Primary Amenorrhea

Author: Maria     Published: 2 years ago 1 Replies

A young female,17yr old presented in opd with primary amenorrhea. On examination patient lacks secondary sexual characteristics.

Labs reveal:

  • Low LH, FSH, estradiol, testoterone,
  • normal DHEA,
  • karyotyping 46 XX,
  • ultrasound pelvis shows absent uterus, blind ended vagina, bilateral normal ovaries, normal bilateral kidneys,
  • cortisol 8am was high 710.8nmol/l,
  • TSH 5.65(slightly high) ,FT3 5.15, FT4 15.91.
  • MRI pitutary in plan.

Needs suggestions and opinion regarding diagnosis and management of this patient?

Whether the diagnosis is Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome or another hypothalamic or pituitary cause, the question is:

  • How can we plan the management of this patient?

Since the patient does not have a uterus, fertility is not possible. We can do two things at most to help her:

  1. Prescribe hormones so that the patient can have a normal female physical appearance.
  2. Assisted reproduction methods can be used.

Assisted reproduction method is very tricky in this case. It is certain that the patient can not have normal babies. We can stimulate her ovaries to produce eggs that can be invitro fertilized with her husband's sperm and placed in another woman's uterus. 

From an Islamic point of view, this is only possible if her husband has another wife as well. The baby will be born to her husband's other wife but the genetic material will be her's.

If there are other expert opinions here, we shall have a consensus and plan this patient's management.

Dr. Ahmed Farhan, 2 years ago

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