A prize winning presentation made by me in 2013 at Osmania medical college about an interesting case recorded in GGH, Guntur.

1. PRIMARY AMENORRHOEA
WITH
MENOURIA
By-
Akash Srivatsav.T ,Final year(II)
Moderator-
Dr. Chandra Sekhar Rao, MD.
Prof. and HOD, dept of OBG,
GGH. Guntur

2. Primary amenorrhea is when a girl has not yet
started her monthly periods,
She has gone through other normal changes
that occur during puberty and
Is older than 16yrs

3. Congenital urogenital fistulas are rare.
Coexistence of congenital urogenital fistula
with vaginal atresia presenting as primary
amenorrhoea with menouria is of extremely
uncommon occurence.
We are reporting a case of primary
amenorrhoea with menouria diagnosed and
being treated in our hospital.

4. Particulars of patient
Name Mrs.SFR
Age 20 years
Occupation Daily wage labourer
Address Bapatla
Marital status Married
Regd no 13023
DOA 18-03-2013
Referred from Bapatla govt hospital

5. She complained of-
Not attaining menarche
Passing blood stained urine for 3-4 days every
month for the past 7 years
Backache and abdominal pain for 3 days while
passing blood stained urine.

6. Gynaecology history-
Not attained menarche
Married for 14 months
Last episode of blood stained urine-19-03-
2013

7. Past history-
No histories of- diabetes
hypertension
jaundice
tuberculosis
epilepsy
blood transfusions
bronchial asthma
surgeries

8. Personal history-
She takes mixed diet
Sleep and appetite are normal
Bowel and bladder habits are
regular
Family history-
Her mother is an
epileptic

9. General physical examination-
Conscious and coherent
Moderately built and
nourished
No- pallor
icterus
cyanosis
clubbing
lymphadenopathy
oedma
Thyroid,breast,spine are
normal

10. Secondary sexual characters-
Axillary hair
 Pubic hair
Breast development
Vitals-
Temperature-afebrile
Pulse-82 /min
Respiratory rate-15bpm
Blood pressure-100/70 mm of Hg

11. Abdominal examination did not reveal any abnormal
masses.
Genital examination ---A small blind ending pouch
was identified in place of vagina.
Rectal examination--- Retroverted uterus felt. Cervix
felt as cylindrical structure about 5 cm above the level
of introitus. No other abnormalities detected.

14. Provisional diagnosis
? Vaginal atresia
with
Urogenital fistula

15. The possible differential diagnoses are-
 imperforate hymen,
Rokitansky-Kustner-Hauser Syndrome,
Testicular-feminisation syndrome,
 Youseff's syndrome.

16. Investigations -
Haemoglobin - 9gm%
TC ---7500/mm, DC—P 51%,L 37%,E 4%.
Blood group and Rh type –AB+
ESR ----25mm/hr
Random blood sugar--- 100mg/dl
HIV---- non reactive
Hep.B & Hep C--- Negative
BT –2min,.CT– 3 min.
Platelets –1,26,000/mm3.
Urine routine examination was normal

17. LFT – within normal limits
RFT– within normal limits.
Thyroid function tests----- normal.
FSH,LH,Prolactin levels-normal
Buccal and peripheral smears for sex chromatin---- positive.
Chest X ray --- Normal study.
ECHO --- Normal study.

18. Urine culture and sensitivity examination
showed growth of coagulase +Staphylococcus
sensitive to Ceftazidime, Levulofloxacine and
Piperacillin.

19. USG of abdomen - Liver normal.
Gallbladder, pancrease, left
and right kidneys were normal.
Uterus – measured 70x30x35
mms.
Thin endometrium. Both
ovaries visualised. No
free
fluid noted in the POD.
No abnormal masses

20. Cystoscopy was performed under local anaesthesia
Bladder volume was normal.
Bladder mucosa was normal.
Uterine impression was seen on
posterior wall of bladder.
Interureteric bar is V- shaped ending in a
dimple proximal to bladder neck.

