Monir uddin2, khadija akter1, shameema akter1, monira sarmin3 and sartaj begum1 abstract background. Herlynwernerwunderlich hww syndrome is a rare congenital anomaly characterized by triad of uterus didelphys, hemivagina, and absent ipsilateral kidney. Little is reported about spinal deformity associated with this syndrome. Herlynwernerwunderlich syndrome is a rare mullerian duct and wolffian duct anomaly with uterus didelphys, unilaterally obstructed hemivagina, and. Herlynwernerwunderlich syndrome hwws herlyn and werner 1971.
Herlynwernerwunderlich syndrome radiology reference. The asso ciation of renal agenesis with ipsilateral blind hemivagi na was reported as herlynwerner syndrome in. A rare case of herlyn werner wunderlich syndrome role of. Herlyn werner wunderlich syndrome rita mittal1 received. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos.
Clinical characteristics of patients with herlynwernerwunderlich syndrome hwws and endometriosis em. Awareness is necessary in order to diagnose and treat this disorder properly before complications occur. Obstructed hemivagina and ipsilateral renal anomaly ohvira, also known as herlynwernerwunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since 1922. The herlynwernerwunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Herlynwernerwunderlich hww syndrome, is a rare anomaly characterized by mullerian duct anomalies mda associated with mesonephric duct anomalies 3. Wunderlich syndrome is a rare condition, in which spontaneous nontraumatic renal hemorrhage occurs into the subcapsular and perirenal spaces 2.
Scoliosis in herlynwernerwunderlich syndrome semantic. Herlynwernerwunderlich syndrome tropical journal of. A diagnosis of herlynwernerwunderlich syndrome hwws was performed and the patient underwent laparoscopic vaginal septectomy, where 500 ml of chocolate coloured fluid was drained. The obstructed vagina affects menstrual flow and causes related clinical features after menarche.
Patient with newlydiagnosed uterine anomaly had known unilateral renal agenesis. Herlyn werner wunderlich hww syndrome is a rare congenital disorder in which there is uterus didelphys, obstructed hemivagina and renal agenesis lending its acronym, ohvira syndrome. It is not to be confused with the wunderlich syndrome. Pdf herlynwernerwunderlich hww syndrome is a rare congenital disorder of the mullerian ducts in which there is uterus didelphys. The herlyn werner wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It is most commonly diagnosed in puberty due to pelvic and abdominal pain, but more rarely it can present in neonates or adults, with primary infertility, pyometra, urinary obstruction and. Obstructed hemivagina and ipsilateral renal anomaly ohvira, also known as herlynwernerwunderlich syndrome, is a rare syndrome constituting 0.
Herlynwernerwunderlich syndrome hwws comprises the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. We present the first case report on herlyn werner wunderlich syndrome from bangladesh. A triad of uterus didelphys, unilateral hemivagina obstruction due to a longitudinal septum and ipsilateral renal agenesis is diagnostic 1. Uterus didelphys with blind hemivagina and ipsilateral renal. Herlyn werner wunderlich syndrome with hematocolpos symptom. Herlyn werner wunderlich syndrome hwws, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, is an uncommon combined mullerian and mesonephric duct anomaly. Herlynwernerwunderlich syndrome rita mittal1 received. Herlyn werner wunderlich hww syndrome, is a rare anomaly characterized by mullerian duct anomalies mda associated with mesonephric duct anomalies 3. Late presentation, mr imaging features and surgical. We report the case of a female neonate with ipsilateral renal agenesis and uterus didelphys with blind hemivagina, also known as herlyn. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation institutional and national and with the helsinki declaration of 1975, as revised in 2000 5. Herlyn werner wunderlich syndrome hwws, also known as ohvira syndrome, is a rare congenital defect in the formation of mullerian ducts, the developmental precursors to the fallopian tubes, uterus, cervix, and upper vagina. Herlyn werner wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys.
Wunderlich 1976 is a rare mullerian anomaly consisting of a didelphic uterus, a hemivaginal septum, and an ipsilateral renal agenesis. Endometriosis in association with herlynwernerwunderlich. Mr imaging revealed the typical features of didelphys uterus, obstructed. Herlynwernerwunderlich syndrome europe pmc article. Successful preterm pregnancy in a rare variation of herlyn. Herlynwernerwunderlich hww syndrome is a rare congenital disorder of the mullerian ducts in which there is uterus didelphys, obstructed hemivagina and unilateral renal agenesis. The herlynwernerwunderlich syndrome is a rare, congenital disorder characterised by uterus didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis, all being secondary to mesonephric ductinduced mullerian anomalies. Background uterus didelphys and blind hemivagina associated with ipsilateral renal agenesis are collectively known as herlynwernerwunderlich syndrome hwws. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. This study presents a case of scoliosis occurring in the setting of hwws and explores the possible association between the 2 diseases. The diagnosis should be suspected in adolescent females with a pelvic mass and an ipsilateral renal agenesis.
