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Herlyn-Werner-Wunderlich syndrome: A fertility-sparing approach to a rare mullerian anomaly.

التفاصيل البيبلوغرافية
العنوان: Herlyn-Werner-Wunderlich syndrome: A fertility-sparing approach to a rare mullerian anomaly.
المؤلفون: Melo GPS; Gynecology Department - University Hospital Clementino Fraga Filho - Federal University of Rio de Janeiro, RJ, Brazil., Jandre TFM; Gynecology Department - University Hospital Clementino Fraga Filho - Federal University of Rio de Janeiro, RJ, Brazil., Vilardo ALRH; Gynecology Department - University Hospital Clementino Fraga Filho - Federal University of Rio de Janeiro, RJ, Brazil., Antunes RA; Gynecology Department - University Hospital Clementino Fraga Filho - Federal University of Rio de Janeiro, RJ, Brazil.; Fertipraxis - Human Reproduction Center, Rio de Janeiro, RJ, Brazil., Parente DB; Gynecology Department - University Hospital Clementino Fraga Filho - Federal University of Rio de Janeiro, RJ, Brazil.; Radiology Department of Federal University of Rio de Janeiro, RJ, Brazil., Coelho-Oliveira A; Gynecology Department - University Hospital Clementino Fraga Filho - Federal University of Rio de Janeiro, RJ, Brazil.
المصدر: JBRA assisted reproduction [JBRA Assist Reprod] 2023 Sep 28. Date of Electronic Publication: 2023 Sep 28.
Publication Model: Ahead of Print
نوع المنشور: Case Reports
اللغة: English
بيانات الدورية: Publisher: Brazilian Society of Assisted Reproduction Country of Publication: Brazil NLM ID: 101684552 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1518-0557 (Electronic) Linking ISSN: 15175693 NLM ISO Abbreviation: JBRA Assist Reprod Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Brasília/DF : Brazilian Society of Assisted Reproduction, [2014]-
مستخلص: The Herlyn-Werner-Wunderlich syndrome (HWWS) is characterized by the triad of uterus didelphys, obstructed hemivagina, and renal agenesis. The typical clinical presentation involves chronic pelvic pain, dysmenorrhea, and palpable abdominal mass, related to hematocolpos/hematometra. It is a rare disease, with a challenging clinical and radiological diagnosis. Surgery is the definitive treatment. Complications such as endometriosis, infertility and chronic pelvic pain occur more frequently and severely when diagnosis and treatment are delayed. This is a case report of a twelve-year-old patient admitted to the Gynecology Department of the Federal University of Rio de Janeiro's General Hospital (HUCFF/UFRJ), in March 2021, with progressive symptoms of dysmenorrhea and abdominal distention due to palpable abdominal mass. She had a previous history of congenital solitary kidney. Magnetic Resonance Imaging (MRI) showed a double uterus with hematometra and hematocolpos on the left side, pelvic endometriosis and left renal agenesis. Conservative clinical treatment with inhibition of the hypothalamic-pituitary-ovarian (H-P-O) axis was initiated while a definitive surgical approach was being defined. In June 2022, the patient underwent left hemi-hysterectomy and salpingectomy, achieving full remission of symptoms. Given the rarity of this syndrome and its potential complications, our report aims to familiarize clinicians with it, mostly those who work with children and adolescents, so that more patients have access to early diagnosis and adequate treatment. Consequently, future fertility can be effectively preserved.
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فهرسة مساهمة: Keywords: fertility preservation; mullerian ducts; solitary kidney; uterine anomalies; uterine didelphys
تواريخ الأحداث: Date Created: 20230928 Latest Revision: 20231215
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC10718557
DOI: 10.5935/1518-0557.20230043
PMID: 37768821
قاعدة البيانات: MEDLINE
الوصف
تدمد:1518-0557
DOI:10.5935/1518-0557.20230043