|Year : 2019 | Volume
| Issue : 2 | Page : 88-90
OHVIRA syndrome in an infant girl
Ruba Sahab1, Sherif Mohamed El-Desoky2, Ahmed Alsayyad3, Mazen Kurdi4, Jameela A Kari2
1 Department of Pediatrics, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia
2 Department of Pediatrics; Pediatric Nephrology Center of Excellence, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia
3 Department of Urology, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia
4 Department of Surgery, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia
|Date of Web Publication||4-Dec-2019|
Jameela A Kari
Pediatric Nephrology Center of Excellence, Department of Pediatrics, King Abdulaziz University, PO Box 80215, Jeddah 21589
Kingdom of Saudi Arabia
Source of Support: None, Conflict of Interest: None
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital urogenital anomaly, which usually presents after menarche. We report a 3-month-old infant who presented with recurrent urinary tract infections (UTI) and had an abdominal lump. Radiological investigations revealed absent right kidney, hydronephrotic left kidney, and a cystic pelvic mass. Exploratory laparotomy revealed hydrometrocolpos which was evacuated. During 1-year follow-up, hydronephrosis decreased, there were no further UTI, and kidney function remained normal. Our case illustrates that OHVIRA syndrome may present in early infancy with UTI, renal anomalies, and pelvic mass.
Keywords: Obstructed hemivagina, obstructed hemivagina and ipsilateral renal agenesis, renal agenesis, single kidney
|How to cite this article:|
Sahab R, El-Desoky SM, Alsayyad A, Kurdi M, Kari JA. OHVIRA syndrome in an infant girl. Asian J Pediatr Nephrol 2019;2:88-90
FNx01These authors contributed equally
| Introduction|| |
OHVIRA syndrome is a rare Müllerian duct anomaly, characterized by a triad of uterus didelphys, obstructed hemi-vagina and ipsilateral renal agenesis. First reported in 1922, the condition is also known as Herlyn–Werner–Wunderlich syndrome. Müllerian duct anomalies affect 0.8%–4% live births, and OHVIRA syndrome accounts for 0.1%–3.5% of all Müllerian anomalies. The condition is considered to have unclear but multifactorial etiology.
Renal agenesis is the most commonly reported urologic anomaly in OHVIRA syndrome, although other malformations, including renal duplication and multicystic dysplastic kidney, have also been described. Most patients present after menarche with pelvic or abdominal pain. Rarely, presentation is delayed to adulthood, with primary infertility, pyometra, urinary obstruction, and ischiorectal swelling., The condition is diagnosed upon imaging, most commonly ultrasound, or computed tomography, although magnetic resonance imaging (MRI) has recently emerged as the most sensitive diagnostic tool. We report an OHVIRA case presented in early infancy.
| Case Report|| |
A 3-month-old Filipino girl was brought to the emergency room with intermittent fever and progressive difficulty in passing urine for 2 weeks, associated with abdominal distension, nonbilious vomiting, and loose stools. She also had brief episodes of generalized tonic seizures. Urinalysis confirmed urinary tract infection (UTI) with Enterobacter faecalis. She subsequently suffered recurrent episodes of Gram-negative UTI associated with sepsis and acute kidney injury, with serum creatinine reaching up to 100 μmol/L.
An abdominal radiograph, performed to rule out intestinal obstruction, showed opacity in the pelvis with paucity of bowel gas and absent rectal gas, suggesting a pelvic mass. Abdominal ultrasonography indicated absence of right kidney and severe hydronephrosis of left kidney [Figure 1]a with dilated hydroureter [Figure 1]c. Both on radiograph and ultrasound, the tip of the Foley catheter was above the right iliac fossa, suggesting distended urinary bladder; ultrasound also indicated that it was displaced anteriorly and to the right side. Micturating cystourethrography showed that the urinary bladder had a smooth wall and size but was pushed to the right side of the pelvis by midline opacity; there was no vesicoureteric reflux [Figure 1]b and [Figure 1]d.
