HERLYN WERNER WUNDERLICH SYNDROME: A RARE ANOMALY

 

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NTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWW) is a triad of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. It is a combined anomaly of Mullerian and mesonephric ducts. It usually presents in adolescent females after menarche with nonspecific symptoms of pelvic pain, dysmenorrhea, and rarely a palpable pelvic mass. When untreated, this may result in acute complications such as  pyohematocolpos, pyosalpinx, or pelviperitonitis, urinary obstruction, and long-term complications, such as endometriosis, pelvic adhesions, and increased risk of abortion, or infertility.

 

 

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ASE: An 14-year-old unmarried female presented to the our department with complaints of lower abdominal pain and lump in lower abdomen. She attained menarche at the age of 12 years and had an otherwise normal menstrual history. Basic laboratory tests were within normal limits.

 

 

An abdominal ultrasound was performed, which revealed absent right kidney, didelphic uterus and dilated hemivagina with low-level internal echoes [Figure 1a, 1b, 1c and 1d].

A suspicion of HWW syndrome was made, and CT abdomen and MRI pelvis was performed to rule out any complications.

MRI confirmed uterus didelphys with two separate uterine cavities, cervices, and vaginas. The left uterine cavity, cervix, and vagina were normal. The right uterine cavity was minimally distended and was seen in continuity with dilated right hemicervix and hemivagina, whose contents were hyperintense on both T1-weighted and T2-weighted MRI and showing fluid-fluid level and T2 shading consistent with hematometrocolpos with an obstructing right vaginal opening. Bilateral ovaries were normal. Right hydrosalphinx was also noted. There was no evidence of peritoneal endometriosis detected on MRI. CT scan of abdomen confirmed the absence of right kidney with compensatory hypertrophy of the left kidney [Figure 2A]. Hence, a diagnosis of HWW syndrome was confirmed with the presence of uterus didelphys, right hematometro-trachelo-colpos, right hemivaginal obstruction, and right renal agenesis.