Anovulation

Mayer Rokitansky Kuser Syndrome (MRKH) is the complete failure in the development of the mullerian ducts resulting in absence of the fallopian tubes, uterus and most of vagina° (as of vagina is formed by Mullerian duct).

Mayer Rokitansky-Kuster Hauser syndrome

Karyotype = 46 XX°

Phenotype = Female

Associated abnormalities are :

• Renal ectopy and agenesis (Horse shoe shaped kidney)°
• Skeletal abnormalities° (most common – scoliosis)
• Cardiac anomalies.°

Clinical features :

• Patient present between 15-18 years of age with primary amenorrhoea.° (eugonadotropic amenorrhea).
• Secondary sexual characteristics are normal° as ovaries are normal (because of their separate embryonic origin, so ovulation is also normal.)
• P/V = Vagina is felt like a blind pouch and uterus is absent.°
• Findings are confirmed by USG.°
• Ideal method for demonstrating Uterine malformations is MRI.°

Management :

• Repair of vaginal agenesis done either by frank dilatation or vaginoplasty.
• Vaginoplast should onl be performed when the girls is just married or about to be marripri  

Frank Dilatation :

This non-surgical procedure consists of a woman applying gradual pressure with progressively increasing dilators over the mullerian pit for 15 minutes twice a day. An indentation is created by the end of 3 to 6 month. Some have satisfactory intercourse, but in many, vaginal size is inadequate and they need a surgical procedure eventually.

Surgical management : Vaginoplasty either by Mc Indoe operation

Differential Diagnosis :

• Testicular feminization syndrome.
• Mayer Rokitansky-Kuster Hauser syndrome can be differentiated from testicular feminizing syndrome on the basis of :

None

White epithelium

Ans. is b i.e. Progestogen    

Metropathia hemorrhagica is a specialised form of DUB. Mostly seen in premenopausal women.

Maximum age incidence : between ages 40 – 45 years.

Pathology :

Changes in the Uterus : Symmetrical enlargement of the uterus to a size of 8 – 10 weeks due to hypertrophy of muscles.

Changes in Endometrium : Naked eye appearance : Endometrium looks thick, congested and often polypoidal.

Microscopic appearance :

• Hyperplasia of all endometrial components.
• Intense cystic glandular hypertrophy.
• Some glands are small and some large giving appearance of “swiss cheese°.
• Glands are empty and lined by columnar epithelium.
• Secretory changes are absent.°
• Follicular cysts containing estrogen present on ovaries.

Sign and Symptom : Patient complains of excessive painless bleeding (Anovular bleeding).

Treatment :

• In DUB due to anovular causes : Progesterones are the mainstay of therapy.
• They diminish the effects of estrogen on target cells by inhibiting oestrogen receptors.

To stop Acute severe bleeding :

• Norethisterone (5 mg tab.) is given thrice daily till bleeding stops. Then norethisterone is stopped resulting in withdrawl bleeding (after 2-4 day). This is called as Medical curettage.
• Thereafter cyclical progesterone – MPA (Medroxy Progesterone Acetate) 10 mg or Norethisterone 5 mg is given from day 5 – day 25 of the cycle for 3 cycles.

Fallopian tube

External Os

If there is a disorder in fusion of downgrowing Mullerian duct and upgrowing derivative of urogenital sinus. It results in Transverse vaginal septum which causes imperforate vagina (or vaginal agenesis).

In a series reported :

–       46% septa were located in upper part.° Ott ti2v-^a        

–       40% septa were located in middle part.°

–       14% septa were located in lower part.°

The upper part corresponds to external os therefore it is the option of choice.

Transverse vaginal septum can present either  in :

A. Neonatal age group ?

• The placental transfer of estro^gen results in stimulatin the •lands of the endocervix which results in formation of Mucocolpos it can present as :

1. abdominal tumour.°
2. can compress the ureter resulting in hydroureter followed by hydronephrosis.°
3. can compress the rectum resultina in obstioation / intestinal obstruction.°

B. At puberty ?

• Patient can present with Primary amenorrhea (actually called as Cryptomenorrhea^a as uterus menstruates normally but blood does not come out due to outflow Tract obstruction).°
• Secondary sexual characteristics are normal.°
• Due to Cryptomenorrhoea, blood gradually collects and distends first the vagina (hematocolpos)° then cervix uterus (hematocervix and hematometra) and finally the tube (hematosaipinx)°. All these present as pelvic/ abdominal tumour.
• The abdominal tumour can irritate the bladder followed by compression of internal urinary meatus leading to

complete retention of urine (This occurs 3 – 4 years after the onset of hidden menstruation and therefore patient is generally aged 15 – 18 years°).

• Patient may complain of monthly cyclic pain (backache I lower abdomen pain).°

Management ?

• Once the diagnosis of cryptomenorrhea is made, surgical treatment is urgently required° since every menstrual episode further dilates the genital tract and threatens permanent impairment of reproductive function.
• Obstruction at the level of the cervix is managed by uterovaginoplasty. If uterovaginoplasty is unsuccessful hysterectomy is performed.
• When the outflow menstrual blood is prevented by a thick vaginal membrane at any level, incision followed

by covering the deficiency with either vaginal epithelium or skin grafting is done.

• When the obstructing membrane is thin and low down, the membrane is merely excised.°

Ans:D.)Ovarian agenesis

Mullerian agenesis

• It is Mayer Rokitansky-Kuster Hauser syndrome. .
• Müllerian agenesis is a congenital malformation characterized by a failure of the Müllerian duct to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion. Müllerian agenesis (including absence of the uterus, cervix and/or vagina) is the cause in 15% of cases of primary amenorrhoea.
• Because ovaries do not develop from the Müllerian ducts, affected women might have normal secondary sexual characteristics but are infertile due to the lack of a functional uterus.

Signs and Symptoms:

• An individual with this condition is hormonally normal; that is, the person will enter puberty with development of secondary sexual characteristics including thelarche and adrenarche (pubic hair). The person’s chromosome constellation will be 46,XX. At least one ovary is intact, if not both, and ovulation usually occurs. Typically, the vagina is shortened and intercourse may, in some cases, be difficult and painful. Medical examination supported by gynecologic ultrasonography demonstrates a complete or partial absence of the cervix, uterus, and vagina.

Classification:

• Typical MRKH – Isolated uterovaginal aplasia/hypoplasia
  • Prevalence – 64 percent
• Atypical MRKH – Uterovaginal aplasia/hypoplasia with renal malformation or uterovaginal aplasia/hypoplasia with ovarian dysfunction
  • Prevalence – 24 percent
• MURCS syndrome – Uterovaginal aplasia/hypoplasia with renal malformation, skeletal malformation, and cardiac malformation
  • Prevalence – 12 percent

Gonadoblastoma – Reinke’s crystals

↓ FSH

a and c both

FSH

Oestradiol                          Progesterone                         Inhibin

(inhibin      FSH production)

(Leads to menstruation in menstrual

cycle) & will release the negative                         Increased FSH as

feedback on GnRH, therefore                               compared to LH increases GnRH pulses

L

Another follicular phase begins