Megacystis with an anterior urethral valve: Case report and review of literature

Confer, Stephen D.; Galati, Vincenzo; Frimberger, Dominic; Kropp, Bradley P.

doi:10.1016/j.jpurol.2009.06.017

Cited by 13 publications

(9 citation statements)

References 11 publications

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“…Anterior urethral valves (AUVs) are the most common congenital obstructive disorder affecting the male anterior urethra (1). Nonetheless, it is still quite rare and occur 15–30 times less frequently than posterior urethral valves (PUVs) (2).…”

Section: Introductionmentioning

confidence: 99%

Anterior Urethral Valves: Not Such a Benign Condition…

et al. 2013

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Purpose: Anterior urethral valves (AUVs) is an unusual cause of congenital obstruction of the male urethra, being 15–30 times less common than posterior urethral valves (PUVs). It has been suggested that patients with congenital anterior urethral obstruction have a better prognosis than those with PUV, with less hydronephrosis, and a lower incidence of chronic renal insufficiency (5 vs. 30%). The long-term prognosis of AUVs is not clear in the literature. In this report we describe our experience and long-term follow up of patients with anterior urethral valve.Materials and Methods: We retrospectively identified 13 patients who presented with the diagnosis of AUVs in our institutions between January 1994 and June 2012. Two patients were excluded: one patient had no follow up after intervention; the other had a follow up <1 year. From the 11 patients included, we evaluated the gestational age, prenatal and postnatal ultrasound findings, voiding cystourethrogram findings, age upon valve ablation, micturition pattern, creatinine, and clinical follow up.Results: Between 1994 and 2012 we evaluated 150 patients with the diagnosis of urethral valves. Of this group, 11 patients (7.3%) had AUVs and an adequate follow up. Mean follow up is 6.3 years (2.5–12 years). Five (45.4%) patients had prenatal diagnosis of AUV. The most common prenatal ultrasonographic finding was bilateral hydronephrosis and distended bladder. One patient showed a large perineal cystic mass, which was confirmed to be a dilated anterior urethra. The mean gestational age was 37.6 weeks (27–40 WGA). Postnatally, 90% had trabeculated bladder, 80% hydronephrosis, and 40% renal dysplasia. The most common clinical presentation was urinary tract infection in five patients (45.4%), followed by weak urinary stream found in four patients (36.3%). The age at initial surgical intervention ranged between 7 days and 13 years. Seven (63.6%) patients had primary transurethral valve resection or laser ablation and three patients (27.2%) had primary vesicostomies. One boy (9.1%) had penile urethrostomy with excision of urethral diverticulum. Two (18.2%) patients developed end-stage renal disease.Conclusion: Anterior urethral valve is a rare congenital entity affecting the genitourinary system in males. Early urinary tract obstruction resulted in end-stage renal disease in 18% of our patient population. In our series, the complication rate and the evolution to renal failure are high and similar to patients with PUV. In patients with AUVs we recommend long-term follow up and close evaluation of patient’s bladder and renal function.

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Section: Introductionmentioning

confidence: 99%

Anterior Urethral Valves: Not Such a Benign Condition…

et al. 2013

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“…While congenital obstruction of the anterior urethra is rare, PUVs remain the most frequent obstructive lesion of the lower system causing megacystis, especially in male fetuses, with an incidence of 1 in 2500-5000 births [4,11]. PUV represents 10.7% of all cases, and is the most frequent cause of terminal renal failure in early childhood [8].…”

Section: Discussionmentioning

confidence: 99%

“…Current therapies include percutaneous vesico-amniotic shunting [5,6,8,9,13,16,19] and in utero fetoscopic ablation of valves by percutaneous fetal cystoscopy that allows laser valve ablation [12,15] and mechanical disruption of posterior urethral valves [3,4,8,17,18,20].…”

Section: Discussionmentioning

confidence: 99%

Fetal posterior urethral valve mechanic perforation using an 18-gauge needle: a case-series

Bertin

Cosmi

Visentin

et al. 2014

Case Reports in Perinatal Medicine

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Objective: To assess the effectiveness of using an 18-gauge needle inserted through the perineal surface of the fetus to solve magacystis and posterior urethral valve (PUV). Methods: From February 2008 to January 2012, 15 cases of magacystis were referred to our tertiary referral center, and in ten, PUV was suspected. In the cases where PUV was suspected after a detailed ultrasound scan, women gave consent to proceed with fetal therapy under sonographic guidance using an 18-gauge needle. The procedures lasted for a median time of 30 min (range 15-50 min). The needle was inserted through the perineal surface of the fetus to perforate the dilated posterior ureteral valve, as well as to perform a perineal vescicocentesis. Results: Median gestational age at time of referral was 17 weeks of gestation (range 16-19 weeks). Five fetuses were female and ten male. Nine cases opted for pregnancy termination, as the kidneys were dysplastic showing hyperechoic structure, while six cases were treated, as the kidneys showed a normal functionality as well as corticomedullar differentiation. Five fetuses were male and one female. After fetal therapy, there were no stillbirths. All fetuses were delivered at term and the infants were followed up by routine kidney ultrasonography and functionality after a median follow-up of 16 months. Conclusions: Despite the most commonly used fetal therapy being vesico-amiotic shunting, percutaneous fetal cystoscopy that allows laser valve ablation and mechanical disruption was the procedure used in the present study. This allowed the direct treatment of the urethral obstruction that caused megacystis, without need to repeat the procedure and without the need of any postnatal therapy.

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“…Megacystis is the longitudinal size of a fetal bladder greater than 7 mm in the first trimester (1). The most common cause of megacystis is lower urinary tract obstruction (2). Megacystis can also be associated with chromosomal anomalies (mostly with trisomy 13 and 18) (3).…”

Section: Figure 1 Cystic Mass In the Uterine Cavity Answermentioning

confidence: 99%