Congenital Gynaecological defromities.

1. CONGENITAL DISEASES
tUe tkr fodkj
DR. RAMVEER SHARMA
M.D. Ayu. (Gynae)
I.P.G.T.&R. Jamnagar
MMM Govt. Ayu. College UDAIPUR
Mob. 9414757932
Email. ramveersharma1960@gmail.com
CONGENITAL - DISEASES

2. CONGENITAL - DISEASES
Ex. Genitalia
The Vulva
 Hypo development (Congenital) of the vulva is rarely
possible, in hormonal disorders it may be.
a) Labial fusion resembling scrotum may be possible in
intersexual condition.
b) External genitalia with ambiguity of sex are genetic origin.
DR. RAMVEER SHARMA

3. CONGENITAL - DISEASES
Anus Deformity
Opening
a. In the perineal end of vestibule
b. In the vestibule
c. In the vagina (rarely)
 The opening is usually sufficient, big, continence is present.
 Result - No problem in future reproduction,
 Delivery should be – caesarean section.
Treatment: - Operation – Colostomy.
DR. RAMVEER SHARMA

4. CONGENITAL - DISEASES
THE VAGINA
1) Absence of the Vagina:-
a. Incomplete: - Commonly at lower third part due to Atresia.
 Uterus -Normally developed with menstruate.
 Uterus may be absent, ovaries are normal.
 Vagina may be open in the urethra.
b. Complete: - Absence of entire vagina,
 lower vagina may be present and developed from Urogenital
sinus.
DR. RAMVEER SHARMA

5. CONGENITAL - DISEASES
Rokitansky- Kusters Hauser Syndrome:-
i. Vagina is absent.
ii. Uterus rudimentary
iii.Ovary and tubes are normal
Features:-
 Primary amenorrhoea.
 There is no bulging of hymeneal membrane.
 Difficult intercourse (detected after marriage in upper
vaginal Atresia),
 Primary sterility.
DR. RAMVEER SHARMA

6. CONGENITAL - DISEASES
Rokitansky- Kusters Hauser Syndrome:-
Diagnosis –
Examination: - P/R. – Absence of uterus, or
Presence of uterus with Haematocolpos.
Treatment: - Plastic operation.( McClendon’s or Williams
operation).
DR. RAMVEER SHARMA

7. CONGENITAL - DISEASES
Rokitansky- Kusters Hauser Syndrome
DR. RAMVEER SHARMA

8. CONGENITAL - DISEASES
Agenesis of the Vulva & Vagina
DR. RAMVEER SHARMA

9. CONGENITAL - DISEASES
VAGINOPLASTY
DR. RAMVEER SHARMA

10. CONGENITAL - DISEASES
VAGINOPLASTY
DR. RAMVEER SHARMA

11. CONGENITAL - DISEASES
2) Narrow Introitus
 The problem can be detected after marriage, or during
investigation of infertility.
Clinical features: -Dyspareunia (Unable, Difficult intercourse)
- Difficult vaginal examination.
Treatment:-
a. Manual stretching (Dilatation) under G.A.
b. Dilatation with lubricated metal dilators / G.A.
c. Surgery- surgical enlargement.
 Perineoplasty - Fanto’s operation.
DR. RAMVEER SHARMA

12. CONGENITAL - DISEASES
3) Hymen- Imperforate
Pathology: - The Hymen is imperforate.
Rest of vaginal tract may be normal.
The symptoms are produce after menarche.
Clinical Features:-
a. Features are appears after 14 -16 years of the age.
b. Normal endocrinal study (Secondary characters)
c. Primary amenorrhoea
DR. RAMVEER SHARMA

13. CONGENITAL - DISEASES
3) Hymen- Imperforate
d. May be collection of vaginal and cervical secretion
(hydrocolpus).
e. Laterally it may be converting in Haematocolpos.
f. Pain lower abdomen-Monthly cyclic and continuous.
g. Urine – frequent, may be Dysuria and retention depends on
pressure of distended uterus.
h. Stool- may be constipation and painful defecation
(Dyschezia).
DR. RAMVEER SHARMA

