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Cloaca approach to decision making
1.
2. Cloaca approach & Decision making
Dr. Faheem ul Hassan
Dr. Gowhar Mufti
3. Introduction
A defect in which the rectum,
vagina and the urinary tract
converge into one common
channel CC.
CC opens into a single orifice at
the position where the urethral
opening is normally located.
occurs in 1:250,000 newborns
4. Embryology
The cloaca is usually a
transient structure during
embryonic development.
Forms at 3 weeks by the
confluence of the allantois
and hindgut
5. Embryology
partitioning of the cloaca
occurs by the craniocaudal
growth of a mesenchymal
structure called the urorectal
septum URS
6. Classification
Type I: Forme fruste
Anteposition of anus with ultra
short
UGS and normal female genitalia
Type II: Low cloacal
malformation
Short UGS < 3 cm (confluence
below PC line)
Type III: High cloacal
malformation
long UGS > 3 cm (confluence
at or above PC line)
Type IV: Vagina and/or rectum
into bladder cavity
Rare cloacal
malformations
Posterior cloaca in boys
Cloacal exstrophy
22. Cloaca
In a cloaca, the vagina, urethra,
and rectum are fused creating a
CC
CC opens into a single orifice at
the normal urethral position.
the common denominator is the
presence of a single perineal
orifice.
23. Cloaca
The length of the common channel varies from 1-
10 cm
Average length of approximately 3 cm.
30% of Cloaca patients have hydrocolpos
The reason why the dilated vagina retains fluid is a
mystery
Likely there is a valve mechanism
24. Hydrocolpos
The hydrocolpos may produce complications like
it may compress the trigone of the bladder,
it may produce VUJO, megaureter, and hydronephrosis
hydrocolpos if left undrained may become infected
(pyocolpos).
The resulting inflammation may scar the vagina and
affect the future reconstruction
25. Double Mullarian System DMS
40% of patients have a double
Mullerian system
2 hemiuteri and 2 hemivaginas.
septation may be
partial or total and
symmetric or asymmetric
26. DMS
DMS is frequently associated with a unilateral
atresia of the Mullerian structure.
it may produce hematocolpos at puberty, as well as
Retrograde menstruation into the peritoneal cavity
28. Clinically
only one orifice is present in the
vulva
Meconium and urine issue from the
common orifice
If the size of the introitus appears
smaller than in a normal female, the
possibility of a cloacal anomaly is
very high.
29. Clinically
On probing a rectovestibular fistula with a fine
forceps, the probe will pass posteriorly and
backwards.
The probe in a rectovaginal fistula will pass only
upwards and this has long been stated as a method of
differentiating between the two.
30. Cloacagram
Multiple catheters may be
required to outline the genital
and urinary components with
contrast medium.
Alternatively, a balloon
catheter can be used, with the
balloon inflated outside the
patient and applied to the
cloacal opening to occlude it.
31. Cystogenotoscopy
At the time of colostomy
formation, it is useful to
perform an endoscopy
of the CC to assess the
internal vaginal and
urethral anatomy and
to determine the length
of the common
channel
32. Contrast Study
distal colostogram and micturating cystogram are
usually performed together
33. CT & MRI
CT with contrast opacification of
the bladder, distal colon,
vagina/hydrocolpos and urinary
tract simultaneously,
can allow for 3D reconstructions
which can display the internal
anatomy beautifully and aid in
planning reconstructive surgery
34. MRI
If simultaneous contrast opacification is not possible,
then MRI is the preferred technique
MRI is a valuable tool in exploring the different
internal anatomical features of the cloacal anomaly
without exposure to ionizing radiation.
35. MRI
Routine preoperative use of MRI has been criticized for
low sensitivity for fistula detection,
need for general anesthesia, and
providing no additional information that can affect surgical
decision
However MRI shows higher sensitivity for detection of
Mullerian anomalies which are common in patients with
persistent cloaca
36. Other Investigations
The urinary tract and spine should also be fully
imaged.
Plain radiography of the spine is useful to detect spinal
anomalies, such as
spina bifida and hemivertebrae.
37. Other Investigations
USG of the spine should be performed in all infants
with Cloaca during the first 3 months of life
USG is done also to rule out
hydronephrosis
Megaureters
hydrocolpos.
38. USG
USG features of HDN and megaureters should raise
suspicion of hydrocolpos.
Most of the time, drainage of the hydrocolpos alone
takes care of the problem of megaureter and
hydronephrosis.
If the patient has a hydrocolpos, the first step should
be drainage
39. Colostomy
Colostomy should be preferably
Descending Colostomy
Divided colostomy
Colostomy and drainage of hydrocolpos should be
carried out in the same sitting
40.
