A 6-year-old female presents with sudden oneset of right sided abdominal pain. Her mom notes the patient has a history of polycystic ovarian syndrome. US reveals an asymmetrically enlarged right ovary with adjacent right adnexal soft tissue swirling concerning for ovarian torsion. She is brought to the operating room where the right ovary had a 180 degree torsion. After successful detorsion the remainder of the pelvis is examined and shown below. What is the best next step in the management of this patient?

 

 

 

 

a [size=6]Oopexy of bilateral ovaries

 

b Detorsion of the left ovary

 

c External pelvic examination

 

d Ovarian biopsy

 

e CT scan of the abdomen/pelvis[/size]

A

A

A 6-year-old female presents with sudden oneset of right sided abdominal pain. Her mom notes the patient has a history of polycystic ovarian syndrome. US reveals an asymmetrically enlarged right ovary with adjacent right adnexal soft tissue swirling concerning for ovarian torsion. She is brought to the operating room where the right ovary had a 180 degree torsion. After successful detorsion the remainder of the pelvis is examined and shown below. What is the best next step in the management of this patient?

 

 

[ATTACH=full]15044[/ATTACH]

 

 

a [size=6]Oopexy of bilateral ovaries

 

b Detorsion of the left ovary

 

c External pelvic examination

 

d Ovarian biopsy

 

e CT scan of the abdomen/pelvis[/size]

 

A 6-year-old female presents with sudden oneset of right sided abdominal pain. Her mom notes the patient has a history of polycystic ovarian syndrome. US reveals an asymmetrically enlarged right ovary with adjacent right adnexal soft tissue swirling concerning for ovarian torsion. She is brought to the operating room where the right ovary had a 180 degree torsion. After successful detorsion the remainder of the pelvis is examined and shown below. What is the best next step in the management of this patient?

 

 

[ATTACH type=full" alt="repview (6).jpeg]15044[/ATTACH]

 

 

a [size=6]Oopexy of bilateral ovaries

 

b Detorsion of the left ovary

 

c External pelvic examination

 

d Ovarian biopsy

 

e CT scan of the abdomen/pelvis[/size]

A

correct answer

c External pelvic examination

 

This patient has an absent uterus on examination which is pathognomic for Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH, i.e. Mullerian agenesis). This entity is defined anatomically as the absence of the uterus and upper vagina in an XX female. There is normal development of the ovaries but patients often have associated renal and vertebral anomalies. The incidence of MRKH is thought to be as high as one in 4,500 women. Patients present most often with primary amenorrhea and many are not noted to have a blind ending vaginal dimple on exam. Ovarian torsion is thought to occur in patients with MRKH due to the increased mobility of the ovaries in the setitng of the Mullerian agenesis. No further treatment is required for the ovarian torsion once the ovaries are detorsed.

 

This patient should undergo a pelvic examination. A patient with MRKH syndrome can be expected to have a normal XX karyotype. On examination, the labia are often normal. The urethra may be patulous or enlarged with a central location. The hymenal tissue is usually normal appearing with an indentation in the posterior introitus but no true vaginal opening. Some patients have a flat plate of tissue instead of an indentation.

 

The patient described in the question underwent a pelvic examination under anestheisa after discussion with the family of the findings of the laparoscopy and was noted to ahve no true vaginal opening confirming the diagnosis of MRKH.