Case Report Delayed Diagnosis of Herlyn-Werner-Wunderlich Syndrome due to Microperforation and Pyocolpos in Obstructed Vaginal Canal Ewa Wozniakowska MD, PhD 1,*, Anna Torres MD, PhD 2, Pawel Milart MD, PhD 1, Slawomir Wozniak MD, PhD 1, Piotr Czuczwar MD, PhD 1, Piotr Szkodziak MD, PhD 1, Tomasz Paszkowski MD, PhD 1 1 2
Department of Gynecology, Medical University of Lublin, Lublin, Poland Department of Human Anatomy, Medical University of Lublin, Lublin, Poland
a b s t r a c t Background: To present a rare anomaly consisting of uterus didelphys, longitudinal vaginal septum, obstructed hemivagina with pyocolpos, fistula to the open vaginal canal, and ipsilateral renal agenesis, referred as Herlyn-Werner-Wunderlich syndrome (HWWS). Case: A 14-year-old girl with recurring purulent vaginal discharge lasting for a few months. Preoperative examination revealed one vaginal canal with one cervical opening on the right side. There was a fistula leading from the obstructed vaginal canal to the left vagina. Intravaginal ultrasound examination demonstrated a longitudinal vaginal septum and a closed pyocolpos on the right side. The longitudinal vaginal septum was excised by way of electrocauterization under direct vision. Conclusion: HWWS should be considered in the differential diagnosis in patients with uterus didelphys and unusual symptoms such as pyocolpos and vaginal discharge. Key Words: Herlyn-Werner-Wunderlich syndrome, Longitudinal vaginal septum, Fistula, Pyocolpos, Purulent vaginal discharge
Introduction
Case
Herlyn-Werner-Wunderlich syndrome (HWWS), is a € llerian anomaly characterized by uterus congenital mu didelphys, longitudinal hemivaginal septum creating a blind hemivagina, and ipsilateral renal agenesis.1 Uterus didelphys can be associated with a complete longitudinal vaginal septum. Longitudinal vaginal septum with two separate vaginal openings is frequently asymptomatic and detected only incidentally during a gynecological examination or delivery. Another clinical presentation of a uterus didelphys is the case of one vagina with occlusion of the contralateral vaginal canal caused by the fusion of the septum with vaginal wall, which leads to hematocolpos. In those cases the most common clinical symptoms are pelvic pain with the presence of a vaginal or pelvic mass and a normal menstrual period. Rarely, a vaginal septum can be partially fenestrated and retained menstrual blood can be infected, leading to a condition known as pyocolpos. In such an instance it manifests itself as purulent vaginal discharge and the diagnosis may be difficult. The available literature reports rare cases of pyocolpos in obstructed vagina with purulent discharge and a communication between the hemivaginas.2-4
This case report presents an uncommon anomaly of the € llerian ducts in which the chronic, purulent vaginal mu discharge was the dominant symptom. A 14-year-old girl was referred to the adolescent gynecology clinic due to chronic, purulent, foul-smelling vaginal discharge without any pelvic pain. The symptoms occurred a few weeks following her menarche, which she had at 13 years, and were observed ever since, either constantly or at intervals of a few days. The symptoms were not relieved despite several attempts of oral antibiotic treatment. Her menses had been regular, lasting for 3 to 4 days and occurring at 30-day intervals. At the age of 12 years she had undergone an emergent appendectomy. During this operation the uterine malformation (class III, didelphys uterus) had been detected. The abdominal/pelvic ultrasound performed at that time had revealed renal agenesis on the right side. The patient was not followed up, further diagnosed, or counseled regarding her malformations. At the time of her visit to our facility the patient did not complain of any pain. Palpation of the abdomen did not reveal any masses or abdominal tenderness. The external genitalia appeared normal. The hymen was intact. A firm, large mass was palpable on rectal examination slightly to the right from the midline. Rectal pelvic ultrasound showed a double uterus with a double cervix without hematometras. In addition, we found an echogenic structure (5.0 cm 4.0 cm) on the right side of the vagina, in the place where the pelvic mass was detected during the rectal examination.
