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Case of mrkh with vaginal hypoplasia for vaginoplasty
- 1. CASE OF MRKH WITH VAGINAL ATRESIA WITH VAGINOPLASTY Dr SNEHA JADHAV (PG RESIDENT ) Dr ARTI SHIRSATH (ASSOCIATE PROFESSOR) Dr AJAY NAIK ( PROFESSOR SURGERY) DEPT. OF OBSTETRICS & GYNECOLOGY MEDIACE 2016
- 2. HISTORY 19 years married female Complaints – Primary amenorrhea Difficulty and pain during sexual intercourse Abdominal pain on and off since 4 years No h/o urinary complaints , joint pains , breathlessness or dyspnea .
- 3. HISTORY Menstrual h/o- primary amenorrhea Obstetric h/o – Nulligravida Married since 6 months. No history of Tuberculosis, Hypertension, Diabetes mellitus, Thyroid disease or Heart disease or Allergies. Personal h/o – mixed diet, no addictions, bowel bladder habits are normal. Family h/o – no significant major illnesses or similar complaints in sibling sisters.
- 4. GENERAL EXAMINATION Height – 158 cm, Weight – 49 kg, BMI – 19.62 kg/m2 Afebrile Pulse – 76 beats/minute, regular, good volume BP – 110/76 mm Hg measured on left arm in supine position. Respiratory rate – 14 /minute No pallor, cyanosis, oedema, Lymphadenopathy, Clubbing or Thyromegaly seen
- 5. SYSTEMIC EXAMINATION Respiratory system – Air entry equal on both sides normal breath sounds Cardiovascular system- S1S2 heard no murmur Central nervous system- oriented to place person and time. Per abdomen – soft No guarding / tenderness / rigidity hernia sites were normal. Local - secondary sexual characters well developed Breast – Tanners stage 4 Axillary / pubic hair Tanners stage 4
- 6. LOCAL EXAMINATION Per vaginum- blind vaginal pouch of 1cm depth. Per speculum – blind vaginal pouch of 1 cm urethral opening is normal Per rectum –small size uterus felt ,mobile , non tender , no anterior bulge felt.
- 7. Provisional diagnosis Primary amennorhea with vaginal atresia which was subjected to further investigations .
- 8. INVESTIGATIONS Hemoglobin – 14.4 gm/dL TLC – 5600/cumm Platelet count – 2,92,000/cumm Blood sugar level random – 84 mg/dl RFT – Urea – 19 mg/dL; Creatinine – 0.8 mg/dL LFT – Bilirubin ( Total ) – 0.3mg/dL ( Direct – 0.1 mg/dL, Indirect – 0.2 mg/dL SGOT – 14 IU/L, SGPT – 10 IU/L, ALP – 46 IU/L Serum electrolytes (in mmol/L) – Sodium – 139,Potassium – 4.4,Chloride – 106 Prothrombin time – 14.7 seconds (control 13.1 sec); INR – 1.12 HIV & HbsAg – Non-reactive Urine routine – Within normal limits.
- 9. INVESTIGATIONS FSH ,LH, TSH , prolactin were within normal limits. ULTRASONOGAPHY – Unicornuate uterus with rudimentary left horn with endometrial thickness of 4.2 mm , cervical canal atretic with vaginal atresia. Both ovaries appear normal. Right kidney agenesis seen with compensatory enlargement of left kidney . Rest within normal limits. Xray chest – Normal . Xray neck and lumbosacral spine – Normal.
- 10. INVESTIGATIONS MRI PELVIS - Unicornuate uterus 70mm x34mm x 50 mm with absent left horn . Atresia / hypoplasia of vagina Right paraovarian 29mm x26 mm cyst Bilateral ovaries normal with multiple follicles. MRI SELLA AND BRAIN - NORMAL
- 11. MRI IMAGES
- 12. MRI IMAGE
- 13. Final Diagnosis MRKH with Non functional Unicornuate uterus with vaginal atresia planned for vaginal reconstruction surgery to restore sexual function. Mc Indoe’s vaginoplasty was planned for the patient using skin graft.
- 14. VAGINOPLASTY OPERATION Mc Indoe’s vaginoplasty was done on 9th august under spinal with epidural anesthesia An elliptical shape Full thickness skin graft was harvested from suprapubic region from the abdomen . Vaginal mould was created using sterile foam filled in condom. Graft defattened upto dermis and sutured over vaginal mould . Space was created between the rectum and bladder for fixing the mould . Graft with mould stiched to the space created.
- 15. Graft defattened Graft stiched over a mould
- 16. Space created between rectum and bladder Condom mould inserted into the space created
- 17. Skin Graft sutured with the passage and the interoitus circumferentially.
- 18. Postoperative care She was on IV antibiotics for 5 days Soft diet started on post op day 2 Every alternate day dressing was done . Mould expelled spontaneously on day5 Foleys was removed on day 8
- 19. Post op day 8
- 20. MRKH Müllerian agenesis, also called Mayer-Rokitansky-Küster-Hauser syndrome or MRKH is a congenital malformation characterized by failure of the Müllerian duct to develop. There is absent or anomalous uterus and variable degrees of vaginal hypoplasia. Incidence 1 : 5000 female child. In this condition hormone levels are normal; they enter puberty with normal development of secondary sexual characters. Congenital absence or anomalies of uterus and vagina ,normal ovaries, phenotypically and genetically female with associated skeletal or renal anomalies .
- 22. References Griffin JE , Edwards C, Madden JD, et al congenital absence of vagina . Ann intern med 1976;85:224-236 Abbe R. New method of creating a vagina in a case of congenital absence. Med Rec 1898;54:836 American congress of Obstetricians and Gynecologists . Mullerian agenesis : diagnosis management and treatment. Committee opinion no 562 obstet gynecol2013;121:1134 Cramer DW , Goldstien DP , Fraer C et al . Vaginal agenesis ( Mayer – Rokitansky- Kuster- Hauser syndrome)associated with the N314D mutation of GALT . Mol Hum Reprod 1996;2:145-148 Rock JA . Anamolous development of vagina . Semin Reprod Endocrinology 2002; 198;61-67 Frank RT the formation of artificial vagina . Am J Obstet Gynecol 1982;59:448- 451 McIndoe A . The treatment of congenital absence and obliterative condition of vagina .