Abstract
Study Objective
To assess the clinical course of obstructive Müllerian anomalies found in girls after
menarche.
Design
A retrospective case series of adolescents who, between 2009 and 2016, were treated
for vaginal or uterine obstructive malformations diagnosed after menarche.
Setting
Division of Gynecology, Poznań University of Medical Sciences, Poznań, Poland.
Participants and Interventions
Twenty-two patients who, at the age range between 11.4 and 18.2 (median, 13.1) years,
between 2 and 74 (median 7.5) months after menarche, underwent surgical repair of
obstructive genital anomaly.
Main Outcome Measures
Müllerian defect type, presentation, radiologic findings, pre- and postoperative course.
Results
Eighteen patients (18 of 22; 81.8%) were diagnosed with obstructed hemivagina ipsilateral
renal anomaly syndrome. One patient (1 of 22; 4.5%) was diagnosed with uterus didelphys
and unilateral cervical atresia. Three patients (3 of 22; 13.6%) had unicornuate uterus
with a cavitated, noncommunicating rudimentary horn. The right side was affected in
13 patients (13 of 22; 59.1%), and the left side in 9 patients (9 of 22; 40.9%; P > .05). All but 1 patient had renal agenesis on the side of obstruction. Before repair
of the obstructive genital anomaly, 4 patients underwent unnecessary surgeries for
misdiagnosed ovarian cysts. Serious complications (pelvic inflammatory disease, vesicovaginal
fistula) occurred in 2 patients with microperforated pyocolpos. Pelvic endometriosis
was found in 4 of our patients.
Conclusion
Our case series suggests that obstructed hemivagina ipsilateral renal anomaly syndrome
is the most common obstructive Müllerian anomaly diagnosed in adolescents after menarche.
The differential diagnosis for unilateral kidney agenesis accompanied by dysmenorrhea
in adolescent girls should include obstructive genital tract anomaly. Accurate diagnosis
of an obstructive genital anomaly early after menarche might help prevent unnecessary
surgeries and infection-related complications. Meanwhile, prompt surgical correction
of an obstructive genital tract anomaly results in relief of symptoms and might reduce
the risk of endometriosis.
Key Words
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of Pediatric and Adolescent GynecologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities.Geburtshilfe Frauenheilkd. 1971; 31: 430
- Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome).J Pediatr Surg. 2006; 41: 987
- Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up.Fertil Steril. 2007; 87: 918
- Unusual form of genital malformation with aplasia of the right kidney.Zentralbl Gynakol. 1976; 98: 559
- Clinical implications of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in the prepubertal age group.PLoS One. 2016; 11: e0166776
- Obstructed hemivagina with ipsilateral renal anomaly.J Pediatr Adolesc Gynecol. 2015; 29: 7
- Clinical characteristics and treatment of Herlyn-Werner-Wunderlich syndrome.Arch Gynecol Obstet. 2014; 290: 947
- New classification of Herlyn-Werner-Wunderlich syndrome.Chin Med J (Engl). 2015; 128: 222
- Case series of laparoscopically resected noncommunicating functional uterine horns.J Pediatr Adolesc Gynecol. 2009; 22: e23
- Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases.Hum Reprod. 2013; 28: 1580
- Laparoscopic removal of the cavitated noncommunicating rudimentary uterine horn: surgical aspects in 10 cases.Fertil Steril. 2005; 83: 432
- Delayed diagnosis of Herlyn-Werner-Wunderlich syndrome due to microperforation and pyocolpos in obstructed vaginal canal.J Pediatr Adolesc Gynecol. 2014; 27: e79
- NASPAG Committee Opinion: Obstructive reproductive tract anomalies.J Pediatr Adolesc Gynecol. 2014; 27: 396
- The presentation and early diagnosis of the rudimentary uterine horn.Obstet Gynecol. 2005; 105: 1456
- Asymmetric lateral distribution and renal agenesis in women with uterus didelphys: institutional case series and a systemic literature review.Fertil Steril. 2007; 87: 719
- Unilateral renal agenesis and female genital tract pathologies.Acta Obstet Gynecol Scand. 2010; 89: 1424
- Endometriosis in association with Herlyn-Werner-Wunderlich syndrome.Fertil Steril. 2014; 201: 790
- Persistence of endometriosis after correction of an obstructed reproductive tract anomaly.J Pediatr Adolesc Gynecol. 2013; 26: e93
Article info
Publication history
Published online: October 10, 2017
Footnotes
The authors indicate no conflicts of interest.
Identification
Copyright
© 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc.