Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2018 Sep;21(3):249-252.
doi: 10.1007/s40477-018-0290-9. Epub 2018 Mar 3.

A rare case of hydrometrocolpos from persistent urogenital sinus in patient affected by adrenogenital syndrome

Igino Simonetti  1 , Piero Trovato  2 , Francesco Verde  2 , Luca Tarotto  2 , Roberto Della Casa  3 , Maria Concetta Lonardo  3 , Gianfranco Vallone  4 , Maria Grazia Caprio  4   5
Affiliations
Case Reports

A rare case of hydrometrocolpos from persistent urogenital sinus in patient affected by adrenogenital syndrome

Igino Simonetti et al. J Ultrasound. 2018 Sep.

Abstract
in English, Italian

Persistent urogenital sinus (PUGS) is a congenital pathological condition characterized by an abnormal communication between the urethra and vagina, which has an estimated incidence of 0.6/10,000 female births. It could be the only known malformation or part of a syndrome. PUGS is commonly shown by a pelvic mass, related to a distended bladder, hydrometrocolpos which is due to an obstruction leading to the dilation of the vagina and uterus (i.e., imperforate hymen, transverse vaginal septum or atresia, and PUGS) or both. We present a case of female patient with classical congenital adrenal hyperplasia, diagnosed on the 7th day of life, with ambiguous genitalia, untreated surgically only with hormone therapy by parental decision. The patient, at the age of 5 years and 5 months, came to our observation for a pelvic ultrasound, which revealed retrovesical neoformation with anechoic content and regular walls. We performed the ultrasound examination that showed the dilation of the cervix and the vaginal canal with anechoic finely corpuscolated content in the declining portion, compatible with hydrometrocolpos from probable persistence of the urogenital sinus. The voiding cystourethrography (VCUG) confirmed the ultrasound diagnosis, with evidence of urogenital sinus. In conclusion, ultrasound is the first diagnostic tool, but need to be completed by other technical procedures, which VCUG or magnetic resonance imaging to observe the site of fusion of the urinary and genital tract.

La persistenza del seno urogenitale (PUGS) è una rara condizione patologica congenita, caratterizzata da un’anomala comunicazione tra uretra e vagina, la cui incidenza è di 0.6/10.000 neonate. Clinicamente, può rappresentare l’unica malformazione rilevabile o far parte di un quadro sindromico. Si presenta comunemente come una massa pelvica, associata a distensione vescicale e/o idrometrocolpo, ossia dilatazione della vagina e dell’utero causata dall’accumulo di secrezioni cervicali conseguente ad un’ostruzione (imene imperforato, setto vaginale trasverso, atresia vaginale e PGUS). Viene presentato il caso di una paziente di sesso femminile, affetta da Sindrome Adreno-Genitale (SAG) forma classica, diagnosticata al 7° giorno di vita, con genitali ambigui (ipertrofia clitoridea, grandi labbra scrotalizzate) non trattati chirurgicamente per decisione parentale, in terapia ormonale sostitutiva. La paziente, all’età di 5 anni e 5 mesi, giunge alla nostra osservazione per una richiesta di ecografia della regione pelvica richiesta, come consulenza, per una neoformazione retrovescicale a contenuto anecogeno e pareti regolari. L’esame ecografico ha mostrato una dilatazione del collo e del canale vaginale a contenuto anecogeno finemente corpuscolato nella porzione declive, compatibile con idrometrocolpo da verosimile persistenza del seno urogenitale. La cistouretrografia ha confermato il reperto ecografico, con evidenza del seno uro-genitale. In conclusione, l’ecografia è la metodica di prima istanza utilizzata, tuttavia necessita di ulteriori metodiche, quali la Cistouretrografia e/o la Risonanza Magnetica per rilevare il punto di comunicazione tra il tratto urinario ed il tratto genitale.

Keywords: Hydrometrocolpos; Magnetic resonance imaging (MRI); Persistent urogenital sinus (PUGS); Ultrasound (US); Voiding cystourethrography (VCUG).

PubMed Disclaimer

Conflict of interest statement

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Human and animal rights

This article does not contain any studies with animals performed by any of the authors.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Figures

Fig. 1
Fig. 1
Longitudinal sonographic image of pelvis demonstrates a dilatation of the cervix and of the vaginal canal (Dl: 69 mm, Dap: 32 mm) with anechoic finely corpuscolated content in the declining portion
Fig. 2
Fig. 2
Transverse sonographic image of pelvis demonstrates a dilatation of the cervix and of the vaginal canal (Dap: 32 mm, Dt: 45 mm) with anechoic finely corpuscolated content in the declining portion
Fig. 3
Fig. 3
Voiding cystourethrography (VCUG), lateral view: after the opacification of the bladder and the vagina, the contrast medium shows an oblong structure which is the vagina overcame by the uterine cavity (urogenital sinus) of about 4 cm
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Valentini AL, Giuliani M, Gui B, Laino M, Zecchi V, Rodolfino E, Ninivaggi V, Manzoni C, Bonomo L. Persistent urogenital sinus: diagnostic imaging for clinical management. What does the radiologist need to know? Am J Perinatol. 2016;33:425–432. - PubMed
    1. Kitta T, Kakizaki H, Iwami D, Tanda K. Successful bladder management for a pure urogenital sinus anomaly. Int J Urol. 2004;11:340–342. doi: 10.1111/j.1442-2042.2004.00785.x. - DOI - PubMed
    1. Taori K, Krishnan V, Sharbidre KG, Andhare A, Kulkarni BR, Bopche S, Patil V. Prenatal sonographic diagnosis of fetal persistent urogenital sinus with congenital hydrocolpos. Ultrasound Obstet Gynecol. 2010;36:641–643. doi: 10.1002/uog.7721. - DOI - PubMed
    1. Vitale V, Cigliano B, Vallone G. Imperforate hymen causing congenital hydrometrocolpos. J Ultrasound. 2013;16:37–39. doi: 10.1007/s40477-013-0009-x. - DOI - PMC - PubMed
    1. Sidatt M, Wedih AOSM, Boubaccar AO, Litime AOE, Feil A, Moussa AO. Hydrocolpos and hydrometrocolpos in newborns. Arch pédiatrie organe Off la Sociéte Fr pédiatrie. 2013;20:176–180. doi: 10.1016/j.arcped.2012.11.002. - DOI - PubMed

Publication types

MeSH terms

Supplementary concepts

LinkOut - more resources