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]
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]
BACKGROUND OF THE PRESENT INVENTION 1. [google.ch]
Clinical presentation was similar to that of posterior urethral valves. [pjms.com.pk]
Entire Body System
- Asymptomatic
Sometimes, a small diverticulum goes unnoticed until adult life [10] or remains asymptomatic, found co-incidentally. Seldom, a stone-filled diverticulum mimicks a firm penile mass [14]. [ispub.com]
[…] valve that separates the left ventricle and the aorta; it opens with end diastole, causing the second heart sound. bicuspid aortic valve a congenital anomaly of the aortic valve, caused by incomplete separation of two of the three cusps; it is generally asymptomatic [medical-dictionary.thefreedictionary.com]
Currently this patient is asymptomatic and has a normal voiding pattern. None of our patients developed urethrocutaneous fistulae. [frontiersin.org]
Other conditions like posterior urethral diverticulum or utricle can be relatively asymptomatic and therefore present late in childhood. We present the spectrum of imaging findings of common and uncommon anomalies involving the male urethra. [ijri.org]
As this congenital anomaly may occur due to a mixed effect of a few minor genes, so it is recommended that PUV should be evaluated in all males in the family, even in asymptomatic ones [ 8 ]. [ncbi.nlm.nih.gov]
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]
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]
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]
They may also present at an older age with febrile UTIs, incontinence, hematuria, failure to thrive, abdominal pain, or pelvic pain. [hawaii.edu]
- Diarrhea
Non-specific symptoms can include poor feeding, irritability, lethargy, vomiting, diarrhea, ill appearance, and abdominal distension (Table 1). [auanet.org]
Cardiovascular
- Hypertension
Wallace reports that hypertension develops in 10% of children with unilateral scars and in 18.5% with bilateral scars. Among adults with reflux nephropathy, 34% ultimately develop hypertension. [emedicine.medscape.com]
Over time, these conditions may lead to chronic kidney disease and hypertension, which can affect a child’s growth and development. Our kidney specialists perform a variety of diagnostic tests to evaluate your child’s kidney and urinary function. [nyulangone.org]
Future hypertension occurs in at least 10-20% of children with pyelonephritogenic scarring. Hypertension in this setting occurs independent of the degree of renal scarring. [auanet.org]
Table 1 Symptoms and signs in posterior urethral valves patients Symptoms Frequency Dribbling and poor stream 50 (51%) Urinary tract infection 40 (40.8%) Fever 5 (5.1%) Hematuria 2 (2.1%) Hypertension 1 (1%) Total 98 (100%) Most common associated anomaly [ncbi.nlm.nih.gov]
Hypertension which settles after valves resection has been commonly noted (Evins and Lorenzo, 1979). Diagnosis at this stage is relatively easy. However the delay in diagnosis may lead to extensive renal damage. [jpma.org.pk]
Eyes
- Prolapse
Pyelolymphatic backflow 402 Frequency of reflux 403 Backflow in acute renal colic 404 Significance of reflux from a roentgendiagnostic point of view 410 Risks associated with reflux 414 R Miscellaneous changes particularly of the ureter 421 Ureteric prolapse [books.google.com]
[…] of urethra Q64.72 Congenital prolapse of urinary meatus Q64.73 Congenital urethrorectal fistula Q64.74 Double urethra Q64.75 Double urinary meatus Q64.79 Other congenital malformations of bladder and urethra Q64.8 Other specified congenital malformations [icd10coded.com]
[…] be required if the diastasis is extreme or after first 2 – 3 days of life as pelvis becomes less malleable Complications Persistent incontinence Urinary tract infections, epididymitis Bladder stones, fistulas Vesicoureteral reflux Vaginal and rectal prolapse [amboss.com]
In females, the most common cause of anatomic bladder outlet obstruction is a ureterocele that has prolapsed into the urethra (urethral prolapse may resemble a large doughnut shaped mass in the perineum). 90% of cases are associated with the upper pole [hawaii.edu]
In women, it tends to be related to pelvic tumours (particularly gynaecological malignancies), prolapse of pelvic structures, or pregnancy. [patient.info]
Skin
- Flushing
