The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–3, 2014 Copyright Ó 2014 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter
http://dx.doi.org/10.1016/j.jemermed.2014.09.064
Clinical Communications: OB/GYN HERLYN-WERNER-WUNDERLICH SYNDROME: A VERY RARE UROGENITAL ANOMALY IN A TEENAGE GIRL Leyla Karaca, MD,* Berhan Pirimoglu, MD,* Ummugulsum Bayraktutan, MD,* Hayri Ogul, MD,* Akgun Oral, MD,† and Mecit Kantarci, MD, PHD* *Department of Radiology, Ataturk University, School of Medicine, Erzurum, Turkey and †Department of Pediatric Surgery, Ataturk University, School of Medicine, Erzurum, Turkey Reprint Address: Mecit Kantarci, MD, PHD, Department of Radiology, Ataturk University, School of Medicine, 200 Evler Mah. 14. Sok No 5, Dadaskent, Erzurum, Turkey
, Abstract—Background: Herlyn-Werner-Wunderlich (HWW) syndrome is an uncommon variant of Mu¨llerian duct anomalies, consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in a post-pubertal adolescent or adult woman in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Case Report: We report the case of a 13-year-old girl who presented to the emergency radiology department with sudden onset of severe pain at the right lower quadrant of the abdomen; imaging confirmed the diagnosis of HWW syndrome. Why should an emergency physician be aware of this?: When unilateral renal agenesis and uterus didelphys coexist, the first thing that the physician should remember is to confirm or refute the presence of a blind vagina for diagnosis of HWW syndrome. Ó 2014 Elsevier Inc.
gina, which produces a mass effect with pain. It can present rarely in prepubertal girls. The presence of other symptoms depends on the existence of uterine or vaginal communications (2,3). We report a case of a 13-year-old girl who presented to our emergency radiology department with acute onset of severe pain in the right lower quadrant of the abdomen. Abdominal ultrasonography (US) and magnetic resonance imaging (MRI) findings revealed a didelphys uterus and renal agenesis consistent with the diagnosis of HWW. CASE REPORT A 13-year-old girl was admitted to our emergency radiology department with a complaint of severe pain in the right lower quadrant of the abdomen for a duration of 2 days. Physical examination revealed right lower abdominal tenderness. On laboratory examinations there was no pathological finding. A gynecologic examination did not reveal any anomalies of the patient’s external genitalia or hymen. Abdominal US examination showed a cystic lesion in the right adnexial region and absence of the right kidney (Figure 1). An MRI revealed a uterine-vaginal malformation consisting of a didelphys uterus communicating with a double vagina; the right vagina was obstructed. There was a collection of fluid that exhibited a high signal intensity on T1 and a low signal
, Keywords—Herlyn Werner Wunderlich syndrome; hematocolpos; renal agenesis; Mu¨llerian duct anomalies
INTRODUCTION The triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly was first described in 1922 (1). Today, this triad is known as Herlyn-WernerWunderlich (HWW) syndrome. Clinically, HWW results in hydrometrocolpos on the side of an obstructed hemiva-
RECEIVED: 26 January 2014; FINAL SUBMISSION RECEIVED: 3 August 2014; ACCEPTED: 30 September 2014 1
2
L. Karaca et al.
Figure 3. This axial T1 weighted image demonstrates the collection of fluid that exhibited high signal intensity on T1 sequence in the right uterus (star) and uterine didelphys (white arrow).
Figure 1. This transverse abdominal ultrasonography image reveals a cystic lesion that is hypoechoic, including the heterogeneous component at the right adnexial region. H = hematometra.
intensity on T2 sequences, both in the right uterus, and in the right obstructed vagina referred to as hematometrocolpos (Figures 2 and 3). This case was discussed with a colleague who is a pediatric surgeon with clinical experience and interest in the topic. In his evaluations, he stated that these appearances,
Figure 2. The coronal T2 weighted image demonstrates the left kidney (K), absence of the right kidney, a collection of fluid that exhibits low signal intensity on T2 sequence (star) and uterine didelphys (black arrow).
