Zeynep Kamil Med J. 2019; 50(4): 233-235 | DOI: 10.16948/zktipb.476364

A Rare Case Report: Herlyn-Werner-Wunderlich Syndrome (HWWS) seen with Primary Amenorrhea and Cervical Agenesis

Emsal Pýnar TOPDAÐI YILMAZ¹, YUNUS EMRE TOPDAÐI¹, SERAY KAYA TOPDAÐI¹, Yakup Kumtepe^2
1Atatürk University School of Medicine, Department of Gynecology and Obstetrics, Erzurum, Turkiye
²Nenehatun Gynecology and Obstetrics Hospital, Clinic of Gynecology and Obstetrics, Erzurum, Turkiye

Herlyn-Werner-Wunderlich sendromunun klasik triadý kör hemivagina, uterin didelphis ve ipsilateral renal agenezidir. Müllerian kanal anomalilerinin bir varyantýdýr. Sebebi ve etiyopatogenezi bilinmemektedir. Klinik prezentasyonu genellikle menarj ile baþlayan ve hematometrokolposa sekonder geliþen þiddetli dismenore ve ele gelen pelvik kitle þeklindedir. Erken taný ve tedavi hastanýn ilerideki fertilitesinin korunmasýnda ve komplikasyonlarýn önlenmesinde önemlidir. Bu çalýþmada 13 yaþýnda bekar pediatrik yaþ grubundan hastanýn pelvik kitle nedeniyle araþtýrýlmasý yapýlýrken MRG ile tanýsý kesinleþtirilmiþ HWWS olgusu tespit edilmiþtir. Hastaya ve ailesine bilgi verilerek onamlarý alýnmýþ ve diagnostik laparoskopi yapýlmýþtýr. Daha sonra paravaginal onarým ile hematometra boþaltýlmýþ ve þikayetleri giderilmiþtir. Ýlerideki fertilite ve endokrin fonksiyonlar açýsýndan takibe alýnmýþtýr ve ikinci bir operasyon yaþýna uygun olarak yapýlabileceði yönünde fikir bildirilmiþtir. Bu sendrom þiddetli dismenore, primer amenore veya pelvik kitle nedeniyle araþtýrýlma yapýlan pediatrik ve adelosan hasta gruplarýnda akýlda bulundurulmalýdýr.

Keywords: mullerian duct anomaly, uterus didelphys, hematometra, ipsilateral renal agenesis

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Primer Amenore ve Servikal Agenezi ile Görülen Herlyn-Werner-Wunderlich Sendromu (HWWS) Nadir Bir Olgu Sunumu

Emsal Pýnar TOPDAÐI YILMAZ¹, YUNUS EMRE TOPDAÐI¹, SERAY KAYA TOPDAÐI¹, Yakup Kumtepe^2
1Atatürk Üniversitesi Týp Fakültesi Kadýn Hastalýklarý ve Doðum Anabilim Dalý, Erzurum, Türkiye
²Nenehatun Kadýn Hastalýklarý ve Doðum Hastanesi, Kadýn Hastalýklarý ve Doðum Kliniði, Erzurum, Türkiye

The classic triad of Herlyn-Werner-Wunderlich syndrome includes blind hemivagina, uterine didelphys and ipsilateral renal agenesis. Herlyn-Werner-Wunderlich syndrome is a variant of Mullerian duct anomalies. Clinical presentation of the Herlyn-Werner-Wunderlich syndrome is usually accompanied by severe dysmenorrhea secondary to hematometrocolpos beginning with menarche and palpable pelvic mass. Early diagnosis and treatment are important in protecting the patient's fertility and preventing complications in the long term. In this study, when a 13-year old patient from the pediatric age group was being investigated for the pelvic mass, HWWS confirmed by MRI was identified. After the patient and his family were informed, and their consent was taken, the diagnostic laparoscopy was performed. Subsequently, after hematometra was removed by paravaginal repair, her complaints were resolved. The patient was followed up regarding fertility and endocrine functions, and a second operation could be performed in accordance with her age. This syndrome should be kept in mind in pediatric and adolescent patient groups who are investigated for severe dysmenorrhea, primary amenorrhea or pelvic mass.

Anahtar Kelimeler: müllerian kanal anomalisi, uterus didelphis, hematometra

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