A Case of Mayer–Rokitansky–Küster–Hauser Syndrome with a Fused Pancake-shaped Pelvic Kidney

Document Type : CASE REPORT

Authors
Ali Reza Eftekhari Moghadam 1
Ghasem Saki 1
Mahin Taheri Moghadam 1
Seyed Mohamad Hossein Mohseni 2
Vajieh Heidari 3
Mohammad Hossein Jamshidi 4
1 Department of Anatomical Science, Faculty of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
2 Golestan Medical Imaging Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
3 School of Health and Biomedical Science, Royal Melbourne Institute of Technology University, Melbourne, Australia
4 Department of Radiologic Technology, Faculty of Paramedicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
Abstract
Mayer–Rokitansky–Küster–Hauser (MRKH) Syndrome is a female reproductive system disorder. It is characterized by a defect in the Müllerian ducts development, and it causes the absence of the uterus in variable degrees in upper vaginal hypoplasia. In addition, it is often associated with the unilateral renal dysplasia. Müllerian agenesis affects 1 in 4500 newborn girls and is considered as a sporadic anomaly. Women with MRKH Syndrome have a normal female chromosome pattern 46, XX with normal ovarian function. The presence of bilateral kidney agenesis with a pelvic pancake-shaped kidney is a rare condition, and a few cases have been reported in medical journals. This case study focuses on a case of MRKH Syndrome with bilateral renal agenesis and a pancake-shaped kidney.
Keywords
Mayer–Rokitansky–Küster–Hauser Syndrome
Müllerian ducts
renal agenesis
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Advanced Biomedical Research
Volume 2019, May
May 2019
Pages 1-4