particularly those related to the obstructed vagina, didelphys uterus, and absence of the kidney, were very similar to HWW syndrome. Surgery confirmed the diagnosis of HWW syndrome, defined by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. In surgery, resection of the obstructing vaginal septum and drainage of the hematocolpos and hematometra was performed. DISCUSSION HWW syndrome is a very rare congenital anomaly of the urogenital tract involving Mu¨llerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. Clinical presentation is usually with severe dysmenorrhea starting after the onset of menstruation; the presence of other symptoms depends on the existence of uterine or vaginal communications. The diagnosis is typically made after menarche due to the recurrent pain; however, incomplete obstruction may cause mild intermittent symptoms that do not worsen until complete obstruction and hematocolpos occur (3,4). The Mu¨llerian ducts migrate to the midline and then fuse to form the uterus, cervix, and upper part of the vagina in the intrauterine 8th week. Incomplete or absent fusion may result in the formation of two hemiuteri at the 8th week. Renal agenesis often accompanies Mu¨llerian duct abnormalities because the kidneys, fallopian tube, ovary, cervix, and upper vagina all generate from the same ureteric bud that fails to form correctly (5,6). Radiological assessment plays an important role in making the diagnosis of HWW (7). US is important in the initial evaluation of undifferentiated pelvic pain, although US was not able to clearly identify the anatomic abnormality in this patient. Computed tomography (CT) or magnetic resonance imaging (MRI) can further characterize structural abnormalities and can aid in planning for surgical management. MRI has high contrast resolution with a lack of ionizing radiation (7). In our routine US
Herlyn-Werner-Wunderlich Syndrome in a Teenage Girl
and MRI examinations, we detected similar appearances to those in the Beer and Carstairs study (3). They reported a case of a 19-year-old woman who presented to the emergency department with sudden onset of severe vaginal pain that was determined to be due to hematocolpos; imaging confirmed the diagnosis of HWW syndrome. Their case had a history of 4 h after the abrupt onset of severe vaginal pain, and they demonstrated their case by CT examination (3). We diagnosed our case after menarche by US and MRI examinations. The approach to the surgical treatment is reconstruction of the vagina, which can be accomplished easily by careful excision of the vaginal septum, with precautions taken against injury to the urethra, bladder, and rectum. Rarely, resection of the vaginal septum may not be enough to relieve the hematometra, and such cases may mandate a hysterectomy (8). WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS? In conclusion, although rare, HWW can result in the acute onset of severe abdominal pain. It is a rare condition that is seen mainly in the post-pubertal woman. It presents with severe abdominal pain and a cystic mass secondary
3
to hematometrocolpos. Ipsilateral renal agenesis with a pelvic mass in a young woman should suggest the diagnosis of the HWW syndrome. MRI should be performed to confirm of the diagnosis. REFERENCES 1. Purslow C. A case of unilateral haematocolpos, haematomethra and haematosalpinx. J Obstet Gynaecol Br Emp 1922;29:643. 2. Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina and ipsilateral renal agenesis: 36 cases and long-term followup. Obstet Gynecol 1997;90:26–32. 3. Beer WM, Carstairs SD. Herlyn Werner Wunderlich syndrome: an unusual presentation of acute vaginal pain. J Emerg Med 2013;45:541–3. 4. Han BH, Park SB, Lee YJ, et al. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich Syndrome) suspected on the presence of hydrocolpos on prenatal sonography. J Clin Ultrasound 2012;41:380. 5. Asha B, Manila K. An unusual presentation of uterus didelphys with obstructed hemivagina with ipsilateral renal agenesis. Fertil Steril 2008;90:849. 6. Takagi H, Matsunami K, Imai A. Uterovaginal duplication with blind hemivagina and ipsilateral renal agenesis: review of unusual presentation. J Obstet Gynaecol 2010;30:350–3. 7. Li S, Qayyum A, Coakley FV, Hricak H. Association of renal agenesis and Mullerian duct abnormalities. J Comput Assist Tomogr 2000;24:829–34. 8. Donnez O, Jadoul P, Squifflet J, et al. Didelphic uterus and obstructed hemivagina: recurrent hematometra in spite of appropriate classic surgical treatment. Gynecol Obstet Invest 2007;63:98.
