Persistent Urogenital Sinus: Diagnostic Imaging for Clinical Management. What Does the Radiologist Need to Know?

Valentini, Anna Lia; Giuliani, Michela; Gui, Benedetta; Laino, Maria Elena; Zecchi, Viola; Rodolfino, Elena; Ninivaggi, Valeria; Manzoni, Carlo; Bonomo, Lorenzo

doi:10.1055/s-0035-1565996

Cited by 13 publications

(19 citation statements)

References 23 publications

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“… [6] It can also be associated with syndromes, including MC-Kusick-Kaufman syndrome, Bardet-Biedel syndrome, and hand-foot-genital syndrome. [7] PUG was associated with uterus didelphys and double vaginal malformation in this case. The urethra and 2 vaginas formed a common channel.…”

Section: Discussionsupporting

confidence: 56%

Multimodal ultrasound imaging of persistent urogenital sinus with uterus didelphys and double vagina malformation

Wang

2021

Medicine

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Rationale: Persistent urogenital sinus (PUG) with uterus didelphys and double vagina is a rare urogenital anomaly. The diagnosis is based on magnetic resonance examination and cystoscopy. To the best of our knowledge, there is no literature report of PUG diagnosed by ultrasound alone. Patient concern: A 23-year-old woman presented with atypical menstruation and recurrent hematuria for 13 years and recurrent lower abdominal pain for 12 years. Diagnosis: PUG was diagnosed through multiple ultrasound modalities, including transabdominal 2-dimensional ultrasound, transrectal bi-plane high-frequency ultrasound, and contrast-enhanced ultrasound. We diagnosed this malformation preoperatively by accurately measuring the length of urethra and common channel through multimodal ultrasound imaging. Interventions: Urethra separation and reconstruction, vaginal pull-through and artificial vaginoplasty, and bilateral hysterosalpingectomy were performed. Outcomes: The postoperative course was uneventful. She was urinating normally after half a year and used continuous vaginal dilatation to avoid stenosis. Lessons: PUG associated with uterus didelphys and double vagina is an extremely rare malformation of the reproductive system. Multimodal ultrasound imaging can be used to diagnose this malformation preoperatively clearly and to accurately measure the length of urethra and common channel, providing an imaging basis for preparing an operative plan.

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Section: Discussionsupporting

confidence: 56%

Multimodal ultrasound imaging of persistent urogenital sinus with uterus didelphys and double vagina malformation

Wang

2021

Medicine

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“…common orifice of the vagina and urethra, while the rectum, anal canal, and anus are well developed. 11 Although anomalies of the urogenital sinus may be sporadic and isolated, sometimes they can be found in association with other anomalies and/or as a part of complex syndromes. 12,13 Most common associated anomalies are renal agenesis, hydronephrosis, polycystic kidneys, uterine anomalies (cervical atresia, bicornuate uterus), vaginal duplication or atresia, esophageal atresia, imperforate anus, and sacral hypoplasia.…”

Section: Discussionmentioning

confidence: 99%

“…9 Moreover, they may be associated with several syndromes, such as the Bardet-Biedl syndrome and the Mckusick-Kaufman syndrome 10 or, in rare cases, ascribed to an arrest of normal cloacal development. 11 Early diagnosis of urogenital sinus anomalies is critical since hydrometrocolpos may determine urinary retention, dilatation of upper urinary tract, and compressive syndrome.…”

Section: Introductionmentioning

confidence: 99%

Perinatal diagnosis of congenital urogenital sinus abnormality

Fiorentino

Bella

Cauzzo

et al. 2023

Congenital Anomalies

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Anomalies of the urogenital sinus, which is a transient feature of the early human embryological development, are rare birth defects. Urogenital sinus abnormalities commonly present as pelvic masses, hydrometrocolpos, or ambiguous genitalia and most commonly occur within the context of congenital adrenal hyperplasia. Anomalies of the urogenital sinus requires surgical repair. We experienced a case of a female newborn with congenital urogenital sinus abnormality in which the early diagnosis helped us to prevent complications by decompressing the vagina soon after birth. Antibiotic prophylaxis was sufficient to avoid infections and to decompress the genitourinary system, thus allowing a deferred elective surgery to correct the sinus.

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“…The presence of a cystic lesion with internal debris posterior to the fetal bladder-which can be detected by sonogram as early as the second trimester of pregnancy-is suggestive of a PUGS. 11 A postnatal ultrasound with similar findings may also help to confirm the diagnosis. In the present case, a postnatal ultrasound when the patient was seven days old did not show any abnormalities; however, this might be due to the fact that the urocolpos had been drained as a result of CIC having been performed prior to the ultrasound.…”

Section: Case Reportmentioning

confidence: 91%

A Case of Persistent Urogenital Sinus: Pitfalls and challenges in diagnosis

Tan

Shunmugan

et al. 2018

Sultan Qaboos Univ Med J

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Persistent urogenital sinus (PUGS) is a rare anomaly whereby the urinary and genital tracts fail to separate during embryonic development. We report a three-year-old female child who was referred to the Sabah Women & Children Hospital, Sabah, Malaysia, in 2016 with a pelvic mass. She had been born prematurely at 36 gestational weeks via spontaneous vaginal delivery in 2013 and initially misdiagnosed with neurogenic bladder dysfunction. The external genitalia appeared normal and an initial sonogram and repeat micturating cystourethrograms did not indicate any urogenital anomalies. She therefore underwent clean intermittent catheterisation. Three years later, the diagnosis was corrected following the investigation of a persistent cystic mass posterior to the bladder. At this time, a clinical examination of the perineum showed a single opening into the introitus. Magnetic resonance imaging of the pelvis revealed gross hydrocolpos and a genitogram confirmed a diagnosis of PUGS, for which the patient underwent surgical separation of the urinary and genital tracts.

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Persistent Urogenital Sinus: Diagnostic Imaging for Clinical Management. What Does the Radiologist Need to Know?

Cited by 13 publications

References 23 publications

Multimodal ultrasound imaging of persistent urogenital sinus with uterus didelphys and double vagina malformation

Multimodal ultrasound imaging of persistent urogenital sinus with uterus didelphys and double vagina malformation

Perinatal diagnosis of congenital urogenital sinus abnormality

A Case of Persistent Urogenital Sinus: Pitfalls and challenges in diagnosis

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