September 5, 2010

Dr. Rahul Manchanda

Dr. K.V. Mahalakshmi

This 18 year old brave girl “X” actually “brought her parents” to the doctor as she was suffering from primary amenorrhea. Naturally distressed especially with her parents not wanting to accept this she taking matters in her hand presented at the out patients. She came to us with chief complaints of not having started with menses and poorly developed breasts.  There was no history to suggest any insidious/ ongoing disease process /radiation exposure. Tuberculosis and thyroid illness were ruled out.
Her ultrasound and MRI showed smaller ovaries and a hypoplastic uterus (33mm) with the endometrium not being well defined. Her chromosomal analysis was normal and on examination, breasts were a little less developed but rest of the secondary sexual characters were within the range of development.
Hormonal profile was within normal but on the lower side.
She was taken up for a hysteroscopy and laparoscopy for further management on 5/9/10.
Hysteroscopy showed a very small cavity with endometrium being in proliferative phase and thin.
On laparoscopy ovaries were a tad smaller and the uterus too appeared smaller.
Hysteroscopic cutting of septum with bilateral lateral wall metroplasty was done.
She was put on high doses of sequential estrogen and progesterone therapy and was asked to follow up.
She did very well and got her first period after her surgery and has been regularly menstruating since then, much to her and her family’s joy!
Follow up USG was done in Feb. 2011 which interestingly showed her ovaries’ size to be larger and normal than before and a uterus of size 66 x 40 x 27mm.
Endometrium now was being well formed and typically triple layered.
Discussion:
Clinical aspect
“X” has done well and stands a normal chance as far as fertility in future goes.
Hypogonadism may present with a range of anatomical abnormalities which need to be diagnosed and managed according to the individuals’ problem/anatomy.
Anatomic abnormalities that may present as primary amenorrhea can broadly be classified as inherited and acquired disorders of the outflow tract (uterus, cervix, vagina and introitus).
Inherited causes of amenorrhea in adolescents in the form of abnormal pelvic anatomy are seen in 15% of girls. In this case partial Mullerian agenesis leading to lack of endometrium and hypoplastic uterus could have been the cause of primary amenorrhea.
Hence after surgical correction in the form of metroplasty, the patient responded well to hormonal therapy, thus regenerating the endometrium and restoring normal menses for this patient.
Lateral metroplasty and hysteroscopy gives us a great ability to treat and manage these patients who otherwise may suffer from infertility /subfertility.

Social aspects
Social stigma associated with problems related Sexual/reproductive system is great in our country and hence very difficult to get to manage and treat these conditions which are not talked about and swept under the carpet.
Prevarication at times can lead to irreversible problems and these needs to be told to people and a concerted education (IEC) Programme by health professionals should be instituted.