Jul 15, 2011 the herlynwernerwunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Herlynwernerwunderlich syndrome is a rare mullerianduct anomaly consisting of uterine didelphys, a unilateral obstructed hemivagina, and ipsilateral renal agenesis. Herlynwernerwunderlich syndrome hwws, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, is an uncommon combined mullerian and mesonephric duct anomaly. Uterus didelphys with obstructed hemivagina frequently associated with ipsilateral renal agenesis is a rare congenital anomaly known as herlynwernerwunderlich syndrome hww. Case report open access herlynwernerwunderlich syndrome presenting with dysmenorrhea. Anatomically, hwws is characterized by three key anomalies of the female reproductive tract.
Perioperative evaluation in herlynwernerwunderlich syndrome. The herlyn werner wunderlich syndrome is a rare, congenital disorder characterised by uterus didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis, all being secondary to mesonephric ductinduced mullerian anomalies. Herlynwernerwunderlich 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. Herlynwernerwunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral. Herlynwernerwunderlich hww syndrome is a combined anomaly syndrome with uterine didelphys, blind hemivagina, and ipsilateral renal. Scoliosis in herlynwernerwunderlich syndrome semantic scholar. It is most commonly diagnosed in puberty due to pelvic and abdominal pain, but more rarely. 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.
Pdf new classification of herlynwernerwunderlich syndrome. New classification of herlynwernerwunderlich syndrome ncbi. In the literature, the syndrome often appears as a single case report or as a small series. Clinical characteristics of 70 patients with herlynwernerwunderlich syndrome. It generally occurs at puberty and exhibits nonspecific and variable symptoms with acute or pelvic pain shortly following menarche. Herlyn werner wunderlich syndrome is defined by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Herlynwernerwunderlich syndrome is a rare mullerian ductal anomaly that is characterized by the presence of a hemivaginal septum, a didelphic uterus and ipsilateral renal agenesis. Simultaneous occurrence of an open gartnerduct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Herlynwernerwunderlich syndromeearly diagnosis with. It is generally difficult to diagnose the uterine malformation before menarche owing to its small size. The patient became symptom free after the procedure. Herlynwernerwunderlich hww syndrome is a very rare congenital anomaly of the urogenital tract involving mullerian ducts and wolffian structures, and it is characterised by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis 1. Herlynwernerwunderlich syndrome hwws is often a misdiagnosed condition and need a high index of suspicion is required in patients with mullerian and mesonephric duct anomalies. Ohvira also causes renal agenesis, which means that one or both of the kidneys do not properly develop while the baby is in the womb.
Herlynwernerwunderlich hww syndrome is a rare congenital disorder in which there is uterus didelphys, obstructed hemivagina and renal agenesis lending its acronym, ohvira syndrome. Herlyn werner wunderlich hww syndrome is a rare congenital disease characterized by mullerian duct anomalies. The herlynwernerwunderlich syndrome a case report with. Authors present a case of herlynwernerwunderlich syndrome in a 19yearold unmarried woman who presented with pelvic pain and pelvic mass. Herlyn werner wunderlich syndrome is a rare developmental anomaly, in which there is uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. We report the case of a 31yearold woman with no relevant medical history, with scant, intermittent and illsmelling metrorrhagia for the last 23 months. We report the presentation, radiographic findings, and outcomes of three patients with herlyn werner wunderlich syndrome who underwent surgical management at a tertiary care academic medical center. Herlynwernerwunderlich hww syndrome is a very rare congenital anomaly of the urogenital tract involving mullerian ducts and wolffian structures, and it is werner by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal.
A rare urogenital anomaly masquerading as acute abdomen. Anatomic variations among patients were confirmed by intraoperative findings, including complete and incomplete obstructions 2 x 2 tong, j. Herlynwernerwunderlich hww syndrome is a very rare congenital anomaly of the urogenital tract involving mullerian ducts and wolffian. A rare case of herlyn werner wunderlich syndrome journal of chalmeda anand rao institute of medical sciences vol 10 issue 2 july december 2015 1 uterus with multiple low level internal echoes in the endometrial cavity figure 1 with absent right kidney.
Age at em diagnosis y age at hwws diagnosis y type of hwws related surgery followup since last surgery mo 1 complete lap left ovarian endometrial cystectomy 2 14 14 complete lap right ovarian endometrial. The herlynwernerwunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and. Herlynwernerwunderlich hww syndrome is a rare congenital disorder of the mullerian ducts in which there is uterus didelphys, obstructed hemivagina and. The diagnosis of hww syndrome is often delayed because of. Uterus didelphys is a symmetric anomaly in which two completely separate uterine cavities are identified, each with normal zonal anatomy as well as its own endometrial cavity and cervix. Herlynwernerwunderlich hww syndrome is a rare congenital disease characterized by mullerian duct anomalies. Herlynwernerwunderlich syndrome is a rare developmental anomaly, in which there is uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. Uterus didelphys and blind hemivagina associated with ipsilateral renal agenesis are collectively known as herlynwernerwunderlich syndrome hwws. Jun 28, 2017 herlynwernerwunderlich syndrome hwws herlyn and werner 1971.
