Arch Gynecol Obstet (2014) 290:855–858 DOI 10.1007/s00404-014-3339-9
REVIEW
Unusual case of OHVIRA syndrome with a single uterus, unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina ˇ ivkovic´ Kresˇimir Zˇivkovic´ • Matija Prka • Nikica Z • Ana Bucko Dubravko Habek
•
Received: 5 November 2013 / Accepted: 26 June 2014 / Published online: 17 July 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Purpose The acronym for obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) was created to describe patients with an obstructed hemivagina and ipsilateral renal anomaly and enables inclusion of other uterine anomalies except uterus didelphys. The main goal of this article is to present a rare case of OHVIRA syndrome with intrapartal rupture of obstructed hemivagina. Methods We present an unusual case of OHVIRA syndrome with single uterus, uterine septum (previously resected by hysteroscopy) and renal agenesis, unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina. Results and conclusion Various symptoms, included in OHVIRA acronym, are a result of different morphologic variants included in this syndrome. Most likely, in our case, the absence of communication between the cervical canal/ patent vagina and the obstructed hemivagina prevented formation of mucocolpos or hematocolpos. This rare clinical variant made our MRI diagnosis of obstructed hemivagina, as well of the entire OHVIRA syndrome, ineffective. We did not find OHVIRA cases in the literature with a single septate uterus and single cervix, associated with obstructed hemivagina diagnosed during and after labor and presented with intrapartal rupture of hemivagina. Because of lack of guidelines, the final decision about K. Zˇivkovic´ (&) M. Prka A. Bucko D. Habek Department of Gynecology and Obstetrics, ‘‘Sveti Duh’’ University Hospital, University of Zagreb, School of Medicine, Sveti Duh 64, 10000 Zagreb, Croatia e-mail: [email protected] N. Zˇivkovic´ Department of Gynecology and Obstetrics, Sˇibenik General Hospital, Stjepana Radic´a 83, 22000 Sˇibenik, Croatia
management of the labor in such rare cases of OHVIRA syndrome is difficult. Keywords Mu¨llerian anomalies OHVIRA syndrome Single uterus Uterine septum Obstructed hemivagina Vaginal delivery
Introduction Mu¨llerian duct anomalies are congenital anomalies of the female genital tract resulting from non-development or non-fusion of the Mu¨llerian ducts, or failed resorption of the uterine septum [1]. This combination of obstructed hemivagina and uterus didelphys was first reported in 1922 [2]. Currently, there are about 65 papers describing cases of these anomalies in earlier literature and also many incoherent appellations. The syndrome of obstructed hemivagina and uterus didelphys, as well as an ipsilateral renal anomaly, was first reported in 1950 [3] and since 1983, this triad has been called Herlyn-Werner-Wunderlich syndrome [4]. The acronym OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) was created in 2007 and has been used to describe patients with an obstructed hemivagina and ipsilateral renal anomaly [5]. OHVIRA syndrome includes two of the three components of the triad in Herlyn-Werner-Wunderlich syndrome and so enables including other uterine anomalies, other than a uterus didelphys, for example a septated uterus. The septated uterus is present in OHVIRA syndrome in about 22 % of all cases [6]. We are presenting an unusual case of OHVIRA syndrome with single uterus/cervix, a large septum of uterine cavity, renal agenesis with intrapartal rupture of dextrolateral obstructed hemivagina.
