Persistent Urogenital Sinus

Singh, Sonia; Singh, Poonam; Singh, Ravinder

doi:10.1016/s0003-2778(10)80034-6

Cited by 19 publications

(16 citation statements)

References 15 publications

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“…Many syndromes can be associated with PUGS, such as the Mckusick-Kaufman syndrome (MKS), the Bardet-Biedl syndrome (BBS), and the handfoot-genital syndrome. 1 • The MKS is characterized by hydrometrocolpos (dilatation of the vagina and uterus due to accumulation of cervical mucus because of maternal estrogen stimulation), postaxial polydactyl (the presence of additional fingers in ulnar part of the hand, and in the fibular part of the foot), and congenital heart disease (atrioventricular canal, ventricular septal defect, and hypoplastic left heart, seen in 10-20% of reported cases). • In BBS, PUGS is correlated with postaxial polydactyl, progressive rod-cone dystrophy, obesity, renal abnormalities, hypogenitalism mental retardation, and mental development delay.…”

Section: Associated Syndromesmentioning

confidence: 99%

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

et al. 2015

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Section: Associated Syndromesmentioning

confidence: 99%

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

et al. 2015

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“…2 Urogenital sinus occurs as a result of the arrested migration of the Mullerian ducts from the Muller tubercle to the vestibule. 3…”

Section: Description Of Diseasementioning

confidence: 99%

Urogenital Sinus with Mitrofanoff Procedure: Case Review

David¹,

Menaka²,

Subramaniam³

et al. 2019

Journal of Health and Allied Sciences NU

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Urogenital sinus is a rare congenital anomaly. We reviewed a case of urogenital sinus anomaly in 6-year-old girl with recurrent urinary tract infection and small bladder capacity who was referred from another hospital in Coimbatore for further management. The external genitalia appeared normal, and an initial sonogram and repeat micturating cystourethrograms did not indicate any urogenital anomalies. She therefore underwent clean intermittent catheterization. Three years later the child underwent investigations like urodynamic study (UDS) and dimercaptosuccinic acid (DMSA) scan and cystoscopy, followed by ureteric implantation and Mitrofanoff procedure. The presentation of urogenital sinus anomaly with recurrent urinary tract infection is rare and the management is complex.

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“…1 The incidence of this rare congenital anomaly is estimated to be six in every 100,000 female births. 2 While PUGS can present as an isolated anomaly, it has also been associated with other diseases, including congenital adrenal hyperplasia or McKusick-Kaufman syndrome. [3][4][5] The presence of PUGS along with digit anomalies, retinitis pigmentosa, obesity, learning disabilities and male hypogenitalism are phenotypical characteristics of Bardet-Biedl syndrome.…”

Section: ‫عن‬ ‫حالة‬ ‫ثقرير‬ ‫هذا‬ ‫اجلنيني‬ ‫التطور‬ ‫خالل‬ ‫للف�صلmentioning

confidence: 99%

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

Cited by 19 publications

References 15 publications

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

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

Urogenital Sinus with Mitrofanoff Procedure: Case Review

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

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