Direct connect flush system US9555219B2 ( en ) 2011-11-10 2017-01-31 Boston Scientific Scimed, Inc. Direct connect flush system US9642705B2 ( en ) 2011-11-15 2017-05-09 Boston Scientific Scimed Inc. [google.la]
Psychiatrical
- Low Self-Esteem
Abused children may also present with secondary incontinence (i.e., wet after at least 6 months of continence), low self-esteem, and a pathologic fear of examination. [auanet.org]
Neurologic
- Irritability
This method is very undesirable in-that the wearer is continually confronted with the embarrassment of having to wear a bulky pad of absorbant material that-can create skin irritations and offensive odors. [google.com]
Non-specific symptoms can include poor feeding, irritability, lethargy, vomiting, diarrhea, ill appearance, and abdominal distension (Table 1). [auanet.org]
[…] result of urine build up) Underdeveloped lungs as a result of decreased amniotic fluid Vesicoureteral reflux (urine reverses its flow from the bladder to the upper urinary tract) In addition to the above symptoms, fever, vomiting, loss of appetite, and irritability [urologists.org]
The provision of such perforations tends to reduce the likelihood of irritation of the walls of the urethral canal, since there is minimized the possibility of accumulation of any discharge with accompanying discomfort and possible infection, Instead, [google.la]
Urogenital
- Cryptorchidism
[…] male births Symptoms/clinical features : Abnormal foreskin ; (dorsal hood), ventral penile curvature ;, and two meatal openings Proximal hypospadias are associated with bifid scrotum and penoscrotal transposition Associated with inguinal hernias and cryptorchidism [amboss.com]
Urinary tract anomalies, Fetus, Fetal pathology Objective: Prune-belly syndrome is a rare congenital disorder characterized by musculature deficiency in the abdominal wall, lower urinary tract obstruction, other urinary tract anomalies, and bilateral cryptorchidism [turkjpath.org]
Lateral VCUG image reveals an extensively dilated anterior and posterior urethra (arrows) Click here to view Prune belly syndrome This refers to a constellation of anomalies, including lax abdominal wall musculature, cryptorchidism, and various lower [ijri.org]
Last updated: Jan 30, 2018 at 21:48 Name Hideo Nakai Nickname H.Nakai E-mail hnakai jichi.ac.jp Affiliation Jichi Medical University Research funding number 50167540 Research Interests cryptorchidism(1), nocturnal enuresis(2), urinary incontinence(3), [researchmap.jp]
• Megalourethra is often associated with other congenital abnormalities including cryptorchidism, renal agenesis etc. • These patients have a functional rather than anatomic urethral obstruction, causing stasis and back pressure into the upper urinary [slideshare.net]
- 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
It results in bladder obstruction. [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]
[…] 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]
Treatment
one death due to sepsis prior to treatment). [bradyurology.blogspot.com]
Endoscopic treatment is the best treatment but some newborns may have to wait for this procedure until they have grown. [chw.org]
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]
However, despite treatment, many patients will develop chronic kidney disease. [amboss.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]
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]
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]
[…] days of life as pelvis becomes less malleable Complications Persistent incontinence Urinary tract infections, epididymitis Bladder stones, fistulas Vesicoureteral reflux Vaginal and rectal prolapse Possible infertility in males Bladder adenocarcinoma Prognosis [amboss.com]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
More studies are required on the inheritance, etiology and pathogenesis of the prune belly syndrome. [turkjpath.org]
Erectile dysfunction and infertility in males UTI References: [1] [2] [4] Hypospadias Definition : common congenital malformation with incorrect positioning of the urethral meatus ; due to failure of urethral folds and foreskin to fuse on ventral penis Etiology [amboss.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]
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]
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 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]
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]
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]