Mayer rokitansky kuster hauser syndrome

I was brought into this world from the womb of my mother…………………………

I am a female baby………..

I thought…… I am a

girl…… but the thought

remained as a thought in my life…………………

The period when she realized that she is different from all other girls…………

She did not have something which others had………….

The day when she knew that she had…………

MAYER ROKITANSKY KUSTER HAUSER

SYNDROME

Imagine what it is for a woman to know that she has no uterus and no vagina….

it’s TERRIBLE………..

Named after……..

August Franz Joseph Karl Mayer Carl Frieherr von Rokitansky Hermann Kuster G.A .Hauser

A rare disorder characterized by congenital absence of uterus and vagina

You cry because of pain but they cry because…………………….

THEY DON’T

HAVE PAIN………

Will u stop menstruating if you could?????

…………………a question asked by a

MRKH girl to all normal women who complain about menstruating……………….

Prevalence

1 in 4000-5000 female births

IS IT MY FAULT?

The cause is unknown……… a blank space still

remains…..

Can you find the answer?........

Signs & Symptoms……

Primary amenorrhea Complete or partial absence of cervix, uterus

and vagina Painful coitus Hormonally normal Enters puberty with development of

secondary sexual characteristics-adrenarche , thelarche

Chromosomal constellation-46XX Ovaries intact…………ovulation

occurs………………….

But she cannot be……… A MOTHER she cannot carry………. A life in her

womb A life perhaps incomplete………

Forms of MRKH…..

Only absence of vagina and uterus Absence of uterus and vagina + 40% kidney

abnormalities 10% hearing

problems 10-12%

skeletal abnormalities

Solution?

No treatment to let the woman herself become pregnant

Treatment available to make the intercourse comfortable

Surgical Management

Frank technique or perineal dilation apply progressive pressure

to the perineum using a bicycle-seat stool to hold a dilator in place .

…………compliance may be poor………..

McIndoe technique

Split thickness skin graft -skin from thigh or buttocks is used……..

PROCEDUREPocket created between rectum and urethra ↓Place a cylindrical stent covered with skin

graft ↓Fix graft in place by attaching cut edges of

skin incission

↓Labia majora sutured loosely together ↓ 1 week later remove stent ↓ irrigate vagina and cauterize areas of

granulation with silver nitrate sticks

Keep dilator in neovagina every day and night for 3 months;

followed by every night for next three

months………….scarring at donor site………………….neovaginal stenosis…………………….long term dilation……………

Williams Vaginoplasty

Uses vulval flap to make a vaginal tube………needs long term dilation……..

Rotational Flap Procedure

Uses - pudendal thigh - gracilis myocutaneous - labia minora - other fasciocutaneous flaps………..extensive scarring at donor

site……..………...requires patient’s

diligence…………...

Intestinal Neovagina

Uses isolated segment of bowel for vagina; mostly sigmoid

………..high grade of satisfaction……….

Vecchietti Technique

Exert continuous progressive pressure…….. by acrylic olive passed through potential neovaginal space and abdominal wall……..

↓Traction device placed into peritoneal cavity ↓ Draw olive upward ………done laparoscopically………..

Options…….

My baby in other’s womb………SURROGACY

Means……

Surrogacy is an arrangement in which a woman carries and delivers a child for another person or couple……..

Surrogacy varies…….

Traditional surrogacy: woman may be child’s genetic mother

Gestational surrogacy: carry pregnancy to delivery after having an embryo, with no genetic relationship, transferred to her uterus

Commercial surrogacy Altruistic surrogacy

Think of IVF if both have problem…………

Characteristics of a surrogate mother

Relative or close friendBetween 21 and 38 yrs of ageGiven birth to one or more child

previously with no complicationsNo history of STDsGood health………

Average weight for her height A non-smoker who lives in a smoke

free room No illegal drug use

Arguements

Cost of surrogacy Custody Desperate needs home Long process Feel of Guilt

Promise of a mother……

First Major Groundbreaking Surrogacy

Publicized issue of Melissa Stem in 1986, USA…… Biological father: William Stem His wife : Elizabeth Stem Surrogate Mother: Mary Beth

Whitehead

Mary Beth Whitehead is the biological mother………..legal mother…………

Who is the Mother?????

Saudi Arabia sanctioned world’s first Uterus transplant……..

In India……

Commercial surrogacy legal in India……Supreme Court in 2002

leader in international surrogacy……

Contact…….

www.surrogatemothers.comwww.surrogateAlternatives.comwww.GlobalDoctorOptions.comwww.indiansurrogates.com

I didn’t bring you……. butI will build you……..

You are my baby and am your mother….

Adoption……

Life is Life……

Realize what you have………… There are people on earth

around you carrying pain in heart ……….

may be more than yours…….

A relation sopure……

Thank you………..