(Professor and Head, Department of Obstetrics and Hynecology, Seth G S Medical College & K E M Hospital, Mumbai, India.)
The Herlyn–Werner–Wunderlich syndrome is characterized by uterus bicornis bicollis and double vagina, with one of the vagina’s ending as a blind pouch, causing hemihematometra. It usually presents normal onset of menarche, followed by progressively severe primary dysmenorrhea. A high degree of suspicion is necessary to detect it and treat it in time. A case of spontaneous resolution of hemihematometra which recurred and resolved is presented.
The Herlyn–Werner–Wunderlich (HWW) syndrome is characterized by uterus bicornis bicollis and double vagina, with one of the vagina’s ending as a blind pouch, causing hemihematometra. Because menstrual blood from one uterus continues to drain out cyclically through one patent vagina, attention is not drawn to the condition. There is severe primary dysmenorrhea due to hemihematometra. If not treated in time, it may lead to pelvic endometriosis. A case in which the hemihematometra drained into vagina spontaneously only to recur and resolve is presented. This is the first case of this type in the world literature.
A 14-year-old girl, presented with severe dysmenorrhea and a lump in lower abdomen. She had undergone ultrasonography (USG) prior to presenting to us. It showed uterus bicornis bicollis, with normal vagina on the right side and a blind pouch of vagina on the left side. There was hemihematometra on the left side, along with hemihematocolpos of upper vagina on the right side. The right kidney was absent. Her medical and surgical history was not contributory. She was not sexually active. Her general and systemic examination revealed no abnormality. There was a tender lump in the suprapubic region reaching 2 cm above the pubis. Her external genitals were normal and hymen was intact. A rectal examination showed a tense bulging cystic lump low in the right part and center of the pelvis, measuring about 5-6 cm in diameter. The uterus was felt separately from the mass. There was no other abnormality. A computerized tomographic (CT) scan of the abdomen and pelvis was done. It showed uterus bicornis bicollis, with normal vagina on the right side and a blind pouch of vagina on the left side. There was hemihematometra on the left side, along with hemihematocolpos of upper vagina on the right side. The right kidney was absent.
Investigations for fitness for anesthesia showed normal reports. She was scheduled to undergo surgical connection of the hematocolpos to the lower vagina. But when she reported to the hospital for admission, there was no pelvic lump. On enquiry she stated that she had menses three days ago, and had passed a lot of blood, which was mainly dark blackish in color. Pelvic ultrasonography was done. It showed no blood in the right hemiuterus and vagina. She was counselled that her condition had resolved by itself and was advised to follow up. She had regular menses subsequently. Her right hemihematometra recurred twice and resolved spontaneously by draining into lower vagina. She was counselled to undergo surgical enlargement of the opening of the hemihematometra into the lower vagina electively. She was subsequently lost to follow up.
Anomalies of the Mullerian (paramesonephric) duct result from non- or faulty development or failure of fusion of the mullerian ducts between the sixth to ninth weeks of fetal gestation.[3,4] The uterus, cervix and the upper four-fifths of the vagina develop from the paramesonephric ducts. The lower one-fifth of the vagina develops from the sinovaginal bulbs. Anomalies of the genital tract and urinary tract (which develops from the mesonephric duct) are often associated. Renal agenesis is the commonest such anomaly. HWW syndrome shows uterus bicornis bicollis and double vagina, with one of the vagina’s ending as a blind pouch, causing hemihematometra. It is seen twice as often on the right side than the left side. Since one uterus is menstruating normally and its outflow tract (cervix and vagina) is patent, the girl menstruates apparently normally, and attention is not drawn to the condition. She has severe dysmenorrhea, which is often treated symptomatically, while she continues to develop hemihematocolpos, followed by hemihematometra. If it is not detected early, she may develop hematosalpinx on the same side and pelvic endometriosis. An USG is easy to perform, noninvasive and diagnostic. The diagnosis can be confirmed by CT scan or magentic resonance imaging. Treatment is excision of the tissue between the hematocolpos and the lower blind vagina and connect the two to each other. There is no treatment for unilateral absence of one kidney, but it is important to know that the girl has that condition, so that adequate precautions can be taken in treatment of her future illnesses so as not to harm the single kidney.
Sheih et al reported two girls with unilateral hematocolpos and ipsilateral vaginal ectopic ureter in whom there was spontaneous rupture of uterovaginal septum. However they did not have intermittent expulsion of the blood/pus. The case presented here was unique in that there was a spontaneous rupture of the right hemihepatocolpos into the lower vagina, so that the hemihematocolpos and hemihematometra drained. Unfortunately the opening was small, and hence it got plugged and the condition recurred. Luckily it opened up again and resolved. In view
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Parulekar SV. Recurrent Hemihematometra In Herlyn–Werner–Wunderlich Syndrome. JPGO 2019. Vol 6 No. 1. Available from: https://www.jpgo.org/2019/01/recurrent-hemihematometra-in.html