Herlyn-Werner-Wunderlich Syndrome With Preterm Pregnancy The Presentation Of A Rare Case
HERLYN-WERNER-WUNDERLICH SYNDROME WITH PRETERM PREGNANCY THE PRESENTATION OF A RARE CASE
Muhammad Rusda
Division of Reproductive Endocrinology and Infertility Department of Obstetrics and Gynecology
School of Medicine, Universitas Sumatera Utara Medan, December 2014
ABSTRACT Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian structures, and characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain right before menarche, causing a delay in diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Mullerian Duct Anomalies (MDA) are infrequently encountered in a routine clinical setting. Case presentation: 19-years old female, primigravida with acute abdomen symptoms. Abdominal ectopic pregnancy was suspected by clinical examination. After that,laparatomy was performed and we found didelphys uterus with one part of them was being pregnant, ruptured at the cornu site and absence of her left kidney. Discussion: The reproductive outcome in cases of a didelphys uterus has been studied and compared with groups of uterine congenital anomalies. In the literature, didelphys uterus was found to give a good chance for a succesful pregnancy, better than other uterine anomalies. As we observed in the literature and our case, fertility in women with a didelphic uterus is not significantly impaired. The prognosis of pregnancies is comparatively good, with a high fetal survival rate, while an increased risk of preterm delivery. Conclusion: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare syndrome of Mullerian and Wolffian duct abnormalities. Because of the rarity of this syndrome, it is frequently misdiagnosed or diagnosed late. Keywords: Herlyn-Werner-Wunderlich Syndrome, Mullerian Duct Anomalies.
Introduction
Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the
urogenital tract involving Mullerian ducts and Wolffian structures, and it is characterized by the
triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally
occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain
right before menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Mullerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting.1,2
It is difficult to estimate the exact incidence of obstructive Mullerian anomalies but it is reported to be around 0,1-3,8% .The incidence of Herlyn-Werner-Wunderlich (HWW) syndrome is also not precisely known, but according to the literature it is estimated as 0,1-3,5% of all Mullerian anomalies.3 The incidence of didelphys uterus, related to HWW, is approximately 1 / 2,000 to 1 / 28,000 , and it is accompanied by unilateral renal agenesis in 43% of cases. The incidence of unilateral renal agenesis is 1 / 1,100, and 25-50% of affected women exhibits associated genital abnormalities .1,4
The potential complications of this syndrome are distinct in acute complications, such as pyohematocolpos, pyosalpinx, or pelvic peritonitis and long-term complications ; such as endometriosis, pelvic adhesions and increased risk of abortion or infertility.1,5,6
Case Presentation A 19- years old javanese female who was primigravida come to emergency room with acute abdomen symptoms. From clinical examination, the patient was suspected with abdominal ectopic pregnancy. When the patient was 10 years old she was being diagnosed with ipsilateral renal agenesis (absence of left kidney) by the internist. Dysmenorhae was found. The LMP was 20 January 2014 (30 -31 wga), the patient was tachycardia, a mass was palpable on left abdomen. Then we done the USG for the patient with conclusions primigravida (30-31 wga) with abdominal ectopic pregnancy. In this case we decided to performed the laparatomy and csection procedure to this patient. In operation we found cullen sign on the peritoneum, didelphys uterus with enlargement and pregnant of the left site uterine, the vessels of left uterine cornu had been ruptured, results of intra-abdominal bleeding then C-section was performed, baby girl was born, 1300 gram, 38 cm of length. There is endometriosis and adhesion at right ovarium in posterior part of uterine.
