Department of Obstetrics and Gynecology, University of Colorado Denver, Aurora, CO, USA (Drs. Stephens, Alvero, Kondapalli). Department of Obstetrics and Gynecology, ME Medical Center, Tufts University School of Medicine, Portland, ME, USA (Dr. Maas). Department of Radiology, University of Colorado Denver, Aurora, CO, USA (Dr. Chang).

ABSTRACT

Introduction: Mu¨llerian anomaly is a result of abnormal elongation, fusion, canalization, or resorption of the paramesonephric ducts during organogenesis. An accurate diagnosis and appropriate treatment planning can be facilitated by imaging modalities; however, direct visualization of the pelvic organs may be necessary for an accurate diagnosis.

Case Description: A 14-year-old girl with primary amenorrhea presented with severe abdominal pain. Magnetic resonance imaging suggested a unicornuate uterus on the right side with a left-sided noncommunicating uterine horn, both with functional endometrium and likely high outflow obstruction. She was counseled on removal of the noncommunicating uterine horn and correction of the outflow obstruction; however, on laparoscopic and vaginoscopic exploration, she was found to have bilateral noncommunicating functional uterine remnants with a normal-length vagina and a septate hymen. She underwent laparoscopic removal of uterine remnants with complete symptom resolution.

Discussion: Preoperative imaging studies can help guide patient counseling and preoperative planning in cases of suspected mu¨llerian anomaly; however, the final diagnosis may not be made until the time of surgery. The patient should be prepared for multiple possibilities during preoperative consultation.

Key Words: Müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, Laparoscopy.

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