Presentation
A teenage girl menstruating since 12 years of age presented with worsening lower abdominal pain for 06 months.
Patient Data
Transabdominal ultrasound demonstrates two uterine cavities with the right hemi-uterus filled with fluid with low-level echoes suggestive of blood products.
The left uterus and cervix are normal.
A large cystic structure with low-level echoes is too noted in the right lower abdomen.
The right renal bed is empty and filled with bowel loops.
The left kidney is normal.
MRI pelvis demonstrates evidence of two uterine cavities, cervices and upper vagina, confirming the presence of uterine didelphys.
The right uterine cavity and cervical canal are filled with T1 and T2 high signal intensity fluid(subacute blood), suggesting right hematometrocolpos secondary to obstructed right hemivagina.
A large right hematosalpinx is seen.
The right ovary is enlarged with multiple cysts containing internal blood products.
The left uterus and ovary are normal.
Right renal agenesis is noted.
The left kidney is normal.
Case Discussion
The Herlyn-werner-wunderlich syndrome is a type III Mullerian anomaly characterized by uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis.
This patient underwent a right hemi-hysterectomy and salpingectomy, and the diagnosis was confirmed.
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