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Fig. 2 Final feature of the vagina with preexistent single cervix after resection of an obstructed hemivagina wall
Fig. 1 Intrapartal presentation of the rupture of obstructed hemivagina, unrecognized before labor
Case report A 30-year old gravida 1 para 0 was admitted to the maternity ward of ‘‘Sveti Duh’’ University Hospital, Zagreb, Croatia, in the early stage of labor at 41 weeks and 2 days of gestation. In her medical history, right kidney agenesis was noticed in her childhood. She had laparoscopy and hysteroscopy procedure in 2012 on account of large uterine septum and primary infertility. A hysteroscopic resection of uterine septum went uneventfully. Magnetic resonance imaging (MRI) was performed preoperatively and renal agenesis was confirmed but without any vaginal deformation. Also, vaginal examination had not revealed any suspicion of vaginal anomaly. Spontaneous pregnancy was achieved promptly after the procedure. She had regular antenatal visits with normal course of pregnancy. On admission, cardiotocography (CTG) recording was normal. The baby was in cephalic position and was at the entrance of pelvis. Cervix was half effaced and 2 cm dilated. The following morning, cervix was fully effaced and 7 cm dilated. CTG was normal and reactive. Having given informed consent, she opted for amniotomy and augmentation of labor. The amniotic fluid was normal. Intravenous infusion of oxytocine was started until her contractions became regular. Two hours later on vaginal exam, the head was above level of spines with some form of ballooned, spastic posterior positioned cervix, 8 cm
123
dilated and epidural analgesia was performed. Again, 2 hours later, another vaginal exam was performed and the fetal head was below the level of spines. On the right side, above the birth canal, there was some unusual form of vaginal mucosa bulge. Immediately after that, the episiotomy was carried out and the woman gave a birth to a live female newborn, 3,610 g/51 cm, the Apgar score was 10/10. Immediately after delivery, a right-sided vaginal rupture was observed. It ended blindly and departed from the front of the cervix between 11 and 12 h lateral and ends back at 6 h toward to the lesser pelvis (Fig. 1). A cervical exam made showed the cervix being intact. We concluded that there was a rupture of dextrolateral obstructed hemivagina. Then the complete wall of this obstructed hemivagina was resected and the entire vaginal injury was sutured in epidural analgesia (Fig. 2). The episiotomy wound was sutured. The genital clinical status and rectovaginal exam, after this surgical procedure, were satisfactory. Antibiotics and uterotonics were administered. After the procedure, postpartal hospital course went orderly. One month later, on the exam, normal vaginal findings and the regular healing of wound in the vagina were found. Histopathology confirmed vaginal tissue in a resected sample.
Discussion This case report presents a unique intrapartal clinical manifestation of an extremely rare morphologic variant of the OHVIRA syndrome (Fig. 3). The classic clinical scenario of this syndrome is an adolescent girl with pelvic pain who, on gynaecologic examination, is found to have a vaginal bulge. Although women with hematocolpos typically have early clinical presentation due to stasis of menstrual blood, oligomenorrhea and pelvic pain, some
Arch Gynecol Obstet (2014) 290:855–858
857
unrecognized obstructed hemivaginas during labor, including postpartum hemorrhage from a massive vaginal rupture. In our case, it was necessary to resect complete tissue of obstructed hemivagina and to create a single vagina promptly after delivery due to a huge intrapartal rupture, which totally deformed the whole of preexisting vagina. Favorable condition for us was that this woman bled little.
Conclusion
Fig. 3 Feature of OHVIRA syndrome with single uterus/cervix in our case. 1 Uterine septum resected by hysteroscopy 1 year before labor and 2 Unrecognized non-communicating obstructed hemivagina
women with an obstructed hemivagina and ipsilateral renal agenesis will have regular menses because menstrual blood from the uterus can flow through its nonobstructed cervix [5] or single cervix such as in our case. Much later, they may present with symptoms of persistent vaginal discharge [7]. Cyclic pain due to stasis of blood in the obstructed hemivagina, as a very common sign in women with obstructed hemivagina [8], was not present in our patient. Most likely in our case, the absence of communication between the single cervix/patent vagina and the obstructed hemivagina prevented forming mucocolpos or hematocolpos. Also, there are some reports of OHVIRA syndrome with acute retention of urine in adolescent caused by obstructed hemivagina [9]. We found one case of OHVIRA in literature with a single septate uterus and a single cervix [10], but with different intrapartal presentation and mode of delivery. The diagnosis of this condition can be challenging due to its rarity and heterogeneous presentation. Relevant radiologic studies confirmed that MRI is obligatory when diagnosing OHVIRA patients, because MRI provides the best insight in anatomic variants closely associated with OHVIRA [11, 12]. We think that non-communicating obstructed hemivagina in our patient, without significant liquid storage, made MRI diagnosis of such a rare variant of OHVIRA syndrome ineffective. Ballooned formation, which had been observed during labor was certainly obstructed hemivagina, distended by passage of fetal head and ruptured later. Also, this bizarre intrapartal presentation made difficulties in final diagnosis of OHVIRA. A final decision about managing labor in such rare cases of OHVIRA syndrome is difficult, because of lack of guidelines. The most important thing regarding vaginal delivery is the presence of experienced obstetricians in labor wards, because serious risks lay in
The presentations and effects of congenital anomalies of the female genitourinary tract are enormously variable. For understanding the pathogenesis and clinical manifestation of Mu¨llerian anomalies, obstetrics knowledge of basic embryology is needed. In this case report we focused on: (1) its rarity in a sense of morphology, and (2) intrapartal clinical presentation and treatment. Certain Mu¨llerian anomalies could be easily recognized during standard diagnostic procedures but, on the other side, could represent a very hard diagnostic task as showed in our case, resulting in totally unrecognized obstructed hemivagina within an extremely rare variant of OHVIRA syndrome. This obstacle delayed final diagnosis and therapy of our patient till the beginning of spontaneous labor, by putting present obstetric staff in a very delicate position. According to us, in such situations vaginal delivery as an option could be considered only in presence of experienced and skillful obstetricians. Conflict of interest
None.
