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Case Reports
. 2021 Sep 8:70:102843.
doi: 10.1016/j.amsu.2021.102843. eCollection 2021 Oct.

A challenging diagnosis and management of Herlyn-Werner-Wunderlich Syndrome in low-resource Settings: A case report complicated with hydronephrosis

Sami Jomaa  1 , Kenana Tawashi  2 , Fatima Alzhraa All Rass  2 , Eyad Abdallah  3 , Nazih Tawashi  2
Affiliations
Case Reports

A challenging diagnosis and management of Herlyn-Werner-Wunderlich Syndrome in low-resource Settings: A case report complicated with hydronephrosis

Sami Jomaa et al. Ann Med Surg (Lond). .

Abstract

Introduction: Herlyn-Werner-Wunderlich syndrome is a rare congenital disorder that consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Both diagnosis and management can be challenging in low-income countries.

Case presentation: a 13-year-old female who experienced recurrent pelvic pain, dysmenorrhea, and abdominal mass for one month. We could not obtain the MRI as it was unavailable in our hospital, and the patient cannot afford its expensive cost in a private center. Instead, US showed an absent right kidney and doubled uterus with a heterogeneous cystic formation. A pelvic CT scan revealed an absent right kidney, an enlarged left kidney, a double uterus, and a left hematocolpos. We established the diagnosis of Herlyn-Werner-Wunderlich syndrome and started planning for correcting the anomalies via laparotomy.

Discussion: ultrasound detects uterus didelphys, hematocolpos, hematometrocolpos, and renal agenesis but cannot identify the vaginal septum and the type of müllerian defect. The MRI is more convenient for a thorough assessment, but it is a high-priced diagnostic method.

Conclusion: although MRI is the gold standard for establishing the diagnosis and preoperative planning, ultrasound and CT scan can be an alternative, less expensive, and reliable approach. In addition, Laparoscopy or vaginoscopy may not be available in these countries. Therefore, correcting the anomalies can be achieved with laparotomy, which results in acceptable outcomes without any complications.

Keywords: Background; Didelphic uterus; Herlyn-Werner-Wunderlich syndrome; Müllerian anomalies; OHVIRA syndrome; Renal agenesis.

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Conflict of interest statement

The authors have no conflict of interest.

Figures

Fig. 1
Fig. 1
Shows a hydronephrosis of the left kidney (Blue arrow), and an absent right kidney (Red arrow). (For interpretation of the references to color in this figure legend, the reader is referred to the Web version of this article.)
Fig. 2
Fig. 2
Exhibits the didelphic uterus (Blue arrow). (For interpretation of the references to color in this figure legend, the reader is referred to the Web version of this article.)
Fig. 3
Fig. 3
Shows the left hematocolpos (Blue arrow). (For interpretation of the references to color in this figure legend, the reader is referred to the Web version of this article.)
Fig. 4
Fig. 4
Illustrates the opening between the two vaginas that allows for blood passage.
See this image and copyright information in PMC

References

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