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Müllerian agenesis

Guidelines

Key sources

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).
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Screening and diagnosis

Diagnosis: as per ACOG 2018 guidelines, recognize that patients with Müllerian agenesis usually are identified during evaluations for primary amenorrhea with otherwise typical growth and pubertal development.
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Diagnostic investigations

Diagnostic imaging: as per ACOG 2018 guidelines, 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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More topics in this section

  • Evaluation for other congenital anomalies

  • Vaginal cytology

Therapeutic procedures

Vaginal dilation: as per ACOG 2018 guidelines, 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.
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Surgical interventions

Indications for surgery: as per ACOG 2018 guidelines, 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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Patient education

General counseling: as per ACOG 2018 guidelines, provide counseling to all patients with Müllerian agenesis and encourage connecting with peer support groups.
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