Urogenial sinus and vagial atresias

1. Urogenital sinus and vaginal
atresias
By Dr Sasidhar
Moderator: Dr Rajesh

2. Introduction
• Abnormalities of the female Urogenital tract can present
and be identified at birth through adult hood.
• Most of them are congenital.
• Many of them are now identified even in the fetal life
owing to the advancement in the diagnostic procedures.
• A large number of these patients may also present at
puberty with primary amenorrhea, pelvic/abdominal pain,
and difficulty using a tampon or having sex.

3. embryology
• Functionally the urogenital system can be divided into the urinary
system and the genital system.
• Embryologically and anatomically, however, they are intimately
interwoven.
• Two embryonic structures play an important role in the
development of the urogenital system, intermediate mesoderm and
cloaca.
• Both urinary system and the genital system develop from a
common mesodermal ridge (intermediate mesoderm) along the
posterior wall of the abdominal cavity, and initially enter into a
common cavity, the cloaca.

5. Intra embryonic mesoderm:
•Paraxial mesoderm
•Lateral plate mesodem
•intermediate mesoderm lying between
these two.

6. • Intermediate mesoderm now forms a bulging
on the posterior abdominal wall lateral to the
attachment of the dorsal mesentery–
nephrogenic cord.
• It extends from the cervical to sacral region of
the embryo.

7. Gonad develops from the coelomic epithelium lining the medial side of the
nephrogenic cord.
Excretory tubules (mesonephros) are associated with the development of the
kidney.
The nephric duct is formed in relation to the developing excretory tubules. At
later stages it becomes the mesonephric duct.( 3rd week)
Paramesonephric duct is formed lateral to the nephric duct (6th week)

10. Cloaca:
• The part of the hind gut caudal to the attachment
of the allantoic diverticulum is called the cloaca.
• This cloaca soon subdivides into a broad ventral
part (primitive urogenital sinus) and a narrow
dorsal part (primitive rectum).
• These two parts are separated from each other
by the formation of the urorectal septum.

12. • The urorectal septum grows towards the cloacal
membrane and eventually fuses with it.
• The cloacal membrane is now divided into a ventral
urogenital membrane and dorsal anal membrane.
• The mesoderm around the anal membrane becomes
heaped up with the result that the anal membrane
comes to lie at the bottom of a pit called the anal pit or
proctodeum. The anal pit contributes to the formation
of the anal canal.

13. • In further development the primitive urogenital
sinus is subdivided into a cranial part called the
vesico-urethral part and caudal part called the
definitive urogenital sinus.
• The openings of the mesonephric ducts lie at the
junction of these two.
• Still later the definitive UGS shows a subdivision
into cranial pelvic part and caudal phallic part.

18. Development of the uterus
PARAMESONEPHRIC DUCTS:
• These ducts are present in the intermediate
mesoderm formed by the invagination of the
coelomic epithellium.(early 6th week)
• The paramesonephric ducts develop into the
main genital ducts of the female.

19. • Initially, three parts can be recognized in each duct:
– (a) a cranial vertical portion that opens into the abdominal cavity,
– (b) a horizontal part that crosses the mesonephric duct, and
– (c) a caudal vertical part that fuses with its partner from the opposite
side.
• With descent of the ovary, the first two parts develop into the
uterine tube and the caudal parts fuse to form the uterine canal.
• When the second part of the paramesonephric ducts moves
mediocaudally, the urogenital ridges gradually come to lie in a
transverse plane. ( end of 6th week).

24. • After the ducts fuse in the midline, a broad transverse pelvic fold is
established - the broad ligament of the uterus.
• The uterine tube lies in its upper border, and the ovary lies on its
posterior surface. The original points of invagination of the ducts into the
coelomic epithelium remain as the abdominal openings of the tubes.
Fimbria are formed in this situation.
• The uterus and broad ligaments divide the pelvic cavity into the
uterorectal pouch and the uterovesical pouch. The fused paramesonephric
ducts give rise to the corpus and cervix of the uterus.
• They are surrounded by a layer of mesenchyme that forms the muscular
coat of the uterus, the myometrium, and its peritoneal covering, the
perimetrium.