Discussion The specific association of uterus didelphys, obstructed hemivagina with ipsilateral renal anomaly is well recognized. The triad has been described as Herlyn-Werner Wunderlich syndrome. The acronym obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is used to describe patients with OHVIRA, that is ,occurrence of two out of three components of the
triad.7,8 OHVIRA is a rare congenital anomaly, patients present with pelvic pain, worsening dysmenorrhea and an associated pelvic mass. As in our case, this is the only case in the last three year. The diagnosis of OHVIRA should be suspected in cases of women presenting with pelvic pain and a pelvic mass with ipsilateral renal agenesis, in our cases the patient come to emergency room with acute abdomen symptoms which from clinical examination the patient was suspected with abdominal ectopic pregnancy and when the patient was 10 years old she was being diagnosed with ipsilateral renal agenesis (absence of left kidney) by the internist, dysmenorhae was found and a mass was palpable on the left abdomen. 9,10
USG and MRI are the modalities of choice for the diagnosis and surgical planning of OHVIRA. Although USG can be used to diagnose this condition, MRI plays an important role in further characterizing the didelphic uterus, obstructed hemivagina and ipsilateral renal agenesis. Furthermore, MRI is superior to ultrasound by allowing for better characterization of anatomic relationships given its multiplanar capabilities and wider field of view.10 The USG has been done in this case with the result abdominal ectopic pregnancy with 29-30wga of pregnancy. MRI was not done in our case eventhough it is the gold standart to diagnosis this case because there is no MRI in our hospital and the patient came with emergency outcome. 7,9
Laparascopy prior to resection helps to rule out additional adnexal pathology and confirm the diagnosis if pre-operative MRI is not feasible. Resection under laparascopic control also help to avoid or detect an inadvertent bladder injury as the paravesical anatomy is distorted.9 Laparascopy can be done in order to clearly identify the uterine anomaly and endometriosis is frequent.11 in our case laparoscopy cannot be performed due to the patient came with acute abdomen. We decided to performed laparatomy and C-section procedure to this patient. In laparotomy, we found cullen sign on the peritoneum, didelphys uterus with enlargement and pregnant of the left site uterine, the vessels of left uterine cornu had been ruptured, results of intraabdominal bleeding then C-section was performed, baby girl was born, 1300gram, 38cm of length. There is endometriosis and adhesion at right ovarium in posterior part of uterine.
The potential complications of this syndrome are distinct in acute complications, such as pyohematocolpos, pyosalpinx, or pelvic peritonitis and long-term complications, such as
endometriosis, pelvic adhesions and increased risk of abortion or infertility.1 Fertility in women
with a didelphic uterus is not significantly impaired. The prognosis of pregnancies is
comparatively good, with a high fetal survival rate, while and increased risk of preterm delivery
(as described in the present case), premature rupture of membranes, fetal growth retardation and malpresentation indicates the need of meticulous prenatal care.12,13
Conclusion
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare syndrome of Mullerian and Wolffian
duct abnormalities . Because of the rarity of this syndrome, it is frequently misdiagnosed or
diagnosed late. Ultrasound and MRI findings can collectively delineate uterine morphology,
absence of the ipsilateral kidney and the nature of the fluid content in the obstructed hemivagina
in the end the best way to figure it out is from laparotomy.
Reference
1. Vescovo R, et al. Herlyn-Werner-Wunderlinch Syndrome: MRI Findings, Radiological Guide (Two Cases and Literature Review), and Differential Diagnosis. BioMed Central, 2012.
2. Hoeffel C, Olivier M, Scheffler C, et al. Uterus Didelphys with Unilateral Distal Vaginal Agenesis and Ipsilateral Renal Agenesis. European Journal of Radiology, 1997.
3. Agwany ASE. Herlyn-Werner-Wunderlinch Syndrome: A Case of Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA Syndrome) and Didelphys Uterus. African Journal of Urology, 2014.
4. Alan JW, Louis RK. Campbell Walsh Urology. Philadelphia: Saunders, 93270-6. 5. Kim TE, et al. Hysteroscopic Resection of The Vaginal Septum in Uterus Didelphys with
Obstructed Hemivagina: A Case Report, J Korean Med; 2007, 22:766-769. 6. Grimbizis GF, et al. Clinical Implication of Uterine Malformations and Hysteroscopic
Treatment Results. Hum Reprod Update; 2001, 7:161-174 7. Sharma D, et al. Herlyn-Werner-Wunderlinch Syndrome Complicated with Pyocolpos: An
Unusual Cause of Postabortal Sepsis. Journal of Gynaecological Endoscopy and Surgery, 2011. 8. Smith NA, Laufer MR. Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome: Management and Follow Up. Fertil Steril, 2007; 87; 918-22. 9. Burgin, J. Obstructed Mullerian Anomalies: Case Report, Diagnosis, and Management. Am J ObstetGynaecol, 2001; 185: 338-44. 10. Cox D, Ching BH. Herlyn-Werner-Wunderlinch Syndrome: A Rare Presentation with Pyocolpos. Journal of Radiology Case Reports., 2012. 11. Cetinkya SE, et al. Hysteroscopic Management of Vaginal Septum in a Vaginal Patients with Uterus Didelphys and Obstructed Hemivagina. Fertil Steril, 2011, 96: 16-8.
12. Cozzolino M, et al. Two Successful Pregnancies in Herlyn-Werner-Wunderlich Syndrome. Obstetric Case Reports, 2014.
13. Heinonen PK. Clinical Implications of The Didelphys Uterus Long Term Follow Up of 49 Cases. European Journal of Obstetrics Gynaecology and Reproductive Biology, 2000, 91;183-190.
