P3C377: The Diagnostic Dilemma of a Teenage Girl with Right Lower Quadrant Abdominal Pain

Introduction:

Introduction: Abdominal pain is a common presentation of children presenting to the pediatric emergency department. The differential for a teenage female presenting to the ER with right lower quadrant pain can be quite broad. Didelphys uterus, obstruction of a hemivagina, and ipsilateral renal agenesis are collectively known as OHVIRA syndrome, or Herlyn-Werner-Wunderlich Syndrome. The initial clinical presentation is frequently cyclic abdominal pain; however, it can vary widely. These abnormalities are rare and can be easily mis-diagnosed. Due to the variety of complications associated with didelphys uterus and Mullerian duct anomalies; such as, uterine rupture, urinary retention, spontaneous abortion, and premature delivery, it is important to accurately diagnose patients.

Case Description:

Case Description: A fifteen-year-old female presented to the ER with acute right-sided abdominal pain concerning for appendicitis. In the ER, labs and imaging were performed. A CBC showed no leukocytosis. BMP and HFP were grossly within normal limits. A urinalysis did not show any significant abnormalities and HCG was negative. Lipase and CRP were not elevated. An ultrasound of the appendix did not show any signs of appendicitis.

After the initial workup resulted, a pelvic ultrasound was performed which showed a possible bicornate uterus and concern for hematocolpos. Bimanual and speculum exams were unremarkable for any obvious abnormalities. The patient’s first menarche was 5 months prior to presentation and had occurred monthly.

Upon admission, MRI was obtained to confirm anatomical abnormalities and rule out other causes of abdominal pain. MRI showed uterine didelphys with two cervices and two vaginal cavities, signs consistent with vaginal obstruction, absence of the left kidney, a beaded tubular fluid structure in the left hemipelvis, and an adjacent cyst that measures 4 cm. Pediatric surgery and pediatric gynecology were consulted. Together they performed a vaginal exam under anesthesia with division of the vaginal septum and evacuation of the hematocolpos. The patient tolerated the procedure well and was discharged home with close follow up.

Discussion:

Discussion: OHVIRA can be easily confused with a more common etiology of lower abdominal pain in young women. This case is a reminder of the importance of keeping your differential broad and to not make an availability bias or other biases when similar patient’s present to the ER. Ultrasound and MRI are often used as the diagnostic tools for OHVIRA, with the gold standard being laparoscopy. The earlier the diagnosis and surgical correction, the less likely there are to be any complications.

Conclusion:

Conclusion: It is important to remember to keep the differential broad when a young female of reproductive age presents with acute onset abdominal pain. Although didelphys uterus with hematocolpos of an imperforate vagina is rare, it is important not to miss the diagnosis as untreated cases can result in significant adverse outcomes.