Bilateral single system ectopic ureters opening into vestibule with bladder agenesis

Baheti, Vishwas; Singh, Janak; Yadav, Sher Singh; Tomar, Vinay

doi:10.1007/s11255-014-0648-7

Cited by 7 publications

(9 citation statements)

References 11 publications

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“…HSG will show a normal endometrial cavity and 3D-US and especially MR allow the right diagnosis. Anomalies of the cloaca and urogenital sinus (including congenital vagino-vesical fistulas) : This category includes cases as simple as the imperforate hymen due to a persistent urogenital membrane together with blind hemibladder [ 54 ], bladder duplication [ 55 , 56 ], bilateral single system ectopic ureters opening into the vestibule or a vaginalised urogenital sinus with bladder agenesis or hypoplastic bladder [ 57 – 59 ] or congenital vesico-vaginal or vesico-uterine fistulas (pseudofistula with menuria [ 60 , 61 ]) and cloacal exstrophy [ 62 , 63 ] (Fig. 8 ).…”

Section: Diagnostic Imagingmentioning

confidence: 99%

Diagnostic imaging and cataloguing of female genital malformations

Acién

2016

Insights Imaging

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To help physicians and radiologists in the diagnosis of female genito-urinary malformations, especially of complex cases, the embryology of the female genital tract, the basis for Müllerian development anomalies, the current classifications for such anomalies and the comparison for inclusion and cataloguing of female genital malformations are briefly reviewed. The use of the embryological system to catalogue female genito-urinary malformations may ultimately be more useful in correlations with clinical presentations and in helping with the appropriate diagnosis and treatment. Diagnostic imaging of the different genito-urinary anomalies are exposed, placing particular emphasis on the anomalies within group II of the embryological and clinical classification (distal mesonephric anomalies), all of them associated with unilateral renal agenesis or dysplasia. Similarly, emphasis is placed on cases of cervico-vaginal agenesis, cavitated noncommunicated uterine horns, and cloacal and urogenital sinus anomalies and malformative combinations, all of them complex malformations. Diagnostic imaging for all these anomalies is essential. The best imaging tools and when to evaluate for other anomalies are also analysed in this review.Teaching points• The appropriate cataloguing of female genital malformations is controversial.• An embryological classification system suggests the best diagnosis and appropriate management.• The anomalies most frequently diagnosed incorrectly are the distal mesonephric anomalies (DMAs).• DMAs are associated with unilateral renal agenesis or renal dysplasia with ectopic ureter.• We analyse other complex malformations. Diagnostic imaging for these anomalies is essential.

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Section: Diagnostic Imagingmentioning

confidence: 99%

Diagnostic imaging and cataloguing of female genital malformations

Acién

2016

Insights Imaging

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“…The most common associated anomalies were those of the urinary system, including renal dysplasia/hypoplasia, pelvic kidneys, crossed fused renal ectopia, horseshoe kidneys, ectopic insertion of the ureters and urethral agenesis [ 2 , 3 , 5 – 7 , 10 , 13 – 15 ]. In females, most ectopic ureters drained to the vagina [ 2 , 8 , 14 , 16 , 17 ], urogenital sinus [ 5 , 6 , 18 ] orvestibule [ 7 , 13 , 19 , 20 ] and rarely to the skin [ 21 ]. In males, ureters drained to the anterior wall of the rectum [ 11 , 12 ], to the seminal vesicles [ 10 ] or to the prostatic urethra [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…In males, ureters drained to the anterior wall of the rectum [ 11 , 12 ], to the seminal vesicles [ 10 ] or to the prostatic urethra [ 9 ]. Various genital anomalies were also found in association with urinary bladder agenesis, including absent uterus [ 19 ], unicornuateand bicornuate uterus [ 2 , 18 ], vaginal agenesis [ 22 ] and penile agenesis [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…The diagnosis of bladder agenesis was made by MRI in most reported cases [ 6 , 8 – 10 , 12 , 14 , 16 ]. Few cases were diagnosed on CT [ 2 ], retrograde ureterogram [ 7 ], intravenous urography (IVU) [ 13 , 19 ] or surgery [ 11 , 18 ]. These results illustrate the role of MRI in the imaging workup of patients with ambiguous genitalia, as it can detail the anatomy of the urogenital system and assess for possible associated anomalies at the same time.…”