Muhammad Rusda
Division of Reproductive Endocrinology and Infertility Department of Obstetrics and Gynecology
School of Medicine, Universitas Sumatera Utara Medan, December 2014
ABSTRACT Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian structures, and characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain right before menarche, causing a delay in diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Mullerian Duct Anomalies (MDA) are infrequently encountered in a routine clinical setting. Case presentation: 19-years old female, primigravida with acute abdomen symptoms. Abdominal ectopic pregnancy was suspected by clinical examination. After that,laparatomy was performed and we found didelphys uterus with one part of them was being pregnant, ruptured at the cornu site and absence of her left kidney. Discussion: The reproductive outcome in cases of a didelphys uterus has been studied and compared with groups of uterine congenital anomalies. In the literature, didelphys uterus was found to give a good chance for a succesful pregnancy, better than other uterine anomalies. As we observed in the literature and our case, fertility in women with a didelphic uterus is not significantly impaired. The prognosis of pregnancies is comparatively good, with a high fetal survival rate, while an increased risk of preterm delivery. Conclusion: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare syndrome of Mullerian and Wolffian duct abnormalities. Because of the rarity of this syndrome, it is frequently misdiagnosed or diagnosed late. Keywords: Herlyn-Werner-Wunderlich Syndrome, Mullerian Duct Anomalies.
Introduction
Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the
urogenital tract involving Mullerian ducts and Wolffian structures, and it is characterized by the
triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally
occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain
right before menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Mullerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting.1,2
It is difficult to estimate the exact incidence of obstructive Mullerian anomalies but it is reported to be around 0,1-3,8% .The incidence of Herlyn-Werner-Wunderlich (HWW) syndrome is also not precisely known, but according to the literature it is estimated as 0,1-3,5% of all Mullerian anomalies.3 The incidence of didelphys uterus, related to HWW, is approximately 1 / 2,000 to 1 / 28,000 , and it is accompanied by unilateral renal agenesis in 43% of cases. The incidence of unilateral renal agenesis is 1 / 1,100, and 25-50% of affected women exhibits associated genital abnormalities .1,4
The potential complications of this syndrome are distinct in acute complications, such as pyohematocolpos, pyosalpinx, or pelvic peritonitis and long-term complications ; such as endometriosis, pelvic adhesions and increased risk of abortion or infertility.1,5,6
Case Presentation A 19- years old javanese female who was primigravida come to emergency room with acute abdomen symptoms. From clinical examination, the patient was suspected with abdominal ectopic pregnancy. When the patient was 10 years old she was being diagnosed with ipsilateral renal agenesis (absence of left kidney) by the internist. Dysmenorhae was found. The LMP was 20 January 2014 (30 -31 wga), the patient was tachycardia, a mass was palpable on left abdomen. Then we done the USG for the patient with conclusions primigravida (30-31 wga) with abdominal ectopic pregnancy. In this case we decided to performed the laparatomy and csection procedure to this patient. In operation we found cullen sign on the peritoneum, didelphys uterus with enlargement and pregnant of the left site uterine, the vessels of left uterine cornu had been ruptured, results of intra-abdominal bleeding then C-section was performed, baby girl was born, 1300 gram, 38 cm of length. There is endometriosis and adhesion at right ovarium in posterior part of uterine.
