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

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

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.

Particulars of patientName 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

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.

Gynaecology history-

Not attained menarche

Married for 14 months

Last episode of blood stained urine-19-03-2013

Past history-

No histories of- diabetes hypertension jaundice tuberculosis epilepsy blood transfusions bronchial asthma surgeries

Personal history-She takes mixed dietSleep and appetite are normalBowel and bladder habits are regular

Family history-

Her mother is an epileptic

General physical examination- Conscious and coherentModerately built and nourishedNo- pallor icterus cyanosis clubbing lymphadenopathy oedmaThyroid,breast,spine are normal

Secondary sexual characters-

Axillary hair Pubic hairBreast development

Vitals-Temperature-afebrilePulse-82 /minRespiratory rate-15bpmBlood pressure-100/70 mm of Hg

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.

Provisional diagnosis

? Vaginal atresia with Urogenital fistula

The possible differential diagnoses are-

imperforate hymen,

Rokitansky-Kustner-Hauser Syndrome,

Testicular-feminisation syndrome,

Youseff's syndrome.

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

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.

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

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

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.

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

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

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.

Diagnosis -

Congenital vesico-vaginal

fistula with distal Vaginal

atresia.

Management -

Planned to undertake stepwise

1. Reconstruction of vagina

2.Restoration of continuity of genital

tract.

3. Repair of vesico-vaginal fistula

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.

A VVF repair will be done by O’Conor’s

method on a future date

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

The relative prevalence of primary amenorrhea includes

Hyper-go-

nadotrohic48%

Eugo-nadotrophic

24%

Hypogo-nadotrohic

28%

Percentage of prevalence-

Eugonadism may result from –

a. Absence of Mullerian development

b. Normal Mullerian development

c. Cryptomenorrhoea

The work up of eugonadotrophic amenorrhoea includes-

Clinical examination for the presence of secondary sexual characters and external genitaliaBuccal smear for Barr bodyGonadotrophin assayImaging studies- USG MRI

Urogenital fistulaAcquired causes are- • obstructed labour • pelvic surgery• malignancy of genital tract • Pelvic irradiationCongenital genital fistulas are

extremely rare

Diagnosis of genital fistulas involve

Detailed historyClinical examinationThree swab test CystoscopyIVUMRI with contrast

In our patient due to vaginal atresia three swab test was not possible

Summary

All women with menouria need complete investigationwith 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.

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

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

First page of the patient’s case sheet