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Megacystis - Megaureter Syndrome
Megaureter-Megacystis Syndrome

Presentation

Patients rarely present with signs of renal failure. Most PM in neonates is nonrefluxing and unobstructed. [orpha.net]

Urinary tract infection was present in 74 per cent of the cases. Of these children who presented before 1975, 14 were… CONTINUE READING [semanticscholar.org]

The megaureter, originating in the late embryonal phase, presents obstruction only. The corresponding nephropathy shows obstructive, refluxive and dysplastic features. [pubmed.ncbi.nlm.nih.gov]

Workup

The main consideration at the time of the workup was posterior urethral valves, although urethral stenosis from other etiology or even atresia could not be excluded, given the severity of dilatation and early oligohydramnios. [kundoc.com]

Treatment

Incorrect diagnoses, including obstruction, neurogenic bladder dysfunction, and "megacystis," were made in 16 children; treatment was inappropriate in 14. In only eight boys were diagnosis and treatment prompt and correct. [ajronline.org]

Gram-negative rods were detected in his urine, and antibiotic treatment was started. Subsequently, both urine and blood culture grew P. aeruginosa. After 2 weeks of treatment, he was scheduled for the surgery to correct VUR and discharged. [jstage.jst.go.jp]

Management and treatment Treatment depends on the type of PM. For refluxing PM, medical management is appropriate during infancy and is continued if there is a trend to resolution. [orpha.net]

Prognosis

Megacystis-microcolon-hypoperistalsis syndrome is a rare condition occurring in female fetuses with a poor postnatal prognosis (see later). 46 – 49 Table 16-2 Causes of Large Bladder Prognosis and In Utero Treatment. [radiologykey.com]

The overall prognosis can be variable from progressive obstruction to spontaneous resolution. [radiopaedia.org]

The Prune Belly syndrome: A review of its etiology, defects, treatment and prognosis. J Urol 1988;140:707-12. 7. Reinberg Y, Shapiro E, Manivel JC, Manley CB, Pettinato G, Gonzalez R. [jnsbm.org]

Prognosis Many cases of PM resolve spontaneously within the first two years of life, with the maturation of the urinary tract. The documents contained in this web site are presented for information purposes only. [orpha.net]

Etiology

Nonobstructive etiologies included the megacystis-megaureter association in 6 cases and the prune-belly syndrome in 1. [indiana.pure.elsevier.com]

The exact etiology of this aperistatic segment is not known. Several etiological theories have been proposed including: Excessive collagen deposition. [abdominalkey.com]

History of consanguinity and the mother being elderly gravida indicate the hereditary etiology in our case. [jnsbm.org]

Etiology The cause is unknown but it may be due to high fetal urine outflow, changes in the ureter pre- and postnatal or transient anatomical obstructions that improve with postnatal development, such as ureteral folds. [orpha.net]

Is of unknown etiology 4. Which of the following is an incorrect statement? a. Only two of the major features of prune belly syndrome can be diagnosed by CT. b. [docslide.com.br]

Epidemiology

Summary Epidemiology Prevalence is unknown, but PM is the second most common cause of neonatal hydronephrosis. The male to female ratio is nearly 4:1 and the left side is more often affected than right (1.6-4.5 times). [orpha.net]

It is generally defined as a bladder diameter > 7 mm in the first trimester 3 bladder diameter > 30 mm in the second trimester bladder diameter > 60 mm in the third trimester Epidemiology The estimated incidence of antenatal imaging is at ~ 1 : 1500 pregnancies [kriznanultrasoundimages.com]

Prune Belly syndrome: Epidemiologic, clinic and therapeutic aspects. Prog Urol 2008;18:470-4. 10. Woodhouse CR. Prospects for fertility in patients born with genitourinary anomalies. J Urol 2001;165:2354-60. 11. [jnsbm.org]

Contemporary epidemiology and characterization of newborn male with prune belly syndrome. J Urol. 2010;76:44–8. View Article Google Scholar Lloyd JC, Wiener JS, Gargollo PC, et al. [jmedicalcasereports.biomedcentral.com]

Gender- difficult to assess because of the epidemiology of UTIs in children, as boys and girls may present with reflux at different ages. [cram.com]

Pathophysiology

Pathology The pathophysiology of these massively dilated ureters and the large capacity bladder is the constant recycling of large volumes of refluxed urine. References Promoted articles (advertising) [radiopaedia.org]

The pathophysiology of these massively dilated ureters and the large capacity bladder is the constant recycling of large volumes of refluxed urine. We treated 29 children between 1 day and 13 years old with this syndrome. [semanticscholar.org]

The causative distal obstruction ascends, its urodynamic reaction, however, descends in accordance with the rules of the pathophysiology of the smooth muscles of cavitary organs. [pubmed.ncbi.nlm.nih.gov]

Prevention

An appropriate preventive management of bladder and intestinal emptying are often effective to prevent the development of VUR and it’s parenchymal complications. [scielo.isciii.es]

[…] the development of PBS, as reported by Leeners et al. [11] PBS is a rare congenital anomaly which has no known prevention other than the routine use of screening for fetal anomalies. [jnsbm.org]

Prevention of infection is essential to minimize the risk of renal damage; therefore, continuous antibiotic prophylaxis is usually used in children with high-grade VUR while awaiting spontaneous resolution. [emedicine.medscape.com]

However, infections must be prevented, especially those due to Proteus mirabilis and other urea-splitting organisms, which can lead to hyperammonemic encephalopathy 14 in PBS patients without liver dysfunction. [nature.com]

The ureteral attachment to the bladder normally is oblique, between the bladder mucosa and detrusor muscle, creating a flap-valve mechanism that prevents reflux ( Fig. 533-1 ). [obgynkey.com]

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