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Absent Uterus and Ovaries

Absent Uterus and Ovaries Posted by Sivapriya S.
Busy SpR :. Essay Question ::

A 20 year old sexually active woman complains of primary amenorrhoea. Clinical examination and ultrasound scanning have confirmed a blind-ending vagina with absent uterus and absent ovaries. Justify your management.

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The Primary Amenorrhea with absent uterus and Ovaries suggests a male karyotype with a default female phenotype. The Aims of management are to reach a firm diagnosis, treat underlying cause, maximise physiological function and discuss gender issues when appropriate.

I would examine her with regard to Palpable gonads in inguinal canal and /or presence of abdomino-pelvic mass .
I would confirm the diagnosis with a chromosomal analysis. If 46XY (androgen insensitivity) or 46XX/XY mosaics - I would discuss about removal of dysgenetic gonads as risk of malignancy, discuss sexual assignment and provide oestradiol replacement.
I would inform her in an empathetic way that sexual functon can be maintained with dilators or surgery and that Her wishes regarding reproductive functions cannot be ascertained.

I would arrange psychosexual counselling in a dedicated clinic. I would Ascertain her wishes of non disclosure, secrecy and wishes regarding her vaginal requirements.
She would be managed by a MDT team involving a plastic surgeon and pschosexual counsellor.

Vaginal dilators are the first line of choice .It has 80 % success rate, but the outcome regarding sexual satisfaction have not been evaluated. This method is the first choice of treatment because it avoids the risks of surgery and when successful, results in a vagina with a moist lining.The success of dilators depends on the motivation of the patient and support provided during therapy.

Surgery is reserved if she is unable to attain the comfortable intercourse. I would inform her that many surgeries are available and any one method lacks ideal results. I would involve the patient in the decisions about surgery. Vaginoplasty by Vecchietti or Davyvov procedures can be performed through Laparoscopy and removal of gonads at the same sitting of operative laparoscopy.

There is a risk of malignancy in the dysgenetic gonads. Hence a lapascopy or a laparotomy is essential to remove the gonads.I would inform her that there is a risk of malignancy in the neovagina in later years.

I would discuss with her regarding risk of osteoporosis in the long term and hence estradiol treatment, vit D and calcium supplementation, weight bearing exercises.
Involvement of a clinical psychologist throughout is essential and helps her to have realistic expectations of her treatment.