診断および治療に難渋したWunderlich症候群の一例

抄録

　Wunderlich syndrome is a disorder of a duplicated uterus with unilateral cervical obstruction and ipsilateral kidney agenesis, and its frequency is reported to be 0.1-3.8％ in the general population. We report a case of Wunderlich syndrome that was difficult to diagnose and treat at our hospital. The patient, an 11-year-old girl, was diagnosed with OHVIRA syndrome on imaging findings and underwent four vaginal septectomies, but the recurrence was repeated. Careful evaluation of pathology specimens led to the diagnosis of Wunderlich syndrome. The OHVIRA syndrome and Herlyn-Werner syndrome are often confused with Wunderlich syndrome because of their similar clinical manifestations and imaging findings, and it is important to submit the closed cyst wall and vaginal wall to pathology for histopathologic differentiation to obtain a final diagnosis. The basic treatment is septal resection. However, signs such as dysmenorrhea of Wunderlich syndrome and OHVIRA syndrome often occur at an early age without a history of sexual intercourse, and in conventional surgery performed under direct vision with a narrow vaginal canal it is difficult to expand the operative field and may result in hymen or perineal lacerations. We devised surgical techniques using not only conventional methods, but also a combined use of the resectoscope, laparoscope, and Harmonic HD1000i shears. The recurrence rate has not been fully investigated, and further research is needed.