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CASE REPORT
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome–A “no-touch-approach”: A case report
1
Department of obstetrics and gynecology, Imam Abdulrahman Bin Faisal University, Dammam, SAUDI ARABIA
Online publication date: 2023-09-24
Publication date: 2023-11-01
Electron J Gen Med 2023;20(6):em544
KEYWORDS
ABSTRACT
Introduction:
Herlyn-Werner-Wunderlich (HWW)/obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare Müllerian duct anomaly (MDA). Usually presenting after menarche classically with dysmenorrhea. Here we present a challenging case, where the diagnosis was missed initially.
Case presentation:
A 10-year-old girl presenting with dysmenorrhea and constipation, treated repeatedly with analgesics, laxatives and enema. A pelvic ultrasound and magnetic resonance imaging (MRI) confirmed the diagnosis of OHVIRA syndrome, which requires surgical intervention. We describe a “no -touch- technique” for resecting the septum. The patient had immediate relief of symptoms and a smooth post-operative recovery.
Conclusions:
Early diagnosis and surgical management are crucial not only to relieve symptoms but to prevent long term complications that may affect the patients’ reproductive potential. Despite pelvic ultrasound being an affordable and a useful diagnostic tool, the diagnosis can be missed by inexperienced operators. Therefore, MRI is recommended to confirm and classify MDAs. The hysteroscopic “no-touch-technique” to resect the septum is an effective, safe and a convenient approach to treat the condition while preserving hymen integrity.
Herlyn-Werner-Wunderlich (HWW)/obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare Müllerian duct anomaly (MDA). Usually presenting after menarche classically with dysmenorrhea. Here we present a challenging case, where the diagnosis was missed initially.
Case presentation:
A 10-year-old girl presenting with dysmenorrhea and constipation, treated repeatedly with analgesics, laxatives and enema. A pelvic ultrasound and magnetic resonance imaging (MRI) confirmed the diagnosis of OHVIRA syndrome, which requires surgical intervention. We describe a “no -touch- technique” for resecting the septum. The patient had immediate relief of symptoms and a smooth post-operative recovery.
Conclusions:
Early diagnosis and surgical management are crucial not only to relieve symptoms but to prevent long term complications that may affect the patients’ reproductive potential. Despite pelvic ultrasound being an affordable and a useful diagnostic tool, the diagnosis can be missed by inexperienced operators. Therefore, MRI is recommended to confirm and classify MDAs. The hysteroscopic “no-touch-technique” to resect the septum is an effective, safe and a convenient approach to treat the condition while preserving hymen integrity.
REFERENCES (20)
1.
Zhu L, Chen N, Tong JL, et al. New classification of Herlyn-Werner-Wunderlich syndrome. Chin Med J. 2015; 128(2):222-25. https://doi.org/10.4103/0366-6... PMid:25591566 PMCid:PMC4837842.
2.
Purslow CE. A case of unilateral hæmatokolpos, hæmatometra and hæmatosalpinx. Int J Obstet Gynaecol. 1922;29:643. https://doi.org/10.1111/j.1471....
3.
Acién P, Acién M. The presentation and management of complex female genital malformations. Hum Reprod Update. 2016;22(1):48-69. https://doi.org/10.1093/humupd... PMid:26537987.
4.
Buttram Jr VC, Gibbons WE. Müllerian anomalies: A proposed classification (an analysis of 144 cases). Fertil Steril. 1979;32(1):40-6. https://doi.org/10.1016/S0015-... PMid:456629.
5.
Golan A, Langer R, Bukovsky I, Caspi E. Congenital anomalies of the Müllerian system. Fertil Steril. 1989;51(5):747-55. https://doi.org/10.1016/S0015-... PMid:2651163.
6.
Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: Management and follow-up. Fertil Steril. 2007;87(4):918-22. https://doi.org/10.1016/j.fert... PMid:17320871.
7.
