Congenital Malformations of Female Genital Organs: DR Sonia Aluvilayil Assistant Professor (Obgy)

Congenital
Malformations
of
Female Genital
Organs
DR SONIA ALUVILAYIL
ASSISTANT PROFESSOR(OBGY)
Developmental
Anomalies
of the
External
Genitalia
PERINEAL OR VESTIBULAR ANUS
 • Detected at the time of birth

 • Usual anal opening site is evidenced by anal pit
 • Either anus will be situated close to the posterior end
 of the vestibule or in the vestibule
ECTOPIC URETER
Vaginal abnormalities
 NARROW INTROITUS
 HYMEN ABNORMALITIES
Imperforate hymen of significance
abnormality.
• Always unnoticed until 14 – 16 yrs.
• Uterine functioning is normal menstrual
blow is pent up inside vagina behind the hymen
(CRYPTOMENORRHOEA)
((HAEMATOCOLPOS)
• The uterus is next involved and cavity dilated
(HAEMATOMETRA)
• If late and neglected, tubes may also be
distended after the fimbrial ends becoming
closed by adhesions (HAEMATOSALPHINX
Clinical features
 Age – 14 -16
 • Periodic lower abdominal pain which may be
 continuous.
 • Primary amenorrhoea
 • Urinary symptoms
Treatment
 Cruciate incision is made in the hymen.

 • Quadrants of the hymen are partially excised not too
 close to the vaginal mucosa
 • Spontaneous escape of dark tarry coloured blood is
 allowed.
 • Patient should be made to lie down with the head end
 raised.
TRANSVERSE SEPTUM ABOVE THE
LOWER ONE – THIRD
SEPTATE VAGINA
 • Complete or incomplete longitudinal septum

 • May be assoc with double uterus and double cervix
 • May be asymptomatic or may produce dyspareunia o
 may obstruct delivery
 • Treatment: septum to be excised
PARTIAL ATRESIA OF THE VAGINA
 A segment of the vagina may be atretic in the upper

 third. May be assoc with cervical atresia
 • Functioning uterus (usually)
 • Primary amenorrhoea, periodic pain in lower
 abdomen with a mass felt per abdomen or rectal
 examination point to the diagnosis.
 • Excision of the atretic portion from below and
 suturing the upper and lower margins of the vaginal
 walls are usually effective
COMPLETE ATRESIA
 Assoc with absence of uterus

 • Healthy gonads and fallopian tubes
 • Pt is phenotypically female with normal karyotype
 pattern
 • Entity is often assoc with urinary tract abnormality
 called Mayer Rokitansky Kuster Hauser syndrome
Uterine
Anomalies
MULLERIAN DUCTS
 In majority, the presence of deformity escapes

 attention.
 • Detection is made accidently during the investigation
 of infertility or repeated pregnancy wastage.
 • In other cases, diagnosis is made during D + E
 operation , manual removal of placenta or during
 Caesarean section.
TYPES:
Arcuate
• Fundus looks concave with heart
shaped cavity outline
• This looks more like a normal uterus,
except it has a dip, or
slight indentation at the top.
• It is a common abnormality, affecting
about one in 25 women
in the general population.
• It doesn't usually make conceiving
difficult
Uterus didelphys
 • Complete lack of fusion of the Mullerian ducts with a double

 uterus and a double vagina.
 • This is when the uterus has two inner cavities.
 • Each cavity may lead to its own cervix and vagina, so there
 are two cervixes and two vaginas.
 • It is uncommon, affecting about one in 350 women.
 • It is possible to conceive if you have this type of abnormality,
 and have a straight forward pregnancy
Septate uterus
 • 2 mullerian ducts fused together but there is persistence of

 septum in between the two either partially or completely
Clinical Features
 May not produce any clinical manifestation.

 • Gynaecological:
 • Infertility and dyspareunia often related – vaginal septum
 • Menorrhagia – due to increased surface area in bicornuate
 uterus
 Obstetrical
 • Midtrimester abortion – may be recurrent
 • Increased incidence of malpresentation – transverse lie in
 arcuate or septate, breech in bicornuate, unicornuate or
 complete or septate uterus
 • Preterm labour
Prolonged labour – due to incordinate uterine action
• Obstructed labour
• Retained placenta and postpartum haemorrhage
where the
placenta is implanted over the uterine septum
Treatment
 Rudimentary horn can be excised to prevent

any
 obstetrical complications
 • Uterine septum can be effectively resected
 hysteroscopically either by scissors or laser
THANK YOU

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Congenital Malformations of Female Genital Organs: DR Sonia Aluvilayil Assistant Professor (Obgy)

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Congenital

 • Detected at the time of birth

 Cruciate incision is made in the hymen.

 • Complete or incomplete longitudinal septum

 A segment of the vagina may be atretic in the upper

 Assoc with absence of uterus

 In majority, the presence of deformity escapes

 • Complete lack of fusion of the Mullerian ducts with a double

 • 2 mullerian ducts fused together but there is persistence of

 May not produce any clinical manifestation.

 Rudimentary horn can be excised to prevent

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