The authors indicate no conflicts of interests. * Address correspondience to: Ewa Wozniakowska, MD, PhD, III Katedra i Klinika Ginekologii Uniwersytetu Medycznego w Lublinie, ul. Jaczewskiego 8, 20-090 Lublin, Poland; Phone: þ48 81 7244848; fax: þ48 81 7244847 E-mail address: [email protected] (E. Wozniakowska).
1083-3188/$ - see front matter Ó 2013 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2013.07.009
2
E. Wozniakowska et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e3
It indicated a possibility of pyocolpos in the right, occluded hemivagina. MRI of the abdomen and pelvis was performed in order to obtain more detailed information. MRI revealed normal ovaries, didelphic uterus without distension of uterine cavities, normal left cervix and hemivagina and a largely distended right hemivagina (2.6 cm 4.3 cm 2.5 cm). Signal intensity of the contents of right hemivagina was suggestive of hemato-pyocolpos. In addition, on this occasion, right renal agenesis was confirmed and other possible urinary tract anomalies were excluded (Fig. 1). The patient was scheduled for surgical treatment. Prior to operation, vaginal examination was performed under general anesthesia without breaking the hymenal membrane. This procedure revealed one vaginal canal with one cervical opening. The vaginal wall on the right side was bulging. On the right side of the examined vaginal canal there was a fistula leading from the obstructed vaginal canal to the left open hemivagina (Fig. 2). The longitudinal vaginal septum was excised by means of electrocauterization under direct visualization. After the incision, a purulent fluid flowed out of the right hemivagina. At this stage we examined the right hemivagina with the use of a hysteroscope. This examination confirmed the presence of the right uterine cervix communicating with the right hemivagina. We concluded that the pyocolpos was slowly draining through the fistula in the vaginal septum resulting in an infection and chronic discharge. After the successful surgery, a follow-up examination was carried out in the outpatient clinic. No complications were found 1 month and 3 months after surgery. As of the date of submission of this manuscript the patient is still asymptomatic and has regular menses. Summary and Conclusion
A syndrome consisting of renal agenesis and obstructed hemivagina was first described in 1971 by Herlyn and Werner. In 1976, Wunderlich broadened the description of the syndrome by adding bicornate uterus. Hence, HWWS € llerian anomaly consisting of denotes a rare congenital mu
Fig. 1. Transversal MRI demonstrating left hemivagina and pyocolpos on right side. B, urinary bladder, R, right hemivagina, *, left hemivagina.
Fig. 2. Vaginoscopic view of fistula/perforation in longitudinal septum.Ă
uterus didelphys, longitudinal hemivaginal septum creating a blind hemivagina, and renal agenesis on the same side. The syndrome is also described in the literature as OHVIRA (obstructed hemivagina and ipsilateral renal agenesis).1,5 € llerian anomaly usually Patients with an obstructing mu suffer from pelvic pain and have an abnormal abdominal mass. Other signs and symptoms are rare and include: fever, pyocolpos, peritonitis, hematometra, vaginal discharge and urinary incontinence.6,7 In 2006, Gholoum et al1 presented their experience with HWWS. During the period between 1982 and 2004 twelve patients were diagnosed with this syndrome and it is one of the largest groups ever described in the literature with this € llerian anomaly. Anatomically, 10 of the 12 variant of mu patients had classic symptoms of HWWS. Abdominal pain and a pelvic mass were the major reported complaints. All patients were treated by septectomy and the treatment was effective in all the cases. In conclusion, surgical excision of the longitudinal vaginal septum is recommended as the procedure of choice; however, in some cases pyocolpos caused by the closure of the septal incision has been reported as a complication after surgery. Varras et al5 demonstrated the difficulty in reaching the correct diagnosis of HWWS, especially one occurring after many years since menarche. They described a 24-year-old woman admitted to the emergency room with vomiting, severe and increasing lower abdominal pain, and purulent vaginal discharge. The preoperative diagnosis was ruptured tubo-ovarian abscess, for which an emergency laparotomy was performed. Vaginal examination under general anesthesia revealed an obstructed right hemivagina with vaginal purulent discharge from a small fistula in the septate vagina. The final diagnosis was uterus didelphys with unilateral perforated right hemivagina and pyocolpos. The diagnosis of our case was difficult because the classic HWWS symptoms were absent and the only symptom was vaginal discharge. The lack of classical HWWS symptoms had resulted in misdiagnosis and long-term antibiotic therapy in an outpatient clinic before her visit to our facility.