25. • Anomalies of the uterus:
– Duplication- complete (uterus didelphys) or partial.
– The lumen may be completely or partially subdivided by septum
– Uterine agenesis
– Unicornuate
– Rhudimentary uterus
– Atresia of the lumen either body of the cervix
• Anomalies of the uterine tubes:
– Absent on one or both sides
– Partial or complete duplication
– Atresia.

26. Development of the vagina
• Shortly after the solid tip of the paramesonephric ducts reaches the
urogenital sinus, two solid evaginations grow out from the pelvic part of
the sinus, the sinovaginal bulbs, proliferate and form a solid vaginal plate.
(9th week)
• Proliferation continues at the cranial end of the plate, increasing the
distance between the uterus and the urogenital sinus. (15th to 26th week)
• By the 5th month, the vaginal outgrowth is entirely canalized. The winglike
expansions of the vagina around the end of the uterus, the vaginal
fornices, are of paramesonephric origin.
• Thus, the vagina has a dual origin, with the upper portion derived from
the uterine canal and the lower portion derived from the urogenital sinus

29. Anomalies of the vagina
• Duplication (usually associated with duplication of the uterus).
• Lumen may be subdivided into longitudinally or transversely by a
septum.
• Absent (may or may not be associated with the absence of uterus).
• Imperforate hymen
• Vesicovaginal or rectoveginal fistula (usually associated with ARM).

30. Urogenital sinus abnormalities
• A urogenital sinus abnormality is present when a persistent
communication occurs between the genital and urinary tract in the
female.
• Typically, the urethra and vagina are joined and exit to the perineum as a
common channel.
• If there is associated rectal involvement and imperforate anus with a
single perineal opening for all three structures, a persistent cloaca or
cloacal anomaly exists.
• Patients with urogenital sinus anomalies commonly have associated
abnormalities of the clitoris, labia, and external genitalia.
• The most common etiology of a persistent urogenital sinus is congenital
adrenal hyperplasia (CAH)

31. classification
• relate to the level of the confluence of the urethra and
vagina.
• Hendren divided urogenital anomalies into those with a
confluence above or below the female external sphincter.
• In most females, the sphincter mechanism may be
indistinct and the level of the pelvic floor a more reliable
landmark.
• It is important to recognize that the level of the confluence
should be considered relative to the bladder neck and not
the external meatus.

34. • Virilization of the distal urethra or urogenital sinus may create a
confluence that is distant from the meatus yet still relatively low.
The critical determinant, therefore, is the distance from the bladder
neck to the confluence as it affects reconstructive surgery.
• The nature of the urogenital sinus distal to the confluence should
also be considered. Hendren described the urogenital sinus distal to
the confluence as urethra- like or vagina-like based on
circumference.
• The difference between the two has potential ramifications on
surgical reconstruction in terms of the amount of local tissue
available for reconstruction of the urethra or vagina.

35. evaluation
• Immediate attention to and stabilization of any fluid or
electrolyte imbalance is paramount.
• Initial screening studies in such patients include assessment
of serum electrolytes, biochemical assays screening for
CAH, and evaluation of karyotype using cultured peripheral
leukocytes.
• Multidisciplinary assessment is initiated.
• Gender assignment based on karyotype and potential for
genitourinary function. child should not be named until the
appropriate gender assignment is made..

36. History/physical examination
• maternal and family history –
– Maternal use of alcohol or drugs and exposure to
androgenic substances
– family history of previous neonatal death,
infertility, genital ambiguity, abnormal pubertal
development or inguinal hernias associated with
retained müllerian structures

37. • Physical examination
– general well being, signs of dehydration and lethargy,
– Patients with 11 β-hydroxylase deficiency may be hypertensive
– abdominal examination should note any lower, midline
abdominal masses
– back should be assessed for any findings associated with an
underlying neurologic anomaly
– Gonads are palpated to determine location, number, size, and
associated structures such as an epididymis and vas deferens
– Rectal examination
– Careful inspection of the female introitus is required to identify
an isolated urogenital sinus if no clitoral or labial abnormalities
are present