References 1. Madureira AJ, Mariz CM, Bernardes JC, Ramos IM (2006) Case 94: uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis. Radiology 239(2):602–606 2. Purslow CE (1922) A case of unilateral haematocolpos, hematometra and haematosalpinx. J Obstet Gynaecol Br Emp 29:643 3. Embrey MP (1950) A case of uterus didelphys with unilateral gynatresia. Br Med J 1:820–821 4. Karag’ozov I (1983) Herlyn-Werner-Wunderlich syndrome. Akush Ginekol (Sofiia) 22(1):70–76 5. Smith N, Laufer M (2007) Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and followup. Fertil Steril 87:918–922 6. Haddad B, Barranger E, Paniel BJ (1999) Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod 14:1962–1964 7. Shah DK, Laufer MR (2011) Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus. Fertil Steril 96(1):e39–e41 8. Stassart JP, Nagel TC, Prem KA, Phipps WR (1992) Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience. Fertil Steril 57:756–761
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858 9. Mandava A, Prabhakar RR, Smitha S (2012) OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 25(2):e23–e25 10. Shavell VI, Montgomery SE, Johnson SC, Diamond MP, Berman JM (2009) Complete septate uterus, obstructed hemivagina, and ipsilateral renal anomaly: pregnancy course complicated by a rare urogenital anomaly. Arch Gynecol Obstet 280(3):449–452 11. Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F (2007) Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind
123
Arch Gynecol Obstet (2014) 290:855–858 hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 37(7):657–665 12. Pellerito JS, McCarthy SM, Doyle MB, Glickman MG, DeCherney AH (1992) Diagnosis of uterine anomalies: relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology 183(3):795–800
REVIEW
Unusual case of OHVIRA syndrome with a single uterus, unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina ˇ ivkovic´ Kresˇimir Zˇivkovic´ • Matija Prka • Nikica Z • Ana Bucko Dubravko Habek
•
Received: 5 November 2013 / Accepted: 26 June 2014 / Published online: 17 July 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Purpose The acronym for obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) was created to describe patients with an obstructed hemivagina and ipsilateral renal anomaly and enables inclusion of other uterine anomalies except uterus didelphys. The main goal of this article is to present a rare case of OHVIRA syndrome with intrapartal rupture of obstructed hemivagina. Methods We present an unusual case of OHVIRA syndrome with single uterus, uterine septum (previously resected by hysteroscopy) and renal agenesis, unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina. Results and conclusion Various symptoms, included in OHVIRA acronym, are a result of different morphologic variants included in this syndrome. Most likely, in our case, the absence of communication between the cervical canal/ patent vagina and the obstructed hemivagina prevented formation of mucocolpos or hematocolpos. This rare clinical variant made our MRI diagnosis of obstructed hemivagina, as well of the entire OHVIRA syndrome, ineffective. We did not find OHVIRA cases in the literature with a single septate uterus and single cervix, associated with obstructed hemivagina diagnosed during and after labor and presented with intrapartal rupture of hemivagina. Because of lack of guidelines, the final decision about K. Zˇivkovic´ (&) M. Prka A. Bucko D. Habek Department of Gynecology and Obstetrics, ‘‘Sveti Duh’’ University Hospital, University of Zagreb, School of Medicine, Sveti Duh 64, 10000 Zagreb, Croatia e-mail: [email protected] N. Zˇivkovic´ Department of Gynecology and Obstetrics, Sˇibenik General Hospital, Stjepana Radic´a 83, 22000 Sˇibenik, Croatia
management of the labor in such rare cases of OHVIRA syndrome is difficult. Keywords Mu¨llerian anomalies OHVIRA syndrome Single uterus Uterine septum Obstructed hemivagina Vaginal delivery
Introduction Mu¨llerian duct anomalies are congenital anomalies of the female genital tract resulting from non-development or non-fusion of the Mu¨llerian ducts, or failed resorption of the uterine septum [1]. This combination of obstructed hemivagina and uterus didelphys was first reported in 1922 [2]. Currently, there are about 65 papers describing cases of these anomalies in earlier literature and also many incoherent appellations. The syndrome of obstructed hemivagina and uterus didelphys, as well as an ipsilateral renal anomaly, was first reported in 1950 [3] and since 1983, this triad has been called Herlyn-Werner-Wunderlich syndrome [4]. The acronym OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) was created in 2007 and has been used to describe patients with an obstructed hemivagina and ipsilateral renal anomaly [5]. OHVIRA syndrome includes two of the three components of the triad in Herlyn-Werner-Wunderlich syndrome and so enables including other uterine anomalies, other than a uterus didelphys, for example a septated uterus. The septated uterus is present in OHVIRA syndrome in about 22 % of all cases [6]. We are presenting an unusual case of OHVIRA syndrome with single uterus/cervix, a large septum of uterine cavity, renal agenesis with intrapartal rupture of dextrolateral obstructed hemivagina.