E. Wozniakowska et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e3
Precise physical examination and imaging lead to the correct diagnosis. Recognizing Herlyn-Werner-Wunderlich syndrome can be difficult, because its symptoms may not be typical and vaginal discharge can have many other causes, leading to misdiagnosis. Microperforation may occur in the hemivaginal septum on the obstructed side, thereby draining into the nonobstructed vagina, resulting in ascending infection. However, most authors agree that HWWS should be considered to prevent misdiagnosis or suboptimal treatment, to decrease morbidity and to avoid unnecessary surgical procedures. Our case confirms that, at minimum, it should be routinely considered in the differential diagnosis in young patients with uterus didelphys or bicornate uterus and unusual symptoms such as vaginal discharge.
3
References 1. Gholoum S, Puligandla PS, Hui T, et al: 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 2. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, € llerian anomalies and intrauterine adhesions. Fertil Steril 1988; 49:944 mu 3. Smith NA, Laufer MR: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007; 87:918 4. Wu TH, Wu TT, Ng YY, et al: Herlyn-Werner-Wunderlich syndrome consisting of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis in a newborn. Pediatr Neonatol 2012; 53:68 5. Varras M, Akrivis Ch, Karadaglis S, et al: Uterus didelphys with blind hemivagina and ipsilateral renal agenesis complicated by pyocolpos and presenting as acute abdomen 11 years after menarche: presentation of a rare case with review of the literature. Clin Exp Obstet Gynecol 2008; 35:156 6. Olsen ME, Breuel KF, Thatcher SS: Communicating double uterus with obstructed hemivagina and subsequent abscess formation: A case report. J Pediatr Adolesc Gynecol 1995; 8:35 7. Savitski JL, Olsen ME: Successful pregnancy in an adolescent woman with a communicating double uterine anomaly. J Pediatr Adolesc Gynecol 2001; 14:181
Department of Gynecology, Medical University of Lublin, Lublin, Poland Department of Human Anatomy, Medical University of Lublin, Lublin, Poland
a b s t r a c t Background: To present a rare anomaly consisting of uterus didelphys, longitudinal vaginal septum, obstructed hemivagina with pyocolpos, fistula to the open vaginal canal, and ipsilateral renal agenesis, referred as Herlyn-Werner-Wunderlich syndrome (HWWS). Case: A 14-year-old girl with recurring purulent vaginal discharge lasting for a few months. Preoperative examination revealed one vaginal canal with one cervical opening on the right side. There was a fistula leading from the obstructed vaginal canal to the left vagina. Intravaginal ultrasound examination demonstrated a longitudinal vaginal septum and a closed pyocolpos on the right side. The longitudinal vaginal septum was excised by way of electrocauterization under direct vision. Conclusion: HWWS should be considered in the differential diagnosis in patients with uterus didelphys and unusual symptoms such as pyocolpos and vaginal discharge. Key Words: Herlyn-Werner-Wunderlich syndrome, Longitudinal vaginal septum, Fistula, Pyocolpos, Purulent vaginal discharge
Introduction
Case
Herlyn-Werner-Wunderlich syndrome (HWWS), is a € llerian anomaly characterized by uterus congenital mu didelphys, longitudinal hemivaginal septum creating a blind hemivagina, and ipsilateral renal agenesis.1 Uterus didelphys can be associated with a complete longitudinal vaginal septum. Longitudinal vaginal septum with two separate vaginal openings is frequently asymptomatic and detected only incidentally during a gynecological examination or delivery. Another clinical presentation of a uterus didelphys is the case of one vagina with occlusion of the contralateral vaginal canal caused by the fusion of the septum with vaginal wall, which leads to hematocolpos. In those cases the most common clinical symptoms are pelvic pain with the presence of a vaginal or pelvic mass and a normal menstrual period. Rarely, a vaginal septum can be partially fenestrated and retained menstrual blood can be infected, leading to a condition known as pyocolpos. In such an instance it manifests itself as purulent vaginal discharge and the diagnosis may be difficult. The available literature reports rare cases of pyocolpos in obstructed vagina with purulent discharge and a communication between the hemivaginas.2-4