38. Radiographic evaluation
• Ultrasound-
– The newborn period is the optimal time for evaluation
because maternal hormones make the ovaries and
uterus more prominent.(sensitivity for locating is at
best 60%).
– Hydrocolpos, bladder distention, hydronephrosis,
dysplasia, and agenesis of kidneys.
– patients with CAH usually have a cerebriform
appearance of their adrenal glands that may be
helpful in diagnosis
• MRI
• contrast genitography

40. • Endoscopic evaluation- If the anatomy is not well
understood after radiographic evaluation, particularly in
the presence of a high confluence or after failure to identify
any vaginal structure on genitography, cystoscopy may be
performed alone in order to better understand or delineate
the anatomy.
• Further studies –
– hormonal stimulation,
– level of 17- OH-progesterone, level of other steroids
(testosterone, dihydrotestosterone, delta-4-androstenedione,
DHEA, 17-OH-progesterone), ACTH, cortisol, and renin will allow
a precise picture of the gonadal and adrenal steroidogenesis,
– Gonadal biopsy.

41. • The most valuable stimulation tests for evaluating
steroid genesis are:
– hCG test for the study of testicular functionality.
– ACTH test for the study of adrenal steroid genesis.
– Normal or elevated levels of testosterone and
dihydrotestosterone may be suggestive of receptor
resistance to androgens.
• Molecular biology techniques are more sensitive and
specific tests for assessment of the tissue sensibility to
androgens, but are not always available.

42. Surgical techniques
Timing
• Controversies remain
• It is generally accepted that clitoroplasty for CAH
patients is best performed in the first few months of
life.
• Relatively early intervention may carry the benefit of
thicker, more vascular skin and easier dissection or
manipulation of the clitoris and paravaginal tissue
related to maternal estrogen stimulation.
• In contrast to timing of clitoral reduction, the optimal
age for vaginal reconstruction is more controversial.

43. • Patients with a low-confluence urogenital
sinus can be operated once their metabolic
management is well controlled; in most cases
an elective reconstruction at 3 to 6 months of
age is done.
• Patients with a midlevel or high confluence
can be electively repaired at 9 to 12 months of
age.

44. VAGINOPLASTY- EARLY INTERVENSION
PROPONENTS
• improved tissue transfer
and reduced scarring
secondary to estrogen
stimulation
• minimization of parental
anxiety over the child’s
condition, and
• earlier self-acceptance of
the child’s gender identity
and genital anatomy
OPPONENTS
• May carry an increased risk
for an element of vaginal
stenosis

45. Patient preparation
• full bowel preparation (unless the confluence is extremely) low.
• series of oral antibiotic doses are often administered as well as
perioperative broad-spectrum intravenous antibiotics
• dorsal lithotomy position for endoscopy.
• For higher lesions, a Foley catheter is placed into the bladder, and a
second catheter, either a Fogarty or Foley, is placed into the vagina
• Surgical preparation- full, lower body prep from the nipple to toes,
front and back

47. Feminizing genitoplasty
• Has three components
– Clitoroplasty
– Labioplasty
– Vaginoplasty

48. clitoroplasty
• clitoral amputation - impairment in orgasm and psychosexual
development were later noted.
• The goal of current techniques is to preserve sensation
(neurovascular integrity) for future orgasms, provide an acceptable
cosmesis, and avoid painful erections
• Kogan described a subtunical excision of the erectile tissue, which
has been used extensively and led to newer nerve-sparing
techniques.
• Baskin and colleagues (1999) found that distribution of the sensory
nerves of the clitoris is similar to the sensory nerves of the penis

49. These nerves are found on the top or dorsal aspect of the clitoris and course
under the pubis; circumferential branches from the dorsal neurovascular
bundle encircle the clitoral shaft toward the ventrum.
In most cases we try to avoid reduction of the glans clitoris, aiming to preserve
sensation, but in patients with a large clitoris a wedge of glans tissue may be
cautiously excised from its ventral aspect.

