{"paper_id":"781561fc-e73c-4bc1-9dd3-046202e390e0","body_text":"Herlyn Werner Wunderlich (HWW) syndrome,\nalso known as obstructed hemi-vagina and ipsilateral\nrenal agenesis (OHVIRA) syndrome, is an\nuncommon combined müllerian duct anomalies\n(MDAs) ( Table 1 ) and mesonephric duct malformation\nof female urogenital tract.\nMDAs classification\nMDAs; Müllerian duct anomalies.\nThe exact incidence of this syndrome is unknown\n( 1 ); however, the incidence of uterus didelphys\n( Fig .1 ) as a part of this syndrome is about 1/2000\nto 1/28000 that is accompanied by unilateral renal\nagenesis with the incidence of approximately\n1/1100, while 25 to 50% of affected women have\nshowed to have genital abnormalities ( 2 - 5 ).\nAlthough HWW syndrome includes variability of\nthe anatomic structures like uterine, cervical, vaginal\nand/or renal anomalies, it is characterized by the\npresence of uterus duplicity and OHVIRA syndrome.\nClass III MDAs-uterine didelphys. MDAs; Müllerian duct\nanomalies.\n\nA 19 year-old unmarried female presented\nto Dr. Rajendra Prasad, Government Medical\nCollege, Kangra, HP, India, in June 2013.\nShe complained of abdominal pain gradually\nincreasing in intensity and scanty periods\nsince the last 6 months. Patient reached\nmenarche at 16 years with normal menstrual\ncycles until 6 months ago. She also complained\nof periodic pain in lower abdomen\naccompanying her menstrual cycles beginning\nfrom around the time of her menarche.\nInitially for the first three-four months, she\nwas being symptomatically managed for\ndysmenorrhea, but ultrasound scans done in\na referral center revealed multiple cystic lesions\nin bilateral adnexa with low level internal\nechoes suggestive of endometriosis.\nThereafter she was being managed medically\nfor endometriosis (in the scans, her\nuterus was reported as normal). Her urine\npr egnancy test was negative.\nAn ultrasound scan done for pelvic organs at our institute revealed uterus didelphys ( Fig .2 ) and a cystic fluid collection with low level internal echoes arising from the pelvis consistent with associated haematocolpos ( Fig .3 ). Cystic lesion was noted in the right adnexa consistent with endometrioma. Right kidney was not visualized ( Fig .4 ).\nTransverse ultrasound image showing two uterine cavities\nwith echogenic endometrium.\nLongitudinal ultrasound image depicting a cystic lesion\nposterior to urinary bladder with low level echoes and communication\nwith endometrial cavity through the cervix.\nTransverse ultrasound of right hepatorenal space showing\nabsent kidney in the right renal fossa.\nSubsequently magnetic resonance imaging (MRI)\nwas performed to better characterize the pelvic\nanatomy and better identify the anatomic location\nof this pelvic fluid collection. MRI revealed uterine\ndidelphys with two separate cervices ( Fig .5 ). The\nright cervix and proximal hemi-vagina were distended\nthat led to the comparison of the left cervix\nand hemi-vagina ( Fig .6A ). The left endometrial\ncavity appeared normal; however, the left cervix/\nhemi-vagina was constricted in its lower part due to\npressure from the distended cervix and hemi-vagina\non the right side resulting in partial obstruction\nof menstrual blood outflow, as seen in the patient\n( Fig .6B ). The high T2 MRI signal characteristics in Transverse ultrasound of right hepatorenal space showing\nabsent kidney in the right renal fossa.\nAxial T2W MRI image showing two uterine cavities with\ndistended right cervix and hemi-vagina. MRI; Magnetic resonance\nimaging.\nA right adnexal cystic lesion with blood products\nwas seen suggestive of endometriotic cyst ( Fig .7 ).\nSubsequent gynecological examination revealed\nan obstructed right hemi-vagina and a fluid wave\npalpable through inferior septum. This hematocolpos\nwas surgically drained and about 400 ml of\nold blood was evacuated. The patient recovered\nuneventfully and no further surgery was done.\nA written consent was taken from the patient for\npublication of this report.\nA. Coronal and B. Axia T2W MRI images showing distended\nright cervix and hemi-vagina compressing the normal left\nhemi-vagina which shows differential signal intensity resulting\nin layering.