Acute appendicitis in a patient with obstructed hemivagina and ipsilateral renal anomaly syndrome: Diagnostic overshadowing in recurrent pelvic pain

Vollständiger Abstract

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Abstract Herlyn–Werner–Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare congenital Müllerian duct anomaly, occurring in approximately 1 in 1,000,000 females. It is characterised by a triad of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. Patients typically present with chronic pelvic pain, dysmenorrhea and endometriosis, which can lead to diagnostic bias and the misattribution of all pelvic pain to their underlying gynaecological condition. We present the case of a 19-year-old female with a known diagnosis of OHVIRA syndrome who presented with acute right iliac fossa (RIF) pain and raised inflammatory markers. Despite her complex gynaecological background, imaging confirmed a diagnosis of acute appendicitis with impending perforation. She underwent a successful laparoscopic appendicectomy. The patient had experienced multiple prior episodes of RIF pain, previously attributed to her gynaecological condition, without further surgical evaluation. This case underscores the importance of maintaining a broad differential diagnosis in patients with complex gynaecological anomalies and highlights the risk of diagnostic overshadowing. A comprehensive assessment for acute surgical pathology should always be considered in this cohort to avoid delays in diagnosis and treatment.