14. CONGENITAL - DISEASES
Examination: - After catheterization
Par abdomen (P.A.) - Supra pubic swelling may be present.
Per vagina (P/V) :- Vulva Tense,
 Bulging membrane of bluish colouration (depends on
Haematocolpos)
Per rectum(P/R-) – reveals the bulged vagina.
DR. RAMVEER SHARMA

15. CONGENITAL - DISEASES
Treatment:- Hymenectomy > Circular incision.
a) Quadrants of the hymen are partially excised
b) No pressure on the lower abdomen.
c) No internal examination done.
d) Patient lies in bed with head raised position.
e) Proper antibiotic should be given
f) Check-up after next period.
DR. RAMVEER SHARMA

16. CONGENITAL - DISEASES
(4) Vaginal Septum
a) Transverse:-
 The septum can be developing at any site of the vagina.
 Commonly at the junction of the -
• Middle and upper third of vagina,
• Or at the Middle and Lower third of vagina.
 The septum can be complete or with an aperture allowing
menstrual blood to drain.
DR. RAMVEER SHARMA

17. CONGENITAL - DISEASES
(4) Vaginal Septum
Symptoms: -
• Complete - Coital problems and like that imperforated
hymen.
• Incomplete - Coital problems and difficulty during labour.
DR. RAMVEER SHARMA

18. CONGENITAL - DISEASES
(2) Horizontal:-
 There are two separate vaginal canals with a septum in
between.
 One canal is often narrow than the other.
 There may be two cervixes.
Symptoms:-
• The condition may be asymptomatic.
• Rarely produce coital difficulty and obstruction during labour.
Treatment:- Surgical excision of the septum.
DR. RAMVEER SHARMA

19. CONGENITAL - DISEASES
(5)Uterine And Vaginal Malformations
Etiology :- Imperfect fusion of Mullerian ducts.
Types – U – Uterus, C – Cervix, V - Vagina
DR. RAMVEER SHARMA
Type Contents Description
Uterus Didelphis 2U + 2C + 2V Separated by broad mass of tissue.
Uterus Bicornis Bicolis 2U + 2C + 2V Separated wholly or partially by
medium septum.
Uterus Bicornis
Unicolis
2U + 1C + 1V Separated by medium septum
Septate uterus 1U + 1C + 1V Medium septum at cavity

20. CONGENITAL - DISEASES
(5)Uterine And Vaginal Malformations
Types – U – Uterus, C – Cervix, V - Vagina
DR. RAMVEER SHARMA
Type Contents Description
Subseptate uterus 1U + 1C + 1V Partially Septate uterine cavity.
Uterus Unicornis One rudimentary horn from one
Mullerian duct.

21. CONGENITAL - DISEASES
Mullerian Anomalies
 Uterus Didelphis
 Uterus Bicornis Bicolis
 Uterus Bicornis Unicolis
 Septate uterus
 Sub septate uterus
 Uterus Unicornis
DR. RAMVEER SHARMA

22. CONGENITAL - DISEASES
 Aplasia and Hypoplasia of Mullerian duct:-
a. Aplasia - Uterus can be completely absence.
b. Hypoplasia of the uterus –
• Rudimentary Uterus,
• Infantile Uterus,
• Hypo plastic Uterus,
• Atresia of cervix,
• Congenital elongation of the cervix.
DR. RAMVEER SHARMA

23. CONGENITAL - DISEASES
Clinical Features:-
 Primary Amenorrhoea
 May be haematomatra,
 Dysmenorrhoea, Menorrhoea.
 Infertility,
 Mid pregnancy habitual abortion,
 Pre mature delivery or transverse lie.
 May not be any symptom at all.
DR. RAMVEER SHARMA

24. CONGENITAL - DISEASES
Diagnosis:-
Clinically: - Through P/V examination under G.A.
Vagina – May be horizontally Septate .
Cervix - Double,
Uterus - Double.
Uterus - may be –
 Absent,
 Hypoplastic – Small in size.
 Rudimentary – One side may be undeveloped.
 Infantile – Altered cervical uterine ratio.
DR. RAMVEER SHARMA

