibiotics have recently been shown to reduce the risk of future infections.[nyulangone.org]
  • Hydronephrosis is dilatation of the renal pelvis and calices due to pathologies preventing outflow of urine together with progressive atrophy of the renal parenchyma 13.[turkjpath.org]
  • Oesophageal atresia (EA): EA is a birth defect which prevents the complete development of the oesophagus, which is the tube that takes food from the orifice towards the stomach.[medic8.com]

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