\nAxial T2W MRI image showing large hyperintense right adnexal\ncyst (endometriotic cyst). MRI; Magnetic resonance imaging.\n\nMost common type of MDAs is the lateral fusion\ndefects which range from symmetric/asymmetric\nto obstructed/unobstructed fusion anomalies. A\nuseful classification based on the degree of failure\nof normal development was proposed by Buttram\nand Gibbons ( 6 ).\nDevelopment of urinary system and müllerian\nduct system are closely related with which accounts\nfor the frequent association of anomalies\ninvolving both the systems ( 2 ,  3 ).\nUterine didelphys results from complete failure of fusion of the müllerian ducts and their normal differentiation to form a cervix and uterus during the 8th week of gestation ( 7 ). Uterine didelphys (Class III MDA) occurs in case of complete failure of fusion as also seen in our case.\nThe Wolffian duct gives rise to the ipsilateral ureteric bud and thus is responsible for the formation of the kidney. Accordingly, in the absence of the Wolffian duct on one side, the kidney and ureter (of the same side) will fail to fuse ( 3 , 4 ). On the side on which the Wolffian duct is missing, the müllerian duct is displaced laterally and fails to adequately fuse with the urogenital sinus, leading to the formation of a blind sac, imperforate or obstructed hemivagina ( 3 ), right side in the present case. The distal part of vagina which arises from the urogenital sinus is not affected and develops normally.\nPatients with OHVIRA syndrome are usually asymptomatic until puberty, when they present with acute lower abdominal pain. Diagnosis is usually made soon after menarche (most patients are diagnosed from 2 months to 2 year after menarche) and the presenting symptoms are pelvic pain, dysmenorrhea, foul-smelling discharge and pelvic mass ( 7 , 8 ). If not treated, complications leading to infertility, endometriosis, pelvic adhesions, and pyosalpinx or pyocolpos may present in the late phase with a high miscarriage rate ( 7 ).\nThe choice of investigation for the diagnosis and operative planning of OHVIRA syndrome are ultrasound and MRI, both of which have an added advantage of being non-invasive ( 1 , 5 ).\nThe role of computed tomography (CT) is limited due to radiation exposure and limited soft-tissue resolution. Ultrasound may reveal uterine didelphus and pelvic fluid collection with low level internal echoes, contiguous with the endocervix (haemato/pyocolpos). Due to retrograde menstruation, features of endometriosis in form of well defined, unilocular or multilocular, predominantly cystic masses containing diffuse, homogeneous, low level internal echoes (endometrioma/chocolate cyst) may also be seen ( 9 ).\nMRI plays an important role in characterizing the didelphic uterus, obstructed hemivagina, and ipsilateral renal agenesis ( 1 , 10 ). MRI findings of OHVIRA syndrome are characterized by iso/high T1W signal and high T2W signal that indicate pelvic fluid collection is contiguous with the endocervix along with didelphic uterus and an absent kidney on the affected side ( 1 , 2 ).\nMRI is far better than ultrasound for characterizing anatomical relationships due to its multiplanar capabilities and larger field of view ( 2 ). However, the gold standard for diagnosis is laproscopy through which has the added benefit of performing therapeutic drainage of hematometra/hematocolpos, vaginal septotomy and marsupialisation ( 10 ). Treatment usually involves surgery in the form of excision of the vaginal septum which helps in relieving obstruction ( 11 ). Surgical intervention also decreases the chances of pelvic endometriosis due to retrograde menstrual seeding. About 87% of patients go on to have a successful pregnancy; however, 23% of patients carry the risk of subsequent abortion ( 12 ).\nThe rarity of OHVIRA syndrome complicates its diagnosis, and hence clinicians and radiologists should consider MDAs among the differential diagnosis in young female patients presenting with abdominal symptoms, especially when associated with renal anomaly/agenesis. Understanding the imaging findings is critical for early diagnosis in an attempt to prevent complications such as endometriosis or adhesions from chronic infections with subsequent infertility.","source_license":"CC-BY-4.0","license_restricted":false}