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

Abstract

Background Persistent urogenital sinus (PUGS) is a congenital pathological condition characterized by an abnormal communication between the urethra and vagina. It may be a part of a complex syndrome and can be more often associated with congenital malformations affecting the genitourinary tract system (33%) such as intersex, rectovaginal communication, bladder agenesis, absence of vagina, and hydrocolpos. The correct radiological assessment of PUGS is especially useful for clinicians since the exact anatomical evaluation of this abnormality is a crucial factor for surgical planning. The imaging study modalities, which are essentially based on ultrasonography, voiding cystourethrography, and magnetic resonance imaging, could be misinterpreted if not correctly performed.

Aim The aim of this article is to highlight this rare pathological condition and to help general radiologists in achieving the correct technical approach for the diagnosis. Special attention will be paid in discussing the role of different imaging modalities and their contribution to the diagnosis and clinical management of patients.

Keywords

magnetic resonance imaging - ultrasound - urogenital sinus - voiding cystourethrography

• References

• 1 Singh S, Singh P, Singh RJ. Persistent urogenital sinus. J Anat Soc India 2010; 59 (02) 242-244
• 2 Kitta T, Kakizaki H, Iwami D, Tanda K. Successful bladder management for a pure urogenital sinus anomaly. Int J Urol 2004; 11 (05) 340-342
• 3 Patankar JZ, Vidyadhar M, Prabhakaran K, Bo L, Lsk Loh D. Urogenital sinus, rectovaginal fistula, and an anterior stenosed anus—another cloacal variant. Pediatr Surg Int 2004; 20 (07) 556-558
• 4 Savanelli A, Esposito C, Tilemis S, Franzese A, Guys J, Settimi A. Persistent urogenital sinus with bladder agenesis and absence of vagina. BJU Int 2003; 92 (Suppl. 03) e48-e49
• 5 Taori K, Krishnan V, Sharbidre KG. , et al. Prenatal sonographic diagnosis of fetal persistent urogenital sinus with congenital hydrocolpos. Ultrasound Obstet Gynecol 2010; 36 (05) 641-643
• 6 Alamo L, Meyrat BJ, Meuwly JY, Meuli RA, Gudinchet F. Anorectal Malformations: Finding the Pathway out of the Labyrinth. Radiographics 2013; 33 (02) 491-512
• 7 Goglia G. Embriologia umana. Padova: Piccin; 1997: 231-233 ; 247–248
• 8 Kenney PJ, Spirt BA, Leeson MD. Genitourinary anomalies: Radiologic anatomic correlations. Radiographics 1984; 4 (02) 233-260
• 9 Berrocal T, López-Pereira P, Arjonilla A, Gutiérrez J. Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. Radiographics 2002; 22 (05) 1139-1164
• 10 Powell DM, Newman KD, Randolph J. A proposed classification of vaginal anomalies and their surgical correction. J Pediatr Surg 1995; 30 (02) 271-275 , discussion 275–276
• 11 Rink RC, Adams MC, Misseri R. A new classification for genital ambiguity and urogenital sinus anomalies. BJU Int 2005; 95 (04) 638-642
• 12 Geifman-Holtzman O, Crane SS, Winderl L, Holmes M. Persistent urogenital sinus: prenatal diagnosis and pregnancy complications. Am J Obstet Gynecol 1997; 176 (03) 709-711
• 13 Cherian MP, Al-Sanna'a NA, Ayyat FM. Hydrometrocolpos and acute renal failure: a rare neonatal presentation of Bardet-Biedl syndrome. J Pediatr Urol 2008; 4 (04) 313-316
• 14 Innis JW. Hand-Foot-Genital Syndrome. In: Pagon RA, Adam MP, Ardinger HH. , et al, eds. GeneReviews. Seattle: University of Washington; 2006: 1993-2014
• 15 Gul A, Yildirim G, Gedikbasi A, Gungorduk K, Ceylan Y. Prenatal ultrasonographic features of persistent urogenital sinus with hydrometrocolpos and ascites. Arch Gynecol Obstet 2008; 278 (05) 493-496
• 16 Rink RC, Metcalfe PD, Cain MP, Meldrum KK, Kaefer MA, Casale AJ. Use of the mobilized sinus with total urogenital mobilization. J Urol 2006; 176 (05) 2205-2211
• 17 Sultan C, Paris F, Jeandel C, Lumbroso S, Galifer RB. Ambiguous genitalia in the newborn. Semin Reprod Med 2002; 20 (03) 181-188
• 18 Arena F, Romeo C, Cruccetti A, Antonuccio P, Basile M, Romeo G. The neonatal management and surgical correction of urinary hydrometrocolpos caused by a persistent urogenital sinus. BJU Int 1999; 84 (09) 1063-1068
• 19 Picone O, Laperelle J, Sonigo P, Levaillant JM, Frydman R, Senat MV. Fetal magnetic resonance imaging in the antenatal diagnosis and management of hydrocolpos. Ultrasound Obstet Gynecol 2007; 30 (01) 105-109
• 20 Dhombres F, Jouannic JM, Brodaty G, Bessiere B, Daffos F, Bénifla JL. Contribution of prenatal imaging to the anatomical assessment of fetal hydrocolpos. Ultrasound Obstet Gynecol 2007; 30 (01) 101-104
• 21 Chavhan GB, Parra DA, Oudjhane K, Miller SF, Babyn PS, Pippi Salle FL. Imaging of ambiguous genitalia: classification and diagnostic approach. Radiographics 2008; 28 (07) 1891-1904
• 22 Riccabona M. Cystography in infants and children: a critical appraisal of the many forms with special regard to voiding cystourethrography. Eur Radiol 2002; 12 (12) 2910-2918
• 23 Adams ME, Hiorns MP, Wilcox DT. Combining MDCT, micturating cystography, and excretory urography for 3D imaging of cloacal malformation. AJR Am J Roentgenol 2006; 187 (04) 1034-1035
• 24 Siegelman ES, Outwater EK, Banner MP, Ramchandani P, Anderson TL, Schnall MD. High-resolution MR imaging of the vagina. Radiographics 1997; 17 (05) 1183-1203
• 25 Ryu J, Kim B. MR imaging of the male and female urethra. Radiographics 2001; 21 (05) 1169-1185
• 26 Hinman Jr F. Vaginoplasty and clitoroplasty for the adrenogenital syndrome. In: Hinman Jr F. eds. Atlas of Pediatric Urologic Surgery. Philadelphia: WB Saunders Company; 1994: 635-648