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.

HERLYN–WERNER–WUNDERLICH SYNDROME: CLINICAL CASE OF A 17 YEAR OLD PATIENT

Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare syndrome in clinical practice in which there is a combination of complete uterus didelphys and OHVIRA syndrome (obstructed hemivagina and ipsilateral renal agenesis). HWWS refers to associative defects of the reproductive and urinary systems and occurs due to anomalies in the development of the Müllerian ducts. The review part of the article presents questions of its epidemiology, and provides historical information. The clinical picture, methods of examination and treatment are considered. Particular attention is paid to the difficulty of diagnosing HWWS and the need for its early diagnosis in order to preserve the reproductive health of girls. A clinical case of a 17-year-old patient with HWWS diagnosed during urgent laparoscopy, the complication of which was hematopiocolpos, is presented. The patient was referred to a level 3 hospital for specialized medical care, where she was operated on.