25. CONGENITAL - DISEASES
Hypoplastic Uterus: -
 Small in size, with subnormal menstrual cycle.
 Non stimulated endometrium with Oestrogene hormone.
 HSG and uterine sound shows Cavity size is less than 6mm.
Infantile uterus: -
 Small size for age and altered body and cervix ratio.
DR. RAMVEER SHARMA

26. CONGENITAL - DISEASES
Investigations:-
 USG uterine :- can detected double Cervix, Cavity, Corn
and Size And Shape.
 I / V USG,
 Hysterography,
 Hysteroscopy,
 Laparoscopy,
 MRI, are useful measures for diagnosis.
DR. RAMVEER SHARMA

27. CONGENITAL - DISEASES
Uterine Deformity
DR. RAMVEER SHARMA

28. CONGENITAL - DISEASES
U.S.G
DR. RAMVEER SHARMA

29. CONGENITAL - DISEASES
U.S.G.UTERUS DIDELPHUS
DR. RAMVEER SHARMA

30. CONGENITAL - DISEASES
H.S.G. Uterus Didelphis
DR. RAMVEER SHARMA

31. CONGENITAL - DISEASES
H.S.G.
DR. RAMVEER SHARMA

32. CONGENITAL - DISEASES
H.S.G. UTERUS DIDELPHUS UNICOLIS
DR. RAMVEER SHARMA

33. CONGENITAL - DISEASES
UTERUS UNICORNIS
DR. RAMVEER SHARMA

34. CONGENITAL - DISEASES
Treatment
a) Absent, Rudimentary, and Infantile uterus – No meanable to
treatment.
b) Hypo-plastic:-
 In young girls- may develop gradually with age.
 Amenorrhoea, Oligomenorrhoea - D&C, and Oestrogene
therapy may be helpful.
 Chance of pregnancy may be possible.
DR. RAMVEER SHARMA

35. CONGENITAL - DISEASES
Surgery
•Indications - Double uterus,
 Haematomatra,
 Pyometra,
 Rudimentary
 Uterine Horn.
a) Excision :-
 Non communicating uterine horn - with Pyometra, or
Haematomatra.
DR. RAMVEER SHARMA

36. CONGENITAL - DISEASES
Surgery
b) Unification of both cavity after excision of tissue, between
two cavity.
 Bicornuate uterus with habitual abortion and infertility.
DR. RAMVEER SHARMA

37. CONGENITAL - DISEASES
C) Hysteroscopic Metroplasty:-
 Resection of the septum can be done either by resectoscope
or by laser.
Advantage –
High success rate (80-89%)
 Short hospital stay
 Less post operative morbidity (infection, adhesions)
 Subsequent chance of vaginal delivery is high.
DR. RAMVEER SHARMA

38. CONGENITAL - DISEASES
Causes (4)
lkekU; gsrq p-fp- 30
feF;kpkjs.k iznq’VsukrZosu cht nks’kkPp nSokPp
vkgkj fogkj Maternal Paternal Idiopathic
(Dietetic) (Behavioural)
Chromosomal Genetic Chromosomal Genetic
feF;kpkjs.k L=h.kka iznq’VsukrZosu pA tk;Urs cht nks’kkPp nSokPp Jq.kq rk i`Fkd~Aa
DR. RAMVEER SHARMA

39. CONGENITAL - DISEASES
 chtkRedekZ”k; dky nks’kS% ekrqLrFkkgkjfogkjnks’kS%A
 dqoZfUr nks’kk% fofo/kkfunq’Vk% laLFkkuo.ksZfUnz; oSd`rkfuAA p-”kk-2@29AA
 Aetiology Congenital defects of
Doshas (Defects ) Vikriti
of of
 Beeja - Chromosomal or Genetic a) Samsthan – Systematic
Aatma – Idiopathic b)Varna – Colour and lustre
 Karma c) Indriya – Organ or Sense.
 Ashaya Environmental
 Kala
 Matu: Ahara Vihara
DR. RAMVEER SHARMA

