This document describes a case of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with vaginal atresia treated with McIndoe's vaginoplasty. A 19-year old woman presented with primary amenorrhea and pain during intercourse. Exams and investigations confirmed MRKH with a unicornuate uterus and vaginal atresia. She underwent a McIndoe's vaginoplasty where a skin graft was harvested from her abdomen and sutured to create a neovagina. Post-operative care included antibiotics and dressing changes. The skin graft took well and a vaginal passage was successfully created.

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 .