41. Goals of Cloacal Surgery
Urinary control,
bowel control,
sexual function, and
obstetrical potential
all patients with anorectal and urogenital malformations
should be clean of stool in the underwear and dry of urine
after the age of 3
43. Cloacas with a common channel 1 cm
The procedure of choice in this type of anomaly is
Posterior Sagittal Anorectovaginoplasty PSAVP
rectum is separated from the vagina as in cases of
vestibular fistulas
the lateral and posterior walls of the vagina are
mobilised
edges of the vagina are sutured to the skin of the neolabia.
urethra or the common wall between the vagina and
urethra is not disturbed in this way.
45. Cloacas with a 1-3 cm common channel
Most cloacas are of this type.
Less than 20% of them require intermittent
catheterization after reconstruction.
Bowel function depends on the quality of the sacrum
and spine.
Repair is carried out anytime between 1-12 months
usually around 3 months.
46. Cloacas with a 1-3 cm common channel
posterior sagittal approach with total urogenital
mobilization TUM is used
The cosmetic and functional resutls are excellent
PSARP incision is made
The CC is opened exactly in the midline which
exposes the internal anatomy of the malformation.
47. Cloacas with a 1-3 cm common channel
5.0 silk stitches are placed around the rectal opening
and rectum is mobilised
Enough length is gained until rectum reaches the
perineum
48. Cloacas with a 1-3 cm common channel
5.0 silk sutures in the edges of the common channel
are applied
Another set of sutures is placed about 5 mm above the
clitoris.
49. Cloacas with a 1-3 cm common channel
The CC is divided between the clitoris and the sutures
using the needle tip cautery
50. Cloacas with a 1-3 cm common channel
A plane of dissection is created between the pubis and the CC
The suspensory ligaments of the urethra as well as their lateral
attachments on vagina divided.
51. Cloacas with a 1-3 cm common channel
By dividing the suspensory ligament a 2 cm length is
gained.
Another 1 cm is gained by dividing posterior and
lateral vaginal bands
TUM achieves a length of 3 cms
Occasionally, cloacas with a 4-5 cm common channel
can be repaired
52. Cloacas with a 1-3 cm common channel
After mobilizing the urogenital sinus, CC is split into 2
lateral flaps.
These 2 lateral flaps become part of the neolabia.
53. Cloacas with a 1-3 cm common channel
urethral meatus is sutured to the tissue behind the clitoris.
lateral walls of the vagina(s) are sutured to the neolabia
54. Cloacas with a 1-3 cm common channel
urethral meatus is sutured to the tissue behind the clitoris.
lateral walls of the vagina(s) are sutured to the neolabia
55. Cloacas with a 1-3 cm common channel
The rectum is then placed within the limits of the
sphincter and in front of the levator mechanism
56. Cloacas with a 3-5 cm common channel
CC length 3-5 cm requires opening of abdomen, in
addition to posterior sagittal approach
With a posterior sagittal incision, the internal anatomy
of the malformation is exposed;
the rectum is separated from the urogenital tract,
Total urogenital mobilization is performed,
Following this abdomen is opened with a midline
infraumbilical incision.
57. Cloacas with a 3-5 cm common channel
Then “Extended Transabdominal Urogenital
Mobilization” is performed
The lateral attachments of the bladder are divided.
The urogenital complex is then brought up between
the bladder and posterior aspect of pubis.
Usually this maneuver allows to gain extra length on
the urogenital mobilization.
58. Carving of the pubic cartilage
If the extended transabdominal TUM is not enough
carving of the pubic cartilage is indicated.
Resecting approximately 50% of the posterior aspect of
the pubic cartilage does not compromise pelvic stability
It allows a more direct trajectory of the urogenital sinus.
This maneuver may allow a tension-free anastomosis
59. Separation of vagina(s) from the urinary tract
This is the most technically demanding maneuver of
the entire cloacal repair.
The separation of these structures through
abdomen is much easier but still technically
demanding.
The bladder is opened in the midline and feeding
tubes are introduced through each of the ureters
60. Separation of vagina(s) from the urinary tract
Ureters are cannulated because in cloaca both
ureters pass through the common wall between the
vagina and the bladder.
To achieve a good repair, it is necessary to separate
these structures with minimal or no damage.
There is a possibility that after the separation, the
vagina(s) reaches the perineum.
61. Vaginal switch
These patients have separated hemiuteri with a vaginal
septum and 2 large hydrocolpi.
If the distance between two hemiuteri is longer than
the vertical length of both hemivaginas, then
patient may be a candidate for the vaginal switch
maneuver
62. Vaginal switch
Both hydrocolpi are tubularized into a single vagina
(switched down vagina.)
the blood supply of the switched vagina will depend on the
blood supply of the opposite hemivagina
63. Vaginal replacement
choice of tissue for the replacement are rectum,
descending colon, sigmoid colon, and finally
small bowel.