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Arch Gynecol Obstet (2014) 290:855–858
Fig. 2 Final feature of the vagina with preexistent single cervix after resection of an obstructed hemivagina wall
Fig. 1 Intrapartal presentation of the rupture of obstructed hemivagina, unrecognized before labor
Case report A 30-year old gravida 1 para 0 was admitted to the maternity ward of ‘‘Sveti Duh’’ University Hospital, Zagreb, Croatia, in the early stage of labor at 41 weeks and 2 days of gestation. In her medical history, right kidney agenesis was noticed in her childhood. She had laparoscopy and hysteroscopy procedure in 2012 on account of large uterine septum and primary infertility. A hysteroscopic resection of uterine septum went uneventfully. Magnetic resonance imaging (MRI) was performed preoperatively and renal agenesis was confirmed but without any vaginal deformation. Also, vaginal examination had not revealed any suspicion of vaginal anomaly. Spontaneous pregnancy was achieved promptly after the procedure. She had regular antenatal visits with normal course of pregnancy. On admission, cardiotocography (CTG) recording was normal. The baby was in cephalic position and was at the entrance of pelvis. Cervix was half effaced and 2 cm dilated. The following morning, cervix was fully effaced and 7 cm dilated. CTG was normal and reactive. Having given informed consent, she opted for amniotomy and augmentation of labor. The amniotic fluid was normal. Intravenous infusion of oxytocine was started until her contractions became regular. Two hours later on vaginal exam, the head was above level of spines with some form of ballooned, spastic posterior positioned cervix, 8 cm
123
dilated and epidural analgesia was performed. Again, 2 hours later, another vaginal exam was performed and the fetal head was below the level of spines. On the right side, above the birth canal, there was some unusual form of vaginal mucosa bulge. Immediately after that, the episiotomy was carried out and the woman gave a birth to a live female newborn, 3,610 g/51 cm, the Apgar score was 10/10. Immediately after delivery, a right-sided vaginal rupture was observed. It ended blindly and departed from the front of the cervix between 11 and 12 h lateral and ends back at 6 h toward to the lesser pelvis (Fig. 1). A cervical exam made showed the cervix being intact. We concluded that there was a rupture of dextrolateral obstructed hemivagina. Then the complete wall of this obstructed hemivagina was resected and the entire vaginal injury was sutured in epidural analgesia (Fig. 2). The episiotomy wound was sutured. The genital clinical status and rectovaginal exam, after this surgical procedure, were satisfactory. Antibiotics and uterotonics were administered. After the procedure, postpartal hospital course went orderly. One month later, on the exam, normal vaginal findings and the regular healing of wound in the vagina were found. Histopathology confirmed vaginal tissue in a resected sample.
Discussion This case report presents a unique intrapartal clinical manifestation of an extremely rare morphologic variant of the OHVIRA syndrome (Fig. 3). The classic clinical scenario of this syndrome is an adolescent girl with pelvic pain who, on gynaecologic examination, is found to have a vaginal bulge. Although women with hematocolpos typically have early clinical presentation due to stasis of menstrual blood, oligomenorrhea and pelvic pain, some
Arch Gynecol Obstet (2014) 290:855–858
857
unrecognized obstructed hemivaginas during labor, including postpartum hemorrhage from a massive vaginal rupture. In our case, it was necessary to resect complete tissue of obstructed hemivagina and to create a single vagina promptly after delivery due to a huge intrapartal rupture, which totally deformed the whole of preexisting vagina. Favorable condition for us was that this woman bled little.