http://dx.doi.org/10.1016/j.jemermed.2014.09.064
Clinical Communications: OB/GYN HERLYN-WERNER-WUNDERLICH SYNDROME: A VERY RARE UROGENITAL ANOMALY IN A TEENAGE GIRL Leyla Karaca, MD,* Berhan Pirimoglu, MD,* Ummugulsum Bayraktutan, MD,* Hayri Ogul, MD,* Akgun Oral, MD,† and Mecit Kantarci, MD, PHD* *Department of Radiology, Ataturk University, School of Medicine, Erzurum, Turkey and †Department of Pediatric Surgery, Ataturk University, School of Medicine, Erzurum, Turkey Reprint Address: Mecit Kantarci, MD, PHD, Department of Radiology, Ataturk University, School of Medicine, 200 Evler Mah. 14. Sok No 5, Dadaskent, Erzurum, Turkey
, Abstract—Background: Herlyn-Werner-Wunderlich (HWW) syndrome is an uncommon variant of Mu¨llerian duct anomalies, consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in a post-pubertal adolescent or adult woman in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Case Report: We report the case of a 13-year-old girl who presented to the emergency radiology department with sudden onset of severe pain at the right lower quadrant of the abdomen; imaging confirmed the diagnosis of HWW syndrome. Why should an emergency physician be aware of this?: When unilateral renal agenesis and uterus didelphys coexist, the first thing that the physician should remember is to confirm or refute the presence of a blind vagina for diagnosis of HWW syndrome. Ó 2014 Elsevier Inc.
gina, which produces a mass effect with pain. It can present rarely in prepubertal girls. The presence of other symptoms depends on the existence of uterine or vaginal communications (2,3). We report a case of a 13-year-old girl who presented to our emergency radiology department with acute onset of severe pain in the right lower quadrant of the abdomen. Abdominal ultrasonography (US) and magnetic resonance imaging (MRI) findings revealed a didelphys uterus and renal agenesis consistent with the diagnosis of HWW. CASE REPORT A 13-year-old girl was admitted to our emergency radiology department with a complaint of severe pain in the right lower quadrant of the abdomen for a duration of 2 days. Physical examination revealed right lower abdominal tenderness. On laboratory examinations there was no pathological finding. A gynecologic examination did not reveal any anomalies of the patient’s external genitalia or hymen. Abdominal US examination showed a cystic lesion in the right adnexial region and absence of the right kidney (Figure 1). An MRI revealed a uterine-vaginal malformation consisting of a didelphys uterus communicating with a double vagina; the right vagina was obstructed. There was a collection of fluid that exhibited a high signal intensity on T1 and a low signal
, Keywords—Herlyn Werner Wunderlich syndrome; hematocolpos; renal agenesis; Mu¨llerian duct anomalies
INTRODUCTION The triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly was first described in 1922 (1). Today, this triad is known as Herlyn-WernerWunderlich (HWW) syndrome. Clinically, HWW results in hydrometrocolpos on the side of an obstructed hemiva-
RECEIVED: 26 January 2014; FINAL SUBMISSION RECEIVED: 3 August 2014; ACCEPTED: 30 September 2014 1
2
L. Karaca et al.
Figure 3. This axial T1 weighted image demonstrates the collection of fluid that exhibited high signal intensity on T1 sequence in the right uterus (star) and uterine didelphys (white arrow).
Figure 1. This transverse abdominal ultrasonography image reveals a cystic lesion that is hypoechoic, including the heterogeneous component at the right adnexial region. H = hematometra.
intensity on T2 sequences, both in the right uterus, and in the right obstructed vagina referred to as hematometrocolpos (Figures 2 and 3). This case was discussed with a colleague who is a pediatric surgeon with clinical experience and interest in the topic. In his evaluations, he stated that these appearances,
Figure 2. The coronal T2 weighted image demonstrates the left kidney (K), absence of the right kidney, a collection of fluid that exhibits low signal intensity on T2 sequence (star) and uterine didelphys (black arrow).
