Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report
The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder characterised by failure of the uterus and vagina to develop properly in women who have normal ovarian function and normal external genitalia and secondary sexual characters. Mullerian agenesis accounts for approximately 10%...
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| Format: | Article |
| Language: | English |
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JCDR Research and Publications Pvt. Ltd.
2016-07-01
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| Series: | National Journal of Laboratory Medicine |
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| Online Access: | http://www.njlm.net/articles/PDF/2133/18926_F(P)_PF1(Om_Vsu)_PFA(GH)_PF2(VsuGH).pdf |
| _version_ | 1818379786965221376 |
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| author | Kaveri Gupta Manisha Sharma Rekha Jain Tarun Sarin Namrata |
| author_facet | Kaveri Gupta Manisha Sharma Rekha Jain Tarun Sarin Namrata |
| author_sort | Kaveri Gupta |
| collection | DOAJ |
| description | The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is
a rare disorder characterised by failure of the uterus and
vagina to develop properly in women who have normal ovarian
function and normal external genitalia and secondary sexual
characters. Mullerian agenesis accounts for approximately
10% cases of primary amenorrhoea. We report a case of
40-year-old female with MRKH syndrome with primary
amenorrhea who presented with lower abdominal pain.
She had well developed secondary sexual characters. Her
ultrasound showed bulky and heterogeneous uterus like
mass with ill defined endometrial-myometrial interface with
multiple uterine fibroids. Primary amenorrhea with normal
secondary sexual characters and phenotype of 44XX lead
us to the diagnosis of MRKH syndrome with uterine myoma
in mullerian remnant.
Surgical intervention showed the presence of transverse
vaginal septum with haematocolpos while the histopathology
of the remnant showed adenomyosis. Because of rarity of
the condition we report this case. |
| first_indexed | 2024-12-14T02:08:20Z |
| format | Article |
| id | doaj.art-afd1f29def2b4708a791d6f93d87700d |
| institution | Directory Open Access Journal |
| issn | 2277-8551 2455-6882 |
| language | English |
| last_indexed | 2024-12-14T02:08:20Z |
| publishDate | 2016-07-01 |
| publisher | JCDR Research and Publications Pvt. Ltd. |
| record_format | Article |
| series | National Journal of Laboratory Medicine |
| spelling | doaj.art-afd1f29def2b4708a791d6f93d87700d2022-12-21T23:20:50ZengJCDR Research and Publications Pvt. Ltd.National Journal of Laboratory Medicine2277-85512455-68822016-07-0153PC09PC1110.7860/NJLM/2016/18926:2133Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case ReportKaveri Gupta0Manisha Sharma1Rekha Jain2Tarun Sarin 3Namrata4Senior Resident, Department of Obstetrics and Gynaecology, NDMC Medical College and Hindu Rao Hospital, NDMC, Delhi, India.Senior Specialist, Department of Obstetrics and Gynaecology, NDMC Medical College and Hindu Rao Hospital, NDMC, Delhi, India.Chief Medical Officer (NFSG), Department of Obstetrics and Gynaecology, NDMC Medical College and Hindu Rao Hospital, NDMC, Delhi, India.Medical Officer, Department of Radiology, NDMC Medical College and Hindu Rao Hospital NDMC, Delhi, India.Senior Specialist, Department of Pathology, NDMC Medical College and Hindu Rao Hospital, NDMC, Delhi, India. The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder characterised by failure of the uterus and vagina to develop properly in women who have normal ovarian function and normal external genitalia and secondary sexual characters. Mullerian agenesis accounts for approximately 10% cases of primary amenorrhoea. We report a case of 40-year-old female with MRKH syndrome with primary amenorrhea who presented with lower abdominal pain. She had well developed secondary sexual characters. Her ultrasound showed bulky and heterogeneous uterus like mass with ill defined endometrial-myometrial interface with multiple uterine fibroids. Primary amenorrhea with normal secondary sexual characters and phenotype of 44XX lead us to the diagnosis of MRKH syndrome with uterine myoma in mullerian remnant. Surgical intervention showed the presence of transverse vaginal septum with haematocolpos while the histopathology of the remnant showed adenomyosis. Because of rarity of the condition we report this case.http://www.njlm.net/articles/PDF/2133/18926_F(P)_PF1(Om_Vsu)_PFA(GH)_PF2(VsuGH).pdfendometrial massmullerian agenesisprimary amenorrhea |
| spellingShingle | Kaveri Gupta Manisha Sharma Rekha Jain Tarun Sarin Namrata Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report National Journal of Laboratory Medicine endometrial mass mullerian agenesis primary amenorrhea |
| title | Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report |
| title_full | Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report |
| title_fullStr | Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report |
| title_full_unstemmed | Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report |
| title_short | Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report |
| title_sort | uterine adenomyosis in mullerian remnant with transverse vaginal septum with haematocolpos in mayer rokitansky kuster hauser syndrome a case report |
| topic | endometrial mass mullerian agenesis primary amenorrhea |
| url | http://www.njlm.net/articles/PDF/2133/18926_F(P)_PF1(Om_Vsu)_PFA(GH)_PF2(VsuGH).pdf |
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