|Figure 1: Radiological images. Ultrasonography showing (a) severe left hydronephrosis and (c) hydroureter with distended urinary bladder. (b) Anterior and (d) lateral films on micturating cystourethrography showing smoothwalled urinary bladder that is displaced anteriorly and to the right side by a midline pelvic opacity, also causing a filling defect. Magnetic resonance imaging seen in (e) and (f), showing a large central pelvic structure with no definite communications, indicates the obstructed hemivagina (V). The urinary bladder (UB) is displaced to the right side of the pelvis, and there is severe left hydroureter (U) and hydronephrosis. The right kidney, ovaries and fallopian tubes are not visualized|
Click here to view
Diuretic renography using technetium99m labeled mercaptoacetyltriglycine showed the absence of uptake on the right side, satisfactory perfusion and outflow without evidence of obstruction in the left hydronephrotic kidney [Figure 2]a, displacement of the urinary bladder to the right side of the pelvis, and no holdup of tracer in the bladder [Figure 2]b. Dimercaptosuccinic acid radionuclide scan confirmed the absence of right kidney and normal tracer distribution on the left kidney. Gastrografin enema indicated normal appearance of the large intestine with minor compression on the rectum anteriorly, without evidence of bowel obstruction. MRI showed a large midline cystic structure, measuring 4 cm × 7 cm × 4.4 cm, in the pelvis [Figure 1]e and [Figure 1]f. This structure peaked inferiorly and seemed to communicate with another small structure anteriorly, possibly the uterus. Cystoscopy with cystogram showed that the urinary bladder was displaced to the right side, but there was no fistulous communication with the pelvic mass.
|Figure 2: Diuretic renography using technetium-99m labeled mercaptoacetyltriglycine showing (a) satisfactory uptake and outflow from the left kidney with no evidence of obstruction. The right kidney was not visualized. (b) Image showing left hydronephrotic kidney with the urinary bladder displaced to the right side of the pelvis|
Click here to view
An exploratory laparotomy was performed to achieve a conclusive diagnosis. This revealed presence of hydrometrocolpos, which was aspirated to remove 60 ml of serosanguinous fluid. There was a high uterine septum splitting the uterus in two parts. Simultaneous vaginoscopy showed direct communication of the posterior uterus with the vagina, while there was a blind pouch anteriorly. The membranous septum was incised with harmonic scalpel, and the uterostomy was closed in two layers in transverse fashion. [Figure 3] indicates the anomalies diagrammatically.
|Figure 3: Diagram depicting obstructed hemivagina, ipsilateral renal agenesis, and uterine didelphys|
Click here to view
There have been no further febrile UTIs during follow-up over 1 year. Repeat ultrasonography showed reduction in the left side hydronephrosis. Renal function returned to normal, with serum creatinine ranging from 25 to 35 μmol/L during follow-up. Parents provided signed informed consent for publishing the case anonymously.
| Discussion|| |
We report a case of OHVIRA syndrome presenting at 3 months of age. This is a rare entity, increasingly recognized as part of the spectrum of Müllerian anomalies, and several case series have been reported, chiefly from tertiary care centers., To our knowledge, this is the first case in which a diagnosis of OHVIRA syndrome was made at this young age. The median age of diagnosis was around 14 years, after menarche; the mean age at presentation for patients with complete hemivaginal obstruction was 12.9 years while those with partial obstruction was 20.7 years., While the syndrome is associated with various renal anomalies, similar to our case, most authors report ipsilateral renal agenesis. Other anomalies, and rarely, no renal anomalies, are also described.,
Patients with OHVIRA syndrome most commonly present with dysmenorrhea, pelvic pain, or pelvic mass secondary to hematocolpos from retained menstrual flow. Acute pelvic or vaginal pain and/or acute abdomen are uncommon presentations. In our patient, the diagnosis was considerably delayed because of the unusual, early and nonspecific presentation. Multiple radiological investigations were performed before the diagnosis was reached. While MRI is reported to be very accurate in diagnosing OHVIRA syndrome,, its sensitivity in the diagnosis of septate uterine or nonmidline Müllerian anomalies is limited. In our case, exploratory laparoscopy was both diagnostic and therapeutic. Evacuation of the hydrometrocolpos and incision of the uterine septum relieved the child of recurrent UTI and allowed recovery of renal function.
