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Case Report of Mayer-Rokitansky-Kuster-Hauser Syndrome associated with Renal Anomoly Diagnosed Incidently in a Patient with Acute Abdomen

Journal: International Journal of Science and Research (IJSR) (Vol.11, No. 2)

Publication Date:

Authors : ; ; ;

Page : 212-214

Keywords : paramesonephric; mullerian; ducts; Mullerian Renal; Cervicothoracic Somite;

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Mayer-Rokitansky-Kuster-Hauser syndrome, also known as Rokitansky syndrome, is a malformation of the female genital tract that is due to interrupted embryonic development of the paramesonephric (m?llerian) ducts. It leads to hypoplasia of the uterus and the upper two-thirds of the vagina. It affects approximately one in 4500 live births (1). It occurs as a purely genital malformation (type 1), but also with associated malformations (type 2 and MURCS association; Mullerian Renal, Cervicothoracic Somite abnormalities.2 Malformations of the kidneys and urinary tract the skeleton, and more rarely of the heart and central nervous system have been described.3Weare reporting a case of incidental finding of MRKH syndrome with associated horseshoe kidney in a 29-year-old female, who came to our emergency department with sudden onset of pain right iliac fossa which is progressive and is non radiating. The clinical diagnosis of Acute appendicitis was kept with MANTERAL score of 5. CECT Abdomen was done in emergency with finding of medially oriented inferior pole of bilateral kidney which is fused in midline at L3 vertebral body level with non visualised uterus and cervix. Appendix is normal. Thus diagnosis of MRKH syndrome is kept with unusual clinical presentation.

Last modified: 2022-05-14 21:00:31