50. steps

53. LABIOPLASTY
Most girls with CAH have labioscrotal swellings that are more anterior than normal
labia majora.
Y- shaped incisions are outlined with an extension posterior
to the swellings
The scrotal flaps are cautiously defatted and moved
posteriorly, beside the introitus, as bilateral Y-V
advancements.
The medial aspects of these skin flaps are then
sutured to the lateral edges of the preputial
skin flaps mobilized during clitoroplasty (now
labia minora)

54. vaginoplasty
• There are six types of vaginoplasty:
– cut-back,
– flap,
– pull-through,
– TUM
– PUM, and
– vaginal replacement

55. Cutback vaginoplasty
• simple procedure performed in the patient with labial
fusion.
• Technique involves incision of the fused skin posteriorly to
the perineum to expose the vaginal orifice.
• The incised lateral edges are oversewn.
• it is only a consideration in patients with a common
urogenital sinus on the phallus with confluence of a normal
vaginal opening at the level of the perineum.

56. Flap vaginoplasty
• Flap vaginoplasty is only indicated for cases with a very-
lowconfluence UGS.
• Fortunoff and colleagues - an inverted perineal skin U-flap
that could be advanced into the opened vagina.
• In a low vaginoplasty there is no need to do a complete
mobilization of the UGS.
• there is no need to insert a finger in the rectum, but a roll
of petroleum jelly (Vaseline) gauze can be inserted in the
rectum to avoid rectal injury

57. inverted perineal skin U-flap
A thick, long, flap is mobilized and then the
posterior wall of the vagina is dissected with care
not to enter the rectum
The apex of the flap is inserted into the apex of
the vagina wall and secured in place

58. PULL-THROUGH VAGINOPLASTY FOR MID AND
HIGH-LEVEL VAGINAL CONFLUENCE
• Before the advent of the urogenital sinus mobilization
procedures, the majority of girls were reconstructed
using a pullthrough vaginoplasty.
• described by Hendren and Crawford
• All patients undergo a total lower body preparation
from nipples to toes, the legs are wrapped.
• Operation begins with a panendoscopy, during which
the confluence is localized.

60. Place a finger in the rectum and dissect against
the rectal wall beneath the external urethral sphincter.
Have your assistant gently pull on the balloon so that
you can identify the junction of the vagina with the
sinus. Incise the vagina almost completely
circumferentially about the base of the balloon (dashed
line). Withdraw the Fogarty catheter. Insert a straight
metal sound into the bladder through the urethra and
complete the division of the vaginal rim, leaving a little
vaginal tissue on the urethra to allow it to be closed
without stricture.

61. close the urethra with interrupted 6-0 chromic catgut
sutures. Replace the sound with a balloon catheter. Dissect
the vagina from the proximal urethra as high as the back of
the trigone.
Suture the posterior flap into the back wall of the vagina
with 5-0 chromic catgut sutures. Incise the perineum
anterior to the vagina as an inverted U to form a second
flap

62. Mobilize this skin flap anteriorly, and suture it to the
vaginal rim. Insert a small suction drain or Penrose drain to
exit through a stab wound. Complete the closure by
joining the lateral margins of the anterior and the
posterior skin flaps to the perineal skin. Place a loose
vaginal pack.
The proximal urogenital sinus at the site of prior confluence is
closed, and the urogenital sinus used as a functional urethra.

63. Total urogenital mobilization
• Total urogenital sinus mobilization (TUM) was described in 1997 by
Alberto Pena.
• Circumferential, partial mobilization of the UGS allows the midlevel
vaginal confluence to be brought down to the perineum without
tension, avoiding the need for separation of the vagina from the
urethra as in the classical pull-through vaginoplasty.
• Fogarty balloon, placed in the vagina during the panendoscopy,
allows the identification of the confluence.
• Urogenital sinus mobilization has the advantage of better
visualization of the merging point.

64. • In this technique, because the confluence is brought
closer to the perineum, the mobilization of skin flaps is
minimized
• The posterior dissection is similar to that done for a
pull-through or flap procedure.
• The anterior dissection in cases requiring a total
urogenital sinus mobilization is done, staying close to
the urogenital sinus under the pubis, and dividing the
ligament from the pubis to the urogenital sinus.

