Yakup KUMTEPE, Emsal Pinar Topdagi YILMAZ, Yunus Emre TOPDAGI, Seray Kaya TOPDAGI

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

Herlyn-Werner-Wunderlich sendromunun klasik triadı kör hemivagina, uterin didelphis ve ipsilateral renal agenezidir. Müllerian kanal anomalilerinin bir varyantıdır. Sebebi ve etiyopatogenezi bilinmemektedir. Klinik prezentasyonu genellikle menarj ile başlayan ve hematometrokolposa sekonder gelişen şiddetli dismenore ve ele gelen pelvik kitle şeklindedir. Erken tanı ve tedavi hastanın ilerideki fertilitesinin korunmasında ve komplikasyonların önlenmesinde önemlidir. Bu çalışmada 13 yaşında bekar pediatrik yaş grubundan hastanın pelvik kitle nedeniyle araştırılması yapılırken MRG ile tanısı kesinleştirilmiş HWWS olgusu tespit edilmiştir. Hastaya ve ailesine bilgi verilerek onamları alınmış ve diagnostik laparoskopi yapılmıştır. Daha sonra paravaginal onarım ile hematometra boşaltılmış ve şikayetleri giderilmiştir. İlerideki fertilite ve endokrin fonksiyonlar açısından takibe alınmıştır ve ikinci bir operasyon yaşına uygun olarak yapılabileceği yönünde fikir bildirilmiştir. Bu sendrom şiddetli dismenore, primer amenore veya pelvik kitle nedeniyle araştırılma yapılan pediatrik ve adelosan hasta gruplarında akılda bulundurulmalıdır

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

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.

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