Our case emphasizes the diversity in age at presentation and symptomatology of OHVIRA syndrome. Long-term outcomes are promising, for cases presenting in adulthood, if diagnosis and surgical intervention are timely.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Vercellini P, Daguati R, Somigliana E, Viganò P, Lanzani A, Fedele L. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: Institutional case series and a systematic literature review. Fertil Steril 2007;87:719-24.
Selter JH, Christianson MS, Washington CI, Resetkova N, Kolp L. Hydronephrosis: A rare presentation of uterine didelphys with obstructed hemivagina and ipsilateral renal anomaly. J Pediatr Adolesc Gynecol 2016;29:e53-5.
Resetkova N, Christianson M, Kolp L. Uterinedidelphys with obstructed hemivagina and ipsilateral renal agenesis with hydronephrosis. Fertil Steril 2012;97:s30-1.
Li S, Qayyum A, Coakley FV, Hricak H. Association of renal agenesis and Mullerian duct anomalies. J Comput Assist Tomogr 2000;24:829-34.
Wang J, Zhu L, Lang J, Liu Z, Sun D, Leng J, et al.
Clinical characteristics and treatment of Herlyn-Werner-Wunderlich syndrome. Arch Gynecol Obstet 2014;290:947-50.
Zurawin RK, Dietrich JE, Heard MJ, Edwards CL. Didelphic uterus and obstructed hemivagina with renal agenesis: Case report and review of the literature. J Pediatr Adolesc Gynecol 2004;17:137-41.
Güdücü N, Gönenç G, Işçi H, Yiǧiter AB, Dünder I. Herlyn-Werner-Wunderlich syndrome – Timely diagnosis is important to preserve fertility. J Pediatr Adolesc Gynecol 2012;25:e111-2.
Epelman M, Dinan D, Gee MS, Servaes S, Lee EY, Darge K. Müllerian duct and related anomalies in children and adolescents. Magn Reson Imaging Clin N
Altintaş A. Uterus didelphys with unilateral imperforate hemivagina and ipsilateral renal agenesis. J Pediatr Adolesc Gynecol 1998;11:25-7.
Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina, and ipsilateral renal agenesis: 36 cases and long-term follow-up. Obstet Gynecol 1997;90:26-32.
Tong J, Zhu L, Lang J. Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome. Int J Gynaecol Obstet 2013;121:173-5.
Haddad B, Barranger E, Paniel BJ. Blind hemivagina: Long-term follow-up and reproductive performance in 42 cases. Hum Reprod 1999;14:1962-4.
Aydin R, Ozdemir AZ, Ozturk B, Bilgici MC, Tosun M. A rare cause of acute abdominal pain: Herlyn-Werner-Wunderlich syndrome. Pediatr Emerg Care 2014;30:40-2.
Gungor Ugurlucan F, Bastu E, Gulsen G, Kurek Eken M, Akhan SE. OHVIRA syndrome presenting with acute abdomen: A case report and review of the literature. Clin Imaging 2014;38:357-9.
Troiano RN, McCarthy SM. Mullerian duct anomalies: Imaging and clinical issues. Radiology 2004;233:19-34.
Economy KE, Barnewolt C, Laufer MR. A comparison of MRI and laparoscopy in detecting pelvic structures in cases of vaginal agenesis. J Pediatr Adolesc Gynecol 2002;15:101-4.
[Figure 1], [Figure 2], [Figure 3]