Incidental diagnosis of Herlyn-Werner-Wunderlich Syndrome in a nulliparous woman: a case report in Bhutan

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital syndrome with features of uterus didelphys, ipsilateral absent kidney and obstructed hemivagina. Ultrasound findings of absent kidney or abnormalities in the kidney should alert the clinicians about the syndrome. Complications like endometriosis, infertility and pelvic inflammation occurs with late diagnosis.A 42-yearold nulliparious married woman who presented with right sided abdominal pain to the National Referral Hospital was diagnosed to have HWWS. The diagnosis was supported by ultrasound, CT and MRI findings. Patient was treated for endometriosis and had symptomatic improvement. The diagnosis of the syndrome is challenging as it is rare but clinicians should suspect the syndrome in women who present with infertility. Ultrasound scan is the basic investigation to screen the syndrome which is available in most hospitals.

Possibilities of Early Ultrasound Diagnostics of the Herlin — Werner — Wunderlich syndrome (Case Report)

Herlin — Werner — Wunderlich syndrome or OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) is a rare type of uterine and vaginal doubling defect (0,1 – 10,0 % of all uterine abnormalities) which is characterized by a triad of symptoms: uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal dysgenesis. The manifestation of the disease usually present after menarche, is accompanied by severe pain syndrome, leads to redoubted complications and organ-carrying operations with decreasing the fertility. At an earlier age, it can manifest itself as an infection of the genitourinary system. The diagnosis is based on clinical and anamnestic data using instrumental methods, the most accessible and safe of which is ultrasound. This article presents three clinical cases of patients 5 – 6 years old with a description of the ultrasound picture of the OHVIRA syndrome.

Ohvira syndrome with left radial hemimelia: a rare association

Ohvira syndrome is rare complex anomaly consisting of uterus didelphys, unilateral ipsilateral obstructed hemivagina and ipsilateral renal agenesis. It typically presents with dysmenorrhea or pelvic pain shortly after menarche due to collection of secretions in the uterus. Reporting a case of 32 years unmarried nulligravida lady with complaints of pain abdomen since 20 days with history of similar complaints 15 years bac. Patient was on Inj. DMPA 2 years back. On examination upper limb skeletal deformity-left radial hemimelia present with a mass of 14-16 weeks gravid uterus on per abdomen examination. USG (February 2020), was suggestive of uterus didelphys bicollis with collection of 108 cc noted in the right uterine cavity with right kidney agenesis with obscured right ovary. Total abdominal hysterectomy with left salphingo-oopherectomy was done. Specimen features were suggestive of uterus didelphys bicornis bicollis with right uterus hematometra with right cervix blind with right sided blind vagina with features suggestive of Ohvira syndrome.