21. Trans perineal USG
Uterus 6.3x3.4cm
Cervix measures– 1.2 cms
Endometrial thickness 0.8 cms
Myometrium normal.
Right and left ovaries normal.
Rudimentary distal vagina,
No evidence of free fluid

22. Retrograde contrast CT cystogram -
Contrast was noted in the distal portion of the
uterus and proximal portion of the vagina.
Entire uterine contour and site of fistulous
communication was not identified.
MRI scan with contrast was suggested

24. MRI scan---
Bladder distended with urine.Wall thickness normal. No calculi. Focal loss of fat
planes between bladder wall and vaginal wall.Anteriorly pulled up vaginal wall
in the right lateral aspect.
Suspicious linear hyperintensities noted on the posterior wall of bladder on the
right side.
Uterus—normal size (5.8x3.4x4.2cm). Weight 41 gms.Endometrium 6mm in
thickness.
Ovaries normal in size and show immature follicles bilaterally.
Vaginal length is 3cm and minimally distended with fluid.
No free fluid, no lymphadenopathy,no masses.
Vertebrae normal.
Anterior abdominal wall normal.
Impression- Suggestive of Vesico-vaginal fistula.

27. Diagnosis -
Congenital vesico-vaginal
fistula with distal Vaginal
atresia.

30. Management -
Planned to undertake stepwise
1. Reconstruction of vagina
2.Restoration of continuity of genital
tract.
3. Repair of vesico-vaginal fistula

31. Abbe-Wharton-McIndoe Vaginoplasty was carried out on
15-07-2013 under epidural anaesthesia. Split skin graft was
raised from the lateral aspect of the right thigh and wrapped
around a mould and secured in the space created between
the bladder and rectum .
Postoperatively patient was managed with antibiotics and
analgesics.
Patient is under follow up for further management.

33. A VVF repair will be done by O’Conor’s
method on a future date

35. Case discussion
• Primary amenorrhea is when a girl has not
yet started her monthly periods,
• She has gone through other normal
changes that occur during puberty and
• Is older than 16yrs

36. The relative prevalence of primary amenorrhea includes
Percentage of prevalence-
Hypergona
dotrohic
48%
Hypogona
dotrohic
28%
Eugonadot
rophic
24%

37. Eugonadism may result from –
a. Absence of Mullerian
development
b. Normal Mullerian development
c. Cryptomenorrhoea

38. The work up of eugonadotrophic amenorrhoea
includes-
Clinical examination for the presence of secondary
sexual characters and external genitalia
Buccal smear for Barr body
Gonadotrophin assay
Imaging studies- USG
MRI

39. Urogenital fistula
Acquired causes are-
• obstructed labour
• pelvic surgery
• malignancy of genital tract
• Pelvic irradiation
Congenital genital fistulas are extremely rare

40. Diagnosis of genital fistulas involve
Detailed history
Clinical examination
Three swab test
Cystoscopy
IVU
MRI with contrast
In our patient due to vaginal atresia three swab test
was not possible

41. Summary
All women with menouria need complete investigation
with exhaustive exploration, analytic evaluation, ultrasound,
imaging tests (principally magnetic resonance) and, very
importantly cystoscopy on the days of menouria.
Surgical treatment must be careful and individualized.
Multidisciplinary input in the management is the cornerstone
for successful reproductive outcome.

42. Bibliography-
1.Shaw’s text book of Gynaecology 15th edition
2.William’s Gynaecology 2nd edition
3.Te Linde’s operative gynecology volume-1,10th edition
4.Female Urology Shlomo Raz and Larissa V.Rodriguez 3rd edition

43. Similar cases-
Primary menouria due to a congenital vesico-vaginal
fistula with distal vaginal agenesis: a
rarity.
Singh V, Sinha RJ, Mehrotra S.
Source
Department of Urology, Chhatrapati Shahuji Maharaj
Medical University (formerly King George's Medical
University), Lucknow, UP 226003, India

44. First page of the patient’s case sheet