Discussion The specific association of uterus didelphys, obstructed hemivagina with ipsilateral renal anomaly is well recognized. The triad has been described as Herlyn-Werner Wunderlich syndrome. The acronym obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is used to describe patients with OHVIRA, that is ,occurrence of two out of three components of the
triad.7,8 OHVIRA is a rare congenital anomaly, patients present with pelvic pain, worsening dysmenorrhea and an associated pelvic mass. As in our case, this is the only case in the last three year. The diagnosis of OHVIRA should be suspected in cases of women presenting with pelvic pain and a pelvic mass with ipsilateral renal agenesis, in our cases the patient come to emergency room with acute abdomen symptoms which from clinical examination the patient was suspected with abdominal ectopic pregnancy and when the patient was 10 years old she was being diagnosed with ipsilateral renal agenesis (absence of left kidney) by the internist, dysmenorhae was found and a mass was palpable on the left abdomen. 9,10
USG and MRI are the modalities of choice for the diagnosis and surgical planning of OHVIRA. Although USG can be used to diagnose this condition, MRI plays an important role in further characterizing the didelphic uterus, obstructed hemivagina and ipsilateral renal agenesis. Furthermore, MRI is superior to ultrasound by allowing for better characterization of anatomic relationships given its multiplanar capabilities and wider field of view.10 The USG has been done in this case with the result abdominal ectopic pregnancy with 29-30wga of pregnancy. MRI was not done in our case eventhough it is the gold standart to diagnosis this case because there is no MRI in our hospital and the patient came with emergency outcome. 7,9
Laparascopy prior to resection helps to rule out additional adnexal pathology and confirm the diagnosis if pre-operative MRI is not feasible. Resection under laparascopic control also help to avoid or detect an inadvertent bladder injury as the paravesical anatomy is distorted.9 Laparascopy can be done in order to clearly identify the uterine anomaly and endometriosis is frequent.11 in our case laparoscopy cannot be performed due to the patient came with acute abdomen. We decided to performed laparatomy and C-section procedure to this patient. In laparotomy, we found cullen sign on the peritoneum, didelphys uterus with enlargement and pregnant of the left site uterine, the vessels of left uterine cornu had been ruptured, results of intraabdominal bleeding then C-section was performed, baby girl was born, 1300gram, 38cm of length. There is endometriosis and adhesion at right ovarium in posterior part of uterine.
The potential complications of this syndrome are distinct in acute complications, such as pyohematocolpos, pyosalpinx, or pelvic peritonitis and long-term complications, such as
endometriosis, pelvic adhesions and increased risk of abortion or infertility.1 Fertility in women
with a didelphic uterus is not significantly impaired. The prognosis of pregnancies is
comparatively good, with a high fetal survival rate, while and increased risk of preterm delivery
(as described in the present case), premature rupture of membranes, fetal growth retardation and malpresentation indicates the need of meticulous prenatal care.12,13
Conclusion
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare syndrome of Mullerian and Wolffian
duct abnormalities . Because of the rarity of this syndrome, it is frequently misdiagnosed or
diagnosed late. Ultrasound and MRI findings can collectively delineate uterine morphology,
absence of the ipsilateral kidney and the nature of the fluid content in the obstructed hemivagina
in the end the best way to figure it out is from laparotomy.
Reference
1. Vescovo R, et al. Herlyn-Werner-Wunderlinch Syndrome: MRI Findings, Radiological Guide (Two Cases and Literature Review), and Differential Diagnosis. BioMed Central, 2012.
2. Hoeffel C, Olivier M, Scheffler C, et al. Uterus Didelphys with Unilateral Distal Vaginal Agenesis and Ipsilateral Renal Agenesis. European Journal of Radiology, 1997.
3. Agwany ASE. Herlyn-Werner-Wunderlinch Syndrome: A Case of Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA Syndrome) and Didelphys Uterus. African Journal of Urology, 2014.
4. Alan JW, Louis RK. Campbell Walsh Urology. Philadelphia: Saunders, 93270-6. 5. Kim TE, et al. Hysteroscopic Resection of The Vaginal Septum in Uterus Didelphys with
Obstructed Hemivagina: A Case Report, J Korean Med; 2007, 22:766-769. 6. Grimbizis GF, et al. Clinical Implication of Uterine Malformations and Hysteroscopic
Treatment Results. Hum Reprod Update; 2001, 7:161-174 7. Sharma D, et al. Herlyn-Werner-Wunderlinch Syndrome Complicated with Pyocolpos: An
Unusual Cause of Postabortal Sepsis. Journal of Gynaecological Endoscopy and Surgery, 2011. 8. Smith NA, Laufer MR. Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome: Management and Follow Up. Fertil Steril, 2007; 87; 918-22. 9. Burgin, J. Obstructed Mullerian Anomalies: Case Report, Diagnosis, and Management. Am J ObstetGynaecol, 2001; 185: 338-44. 10. Cox D, Ching BH. Herlyn-Werner-Wunderlinch Syndrome: A Rare Presentation with Pyocolpos. Journal of Radiology Case Reports., 2012. 11. Cetinkya SE, et al. Hysteroscopic Management of Vaginal Septum in a Vaginal Patients with Uterus Didelphys and Obstructed Hemivagina. Fertil Steril, 2011, 96: 16-8.
12. Cozzolino M, et al. Two Successful Pregnancies in Herlyn-Werner-Wunderlich Syndrome. Obstetric Case Reports, 2014.
13. Heinonen PK. Clinical Implications of The Didelphys Uterus Long Term Follow Up of 49 Cases. European Journal of Obstetrics Gynaecology and Reproductive Biology, 2000, 91;183-190.