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Malformations of the genital tract (Pathophysiology (Failure to form …
Malformations of the
genital tract
Definition
Abnormal development of
the genital tract
Epidemiology
Rare, 3%
Often co-existing renal/urinary tract
abnormalities (~40%)
Pathophysiology
Failure to form
Complete: Rokitansky syndrome
Partial: unicornuate uterus
Failure of duct fusion
Longitudinal vaginal septae
Bicornuate uterus
Uterus didelphys (double system)
Normal
Mullerian ducts form, fuse in the midline,
and fuse with the urogenital sinus
Failure to fuse with sinus
Transverse vaginal septae
Wolffian remenants
Cysts in lateral vaginal wall or broad ligament
(often no clinical significance)
Clinical
presentation
Transverse vaginal septum
Primary amenorrhoea
Cyclical pain
Abdominal mass
Urinary retention
Endometriosis
Imperforate hymen
Primary amenorrhoea
Cyclical pain
Bluish bulge membrane at introitus
Rokitansky syndrome
Primary amenorrhoea
Normal secondary sexual characteristics
Blind-ending/absent vagina
Uterine anomalies (bicornuate, arcuate, septae)
Often asymptomatic
Primary infertility
Recurrent miscarriage
Preterm labour
Abnormal lie
Diagnosis
Examination
Pelvic
Bluish bulging membrane (imperforate hymen)
Absent/blind end vagina (Rokitansky)
Adnexal tenderness (endo due to transverse septum)
Speculum
Inability to pass (Rokitansky)
Abdominal
Mass, urinary symptoms (transverse septum)
Investigations
Bloods
Karyotyping (exclude androgen insensitivity syndrome)
Imaging
Abdo/pelvic/renal USS: abnormal tract
MRI pelvis: gold standard
Vaginoscopy, cystoscopy, hysteroscopy: EUA
Bedside
History
PGH
Menses, irregular bleeding, problems with coitus,
contraception, STIs/PID, smear history
POH
Gravity/parity, conception, delivery,
gestation, weight, complications
PC/HPC
Absent menarche, heavy/painful bleeds, cyclical pain,
abdominal mass, urinary symptoms, recurrent miscarriage/preterm labour, infertility
PMH
Known conditions, surgery
DH
Current meds, allergies
FH
Congenital anomalies
Late menarche, infertility
SH
Occupation, smoking, alcohol
Management
Obstructive
anomalies
Surgical
Laparoscopic surgery
MOA: surgical correction of obstruction
Conservative
Information, advice, support
Vaginal
septae
Conservative
Information, advice, support
Surgical
Indication: transverse or longitudinal
MOA: resection of septae
Rokitansky
syndrome
Conservative
Information, advice, support
Surgical
Vaginal dilatation
Indication: 1L
MOA: create functioning vagina
Vaginoplasty
Indication: 2L
Imperforate
hymen
Surgical
Indication: all
MOA: cruciate incision in membrane,
allowing menses to pass
Conservative
Information, advice, support