40. CONGENITAL - DISEASES
AETIOPATHOGENESIS (Ch.Sh.4/30)
;nkfL=;k% lsoekuk;k When an woman use
nks’k izdksidkU;qDrk∙U;rek Dosha aggravating diet and environment
nks’kk% izdqfirk Dosha become aggravated
“kjhjeqiliZ;Ur% Aggravated Doshas circulate all over body
“kksf.krxHkkZ”k;eqii|Uras Comes in contact with Ovum and Uterus
u p dkRL;Zsu “kksf.kr xHkkZ”k;kS nw’k;fUr Defects the both ovum and uterus
rfn;a xHkZa yHkrs L=h During this time if women get fertilized
DR. RAMVEER SHARMA

41. CONGENITAL - DISEASES
AETIOPATHOGENESIS (Ch.Sh.4/30)
rnk p rL; xHkZL;
ekr`tkukeo;okukeU;reks∙o;oks Maternal or other Organs
fod`freqRik|rs ,dks∙usdks Defect the one or many organs.
DR. RAMVEER SHARMA

42. CONGENITAL - DISEASES
• ;L;;L;kº;o;oL; cht chtHkkxs ok nks’kk% iznks’keki|rs r¥~preo;oe~
fod`fr% vkfo”kfrA
1½ ;nkº;L;% “kksf.krs xHkkZ”k; chtHkkx% iznks’keki|rs rnk cU/;ka tu;frA
 cU/;ka u’VkrZoka fo|kr~A cU/;ka L;knuqidze%AA
2½ ;nkiqujL;% “kksf.krs xHkkZ”k; chtHkkxko;o iznks’keki|rs rnk iwfriztka tu;frA
DR. RAMVEER SHARMA

43. CONGENITAL - DISEASES
3½ ;nkRoL;% “kksf.kr xHkkZ”k; chtHkkxko;o L=hdjk.kka p “kjhj chtHkkxkukesd
ns”k% iznks’keki|Urs rnk L=h vkd`fr Hkwf;’Ba vfL=;ka okrkZa uke tu;fr ra L=h
O;kinkekp{krsA p-”kk- 4@30AA
v½ xHkkZ”k; chtHkkx% (Chromosomal defect ?) cU/;ka
c½ xHkkZ”k; chtHkkxko;o (Genetic defect ?) iwfriztka
l½ xHkkZ”k; chtHkkxko;o chtHkkxkukesd ns”k% (Genetic one part defect ?)
vfL=;ka okrkZa ukeA
DR. RAMVEER SHARMA

44. CONGENITAL - DISEASES
CONGENITAL
‘k.< ¼p-”kk-2@17 &21½ Type – 8
DR. RAMVEER SHARMA
Disease Cause Pathogenesis Feature
1- f}jsrk
Have a both
Gonads
cht% leka”kk
Equal -
Shukra +Artawa
nqrIrchtkr L=hiqalfyaxh Hkofr
XXY.
2- iousfUnz;
Gonads Agenesis
djksfr ok;q% “kqdzk”k;a xHkZxrL; gRok
Agenesis during
uterine life
iousfUnz;Roe~A
Azoospermia ?
Anovulation

45. CONGENITAL - DISEASES
CONGENITAL
‘k.< ¼p-”kk-2@17 &21½ Type – 8
DR. RAMVEER SHARMA
Disease Cause Pathogenesis Feature
3- laLdkjokgh
Treatable
Deformities
dq#rs∙fuy”p “kqdzk”k; }kjfo?kêusu
Defected
Spermatic tract or
Genital Tract
laLdkjokga
Can be treated
4--5 DyhckS uj ‘k.<
ukjh ‘k.<
Pseudo
Hermaphroditism
eUnkYichtkr~
Oligospermia or
Spermatic defects
DyhckS p gsrqfoZd`fr};L;
Absence of gonads
in Male and Female
vcykog’kkZ
Oligospermia,
Lac of erection

