mayer rokitansky kuster hauser syndrome

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

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Page 1: Mayer rokitansky kuster hauser syndrome

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

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I am a female baby………..

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I thought…… I am a

girl…… but the thought

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

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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…………

Page 5: Mayer rokitansky kuster hauser syndrome

MAYER ROKITANSKY KUSTER HAUSER

SYNDROME

Page 6: 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………..

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Named after……..

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

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A rare disorder characterized by congenital absence of uterus and vagina

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

THEY DON’T

HAVE PAIN………

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

…………………a question asked by a

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

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Prevalence

1 in 4000-5000 female births

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IS IT MY FAULT?

The cause is unknown……… a blank space still

remains…..

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

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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………………….

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

womb A life perhaps incomplete………

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Forms of MRKH…..

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

abnormalities 10% hearing

problems 10-12%

skeletal abnormalities

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Solution?

No treatment to let the woman herself become pregnant

Treatment available to make the intercourse comfortable

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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………..

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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

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

granulation with silver nitrate sticks

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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……………

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Williams Vaginoplasty

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

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Rotational Flap Procedure

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

site……..………...requires patient’s

diligence…………...

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Intestinal Neovagina

Uses isolated segment of bowel for vagina; mostly sigmoid

………..high grade of satisfaction……….

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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………..

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Options…….

My baby in other’s womb………SURROGACY

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Means……

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

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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

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Think of IVF if both have problem…………

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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………

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

free room No illegal drug use

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Arguements

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

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Promise of a mother……

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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

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

Who is the Mother?????

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

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In India……

Commercial surrogacy legal in India……Supreme Court in 2002

leader in international surrogacy……

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Contact…….

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

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

You are my baby and am your mother….

Adoption……

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Life is Life……

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

around you carrying pain in heart ……….

may be more than yours…….

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A relation sopure……

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Thank you………..