A girl has primary amenorrhea with normal ovaries, absent internal genitalia but normal external genitalia. Most probable diagnosis?
High-Yield Explanation
Mayer Rokitansky Kuster Hauser's syndrome : karyotype is 46,XX Normal secondary sexual charecteristics Functional ovaries Normal hormonal profile Associated with skeletal and renal anomalies Management involves creation of a functional vagina for coital purposes and can become biological mother by surrogacy. SHAW'S TEXTBOOK OF GYNAECOLOGY,Pg no:286,15th edition