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New classification of Herlyn-Werner-Wunderlich syndrome.

Zhu L, Chen N, Tong JL, Wang W, Zhang L, Lang JH - Chin. Med. J. (2015)

Bottom Line: The clinical details associated with these two types are distinctly different.HWWS patients should be differentiated according to these two classifications.The two classifications could be generalized by gynecologists world-wide.

View Article: PubMed Central - PubMed

Affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.

ABSTRACT

Background: Uterus didelphys and blind hemivagina associated with ipsilateral renal agenesis are collectively known as Herlyn-Werner-Wunderlich syndrome (HWWS). In the literature, the syndrome often appears as a single case report or as a small series. In our study, we reviewed the characteristics of all HWWS patients at Peking Union Medical College Hospital (PUMCH) and suggested a new classification for this syndrome because the clinical characteristics differed significantly between the completely and incompletely obstructed vaginal septum. This new classification allows for earlier diagnosis and treatment.

Methods: From January 1986 to March 2013, all diagnosed cases of HWWS at PUMCH were reviewed. A retrospective long-term follow-up study of the clinical presentation, surgical prognosis, and pregnancy outcomes was performed. Statistical analyses were performed using SPSS, version 15.0 (IBM, Armonk, NY, USA). Between-group comparisons were performed using the χ2 test, Fisher's exact test, and the t-test. The significance level for all analyses was set at P < 0.05.

Results: The clinical data from 79 patients with HWWS were analyzed until March 31, 2013. According to our newly identified characteristics, we recommend that the syndrome be classified by the complete or incomplete obstruction of the hemivagina as follows: Classification 1, a completely obstructed hemivagina and Classification 2, an incompletely obstructed hemivagina. The clinical details associated with these two types are distinctly different.

Conclusions: HWWS patients should be differentiated according to these two classifications. The two classifications could be generalized by gynecologists world-wide.

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Related in: MedlinePlus

Classification 1.1, with blind hemivagina.
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Figure 1: Classification 1.1, with blind hemivagina.

Mentions: In this classification, the hemivagina is completely obstructed; the uterus behind the septum is completely isolated from the contralateral uterus, and no communication is present between the duplicated uterus and vagina. Hematocolpos may occur only a few months after menarche. Hematometra and hematosalpinx occurred in some more severely affected patients, as well as bleeding in the periadnexal and peritoneal space. Patients with this classification have an earlier age of onset, with a short time from menarche to attack. The presenting symptoms may include the acute onset of abdominal pain, fever, and vomiting. Hemoperitoneum, due to bleeding from the fallopian tube, can be found at surgery.[510] Endometriosis can result from blood reflux into the abdominal cavity and may have dire consequences. If not treated in time, the condition can progress to secondary endometriosis, pelvic adhesion, pyosalpinx, and even pyocolpos [Figure 1].[67]


New classification of Herlyn-Werner-Wunderlich syndrome.

Zhu L, Chen N, Tong JL, Wang W, Zhang L, Lang JH - Chin. Med. J. (2015)

Classification 1.1, with blind hemivagina.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4837842&req=5

Figure 1: Classification 1.1, with blind hemivagina.
Mentions: In this classification, the hemivagina is completely obstructed; the uterus behind the septum is completely isolated from the contralateral uterus, and no communication is present between the duplicated uterus and vagina. Hematocolpos may occur only a few months after menarche. Hematometra and hematosalpinx occurred in some more severely affected patients, as well as bleeding in the periadnexal and peritoneal space. Patients with this classification have an earlier age of onset, with a short time from menarche to attack. The presenting symptoms may include the acute onset of abdominal pain, fever, and vomiting. Hemoperitoneum, due to bleeding from the fallopian tube, can be found at surgery.[510] Endometriosis can result from blood reflux into the abdominal cavity and may have dire consequences. If not treated in time, the condition can progress to secondary endometriosis, pelvic adhesion, pyosalpinx, and even pyocolpos [Figure 1].[67]

Bottom Line: The clinical details associated with these two types are distinctly different.HWWS patients should be differentiated according to these two classifications.The two classifications could be generalized by gynecologists world-wide.

View Article: PubMed Central - PubMed

Affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.

ABSTRACT

Background: Uterus didelphys and blind hemivagina associated with ipsilateral renal agenesis are collectively known as Herlyn-Werner-Wunderlich syndrome (HWWS). In the literature, the syndrome often appears as a single case report or as a small series. In our study, we reviewed the characteristics of all HWWS patients at Peking Union Medical College Hospital (PUMCH) and suggested a new classification for this syndrome because the clinical characteristics differed significantly between the completely and incompletely obstructed vaginal septum. This new classification allows for earlier diagnosis and treatment.

Methods: From January 1986 to March 2013, all diagnosed cases of HWWS at PUMCH were reviewed. A retrospective long-term follow-up study of the clinical presentation, surgical prognosis, and pregnancy outcomes was performed. Statistical analyses were performed using SPSS, version 15.0 (IBM, Armonk, NY, USA). Between-group comparisons were performed using the χ2 test, Fisher's exact test, and the t-test. The significance level for all analyses was set at P < 0.05.

Results: The clinical data from 79 patients with HWWS were analyzed until March 31, 2013. According to our newly identified characteristics, we recommend that the syndrome be classified by the complete or incomplete obstruction of the hemivagina as follows: Classification 1, a completely obstructed hemivagina and Classification 2, an incompletely obstructed hemivagina. The clinical details associated with these two types are distinctly different.

Conclusions: HWWS patients should be differentiated according to these two classifications. The two classifications could be generalized by gynecologists world-wide.

Show MeSH
Related in: MedlinePlus