65. Partial urogenital mobilization
• Rink and colleagues (2006) proposed the use of a
PUM.
• In response to concerns for possible
complications of urinary incontinence, resulting
from the circumferential dissection of the UGS
beyond the pubourethral ligament.
• In this technique the anterior dissection stops at
the pubourethral ligament, aiming to avoid
compromising the innervation to the bladder
outlet

66. • In both total and partial urogenital sinus
mobilization, as previously described for low-
confluence vaginoplasty, the distal segment of
the vagina can be quite narrow; hence its
posterior wall must be incised up to a normal-
caliber vagina to avoid a vaginal stricture.

68. Place a temporary 5-0 Prolene holding stitch through
the clitoris

69. Separate the urogenital sinus from the virilized clitoris to
the level of the pubis. Divide the ligaments, attaching the
urogenital sinus to the pubis and thus freeing the
urogenital sinus. Identify the vaginal confluence by
palpation of the Fogarty catheter.
open the vagina posteriorly over the Fogarty
catheter. Continue until a normal caliber is encountered,
as the distal third of the vagina is typically narrow and
prone to stenosis. Complete the anterior dissection of the
urogenital sinus from the corpora of the clitoris
underneath the pubic bone. This frees the anterior
urogenital sinus. Complete the posterior dissection of the
vagina, allowing the entire urogenital sinus to become
mobile and, in most cases, easily able to reach the
perineum.

70. SPLITTING THE UROGENITAL SINUS
• proposed by Rink
• to enhance the feminizing genitoplasty.
• The common urogenital sinus can be used in the following
manners:
– (1) In very-lowconfluence cases we do not mobilize the
urogenital sinus and incise it longitudinally on its ventral aspect
up to the confluence point; the lateral aspects of the opened
sinus are sutured to medial aspects of the prepuce wings, thus
resulting in a more normal anatomic configuration;
– (2) ventrally to fashion a mucosa-lined vestibule,
– (3) dorsally to create a flap to enhance the anterior half of the
vaginoplasty, and
– (4) laterally to create a flap that will be rotated to extend the
vagina, thus completing the vaginoplasty

72. Vaginal agenesis
• pure mullerian agenesis/ can also be androgen
insensitivity
• Mullerian agenesis (also known as Mayer-
Rokitansky-Kuster-Hauser [MRKH] syndrome)
probably results from a failure of the
mullerian ducts to reach the urogenital sinus.
• occurring between the 4th and 12th weeks of
gestation

73. • 1 in 4000 to 1 in 5000 live female births
• By definition, patients are 46,XX females with
normal secondary sex characteristics that
commonly present with amenorrhea.
• Associated with
– Renal anomalies (agenesis, fusion anomalies) – 30%
– Skeletal anomalies
– MURCS association- mullerian duct aplasia, renal
aplasia, and cervicothoracic somite association
– TAR syndrome (thrombocytopenia-absent radius)

74. • TheMcKusick-Kaufman syndrome (MKS)
– Hydrometrocolpos due to vaginal atresia or aplasia,
– postaxial polydactyly, and
– congenital heart disease
• Bardet-Biedl syndrome (BBS)
– genital abnormalities and polydactyly
– in addition to retinitis pigmentosa, learning disabilities,
central
– obesity, and cardiac abnormalities
– Mutations in the MKKS, mutations of BBS6 gene on
chromosome 20p12.

75. diagnosis
• Bimodal distribution – neonatal and pubertal
• Diagnosis of vaginal agenesis is most frequently
made at the time of evaluation of primary
amenorrhea (15% of girls presenting with
primary amenorrhea will have MRKH syndrome)
• It is important to differentiate MRKH and
androgen insensitivity syndrome (AIS), both of
which present with primary amenorrhea and
absence of a functional vagina

76. AIS
• there is absent or scant pubic hair,
• “male range” serum testosterone
• In the prepubertal time period, before elevation of testosterone, a
chromosomal analysis is necessary
• The presence of a Y chromosome increases the risk of
dysgerminoma in the gonadal tissue, and thus removal of gonadal
tissue is indicated.
• a lack of mullerian structures (uterus, cervix, and upper vagina), due
to the presence of mullerian inhibiting substance (MIS).
In contrast, women with MRKH have absence of mullerian structure
with normal functioning ovaries and hormones.

