Paediatric Surgery (Ukraine). 2026. 2(91): 128-135. doi: 10.15574/PS.2026.2(91).128135
Maksymenko O. S.^1,2, Lytvynenko O. V.², Hutnik O. M.², Savchenko R. B.¹, Hryn V. H.^1
1Poltava State Medical University, Ukraine
²Medical Curatively-Diagnostic Center “MEDION”, Poltava, Ukraine

For citation: Maksymenko OS, Lytvynenko OV, Hutnik OM, Savchenko RB, Hryn VH. (2026). Case report of Herlyn-Werner-Wunderlich syndrome in 2-year-old child with completely obstructed hemivagina with pyocolpos. Paediatric Surgery (Ukraine). 2(91): 128-135. doi: 10.15574/PS.2026.2(91).128135.
Article received: Jan 24, 2026. Accepted for publication: Jun 15, 2026.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the development of the female genital organs, characterized by a triad of symptoms: a double uterus, obstructed hemivagina, and ipsilateral renal agenesis. This complex and combined malformation of the genitourinary system is difficult to diagnose, especially in young children.
Aim – analyze and describe the experience of diagnosing and treating a 2-year-old girl with left renal agenesis, a double uterus, and incomplete obstructive pyocolpos, based on a clinical case.
Clinical case. Ultrasound (US) examination revealed the absence of the left kidney, a left ovarian cyst, and an enlarged uterus. Computed tomography (CT) diagnosed a tumor formation of the pelvis and agenesis of the left kidney. During the diagnostic laparoscopy (DL), a doubled uterine body and a tumor formation were found. Additional magnetic resonance imaging (MRI) allowed the diagnosis of HWWS. Vaginoscopy, septotomy with pyocolpos drainage, and complete excision of the vaginal septum were performed. Two cervical ossifications were visualized. HWWS manifests after the onset of menarche, when the obstructed hemivagina fills with menstrual blood. Ultrasound and MRI, supplemented by laparoscopy, are the gold standard for diagnosing this pathology. In our case, ultrasound and CT data did not provide a clear picture. However, DL and MRI confirmed the diagnosis.
Conclusion. Thus, a multidisciplinary and multi-instrumental approach with minimally invasive technologies allowed us to diagnose a rare congenital anomaly of the genitourinary system and quickly eliminate the obstruction of the left hemivagina without adverse gynecological and reproductive consequences.
The study was conducted in accordance with the principles of the Declaration of Helsinki. Informed consent was obtained from the child’s parents participating in the study.
The authors declare no conflicts of interest.
Keywords: Herlyn-Werner-Wunderlich syndrome, OHVIRA syndrome, uterine didelphys, obstructed hemivagina, renal agenesis, pediatric gynecology.

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