46. CONGENITAL - DISEASES
‘k.< ¼p-”kk-2@17 &21½
DR. RAMVEER SHARMA
Disease Cause Pathogenesis Feature
6- odzh
Shishna Vakrata
Hypospadiasis
Vaginal deformities
ekrqO;Zok;izfr?ksu L;k}htnkScZY; rFkk
firq”pA
7- iousfUnz; bZ’;kZfHkHkwrk
ofieUng’kkZoh’;kZj
rsjso
Absence of
Ejaculation
8- Okkfrd ‘k.< ok¸ofXunks’kkn o`’k.kkS rq ;L; uk”ka
xrkS
Have no testes
Agenesis or
Cryptoorchadism

47. CONGENITAL - DISEASES
(1)Suchi Mukhi Yoni :-
 Congenital vaginal Atresia or pin hole uterus.
xHkkZ”k;LFkk% fL=;k% jkS{;k}k;q% ;ksfua iznw’k;uA
ekr`nks’kkn.kq }kjk dq;kZRlwpheq[kh rq lkAA lwphcD=kfrlao`rka AA
Time – During intrauterine time.
Cause – Aggravated Vayu by its Ruksha Guna due to indulging
unwholesome diet by Mother.
Symptoms – Narrow introitus like pin hole.
Chikitsa – lao`rkao)Z;sRiqu%AA Lusgu] Losnu] foo)ZuA
DR. RAMVEER SHARMA

48. CONGENITAL - DISEASES
(2) Shandhi Yoni :-
Time –During intrauterine life xHkZLFk
Cause – Ovum (Chromosomal or Genetic) defects chtnks"kkÙkq and
vitiated Vayu ek#rksigr
Pathogenesis – Defects the genital organs xHkZLFkek:rksigrk”k;kA
• chtnks"kkÙkq xHkZLFkek:rksigrk”k;kAA 34AA
DR. RAMVEER SHARMA

49. CONGENITAL - DISEASES
(2) Shandhi Yoni :-
Features –
 Agenesis of Uterus (Or genital tract) - ek:rksigrxHkkZ”k;k
 Aversion to males or sexual act, - u`}sf"k.;] pdkjkUeSFkqu}s"kfu"ks/k%A-
 Breastless or ill developed breast, and - vLruh] bZ"kRLRkuhA
 Rough in touch and during coitus. [kjLi”kkZ p eSFkqus
 It may have other features like – Roughness, Painful, feeling
hot and itching etc.
 u`}sf"k.;Lruh pSo "k.<h L;knuqiØekAA 35AA¼p-fp-30½ vukrZokLruh "k.<h
[kjLi”kkZ p eSFkqusAA 18AA ¼lq-m-38½ DR. RAMVEER SHARMA

50. CONGENITAL - DISEASES
 chtnks"kkfnfr lkekU;opusukfi vkrZo:ichtnks"k ,o Ks;k%]
 rÌks"kL;So xHkkZ”k;ksi/kkrdjRokr~AAek:rksigrk”k;sfr ek:rksigrxHkkZ”k;k A
 vLruh bZ"kRLRkuhA ¼p-fp-30@34]35 dh pØ-½
DR. RAMVEER SHARMA

51. CONGENITAL - DISEASES
¼2½ -------pdkjkUeSFkqu}s"kfu"ks/k%A--------A loZfyaxksfPNªfrfjfr jkS{;rksnkfndk
okrfyaxksfPNªr;%] pks"kkfndk% fiÙkkfyaxksfPNªr;% Lusgd.M~okfndk%
'ys"efyaxksfPNªr;”p AA ¼lq-m-38@18]20 dh MYg.k] ¼ek-fu-62@11]13( Hkk-iz-
fp-70@14]16 ,oa ;ks-j-;ksfujksx~*½
 Chromosomal or developmental defects –
 Male hermaphroditism.
 Klinefelter Syndrome XXY. Mayer - Rokitansky- Kusters
Hauser (MRKH) Syndrome.
 Treatment – Non treatable. ‘k.<h L;knuqiØekAA 35AA
DR. RAMVEER SHARMA