77. • prenatal ultrasound, Prenatal magnetic
resonance imaging (MRI)
• in the neonatal period- abdominal mass
• In the post pubertal period- primary
amenorrhoea, cyclical abdominal pain,
abdominal mass.

78. Treatment
• Treatment of vaginal agenesis depends on the anatomy
of the individual patient
• The goal of therapy is to provide adequate sexual
function and deal with the psychologic impact that the
patient has no uterus or vagina.
• The timing of creation of a functional vagina is
important. In cases of MRKH and AIS it is best to wait
for intervention until the patient is postpubertal and
the young woman is interested in creating a functional
vagina.

79. • The goal for creation of a functional vagina is
pleasurable sexual intercourse with acceptable
cosmesis of the external genitalia with minimal short-
and long-term morbidity.
• Ideally, the neovagina
– should be located appropriately (posterosuperiorly),
– be of adequate dimensions,
– be lined by elastile tissue (either mucosa or skin),
– be neither constantly moist nor malodorous,
– be hairless, and
– be sensate at least at the introitus

80. • Non operative management and operative
management.
• Non operative management –
– using vaginal dilators for 20 minutes twice daily.
– The dilation technique is best managed by a team
of nurse and physician
– lack of patient compliance affecting the results
– The angle of insertion of the dilator is a key to
success

81. Patients are asked to return monthly for examination to assure that the vagina
is being created in the appropriate location and at the appropriate angle. The
next size dilator is dispensed when appropriate.

82. • Advantages
– Technique is under the control of the young woman,
– it does not require anesthesia or a surgical procedure.
– cost effective
• Critics have concerns regarding
– adequate length of the vagina,
– poor lubrication, and
– Dyspareunia during intercourse

83. Operative procedures
Open procedures:
– insertion of a skin-covered vaginal mold (Abbe-
McIndoe procedure),
– artificial skin grafts,
– various fasciocutaneous and myocutaneous flaps
(gluteal-thigh flap, gracilis, rectus abdominis, Malaga
flap, Singapore flap, vulvovaginoplasty of Williams,
vulvovaginal flap (lotus petal labia majora flap),
horseshoe flap using labia minora,
– labial flaps using tissue expanders
– full-thickness skin grafts with vacuum-assisted wound
closure, and the use of amnion and peritoneum

84. • laparoscopic procedures:
– The Vecchietti procedure consists of intra-
abdominal traction on the perineal membrane
causing invagination of the shallow vaginal
dimple.
– Wharton procedure simply places a condom-
covered mold in a created space.
– Sheares’ modification of the Wharton technique -
adjustable vaginal stent was placed in the space.

85. McIndoe procedure
• The most popular tissue for vaginal
replacement during the past 3 decades has
been the split-thickness skin graft as described
by McIndoe
• uses several split-thickness skin grafts, which
are usually harvested from the buttocks

86. The labia are sutured over the mold to hold it in position. After 7 days of bedrest the
patient is taken back to the operating room for cutting of the labial sutures, removal
of the mold, and sewing of the edge of the perineal skin to the skin grafts
Due to the use of dermis, lubricants are required
significant incidence of inadequate vaginal length, vaginal stenosis, and dyspareunia
due to contraction of the split-thickness skin graft are drawbacks of this procedure

87. • Davydov procedure:
– The peritoneum has been used to line the neovaginal
space.
– but this requires laparotomy or laparoscopy.
• Williams vulvovaginoplasty
– Use of vulvar tissues to create a vagina grossly change
the appearance of the external genitalia
– an abnormal angle for intercourse is problematic,
resulting in dyspareunia and making this operation
undesirable

88. Other materials used
• In order to avoid the need for a skin graft donor site, the
use of an acellular human dermal allograft has been
reported.
• Amnion has been used to avoid the need for autologous
graft sites.
• Use of tissue expanders to create more tissue for a labia
minora graft may prove useful.
• Autologous buccal mucosa has been used successfully for
vaginoplasty