It usually presents at puberty with pelvic pain, the form of pyometra, ischiorectal swelling, urinary obstruction, and primary infertility. Herlyn werner wunderlich syndrome, also known as ohvira obstructed hemivagina and ipsilateral renal anomaly is an extremely rare syndrome characterized by a congenital birth defect of the lower abdominal and pelvic organs. Pediatric obstructed hemivagina and unilateral renal. Herlynwernerwunderlich syndrome is defined by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. An unusual case report of full diagnostic approach and treatment rohit bhoil, m. Herlynwernerwunderlich syndrome presenting with dysmenorrhea. Management and outcome of patients with combined vaginal septum, bifid utery, and ipsilateral renal agenesis herlyn werner wunderlich syndrome. We report the presentation, radiographic findings, and outcomes of three patients with herlynwernerwunderlich syndrome who underwent surgical management at a tertiary care academic medical center. Clinical characteristics of patients with herlyn werner wunderlich syndrome hwws and endometriosis em. The most common presentation is an abdominal mass secondary to hematocolpos, pain and dysmenorrhea.
Uterus didelphys with obstructed hemivagina frequently associated with ipsilateral renal agenesis is a rare congenital anomaly known as herlyn werner wunderlich syndrome hww. It usually presents at puberty with pelvic pain, the form of pyometra, ischiorectal swelling. Mar 29, 2020 herlynwernerwunderlich hww syndrome, is a rare anomaly characterised by mullerian duct anomalies mda associated with mesonephric duct anomalies. Uterodidelphys with obstructed hemivagina and ipsilateral renal agenesis is referred to as the herlyn werner wunderlich hww syndrome. A diagnosis of herlyn werner wunderlich syndrome hwws was performed and the patient underwent laparoscopic vaginal septectomy, where 500 ml of chocolate coloured fluid was drained. Clinical presentation wunderlich syndrome is clinically characterized by lenks triad. Only a handful of these cases have been associated with pyocolpos. Diagnosis and surgical management of herlynwernerwunderlich. This entity is also known as obstructed hemivagina and ipsilateral renal anomaly ohvira. So, in view of uterine pathology and absent right kidney. Background uterus didelphys and blind hemivagina associated with ipsilateral renal agenesis are collectively known as herlyn werner wunderlich syndrome hwws. A previously unreported scoliosis in hwws is described.
Herlynwernerwunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Herlyn werner wunderlich syndrome with hematocolpos. Management and outcome of patients with combined vaginal septum, bifid utery, and ipsilateral renal agenesis herlynwernerwunderlich syndrome. The diagnosis should be suspected in adolescent females.
Herlynwernerwunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys. Herlynwernerwunderlich syndrome consisting of uterine. Perioperative evaluation in herlynwernerwunderlich. This article presents clinical and laparoscopic findings as well as the surgical management of 5 cases with various clinical scenarios. Patients are symptomatic shortly after menarche when hematocolpos develop during menstruation, resulting in dysmenorrhea and a pelvic mass. Abstractherlynwernerwunderlich syndrome hwws is a congenital mullerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We present the first case report on herlynwernerwunderlich syndrome from bangladesh.
The cyst was punctured and instilled with contrast medium for cystography to demonstrate the anatomy. Pdf herlynwernerwunderlich syndrome is a rare developmental anomaly, in which there is uterus didelphys with obstructed hemivagina and ipsilateral. Herlyn wernerwunderlich syndrome hwws is a rare congenital anomaly of the female genital tract, characterised by the triad of uterus didelphys, blind hemivagina, and ipsilateral renal agenesis 1, 2. Obstructed hemivagina and unilateral renal anomaly syndrome ohvira sometimes known as herlyn werner wunderlich syndrome is a rare disorder in which a female baby is born with two uteruses. Herlynwernerwunderlich hww syndrome is a rare variant of mullerian duct anomalies con sisting of uterine didelphys, obstructed hemivagina, and. Herlynwernerwunderlich syndrome is a very rare cause of abdominal pain in young women. Pelvic endometriosis is one of the common complications of hwws. Bilateral ovaries were normally visualised with a small haemorrhagic cyst seen in the left ovary. Herlynwernerwunderlich syndrome hww syndrome, is a rare congenital anomaly consisting of the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. A case of obstructed hemivagina with ipsilateral renal agenesis ohvira syndrome and didelphic uterus.
Herlyn werner wunderlich syndrome hwws is often a misdiagnosed condition and need a high index of suspicion is required in patients with mullerian and mesonephric duct anomalies. Herlyn werner wunderlich syndrome presenting with dysmenorrhea. Herlynwernerwunderlich syndrome is a very rare congenital genitourinary anomaly characterized by uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Wunderlich syndrome hwws is a rare congenital malformation that involves uterus dideslphys, obstructed hemivagina and ipsilateral renal agenesis. The characteristic triad of this syndrome includes didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis hence, also known as obstructed hemivagina and ipsilateral renal agenesis or ohvira syndrome. Herlynwernerwunderlich syndrome confirmed on laparoscopy. Prenatal sonography revealed the absence of the left kidney and a retrovesical cystic lesion suspected as hydrometrocolpos. Uterus didelphys with blind hemivagina and ipsilateral.
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