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]
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]
Clinical presentation was similar to that of posterior urethral valves. [pjms.com.pk]
We present here our experience of two cases that presented with stricture urethra post ablation and were managed successfully. :: Case Reports Case 1 A 13 month-old boy, a follow up case of type 1 posterior urethral valve (PUV) underwent cystoscopic valve [jpgmonline.com]
Young recognized that PUV was a clinical heterogeneous entity, with patients having a variety of clinical presentations and outcomes. [bradyurology.blogspot.com]
Gastrointestinal
- Failure to Thrive
[…] to thrive Diagnostics Voiding cystourethrogram is diagnostic and demonstrates dilation/elongation of posterior urethra while voiding Prenatal ultrasound of the upper urinary tract demonstrates distended bladder, bilateral hydroureters, bilateral hydronephrosis [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]
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]
Urogenital
- 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]
Children may present with clinical pyelonephritis, fever, abdominal/flank pain, malaise, nausea, vomiting, cystitis with dysuria, frequency, urgency, and urge incontinence. [hawaii.edu]
Although rare, adult presentation of PUVs has been described in case reports, with symptoms ranging from obstructive voiding symptoms to postejaculatory dysuria. [emedicine.medscape.com]
- Bladder Neck Obstruction
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]
It results in bladder obstruction. [icd10data.com]
[…] exstrophy of urinary bladder Q64.19 Other exstrophy of urinary bladder Inclusion term(s): Extroversion of bladder Q64.2 Congenital posterior urethral valves Q64.3 Other atresia and stenosis of urethra and bladder neck Q64.31 Congenital bladder neck obstruction [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]
- Incomplete Bladder Emptying
Secondary reflux and incomplete bladder emptying from poor bladder function increases the risk of pyelonephritis. [auanet.org]
emptying is incomplete on serial ultrasounds. [pediatricurologybook.com]
- Urinary Hesitancy
Chronic lower tract obstruction Usual signs and symptoms include: Urinary hesitancy. Narrow and weak urine stream. Dribbling at end of micturition. Feeling of incompletely emptied bladder. [patient.info]
- Urethral Disease
Acquired Inflammatory Diseases • Gonococcal and Nongonococcal Urethritis • Complications associated with gonococcal urethritis are more common and more serious than those associated with nongonococcal urethritis and include urethral stricture, periurethral [slideshare.net]
Workup
Urine
- 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
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]
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]
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]
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]
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]
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]
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]
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]
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]