89. Bowel vaginoplasty
• Rectum, sigmoid, small intestine
• Rectum is not used because of the need for colostomy.
• Sigoid colon is the preferred part currently
Bowel vaginoplasty
• uses a section of bowel approximately 10 cm that is
mobilized and retains its vascular pedicle to reach the
perineum without compromise to the graft or the pedicle.
• Most surgeons advocate anchoring the proximal bowel
segment in order to decrease the risk of prolapse of the
bowel vagina

91. Complications
– Syed and colleagues described the occurrence of
diversion colitis in 3 of 18 children
– Hiroi and colleagues in Japan described the only case
of malignancy occurring in a sigmoid vaginoplasty
– Many women do complain of the natural mucus
production
– Prolapse -avoided by retroperitoneal fixation of the
sigmoid segment

92. Congenital vaginal obstruction
• can result from
– imperforate hymen,
– agenesis of the lower vagina, or
– a transverse vaginal septum

93. IMPERFORATE HYMEN
• Perforation of the hymen usually occurs during the fifth
month of gestation, Failure of perforation to occur
results in an imperforate hymen.
• Present with hydro/ muco/ pyo/ hematocolpos or
metrocolpos.
• Congenital vaginal obstruction is most commonly
caused by simple obstructive anomalies and can
include agenesis of the lower vagina, segmental
agenesis, or transverse vaginal septum.

94. • Diagnosis: prenatal/ newborn period/
pubertal period.
– Ultrasound/ MRI
– Present with midline mass, frequently associated
with urinary tract obstruction.
– Primary amenorrhoea at puberty with cyclical
abdominal pain.
• Treatment- elliptical incision not cruciate

95. Agenesis of the Lower Vagina
• presentation is similar to an imperforate hymen, and
the diagnosis is determined by physical examination
and imaging studies
• Diagnosis- The external appearance of ALV can easily
be confused with an imperforate hymen. The key
means to a correct diagnosis include the observation
that there are normal fronds of hymen and there is not
just a membrane of tissue.
• P/R - appreciate a space between the obstructed
vagina and the location for the introitus.

96. If there is some amount of areolar tissue until the obstructed vagina is reached,
then this is ALV and not an imperforate hymen
Once the obstruction is relieved and the old blood is evacuated, the native upper
vagina will tend to retract back up toward the uterus. This is the reason the native
vaginal tissue must be sewn to the newly created introitus

97. Transverse Vaginal Septum
• can occur at varying levels of the vagina.
• referred to as low, middle, and high transverse
vaginal septum.
• approximately 46% of vaginal septa occur in the
upper vagina, 40% in the middle vagina, and 14%
in the lower vagina.
• The presentation will vary depending on whether
there is a perforation.
• Pelvic ultrasonography is usually diagnostic of
hematocolpos.

98. best treated by draining the vagina by cutting into the septum, resecting the septum,
and performing a simultaneous transperineal vaginal pull-through procedure
attaching the upper and lower vagina using absorbable sutures

99. Anomalies of fusion
• Vaginal duplication
• OHVIRA
• Longitudenal vaginal septum

100. OHVIRA
• obstructed hemivagina with ipsilateral renal anomaly
(OHVIRA) or Herlyn-Werner-Wunderlich syndrome
• Urinary tract anomalies may confound the situation with an
ectopic ureter inserting into a duplicated imperforate
hemivagina, resulting in a mass.
• The mass usually pushes the bladder forward and the
normal vagina in a posterior direction.
• simple incision of the obstructing septum with resection of
a “window of tissue” often provides adequate drainage

102. Longitudinal Vaginal Septum
• For nonobstructed longitudinal vaginal septum, a
surgical excision of the septum is required only if
the young woman desires such a procedure
• Some maintain the longitudinal vaginal septum
and have no issues with sexual activity or vaginal
delivery
• Most have two cervices, so a Papanicolaou smear
must be obtained from both.

103. • The septumshould be excised by “wedging” out
the fibrous septal tissue. Great care is necessary
to avoid the bladder above, the rectum below.
• Once the septal tissue is excised, the normal
vaginal mucosa from one vagina is sewn to the
other vagina to create a smooth vaginal mucosa
and close the resulting defect. Excision of the
septal tissue removes the fibrous tissue to avoid
dyspareunia

105. Thank you