assessment and management of cloaca, a rare congenital anomaly in children

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

7. Urogenital sinus (UGS) variations
Long UGS ending in the tip of the clitoris.

8. Urogenital sinus (UGS) variations
Subclitoral meatus.

9. Urogenital sinus (UGS) variations
Wide opening of the UGS (like a vagina).

10. Urogenital sinus (UGS) variations
With accessory tract

11. Vaginal Variations
Double vagina, side by side.

12. Vaginal Variations
Single vagina terminating in the UGS.

13. Vaginal Variations
Vagina entering the bladder.

14. Vaginal Variations
Absence of a vagina, with a uterus present

15. Rectal Variations
Normal anus – no cloacal malformation.

16. Rectal Variations
Ectopic anus

17. Rectal Variations
Low confluence of the rectum into the
UGS.

18. Rectal Variations
Short common channel

19. Rectal Variations
Long common channel.

20. Rectal Variations
Long fistula with colon at the upper end.

21. Rectal Variations
Rectal fistula entering next to the bladder neck and
anterior to the vagina

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

27. Associated Defects
 Absent kidney,
 VUR,
 horseshoe kidney,
 ectopic ureters,
 double ureters
 hydronephrosis, and megaureters.

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

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

42. P

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.

44. P

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.

71. P

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.

83. Thank you