52. CONGENITAL - DISEASES
(3) Bandhya Yoni
Time –During intrauterine life - xHkkZ”k;LFks
Cause – Ovum (Chromosomal or Genetic) defects.
 ;nk áL;k% 'kksf.krs xHkkZ”k;chtHkkx% iznks"keki|rs]
Pathogenesis – Defects the ovary or genital organs.
 xHkkZ”k;LFks fuoZrZda chta iznq";fr rnk oU/;ka tu;frA ¼v-la-'kk-2@48½
Features – Amenorrhoea - oU/;ka u"VkrZoka fo|kr
 Absent of Uterus and Menses. xHkkZ”k;L; rFkk··rZoL; pksi?kkrsuA
 May or not developed breast. oU/;k"k.<~;ks% lLruRofuLruRos
DR. RAMVEER SHARMA

53. CONGENITAL - DISEASES
(3) Bandhya Yoni
Time –During intrauterine life - xHkkZ”k;LFks
Cause – Ovum (Chromosomal or Genetic) defects –
;nk áL;k% 'kksf.krs xHkkZ”k;chtHkkx% iznks"keki|rs]
Pathogenesis – Defects the ovary or genital organs
xHkkZ”k;LFks fuoZrZda chta iznq";fr rnk oU/;ka tu;frA¼v-la-'kk-2@48½
Features – Amenorrhoea - oU/;ka u"VkrZoka fo|kr
xHkkZ”k;L; rFkk··rZoL; pksi?kkrsu
oU/;k"k.M~;ks% lLruRofuLruRos fo”ks"kr%AA¼lq-m-38@11 MYg.k½
;nk áL;k% 'kksf.krs xHkkZ”k;chtHkkx% iznks"keki|rs] rnk oU/;ka tu;frAA¼p-'kk-
4@30½ ¼vk½ ;nk áL;k% 'kksf.krk[;s chts xHkkZ”k;LFks fuoZrZda chta iznq";fr
rnk oU/;ka tu;frA¼v-la-'kk-2@48½ DR. RAMVEER SHARMA

54. CONGENITAL - DISEASES
 xHkkZ”k;tudks chtHkkxks xHkkZ”k;chtHkkx%] fdaok xHkkZ”kL; rFkk chtL;
pkrZo:iL; tudks Hkkx%A----------A xHkkZ”k;L; rFkk··rZoL; pksi?kkrsu fL=;k
oU/;Roa O;äesoA ¼p-'kk-4@30 pØ- Vhdk½
 oU/;ka u"VkrZoka fo|kr~--------A prl`"ofi pk|klq HkoUR;fuyosnuk%AA ¼lq-m-
38@10]11½
 oU/;k"k.<~;ks% lLruRofuLruRos fo”ks"kr%AA¼lq-m-38@11 MYg.k½
DR. RAMVEER SHARMA

55. CONGENITAL - DISEASES
Yoni Roga by
Acharya Kashyapa:- Su.28 / 6.
Anatomical defects :-
• “kdVkd`fr ;ksfu – Like a cart Shape of vulva.
• mfR{kIrk ;ksfu – Prolapsed or Protruded (Adrenalism or Elongated clitoris)
• foo`rk ;ksfu – Dilatated introitus of Prolapses.
• lao`rk ;ksfu - Constricted or Narrow introitus.
• “kq’dk ;ksfu – Dry vagina
• yEch ;ksfu – Longitudinally arranged Vulva.
• fo’ke ;ksfu – Irregular Genital tract.
• fofy³~xk ;ksfu – Defected external Genitals or lac of secondary sex characters.
DR. RAMVEER SHARMA

56. CONGENITAL - DISEASES
(4)Shushka Revati –
• Amenorrhoea even up to sixteen year.
• Ill developed upper and lower arms and breasts.
 vk’kksM”ko’kkZ;kizkIrk;kL=hiq’ie~uki”;fr k izEykuckgw:dqprekgq%”kq’djsorh kk
(5) Katambhara –
 Amenorrhoea till death.
 Emaciate, Weak strength, Irritated in behavior.
 foukiq’ierq;kukjh ;Fkkdkye~izu”;fr k d`”kk%ghucykdzq)klkfipksDrk%dVEHkjk kk
DR. RAMVEER SHARMA

57. BACKACHE
THANKS
DR.RAMVEER SHARMA