This case report presents an uncommon anomaly of the € llerian ducts in which the chronic, purulent vaginal mu discharge was the dominant symptom. A 14-year-old girl was referred to the adolescent gynecology clinic due to chronic, purulent, foul-smelling vaginal discharge without any pelvic pain. The symptoms occurred a few weeks following her menarche, which she had at 13 years, and were observed ever since, either constantly or at intervals of a few days. The symptoms were not relieved despite several attempts of oral antibiotic treatment. Her menses had been regular, lasting for 3 to 4 days and occurring at 30-day intervals. At the age of 12 years she had undergone an emergent appendectomy. During this operation the uterine malformation (class III, didelphys uterus) had been detected. The abdominal/pelvic ultrasound performed at that time had revealed renal agenesis on the right side. The patient was not followed up, further diagnosed, or counseled regarding her malformations. At the time of her visit to our facility the patient did not complain of any pain. Palpation of the abdomen did not reveal any masses or abdominal tenderness. The external genitalia appeared normal. The hymen was intact. A firm, large mass was palpable on rectal examination slightly to the right from the midline. Rectal pelvic ultrasound showed a double uterus with a double cervix without hematometras. In addition, we found an echogenic structure (5.0 cm 4.0 cm) on the right side of the vagina, in the place where the pelvic mass was detected during the rectal examination.
The authors indicate no conflicts of interests. * Address correspondience to: Ewa Wozniakowska, MD, PhD, III Katedra i Klinika Ginekologii Uniwersytetu Medycznego w Lublinie, ul. Jaczewskiego 8, 20-090 Lublin, Poland; Phone: þ48 81 7244848; fax: þ48 81 7244847 E-mail address: [email protected] (E. Wozniakowska).
1083-3188/$ - see front matter Ó 2013 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2013.07.009
2
E. Wozniakowska et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e3
It indicated a possibility of pyocolpos in the right, occluded hemivagina. MRI of the abdomen and pelvis was performed in order to obtain more detailed information. MRI revealed normal ovaries, didelphic uterus without distension of uterine cavities, normal left cervix and hemivagina and a largely distended right hemivagina (2.6 cm 4.3 cm 2.5 cm). Signal intensity of the contents of right hemivagina was suggestive of hemato-pyocolpos. In addition, on this occasion, right renal agenesis was confirmed and other possible urinary tract anomalies were excluded (Fig. 1). The patient was scheduled for surgical treatment. Prior to operation, vaginal examination was performed under general anesthesia without breaking the hymenal membrane. This procedure revealed one vaginal canal with one cervical opening. The vaginal wall on the right side was bulging. On the right side of the examined vaginal canal there was a fistula leading from the obstructed vaginal canal to the left open hemivagina (Fig. 2). The longitudinal vaginal septum was excised by means of electrocauterization under direct visualization. After the incision, a purulent fluid flowed out of the right hemivagina. At this stage we examined the right hemivagina with the use of a hysteroscope. This examination confirmed the presence of the right uterine cervix communicating with the right hemivagina. We concluded that the pyocolpos was slowly draining through the fistula in the vaginal septum resulting in an infection and chronic discharge. After the successful surgery, a follow-up examination was carried out in the outpatient clinic. No complications were found 1 month and 3 months after surgery. As of the date of submission of this manuscript the patient is still asymptomatic and has regular menses. Summary and Conclusion
A syndrome consisting of renal agenesis and obstructed hemivagina was first described in 1971 by Herlyn and Werner. In 1976, Wunderlich broadened the description of the syndrome by adding bicornate uterus. Hence, HWWS € llerian anomaly consisting of denotes a rare congenital mu
Fig. 1. Transversal MRI demonstrating left hemivagina and pyocolpos on right side. B, urinary bladder, R, right hemivagina, *, left hemivagina.