64. Vaginal replacement
Vaginal replacement with rectum: If the patient has a
very dilated rectum, we can divide it longitudinally, preserving the
blood supply of both portions
Both structures are rotated 90° in opposite directions to avoid the
overlap of suture lines.
65. Vaginal replacement
In a ptients with nondilated rectum most distal part of
the rectum can be used as neovagina and proximal
mobilised as the neorectum.
66. Vaginal replacement with colon
descending colon, which has a nice vascular arcade of
vessels.
A good option is to take the colostomy down and use
the part that used to be the colostomy to replace the
vagina.
67. Vaginal replacement using small bowel
The longest mesentery of the small bowel seems to be
located about 15 cm proximal to the ileocecal valve
this part of the bowel is ideal for vaginal replacement.
68. Colovaginoplasty
Colon, cecum, or ileum may be used for bowel vaginoplasty
but the sigmoid colon is preferred over the others
because of following
it is self-lubricating
mucus production is less of a problem than with the use of
the small bowel
there is a minimal risk of stenosis
it is close to the perineum
it has an easily mobilized vascular pedicle
it does not require moulds or stenting.
69. However ulcerative colitis, diversion colitis and
adenocarcinoma has been seen with sigmoid
vagina
70. Cloacas with greater than 5 cm common channel
When common channel is 5 cm, separation of the
structures (rectum, vagina, and urinary tract) is to be done
at laparotomy than through the posterior sagittal Approach
The rectum opens either into the bladder neck or the
trigone
little hemivaginas also open in the trigone or the bladder
neck
the ureters open in that area as well.
72. Postop care
Patients without fecal diversion are maintained with
nothing by mouth, and receive parenteral nutrition for 7-10
days.
They usually stay in the hospital 2-5 days.
A Foley catheter remains in place for 2 to 3 weeks.
Approximately 20% of these patients may eventually
require intermittent catheterization
Two weeks after the procedure, parent are taught anal
dilatation
73. Pitfalls in the management of newborn cloacas
Failure to recognise & manage hydrocolpos
HDN
Pyocolpos
Rupture of pyocolpos and peritonitis
Pyocolpos and fibrosis of a portentially usable Vagina
For drainage of pyocolpos tube vaginostomy is recommended
Other option is Vaginostomy at or near umbilicus
74. Pitfalls in the management of newborn cloacas
Colostomy problems
Too distal in location
Colostomy prolapse
Divided colostomy in the region of desceding colon is recommended
75. Pitfalls in the management of newborn cloacas
Clinical misdiagnosis
Cloaca may be misdiagosed as imperforate anus with
rectalvaginal fistula
Use of the term rectovaginal fistula in literature is
common
Imperforate anus with rectovaginal fistula is almost
nonexistent.
76. Pitfalls in the management of newborn cloacas
Patients with cloaca often have a hypertrophied
clitoris, and thus a clinician might suspect an intersex
anomaly
However, all patient in a series of 490 cloacas had two
normal ovaries and were chromosomally XX.
77. Road to success
Drainage of a hydrocolpos,
A correctly placed and performed colostomy
and vesicostomy
Precise clinical diagnosis,
78. Outcomes- Renal function
A high incidence of renal failure is observed in cloaca
patients.
In one large retrospective review, half of the cohort
had chronic renal failure by 5.7 years of age,
end stage RF requiring renal transplantation was
repoted in 19%.
Overall mortality rate from RF was 6%
The importance of the early recognition and management of
chronic renal failure cannot be overemphasised.
79. Faecal continence
Around 60% of cloaca patients become continent of
faeces.
Only 28% are continent by spontaneous bowel
movements
Around 30–40% need rigorous bowel management
programmes in the form of rectal washouts or
antegrade enemas to achieve social continence
80. Urinary continence
In one prospective study of anorectal patients, 90% of
cloaca patients had bladder dysfunction on urodynamic
study prior to surgery
only 22% of cloaca patients void spontaneously and are dry
A further 12% have achieved continence by CIC
46% of patients required reconstructive surgery.
Cloaca patients are also potentially at risk of iatrogenic
nerve damage during surgical repair
81. Gynaecological outcome
In a study 86% had an adequate vagina with no
menstrual problems
57% were sexually active
19% of the adult cloaca patients required additional
vaginal surgery to facilitate intercourse.
82. Obstetric outcome
So far, a normal pregnancy and delivery of a healthy
baby been reported in only one series.
Delivery by caesarean section is usually recommended
in patients where vaginoplasty has been performed.