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𝐒𝐮𝐛𝐬𝐜𝐫𝐢𝐛𝐞 𝗙𝐨𝐫 𝗠𝐨𝐫𝐞 𝗜𝐧𝐟𝐨𝐫𝐦𝐚𝐭𝐢𝐨𝐧 𝐨𝐧 𝗛𝐞𝐚𝐥𝐭𝐡 👩⚕ 𝐚𝐧𝐝 𝗠𝐞𝐝𝐢𝐜𝐢𝐧𝐞💉🩺💊 𝐘𝐨𝐮𝐭𝐮𝐛𝐞 : / @draishwaryakelkar 📌𝗙𝗮𝗰𝗲𝗯𝗼𝗼𝗸 : / draishwaryakelkar 📌𝗧𝘄𝗶𝘁𝘁𝗲𝗿: / aishwayadr 📌𝗜𝗻𝘀𝘁𝗮𝗴𝗿𝗮𝗺 : / clinical.learning ✨ Müllerian Agenesis (MRKH) | USMLE Step 2 CK | Primary Amenorrhea, Diagnosis, Differentials & Management YouTube Description: Müllerian agenesis—also called Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome—is a congenital absence or severe hypoplasia of the uterus and upper two-thirds of the vagina in a 46,XX patient with normal ovaries, normal estrogenization, and normal secondary sexual characteristics. Teens typically present with primary amenorrhea and normal breast and pubic hair development; pelvic exam often reveals a short/blind vaginal pouch. Because the ovaries develop from the gonadal ridge (not Müllerian ducts), ovulation and hormone levels are normal. Remember associated anomalies: renal (agenesis, ectopic kidney, duplicated collecting system) and skeletal (vertebral, rib), plus the MURCS spectrum (Müllerian agenesis, renal anomalies, cervicothoracic somite defects). First steps: rule out pregnancy, then evaluate with pelvic ultrasound and confirm with MRI; check karyotype (46,XX) and consider renal imaging. High-yield differentials hinge on phenotype and hair pattern. Complete androgen insensitivity (46,XY) has absent uterus and vagina but scant/absent pubic/axillary hair with intra-abdominal or inguinal testes; imperforate hymen or transverse vaginal septum presents with cyclic pelvic pain, bulging bluish membrane, and hematocolpos on ultrasound; functional hypothalamic amenorrhea and gonadal dysgenesis have low or high FSH/LH respectively with a present uterus. Management prioritizes patient-centered counseling and creation of a functional vagina—progressive vaginal dilation is first-line and highly effective; surgical neovagina is reserved for failures or patient preference. Fertility options include IVF with a gestational carrier (oocytes are viable); uterine transplantation is emerging but limited to specialized centers. Address sexual function, psychosocial support, and screen for renal/skeletal anomalies. For exams: 46,XX primary amenorrhea with normal secondary sexual characteristics and no uterus on imaging = MRKH until proven otherwise. #MRKH #MullerianAgenesis #PrimaryAmenorrhea #USMLEStep2CK #USMLEStep1 #OBGYN #AdolescentGynecology #AmenorrheaWorkup #PelvicUltrasound #VaginalDilation #Neovagina #GestationalCarrier #AndrogenInsensitivity #ImperforateHymen #MURCS #MedicalEducationUSA #USMLEPreparation #Step2CKPrep #WomensHealth #ClinicalPearls Thinking