Fig. 2. Vaginoscopic view of fistula/perforation in longitudinal septum.Ă
uterus didelphys, longitudinal hemivaginal septum creating a blind hemivagina, and renal agenesis on the same side. The syndrome is also described in the literature as OHVIRA (obstructed hemivagina and ipsilateral renal agenesis).1,5 € llerian anomaly usually Patients with an obstructing mu suffer from pelvic pain and have an abnormal abdominal mass. Other signs and symptoms are rare and include: fever, pyocolpos, peritonitis, hematometra, vaginal discharge and urinary incontinence.6,7 In 2006, Gholoum et al1 presented their experience with HWWS. During the period between 1982 and 2004 twelve patients were diagnosed with this syndrome and it is one of the largest groups ever described in the literature with this € llerian anomaly. Anatomically, 10 of the 12 variant of mu patients had classic symptoms of HWWS. Abdominal pain and a pelvic mass were the major reported complaints. All patients were treated by septectomy and the treatment was effective in all the cases. In conclusion, surgical excision of the longitudinal vaginal septum is recommended as the procedure of choice; however, in some cases pyocolpos caused by the closure of the septal incision has been reported as a complication after surgery. Varras et al5 demonstrated the difficulty in reaching the correct diagnosis of HWWS, especially one occurring after many years since menarche. They described a 24-year-old woman admitted to the emergency room with vomiting, severe and increasing lower abdominal pain, and purulent vaginal discharge. The preoperative diagnosis was ruptured tubo-ovarian abscess, for which an emergency laparotomy was performed. Vaginal examination under general anesthesia revealed an obstructed right hemivagina with vaginal purulent discharge from a small fistula in the septate vagina. The final diagnosis was uterus didelphys with unilateral perforated right hemivagina and pyocolpos. The diagnosis of our case was difficult because the classic HWWS symptoms were absent and the only symptom was vaginal discharge. The lack of classical HWWS symptoms had resulted in misdiagnosis and long-term antibiotic therapy in an outpatient clinic before her visit to our facility.
E. Wozniakowska et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e3
Precise physical examination and imaging lead to the correct diagnosis. Recognizing Herlyn-Werner-Wunderlich syndrome can be difficult, because its symptoms may not be typical and vaginal discharge can have many other causes, leading to misdiagnosis. Microperforation may occur in the hemivaginal septum on the obstructed side, thereby draining into the nonobstructed vagina, resulting in ascending infection. However, most authors agree that HWWS should be considered to prevent misdiagnosis or suboptimal treatment, to decrease morbidity and to avoid unnecessary surgical procedures. Our case confirms that, at minimum, it should be routinely considered in the differential diagnosis in young patients with uterus didelphys or bicornate uterus and unusual symptoms such as vaginal discharge.
3
References 1. Gholoum S, Puligandla PS, Hui T, et al: 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 2. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, € llerian anomalies and intrauterine adhesions. Fertil Steril 1988; 49:944 mu 3. Smith NA, Laufer MR: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007; 87:918 4. Wu TH, Wu TT, Ng YY, et al: Herlyn-Werner-Wunderlich syndrome consisting of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis in a newborn. Pediatr Neonatol 2012; 53:68 5. Varras M, Akrivis Ch, Karadaglis S, et al: Uterus didelphys with blind hemivagina and ipsilateral renal agenesis complicated by pyocolpos and presenting as acute abdomen 11 years after menarche: presentation of a rare case with review of the literature. Clin Exp Obstet Gynecol 2008; 35:156 6. Olsen ME, Breuel KF, Thatcher SS: Communicating double uterus with obstructed hemivagina and subsequent abscess formation: A case report. J Pediatr Adolesc Gynecol 1995; 8:35 7. Savitski JL, Olsen ME: Successful pregnancy in an adolescent woman with a communicating double uterine anomaly. J Pediatr Adolesc Gynecol 2001; 14:181