particularly those related to the obstructed vagina, didelphys uterus, and absence of the kidney, were very similar to HWW syndrome. Surgery confirmed the diagnosis of HWW syndrome, defined by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. In surgery, resection of the obstructing vaginal septum and drainage of the hematocolpos and hematometra was performed. DISCUSSION HWW syndrome is a very rare congenital anomaly of the urogenital tract involving Mu¨llerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. Clinical presentation is usually with severe dysmenorrhea starting after the onset of menstruation; the presence of other symptoms depends on the existence of uterine or vaginal communications. The diagnosis is typically made after menarche due to the recurrent pain; however, incomplete obstruction may cause mild intermittent symptoms that do not worsen until complete obstruction and hematocolpos occur (3,4). The Mu¨llerian ducts migrate to the midline and then fuse to form the uterus, cervix, and upper part of the vagina in the intrauterine 8th week. Incomplete or absent fusion may result in the formation of two hemiuteri at the 8th week. Renal agenesis often accompanies Mu¨llerian duct abnormalities because the kidneys, fallopian tube, ovary, cervix, and upper vagina all generate from the same ureteric bud that fails to form correctly (5,6). Radiological assessment plays an important role in making the diagnosis of HWW (7). US is important in the initial evaluation of undifferentiated pelvic pain, although US was not able to clearly identify the anatomic abnormality in this patient. Computed tomography (CT) or magnetic resonance imaging (MRI) can further characterize structural abnormalities and can aid in planning for surgical management. MRI has high contrast resolution with a lack of ionizing radiation (7). In our routine US
Herlyn-Werner-Wunderlich Syndrome in a Teenage Girl
and MRI examinations, we detected similar appearances to those in the Beer and Carstairs study (3). They reported a case of a 19-year-old woman who presented to the emergency department with sudden onset of severe vaginal pain that was determined to be due to hematocolpos; imaging confirmed the diagnosis of HWW syndrome. Their case had a history of 4 h after the abrupt onset of severe vaginal pain, and they demonstrated their case by CT examination (3). We diagnosed our case after menarche by US and MRI examinations. The approach to the surgical treatment is reconstruction of the vagina, which can be accomplished easily by careful excision of the vaginal septum, with precautions taken against injury to the urethra, bladder, and rectum. Rarely, resection of the vaginal septum may not be enough to relieve the hematometra, and such cases may mandate a hysterectomy (8). WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS? In conclusion, although rare, HWW can result in the acute onset of severe abdominal pain. It is a rare condition that is seen mainly in the post-pubertal woman. It presents with severe abdominal pain and a cystic mass secondary
3
to hematometrocolpos. Ipsilateral renal agenesis with a pelvic mass in a young woman should suggest the diagnosis of the HWW syndrome. MRI should be performed to confirm of the diagnosis. REFERENCES 1. Purslow C. A case of unilateral haematocolpos, haematomethra and haematosalpinx. J Obstet Gynaecol Br Emp 1922;29:643. 2. Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina and ipsilateral renal agenesis: 36 cases and long-term followup. Obstet Gynecol 1997;90:26–32. 3. Beer WM, Carstairs SD. Herlyn Werner Wunderlich syndrome: an unusual presentation of acute vaginal pain. J Emerg Med 2013;45:541–3. 4. Han BH, Park SB, Lee YJ, et al. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich Syndrome) suspected on the presence of hydrocolpos on prenatal sonography. J Clin Ultrasound 2012;41:380. 5. Asha B, Manila K. An unusual presentation of uterus didelphys with obstructed hemivagina with ipsilateral renal agenesis. Fertil Steril 2008;90:849. 6. Takagi H, Matsunami K, Imai A. Uterovaginal duplication with blind hemivagina and ipsilateral renal agenesis: review of unusual presentation. J Obstet Gynaecol 2010;30:350–3. 7. Li S, Qayyum A, Coakley FV, Hricak H. Association of renal agenesis and Mullerian duct abnormalities. J Comput Assist Tomogr 2000;24:829–34. 8. Donnez O, Jadoul P, Squifflet J, et al. Didelphic uterus and obstructed hemivagina: recurrent hematometra in spite of appropriate classic surgical treatment. Gynecol Obstet Invest 2007;63:98.
