Herlyn–Werner–Wunderlich syndrome: pre- and post-surgical MRI and US findings

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This paper reviews magnetic resonance imaging and ultrasonography findings of Herlyn–Werner–Wunderlich syndrome, correlating them with embryological, clinical, and surgical features.

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This paper provides a pictorial review of magnetic resonance imaging and ultrasonography findings in Herlyn–Werner–Wunderlich syndrome, correlating imaging features with embryology, clinical presentation, and surgical findings. The reviewed syndrome is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, with common post-menarche symptoms such as pelvic pain, dysmenorrhea, and palpable masses from hematocolpos/hematometra, and potential complications including pyohematocolpos, pyosalpinx, endometriosis, and pelvic adhesions. A stated limitation is that diagnosis can be difficult because physical examination and symptoms lack specificity, making the paper’s emphasis on accurate imaging description crucial. Relevance to endometriosis: the abstract notes endometriosis as a potential long-term complication in HWWS, though the paper’s main focus is on pre- and post-surgical MRI/US imaging findings for this congenital anomaly. This paper is centrally about endometriosis and adenomyosis? Not—This paper does not explicitly discuss adenomyosis; it relates to endometriosis by describing it as a potential long-term complication of Herlyn–Werner–Wunderlich syndrome.

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Abstract

Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Müllerian duct anomalies with mesonephric duct anomalies. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes this syndrome. Patients generally present with non-specific symptoms after menarche. Pelvic pain, dysmenorrhea, and palpable mass due to hematocolpos or hematometra are the most common findings. Pyohematocolpos and pyosalpinx may appear as acute complications, while endometriosis and pelvic adhesions constitute potential long-term complications. When a prenatal diagnosis of unilateral renal agenesis in newborn girls is known, a gynecological imaging study should be performed to exclude uterine and vaginal abnormalities. These patients should be followed up to ensure that a timely surgical correction is performed. The diagnosis of HWWS is difficult due to the lack of specific symptoms or findings upon physical examination. An accurate imaging description of these congenital anomalies is crucial to guide patients toward surgical treatment, relieving acute complications, and preserving the normal fertility. The authors provide a pictorial review of the magnetic resonance imaging and ultrasonography findings of the HWWS with correlation to embryological, clinical, and surgical features. Similar content being viewed by others

References

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Herlyn–Werner–Wunderlich syndrome: pre- and post-surgical MRI and US findings. Abdom Imaging 40, 2667–2682 (2015). https://doi.org/10.1007/s00261-015-0421-0 Published: Issue date: DOI: https://doi.org/10.1007/s00261-015-0421-0

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MeSH descriptors

Magnetic Resonance Imaging Postoperative Complications Postoperative Complications Preoperative Care Urogenital Abnormalities Urogenital Abnormalities Congenital Abnormalities Congenital Abnormalities Congenital Abnormalities Congenital Abnormalities Female Humans Kidney Kidney Kidney Kidney Kidney Kidney Diseases Kidney Diseases Kidney Diseases

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