Developmental of the female genital tract

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broad ligament is the sheeth of periotnuem that the developing paramesonephric duct has ppulled along
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2 cervix 2 utereus ⇒ didelphys
1 cervix 2 uterus ⇒ bicornuate
unicorunate uterus
septate uterus (fundus is intact vs bicorunuate where the fundus is dipped)
mullerian agenesis
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Mullerian abnormalaties

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Diethylstilbestrol Exposure
 
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normal gonads and normal karyotupe and normal breast....vs gonadal dysgeneisis and ASI
 
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can be associated with two vaginas as well if the latter
 
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arcuate ueterus is usually just a radiological anomaly. a dip in the top.
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if the rudimenary horn has endometrium and
if rudimentary horn is not connected then blood can be collected and dusmenohrreha
So this is the anomaly where you can get unilateral dysmenorrhea
but if connected then can even have fertilisation and a cornual pregnancy
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mullerian agensis is a rare anomaly
 
DES was used earlier to prevent aborition and strenghten the pregnancy but wwas terotegenic and abnormalaties seen later
no now threatned miscarriage have to just wait and watch
 
 
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Abortion vs infertiity?
Septate causes most commonly cause abotrion in the second semester but its one abnormalality thsat can cause abortion in t1
causes recurrent abortion because the spetum is less vascular and fribrous and cannot support a placenta
 
spetate is most problematic one reason its rare
arcuate cause duhhh but didelphs just 2 uetreus and 2 cervix and can go undetected for years
 
 
 
 
3d usg is cheaper tho. but MRI is the treatment of choice. Mri > HSG
 
 
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fundus dipped means bicornuate uterus
 
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those 3 are types of metroplasties
 
which i a unification operation
 
that can be done for bicornuate and aslo for septum that fails the spetate lysis answer.
 
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the lap and hyesterocopy can be both diagnostic and therpauetic. can confirm thats its septum and not bcornuate and treat in the same operation without having another
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choice depends on the full clinical profile ...in this case because priamry inferitity then hys + lap...if recurrent then better to do mri and 3d us
 

Development of Sex & XY Females

 
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upregulation of other SOMATIC genes
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testis development needs Y genes and upregulation of other genes while ovariain evelopment needs absemce of y genes and upregulation of other genes. if nothing then dysgentic.
external genitlai by default is a female but in testerone exposure it gets male. That is the internal gentialia is dependant on testerone to get male but external genitalia needs 5 dht. So requires 5alpha reductase as well.
 

Swyers syndrome

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streaky gonads as swyers
 
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no testesterone ⇒ so no pestrogen whatssoever since not aromatised!
 
 
 

AIS

 
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upper vagina is absent so blind vaginal pouch
gonadectomy after puberty instead of immedietly since contributing to the normal female development (vs swey done immetdiately)
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karyotyping to confirm finally because AIS is a XY disorder while mullerian isnt.
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Complete AIS vs partial AIS
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So mullerian vs ais vs sweys vs other dds for hirtusim and virlism and sex disorders
 
 

Mullerian agenesis vs AIS

 
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both of these conditions have no ueterus
 
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Above shows the importance of the definition of delayed puberty. its not pathological yet!
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Definitoon confrimation comes by karyotyping

Delayed Puberty

 
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no use measuring gnrh because its a pulsatile secretion
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gonadectomy should be done with Y chromosome because risk of gonadal cancer but no need for xx
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short height because x chromosome has the shok gene which is required for good ehigh and with one x its haplo insuffiency
 
turners with xy mosaic gonadectomy needs to be done
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they can have menses if taken on hrt ...with ueterus if have hrt then can give menses
 
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in consittuional will have short height ...but before going there must exclude kallmans syndrome.
cant measure gnrh because its pulsatile secretions!
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Disorders of sex developement - Deepal

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