Conclusion
Fig. 3 Feature of OHVIRA syndrome with single uterus/cervix in our case. 1 Uterine septum resected by hysteroscopy 1 year before labor and 2 Unrecognized non-communicating obstructed hemivagina
women with an obstructed hemivagina and ipsilateral renal agenesis will have regular menses because menstrual blood from the uterus can flow through its nonobstructed cervix [5] or single cervix such as in our case. Much later, they may present with symptoms of persistent vaginal discharge [7]. Cyclic pain due to stasis of blood in the obstructed hemivagina, as a very common sign in women with obstructed hemivagina [8], was not present in our patient. Most likely in our case, the absence of communication between the single cervix/patent vagina and the obstructed hemivagina prevented forming mucocolpos or hematocolpos. Also, there are some reports of OHVIRA syndrome with acute retention of urine in adolescent caused by obstructed hemivagina [9]. We found one case of OHVIRA in literature with a single septate uterus and a single cervix [10], but with different intrapartal presentation and mode of delivery. The diagnosis of this condition can be challenging due to its rarity and heterogeneous presentation. Relevant radiologic studies confirmed that MRI is obligatory when diagnosing OHVIRA patients, because MRI provides the best insight in anatomic variants closely associated with OHVIRA [11, 12]. We think that non-communicating obstructed hemivagina in our patient, without significant liquid storage, made MRI diagnosis of such a rare variant of OHVIRA syndrome ineffective. Ballooned formation, which had been observed during labor was certainly obstructed hemivagina, distended by passage of fetal head and ruptured later. Also, this bizarre intrapartal presentation made difficulties in final diagnosis of OHVIRA. A final decision about managing labor in such rare cases of OHVIRA syndrome is difficult, because of lack of guidelines. The most important thing regarding vaginal delivery is the presence of experienced obstetricians in labor wards, because serious risks lay in
The presentations and effects of congenital anomalies of the female genitourinary tract are enormously variable. For understanding the pathogenesis and clinical manifestation of Mu¨llerian anomalies, obstetrics knowledge of basic embryology is needed. In this case report we focused on: (1) its rarity in a sense of morphology, and (2) intrapartal clinical presentation and treatment. Certain Mu¨llerian anomalies could be easily recognized during standard diagnostic procedures but, on the other side, could represent a very hard diagnostic task as showed in our case, resulting in totally unrecognized obstructed hemivagina within an extremely rare variant of OHVIRA syndrome. This obstacle delayed final diagnosis and therapy of our patient till the beginning of spontaneous labor, by putting present obstetric staff in a very delicate position. According to us, in such situations vaginal delivery as an option could be considered only in presence of experienced and skillful obstetricians. Conflict of interest
None.
References 1. Madureira AJ, Mariz CM, Bernardes JC, Ramos IM (2006) Case 94: uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis. Radiology 239(2):602–606 2. Purslow CE (1922) A case of unilateral haematocolpos, hematometra and haematosalpinx. J Obstet Gynaecol Br Emp 29:643 3. Embrey MP (1950) A case of uterus didelphys with unilateral gynatresia. Br Med J 1:820–821 4. Karag’ozov I (1983) Herlyn-Werner-Wunderlich syndrome. Akush Ginekol (Sofiia) 22(1):70–76 5. Smith N, Laufer M (2007) Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and followup. Fertil Steril 87:918–922 6. Haddad B, Barranger E, Paniel BJ (1999) Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod 14:1962–1964 7. Shah DK, Laufer MR (2011) Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus. Fertil Steril 96(1):e39–e41 8. Stassart JP, Nagel TC, Prem KA, Phipps WR (1992) Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience. Fertil Steril 57:756–761
123
858 9. Mandava A, Prabhakar RR, Smitha S (2012) OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 25(2):e23–e25 10. Shavell VI, Montgomery SE, Johnson SC, Diamond MP, Berman JM (2009) Complete septate uterus, obstructed hemivagina, and ipsilateral renal anomaly: pregnancy course complicated by a rare urogenital anomaly. Arch Gynecol Obstet 280(3):449–452 11. Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F (2007) Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind
123
Arch Gynecol Obstet (2014) 290:855–858 hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 37(7):657–665 12. Pellerito JS, McCarthy SM, Doyle MB, Glickman MG, DeCherney AH (1992) Diagnosis of uterine anomalies: relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology 183(3):795–800