Section: Discussionmentioning

confidence: 99%

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A case of urinary bladder agenesis and bilateral ectopic ureters: a case report

et al. 2018

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BackgroundUrinary bladder agenesis is a very rare congenital anomaly with very few cases reported in the literature.Case presentationWe report a one-month-old baby presenting with ambiguous genitalia and recurrent urinary tract infections. Her clinical course was complicated by renal impairment. Magnetic resonant imaging (MRI) revealed a diagnosis of bladder agenesis with bilateral ectopic insertion of the ureters into the vagina, associated with several other anomalies. The patient underwent bilateral high anterior ureterostomies in an hospital abroad at 5.5 months of age. She then developed ureteral necrosis that had to be corrected with left pyeloplasty and by placing a left nephrostomy tube for drainage. Eventually, the patient’s renal function declined, and she developed chronic kidney disease (CKD).The case with its imaging findings and pathogenesis as well as a review of the literature are presented.ConclusionsUrinary bladder agenesis is a rare congenital condition that can be associated with multiple anomalies. Early diagnosis and therapeutic intervention can prevent progression to chronic kidney disease.

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“…Only seven subjects had a twin, five of whom were monozygotic [ 14 , 15 , 21 , 34 , 39 ], and one via in vitro fertilization [ 32 ]. One has hypospadias [ 21 ], one, similarly to their twin, has teratology of Fallot [ 14 ], and five were without complaint [ 15 , 29 , 32 , 37 , 45 ].…”

Section: Reviewmentioning

confidence: 99%

Bladder Agenesis: A Systematic Review

Yahya

2023

Cureus

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Bladder agenesis is a rare congenital deformity characterized by the absence of the bladder. It is primarily observed in postmortem dissections of stillbirths rather than live births. The condition is often associated with other congenital anomalies, leading to the hypothesis that most affected fetuses do not survive to term. However, the exact cause and specific associated anomalies remain unclear and poorly described in the literature. The limited mention of bladder agenesis in textbooks and literature underscores the importance of creating a comprehensive source for future research in this field. Therefore, our objective is to collect and analyze data on bladder agenesis, focusing on associated anomalies and potential causes, to enhance our understanding of the condition. We conducted a thorough review of reports collected from three databases, Google Scholar, PubMed, and Science Direct, last searched on July 30, 2023, starting with 327 reports. Excluding duplicates and records written in languages other than English, veterinary studies, irrelevant reports, or stillbirths. Inclusion criteria were the following: cases must have proven bladder agenesis, not hypoplasia, and must have most of the information, including the age of diagnosis, presenting symptoms, gender, associated anomalies, and management or outcome of the patient. A quality assessment was conducted according to the Joanna Briggs Institute checklist for case reports. A total of 65 case reports from 56 articles were included in the review. Through our manual analysis, we documented a wild array of malformations associated with bladder agenesis. Among the reports reviewed, 93% exhibited urinary system malformations beside bladder agenesis, 77% were found to have reproductive malformations, 44% had gastrointestinal anomalies, 38% showed musculoskeletal malformations, 28% had cardiac malformations, and another 28% had vascular anomalies. The overall mortality rate was 38%, with a higher rate of 74% for males compared to 20% for females. By collating and analyzing those case reports, we aim to contribute to a better understanding of bladder agenesis and its associated anomalies, facilitating further investigations and advancements in the field.

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Bilateral single system ectopic ureters opening into vestibule with bladder agenesis

Cited by 7 publications

References 11 publications

Diagnostic imaging and cataloguing of female genital malformations

Diagnostic imaging and cataloguing of female genital malformations

A case of urinary bladder agenesis and bilateral ectopic ureters: a case report

Bladder Agenesis: A Systematic Review

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