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The following summarized guidelines for the evaluation and management of müllerian agenesis are prepared by our editorial team based on guidelines from the American College of Obstetricians and Gynecologists (ACOG 2018).
1.Screening and diagnosis
Diagnosis: recognize that patients with Müllerian agenesis usually are identified during evaluations for primary amenorrhea with otherwise typical growth and pubertal development.
Diagnostic imaging: recognize that rudimentary Müllerian structures are found in 90% of patients with Müllerian agenesis by MRI, but can be difficult to interpret on ultrasound and may be particularly misleading before puberty.
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Evaluation for other congenital anomalies
Vaginal dilation: offer primary vaginal elongation by dilation as the first-line approach in most patients with Müllerian agenesis because it is safer, patient-controlled, and more cost-effective than surgery, and is successful for > 90-96% of patients.
Indications for surgery: reserve surgery for cases of unsuccessful primary dilation or for patients preferring surgery after a thoroughly informed consent discussion with the gynecologic care provider and the patient's parents or guardians.
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General counseling: provide counseling to all patients with Müllerian agenesis and encourage connecting with peer support groups.
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