Fedele L, Motta F, Frontino G, Restelli E, Bianchi S. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: Pelvic anatomic variants in 87 cases. Hum Reprod. 2013;28(6):1580-3. https://doi.org/10.1093/humrep... PMid:23532323.
8.
Gupta N, Gandhi D, Gupta S, Goyal P, Li S, Kumar Y. A variant of Herlyn-Werner-Wunderlich syndrome presenting with acute abdomen: a case report and review of literature. Glob Pediatr Health. 2018;5:2333794X18769817. https://doi.org/10.1177/233379... PMid:29761137 PMCid:PMC5946351.
9.
Mandava A, Prabhakar R, Smitha S. OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol. 2012;25(2):e23-5. https://doi.org/10.1016/j.jpag... PMid:22421561.
10.
Shavell VI, Montgomery SE, Johnson SC, Diamond MP, Berman JM. Complete septate uterus, obstructed hemivagina, and ipsilateral renal anomaly: Pregnancy course complicated by a rare urogenital anomaly. Arch Gynecol Obstet. 2009;280(3):449-52. https://doi.org/10.1007/s00404... PMid:19132379.
11.
Bajaj SK, Misra R, Thukral BB, Gupta R. OHVIRA: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis: Advantage MRI. J Hum Reprod Sci. 2012;5(1):67-7. https://doi.org/10.4103/0974-1... PMid:22870020 PMCid:PMC3409925.
12.
Zhang H, Qu H, Ning G, et al. MRI in the evaluation of obstructive reproductive tract anomalies in paediatric patients. Clin Radiol. 2017;72(7):612e7-15. https://doi.org/10.1016/j.crad... PMid:28283284.
13.
Lacy J, Correll GR, Walmer DK, Price TM. Simple vaginal mold for use in the postoperative care of patients with a transverse vaginal septum. Fertil Steril. 2007;87(5):1225-6. https://doi.org/10.1016/j.fert... PMid:17418828.
14.
Xu B, Xue M, Xu D. Hysteroscopic management of an oblique vaginal septum in a virgin girl with a rare variant of Herlyn-Werner-Wunderlich syndrome. J Minim Invasive Gynecol. 2015; 22(1):7. https://doi.org/10.1016/j.jmig... PMid:25111519.
15.
Lee C-L, Wang C-J, Swei L-D, Yen C-F, Soong Y-K. Laparoscopic hemi-hysterectomy in treatment of a didelphic uterus with a hypoplastic cervix and obstructed hemivagina: Case report. Human Reprod. 1999;14(7):1741-3. https://doi.org/10.1093/humrep... PMid:10402379.
16.
Fachin CG, Sampaio Rocha JLA, Maltoni AA, et al. Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature. Int J Surg Case Rep. 2019;63:129-34. https://doi.org/10.1016/j.ijsc... PMid:31586891 PMCid:PMC6796699.
17.
Cheng C, Subedi J, Zhang A, et al. Vaginoscopic incision of oblique vaginal septum in adolescents with OHVIRA syndrome. Sci Rep. 2019;9:20042. https://doi.org/10.1038/s41598... PMid:31882725 PMCid:PMC6934495.
18.
AlMulhim J, AlRasheed MR. Herlyn-Werner-Wunderlich syndrome with borderline serous cystadenoma of the ovary: Case report and literature review. Radiol Case Rep. 2020;16(3):744-7. https://doi.org/10.1016/j.radc... PMid:33532015 PMCid:PMC7835497.
19.
Al Ghafri A, Fida A, Al-Gharras A. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Oman Med J. 2018;33(1):69-71. https://doi.org/10.5001/omj.20... PMid:29468003 PMCid:PMC5798792.
20.
Saleh A, Alrashid M, Al Hindi S. Acute abdomen: Unusual presentation of Herlyn-Werner Wunderlich syndrome. Inter J Surg Case Rep. 2021;82:105911. https://doi.org/10.1016/j.ijsc... PMid:33962268 PMCid:PMC8121687.
| eISSN: | 2516-3507 |
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