Prenatal incision of ureterocele causing bladder outlet obstruction: a multicenter case series.

We reviewed data from a cohort of fetuses with ureterocele diagnosed and operated prenatally in four fetal therapy centers. Inclusion criteria were (1) ureterocele confirmed on detailed fetal ultrasound examination, (2) absence of additional fetal malformations, and (3) fetal intervention to decompress the ureterocele with local institutional review boards' approval. Data on sonographic follow-up, obstetrical, neonatal outcome, and postnatal evaluation were collected. Ten cases of prenatally treated ureterocele are described. Six cases benefited from a fetoscopy for laser incision and decompression, two cases had an ultrasound guided puncture before resorting to a fetoscopy with laser incision, one case had a balloon catheterization under ultrasound guidance, and one case had an ultrasound-guided opening of the ureterocele with a laser fiber passed through a 20-gauge needle. Mean gestational age at diagnosis was 21.6 GW. Two cases underwent termination of pregnancy. The remaining eight cases recovered normal amniotic fluid volume and delivered a liveborn child at a mean gestational age of 38.6 GW with normal creatinine levels during the first week of life. Prenatal incision provided complete treatment of severely obstructive ureteroceles in 80% of the cases and allowed improvement of urinary electrolytes, renal size and echogenicity, bladder filling in all survivors, and recollection of normal amniotic fluid volume, in case of oligoanhydramnios. © 2017 John Wiley & Sons, Ltd.

New minimally invasive technique for cystoscopic laser treatment of fetal ureterocele.

Prenatal ultrasound detection of fetal ureterocele with bilateral hydroureteronephrosis, obstruction of the bladder outlet and progressive amniotic fluid reduction have been associated with a significant risk of end-stage chronic renal disease after birth. Fetal cystoscopic laser incision of the ureterocele, using standard 3.5-mm fetoscopic access to the amniotic cavity and the fetal bladder with the aim of relieving the bladder outflow obstruction to preserve renal function, has been reported previously in a case with a favorable outcome. We report on two additional cases of fetal ureterocele treated by cystoscopic laser decompression. In the first case, a standard 3.3-mm uterine entry was used. In the second case, a new approach was adopted using an 'all-seeing needle' 1.6-mm endoscope. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Fetoscopic laser surgery to decompress distal urethral obstruction caused by prolapsed ureterocele.

We report on the successful use of fetoscopic surgery to treat a case of prolapsed ureterocele in a female fetus. At 21 weeks' gestation, a double renal system with an intravesical ureterocele obstructing the bladder outlet was diagnosed, causing severe megacystis, bilateral hydronephrosis and progressive oligohydramnios. Ultrasound evaluation following referral to our center confirmed severe bilateral hydronephrosis with pelvic and calyceal dilatation, but amniotic fluid volume was normal and the ureterocele was not visualized in the bladder. Instead, a cystic mass within the external genitalia was observed, suggestive of a prolapsed ureterocele, causing intermittently severe obstruction of the urethra. The parents were counseled about the uncertain prognosis and fetal surgery to decompress the urinary system was proposed. The procedure involved firing a contact diode laser until perforation of the ureterocele was achieved. Following laser surgery, resolution of megacystis, reduction of hydronephrosis and normalization of amniotic fluid volume were observed. Our report demonstrates that fetoscopic decompression of a distal urethral obstruction is feasible in the rare event of congenital prolapsed ureterocele.

Sonography of fetal micturition.

OBJECTIVE: To describe the sonographic visualization of fetal micturition and its role in the diagnosis of posterior urethral valves and hypospadias. METHODS: This was a prospective study of 25 male fetuses (21 with bilateral pyelectasis, one whose bladder was being studied because of ureterocele and three with hypospadias), and five female fetuses (with bilateral pyelectasis). A midline sagittal scan of the fetal pelvis, perineum and external genitalia was obtained and observed continuously during fetal micturition. RESULTS: In 19 of the 21 male fetuses and the five female fetuses with bilateral pyelectasis micturition was normal, with visualization of urinary bladder contraction, slight fluid distention of the urethra and a urinary stream from the external urethral meatus. In three male fetuses, two with bilateral pyelectasis and the one with ureterocele, the posterior urethra was normal at rest and it ballooned out during micturition, diagnostic of posterior urethral valves. In the three male fetuses with hypospadias the ventral jet of the urinary stream was visualized. CONCLUSION: Fetal micturition can be visualized on sonography. It may be of value in the diagnosis of posterior urethral valves and hypospadias.

Obstructive ureterocele-an ongoing challenge.

Ureterocele is a cystic dilatation of the intravesical ureter that is most commonly observed in females and children, and usually affects the upper moiety of a complete pyeloureteral duplication. According to their position, ureteroceles are divided into intravesical, when the ureterocele is completely contained inside the bladder, and extravesical when part of the cyst extends to the urethra or bladder neck. Most ureteroceles are diagnosed in utero or immediately after birth during an echographic screening of renal malformations. Severe, febrile urinary tract infection is the most common postnatal presentation of ureteroceles, but they may, rarely, prolapse and acutely obstruct the bladder outlet. Once an ureterocele is identified sonographically, a voiding cystourethrogram to detect vesicoureteral reflux (VUR) and a 99m-technetium dimercapto-succinic acid renal scan to evaluate the function of the different portions of the kidney are mandatory. VUR in the lower pole is observed in 50% of cases and in the contralateral kidney in 25%. Simple endoscopic puncture of the ureterocele has recently been advocated as an emergency therapy for infected or obstructing ureteroceles and as an elective therapy for intravesical ureteroceles. The rate of additional surgery after elective endoscopic puncture of an orthotopic ureterocele ranges from 7 to 23%. Treatment of ectopic ureteroceles is more challenging and both endoscopic puncture and upper pole partial nephrectomy frequently require additional surgery at the bladder level. The reoperation rate after endoscopic treatment varies from 48 to 100%. It is 15 to 20% after upper pole partial nephrectomy if VUR was absent before the operation, but is as high as 50-100% when VUR was present. Thus, endoscopic incision is appropriate as an emergency treatment or when dealing with a completely intravesical ureterocele. Upper pole partial nephrectomy is the elective treatment for an ectopic ureterocele without preoperative VUR. In an ectopic ureterocele with VUR, no matter which type of primary therapy has been chosen, a secondary procedure at the bladder level, involving ureterocele removal and reimplantation of the ureter(s), should be anticipated.

Prenatal diagnosis of a prolapsed ureterocele with magnetic resonance imaging.

Ectopic ureterocele with ureteral duplication is the most common type of ureterocele found in children. Early accurate diagnosis is desirable to decrease the risk of urosepsis and renal damage. Prenatal imaging with ultrasonography detects the vast majority of ureteroceles; however, in some cases, the diagnosis may remain in doubt. We report on the use of magnetic resonance imaging to clarify the prenatal diagnosis of a prolapsed ureterocele.

Ultrasound-guided laser treatment for fetal bladder outlet obstruction resulting from ureterocele.

In a case of severe bilateral hydronephrosis in a female fetus associated with ureterocele at 28 weeks, under local anesthetic, ultrasound-guided neodymium:yttrium-aluminum-garnet laser was used to perforate the ureterocele and successfully overcome the obstruction. There was no recurrence of obstruction, and renal function was normal postnatally.

[Classification and treatment of ureteroceles in the adult]. / Classification et traitement des urétérocèles de l'adulte.

Ureterocele or congenital pseudocystic dilatation of the terminal portion of the ureter due to persistence of Chwalla's membrane is frequent in female adults (17-35%) in its orthotopic form. It expands the short submucosal segment of the normally situated ureter and may be intravesical or extravesical. The diagnosis is primarily radiological based on intravenous urography (IVU) and endoscopy (urethrocystoscopy). Treatment depends on the site of the ureterocele, the clinical context, associated anomalies and especially the dimensions. Endoscopic treatment has numerous advantages and deserved to be more widely used.

Antenatal ultrasonographic development of ureteroceles. Implications for management.

Ureteroceles associated with a duplex collecting system may lead to obstructive uropathy, the most common genitourinary anomaly of the fetus. Although most frequently presenting as hydronephrosis, damage from obstruction is a progressive process which may even develop into bilateral renal dysfunction. Antenatal intervention for preservation of fetal renal function may be considered, but must be balanced against procedural risks. Therefore, accurate documentation of the progression may be necessary. In these cases, the development of the classic findings of two ureteroceles are documented over a period of several weeks using serial ultrasonographic evaluation. Postnatal evaluation of the infants confirmed the antenatal findings. The importance of serial ultrasonographic evaluation of fetal obstructive uropathies is dramatically illustrated.

