Pediatric

1. CLOACAL AND ARM PATIENTS
DX AND MANAGEMENT
URINARY PROBLEMS IN

2. • Anorectal malformations are congenital
anomalies that occur in approximately 1 in
4500 live births.

4. VATER & VACTERL
• VATER (acronym of Vertebral and vascular anomalies,
Anal atresia, Tracheoesophageal fistula, Esophageal
atresia, and Renal anomalies, Radial dysplasia) .
• VACTERL (acronym of Vertebral abnormalities, Anal
atresia, Cardiac defects, Tracheoesophageal fistula with
Esophageal atresia, Radial and renal defects, and
Lower-limb abnormalities) associations .
• These children can have both structural and functional
abnormalities of the upper and lower urinary tract as
well as significant genital anomalies

5. • Anomalies of the genitourinary tract can have a dramatic
impact on the length and quality of these children’s live.
• Genitourinary anomalies occur frequently in patients with
ARM and previous retrospective reviews report incidences
from 20 to 50% .
• the embryological development of the rectum and
genitourinary tract develop simultaneously and in close
proximity
• A common embryological insult affecting the caudal
portion of the embryos development at a critical time
during early gestation can cause a spectrum of defects in
the anorectal, genital, urinary, and spinal tract

6. Renal Anomalies
a) Position
Renal ectopia occurs in approximately 5% of
patients and renal fusion in 5%
These patients do not need specific
management of these renal problems;
however, they do predispose to urinary tract
infections and to vesicoureteric reflux, which
needs to be considered

7. b) Duplication
• Renal duplication, either partial or complete,
is seen in between 2 and 5% of patients.
• The management of these patients is the
same as those without ARM.
• An ectopic ureter associated with an upper
pole moiety must be considered as a cause of
incontinence in these children.

8. Hydronephrosis
 2 and 10% .
• In most series hydronephrosis is used to refer to
a uretero-pelvic obstruction
The management approximately 25% requiring
surgical reconstruction
Surgical reconstruction is needed in those patients
with an increasing hydronephrosis, deteriorating
renal function, or symptoms

9. Renal Agenesis
• Unilateral renal agenesis is a common
problem, it is reported in 3–5% of children
with low anomalies and up to 20% of patients
with high anomalies

10. Multicystic Dysplastic Kidney,
• Renal dysplasia is seen in patients with both
low and high ARM
• This abnormality appears less commonly, in
about 1–3% of patients .
• The importance of dysplasia and renal
agenesis cannot be overlooked, as chronic
renal failure is one of the major causes of
mortality in these patients

13. Ureteric Anomalies
Vesicoureteric Reflux
• incidence of vesicoureteric reflux varies greatly
from 2 to nearly 50%
• The management of reflux follows the same
principles as all patients with primary reflux.
• Division of the rectourinary fistula and
subsequent decrease in bacterial contamination
frequently leads to resolution of lower grades of
reflux.

14. Megaureters
• Megaureters are rarely observed
(approximately 1– 3%)
• As with hydronephrosis, a policy of
prophylactic
• antibiotics and observation is appropriate.
• Unless indicated for surgical intervention .

15. Bladder function
• The rate of urinary incontinence varies greatly
in the literature depending on the severity of
the original ARM and on the definitions used
to describe continence.
• Overall incontinence rates in the literature
vary from 10% up to 25%.

16. • Denervation of the lower urinary tract has
been observed following posterior sagittal
anorectoplasty (PSARP) and with other
techniques used to reconstruct.
• The incidence of new neurological damage
following PSARP for anorectal malformations
varies between 5 and 10%
• Constipation may also contribute to abnormal
bladder function

17. • Reducing bladder pressure should first be
attempted medically using anticholinergic
agents; when this fails, bladder augmentation
may be necessary.
• These techniques can be used to achieve
social continence in many of the patients

18. • CIC can be performed either urethrally or via a
Mitrofanoff stoma .
• In the majority of patients with an ARM the
urethra is sensate, consequently urethral CIC
can be uncomfortable, especially in the older
patient.

19. Urethral Problems
• Posterior urethral valves, megaurethra, and
urethral duplication have been reported in
association with ARM
• The most common are iatrogenic and include
urethral strictures, large diverticula, or
remnants of the rectal pouch from incomplete
dissection of the rectal fistula at the time of
pull-through

20. • Stones may form if a urethral diverticulum is
left, precipitating recurrent infections
• it can be technically difficult to catheterize the
urethra in those patients who require CIC for
neurogenic bladder

21. Genital Anomalies
• hypospadias,
• bifid scrotum,
• bilateral undescended testes 10 and 40%
• penoscrotal transposition.
• Megaurethra and ectopic urethra are
uncommon, and penile duplication has
occurred in a few patients

22. CLOACA
• when rectum, vagina and lower urinary tract
fuse into a single common channel.
• Persistent cloaca is a most severe
malformation of anorectal anomalies in girls
and is associated with complex pelvic
malformations

23. • Different patterns of the cloacal anatomy
result from differences in the level of arrest in
the early development of urorectal septum.
• The position of rectal fistula may cause the
vaginal anomalies. The müllerian ducts fail to
fuse in the existence of high rectal fistula and
results in duplication of the uterus and the
vagina

24. • At birth, any girl with a single perineal orifice
requires abdominal ultrasonography to
evaluate the kidneys and the urinary tracts as
well as the association of vaginal dilatation

25. Urological anomiles
• Absent kidney,
• VUR
• horseshoe kidney,
• ectopic ureters,
• double ureters
• hydronephrosis,
• megaureters.

26. 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

35. Urinary incontinence
The rate of incontinence in girls born with a
cloacal anomaly can approach 60–70%
• The incidence of new neurological damage
following a cloacal anomaly who undergo
reconstruction using PSARP and total
urogenital mobilization, with 50% showing
deterioration in bladder function

37. Cloacal exstrophy (OEIS Syndrome)
Omphalocele :a large protrusion outside the cavity of the abdomen,
involving several organs including the spleen, liver and intestine. It may
also be small.
Exstrophy of the bladder and rectum
The colon and rectum are also open with a segment of the rectum
situated between the halves of the bladder and located on the surface
of the abdomen.
Imperforate anus :and the colon may connect to the urethra, bladder
or vagina through a fistula (small tract).
Spinal defects spina bifida.
Diagnosis

38. Diagnosis
• Cloacal exstrophy is usually diagnosed with
fetal ultrasound. It is confirmed upon birth
during physical exam

39. multidisciplinary team of surgeons (pediatric
general surgeons, pediatric urologists, pediatric
orthopedists) will create a treatment plan based
on the type and the extent of your child’s
condition

40. • The goals of treatment include an anatomic
reconstruction and achieving bowel and
urinary control, as well as normal sexual
function.

44. Thank you