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| CASE REPORT |
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| Year : 2016 |
Volume
: 9 | Issue : 4 | Page
: 263-266 |
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A rare case of 46,XX gonadal dysgenesis and Mayer-Rokitansky-Kuster-Hauser syndrome
Sriharibabu Manne1, CH Veeraabhinav1, Mounica Jetti1, Yalamanchali Himabindu2, Kiranmai Donthu2, Mutyalarayudu Badireddy3
1 Department of Medicine, G.S.L. Medical College and General Hospital, Rajahmundry, Andhra Pradesh, India
2 Department of Obstetrics and Gynecology, G.S.L. Medical College and General Hospital, Rajahmundry, Andhra Pradesh, India
3 Department of Radiology, G.S.L. Medical College and General Hospital, Rajahmundry, Andhra Pradesh, India
Correspondence Address:
Yalamanchali Himabindu
Department of Obstetrics and Gynecology, G.S.L. Medical College and General Hospital, Rajahmundry, Andhra Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0974-1208.197694
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46,XX gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46,XX karyotype. The phenotypic manifestations of MRKH syndrome may sometimes overlap with various other syndromes and require accurate delineation. The coexistence of both these disorders is extremely rare. Here, we report a case of 46,XX gonadal dysgenesis and MRKH syndrome with anatomically dispersed congenital anomalies unique among reported cases. |
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