Keywords
cloaca - urethrovaginal fistula - use of Deflux
Introduction
Cloacal anomaly is one of the rarest forms of anorectal malformation found in newborns,
which has an incidence of 1 in 20,000 live births.[1]
[2]
[3] Apart from being at the most severe end of anorectal malformation spectrum, it also
offers a formidable challenge to the surgeons. It requires the surgeon to have in
depth knowledge of newborn female pelvic anatomy as well as considerable amount of
specialized surgical skills to effectively and successfully treat patients with cloacal
anomalies.[4] In spite of best interdisciplinary management and adequate surgical correction,
the incidence of short- and long-term complications still remains high. The surgical
correction needs to be tailored specifically for the patient, as minor variations
in the anomaly does exist.[1] The complications arising also needs to be treated in a way which is patient specific.[5] The most acceptable approach for one-stage total correction of cloacal malformation
is the combined posterior sagittal and abdominoperineal approach.[6] Urethrovaginal fistula is one of the difficult complications of surgical correction
of cloaca.
Case Report
Our patient was born at term and was diagnosed at birth to have cloacal anomaly. She
underwent diverting colostomy and cystovaginoscopy on the second day of her life where
the common channel was 3.5 cm ([Fig. 1]). It was found that she had duplication of uterus and vagina, and her both ureters
were ectopically inserted into the corresponding hemivagina. The urinary bladder was
empty and collapsed ([Fig. 2]). The rectum and confluence of the hemivaginae opened above the bladder neck ([Fig. 3]). The sacral ratio is 0.3. Full repair was done at the age of 10 months. Abdominal
approach was utilized along with posterior sagittal anorecto vaginoplasty (PSARVP),
where both ureters were reimplanted into the bladder, the common channel was left
as urethra, the hemivaginae was combined and pulled down, and the rectum was pulled
through the sphincter complex and repaired in the respective positions.
Fig. 1 Common channel 3.5 cm at the second day of life.
Fig. 2 Duplicated uterus and two hemi-vagina with ectopic ureter.
Fig. 3 Cystoscopy view at the second day of life.
The post-operative cosmetic result was good, but the patient had urine leak through
her vagina, which raised a suspicion of vesicovaginal fistula. This was confirmed
by cystovaginoscopy where vesicovaginal fistula, ∼0.5 cm in diameter, was found. The
fistula was approached through transvesical access and was closed in two layers without
tissue interposition and catheter left for 10 days, 8 months after PSARVP. She eventually
developed small urethrovaginal fistula, most likely as complication of vesicovaginal
fistula repair. This tiny fistula, confirmed by cystoscopy, ∼4 to 4.5 cm distance
from the skin, did not mandate a radical procedure for repair. According to our judgement,
this would be marred with more harm than benefit to our patient.
We devised a novel technique (full detailed explanation given to the family) where
we approached the urethrovaginal fistula through a resectoscope via the vagina at
30 months of age. The edges were diathermised, and Deflux was injected just below
the mucosa to approximate the diathermised edges of the fistula, and urinary catheter
had been left for 5 days ([Figs 4] and [5]). The new idea was discussed with an expert outside the hospital before the attempt.
Fig. 4 The uretherovaginal fistula via vagina.
Fig. 5 Deflux injection.
The patient was referred postoperatively to the same center discussed before. The
girl was passing urine spontaneously, and an ultrasound (US) showed normal kidney
and no residual urine in the bladder.
Subsequent cystoscopy and vaginoscopy examinations did not reveal any recurrence of
the urethrovaginal fistula. The patient was eventually examined by an experienced
pediatric surgeon and pediatric urologist, and both ruled out any recurrence of the
fistula.
Colostomy was closed at 3 years of age and started on CIC.
Because the mother was not compliant with CIC, MITROFANOFF was done for her at three
and half years.
Discussion
Management of complex urethrovaginal or vesicovaginal fistulae traditionally involves
a radical approach where the fistulous tract is excised, and some vascularized graft
or flap is interposed to prevent recurrence of the fistula. Even in the best hands,
the rate of recurrence remains high due to the fact that urethra and vagina share
a common wall, more so in cloacal anomalies,[7] and also due to compromised blood supply in an area that is breached by surgery.
We had decided not to undertake a radical approach for the second time in this area,
as further complication and redevelopment of fistula or complete breakdown of the
repair was anticipated. Instead, a relatively simple and novel technique was adopted,
which proved to be successful in closing the small fistula.
Conclusion
The surgical and medical management of cloacal anomalies remains to have a high morbidity,
such as the development of fistulas between the urethra and the vagina. If these are
small, the injection of Deflux into the fistula may be a novel therapeutic option.
