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An Unusual Case of Uterine FibroidI Adibah, K Norhayati, KC Liew

Department of Obstetrics and Gynaecology, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia

Introduction and Objective

The case

The case continues……

Mayer-Rokitansky-Küster-Hauser (MRKH)

syndrome is an uncommon congenital anomaly,

affecting 1 in 4000 to 5000 of women. It is

characterized by vaginal aplasia with normal

external genitalia, absence of fibromuscular

remnants of the uterus with normally developed

fallopian tubes and ovaries.

The presence of solid pelvic mass in patients with

MRKH is difficult to examine, especially when no

vaginal reconstruction has been performed. An

imaging technique may provide further information

about the origin but may also be misleading. By

describing the uncommon association between

uterine fibroid and MRKH may make gynaecologist

to be aware of its possibility in dealing with pelvic

mass in MRKH cases.

A 51 year old Malay lady was referred to our centre

for further management of primary amenorrhoea. Her

previous gynaecologic history included the diagnosis

of MRKH made by another service hospital, when

she was 18 years of age. She had not undergone any

vaginal operation apart from her diagnostic

laparoscopy. She was advised to come back to the

hospital when she got married which occurred 33

years later.

The patient managed to have a consummative

sexual intercourse. However, she had some

climacteric symptoms.

Physical examination revealed that the secondary

sexual characteristics were well developed, and the

external genitalia were normal. She had a functional

vagina with a length of 4cm achieved through

sexual activity, an absence of cervix, and a palpable

right pelvic mass.

The serum level of oestrogen was normal; however

the levels of follicle-stimulating hormone and

luteinizing hormone were within menopausal range.

The intravenous pyelogram showed duplex right

hydroureters with mild hydronephrosis and a single

left hydroureter. Transvaginal scan revealed a solid

pelvic mass, which could be ovarian in origin. A

rudimentary uterus and an atrophic ovary

measuring 2cm x 1cm were visualized on the left

side. CT scan of the pelvic identified a solid right

ovarian mass measuring 5cm x 6cm.

The patient underwent a laparotomy. The uterus

was substituted by 2 bilateral remnants, of which

the one on the right side showed multiple uterine

fibroids. The remnants were completely separated.

Both fallopian tubes were normal and both ovaries

were noted atrophic.

The case continues…..

Right fallopian tube

Fibroid s

Remnants of the uterus

Myomectomy of the uterine fibroids was performed,

leaving the uterine remnant to the size of 2cm x 1 cm.

The patient was discharged well on the third

postoperative day. Histopathology examination of the

fibroids confirmed the diagnosis.

Discussion

References

Patients with MRKH syndrome have normally

developed ovaries and fallopian tubes, and many of

them have two uterine remnants. These uterine

remnants consist of fibromuscular tissue, which

may be the target tissue for ovarian steroids, from

which tumours like leiomyomas can originate.

A pelvic mass found in MRKH patient needs to be

thoroughly evaluated. As illustrated in this case,

imaging technique may give some value but may

also mislead the physician to the origin of the mass.

A better way to evaluate such case is by performing

diagnostic laparoscopy, provided that the risk of

developing malignancy is low.

In conclusion, although the development of uterine

fibroid is rare in MRKH patients, it needs to be

included in the differential diagnosis of pelvic mass

in these patients.

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2. Efthimios Deligeoroglou, Antonios Kontoravdis, Evangelos Makrakis, Panagiotis Christopoulos, Apastolos Kountouris and George Creatsas. Development of leiomyomas on the uterine mremnants of two women with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril 2004;81:1385-1387

3. Faber M, Stein A, Adashi E. Rokitanski-Küster-Hauser syndrome. Obstet Gynecol; 51 (supp 11): 70-3

4. Marta Lamarca, Ricardo Navario, Maria Eugenia Ballesteros, Salvador Garecia-Agurire, Maria Pilarconte and Jose Antonio Dugue. Leiomyomas in both uterine remnants in a woman with the Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril 2009;91:931 e13-e15

5. Metzger DA, Massad LS, Piseitelli JT. Leiomyoma in a mullerian remnant. A case report. J Reprod Med 1988;33:246-8

Right ovary