[Ureterocele in children]. / Ureterokèles bij kinderen.

Two case reports of children with ectopic ureteroceles are presented. The embryologic development, symptoms, diagnosis and treatment are discussed. Hemi-nephroureterectomy is the treatment of choice. In instances, such as serious urinary tract infections, massive vesicoureteral reflux in other ureters and in single system ureteroceles, endoscopic incision of the ureterocele can be effective in terms of control of infection and decompression of other kidney parts. Definite surgery can be deferred until the child is in a better condition.

Embryogenesis of ureteral anomalies: a unifying theory.

Extensive gross, microscopic and clinical studies of various ureteral anomalies have enabled investigators to set forth theories regarding the aetiologies of these anomalies consistent with observed fact. Synthesis of these observations allows for a simplified classification of mega-ureter (primary obstructed, reflux and non-obstructed, non-reflux mega-ureters), ureterocele, duplex ureters and ectopic ureters based upon some combination of mesenchymal differentiation anomalies and location anomalies. A defect early in mesenchymal differentiation would be expected to result in panureteral disease. A defect later in development would result in a focal abnormality anywhere along the course of the ureter. Influence upon ureteral bud mesenchyme by local expansion factors in the bladder base may result in various types of ureterocele. Position of the ureteral orifice on the trigone or in Wolffian duct derivatives would occur in accordance with the Weigert-Meyer principle and would correlate with upper tract dysplasias. Thus, an attempt has been made to systematize and trace the origins of mega-ureter, ureterocele, duplex ureters and ectopic ureters to defects of differentiation of the basic mesoblastic cell in aberrant locations of the ureteral bud.

Ureteral duplication, ectopy and ureteroceles.

These entities pose some of the most challenging diagnostic and therapeutic objectives involving the urinary system. The clinical presentations are varied, and involve children from prenatal to adolescent age groups. The diagnostic and therapeutic alternatives are presented, along with a classification for the more complex clinical settings.

Morphological study of the ureterocele: a possible clue to its embryogenesis as evidenced by a locally arrested myogenesis.

The wall of a ureterocele and the proximal ureter involved were investigated with light and electron microscopy. Emphasis in our study was on the muscular structure. In most cases, that is in patients with single or duplex, or simple or ectopic ureteroceles, muscle bundles were hardly developed in the dome of the ureterocele but were well developed in the proximal ureter, with and without dilatation. In the dome of the ureterocele muscle cells were much smaller than those in the proximal ureter. Thick and thin myofilaments were demonstrated in muscle cells in the proximal ureter but thick myofilaments were absent in the dome of the ureterocele. These findings may suggest that a ureterocele is a segmental embryonal arrest of the most distal portion of the ureter.

Abnormalities of the ureteral bud.

The development and incorporation of the ureteric bud into the developing bladder is a complex process, and it is influenced by events occurring at the same time in the development of other systems in the perineum. Knowledge of the embryology of the ureteric bud and associated wolffian duct is the key to understanding the development of the entire genitourinary system. If the normal embryology is understood, the dysembryogenesis that results in many of the common anomalies of the lower urinary system is simplified. Many of the concepts proposed in this discussion are well substantiated, others are more difficult to prove conclusively. It is believed, however, that the embryologic approach provides a simplified understanding of many of the common problems encountered in pediatric urology. The key in clinical practice to the diagnosis and understanding of these defects is the cystoscopic examination. Careful assessment of the orifice's position, appearance, and tunnel length will allow the interpretation of how the abnormality came about, and also provides valuable information concerning the state of the associated renal segment. This in turn allow better therapeutic management of the problem based on the knowledge of the severity of the renal anomaly associated with the abnormality found in the bladder.

Ectopic ureterocele, hydroureter, and renal dysplasia: an embryogenic triad.

A review of the literature of the embryogenesis of ectopic ureterocele is presented. A theory linking the triad of ectopic ureterocele, hydroureter, and renal dysplasia to a primary intrinsic developmental abnormality of size and position of the ureteral bud is postulated.

Horseshoe kidney with multicystic dysplasia associated with ureterocele.

This is a case report of a horseshoe kidney one side of which contains a multicystic dysplastic element associated with a ureterocele. The surgical management and embryologic basis are reviewed.