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Case Report

Continent cutaneous diversion and external genitalia

reconstruction in a child with severe variety urogenital sinus
and ambiguous genitalia
Sajni I. Khemchandani
Consultant Pediatric Urologist, Part Time Professor in Transplantation Surgery, Department of Urology, Institute
of Kidney Diseases and Research Center and Dr. H. L. Trivedi Institute of Transplantation Sciences, Civil Hospital
Campus, Ahmedabad, Gujarat, India

Address for correspondence: Dr. Sajni I. Khemchandani, 401, Sudarshan Flats, 13, Shantinagar Society, Usmanpura,
Ahmedabad - 380 013, Gujarat, India. E-mail: dr_sajni@rediffmail.com

ABSTRACT Access this article online

Website: www.jiaps.com
The diagnosis and management of a child with ambiguous genitalia and severe variety DOI: 10.4103/0971-9261.151554
of urogenital sinus with a high vesico-vaginal confluence is challenging. This 4-year- Quick Response Code:
old female child had solitary right kidney with ectopic ureter opening in high variety
of urogenital sinus with hypo-plastic urinary bladder and incontinence. We describe
genitourinary reconstruction with complete functional rehabilitation in this child. This
complex problem was managed with continent urinary diversion with Penn pouch
and refashioning of external genitalia, rendering continence and near normal female
external genitalia. The child and parents are happy with continence and aesthetically
normal external genitalia.

KEY WORDS: Continent urinary diversion, genital ambiguity, urinary incontinence,

urogenital sinus

INTRODUCTION external genitalia, hence was reared like a male child.

The diagnosis and management of a child with
ambiguous genitalia should be considered as an
emergency. We describe complete genito-urinary
reconstruction in a four year old female child with
ambiguous genitalia. The child had right solitary kidney
with ectopic ureter opening in high vesico-vaginal
confluence with hypo-plastic urinary bladder and
continuous incontinence of urine.
CASE REPORT
A 4-year old child presented with continuous
incontinence of urine since birth and ambiguous
Child had continuous dribbling and has never passed
urine in normal stream. The mother had no history of
drug intake or viral fever during pregnancy. Since child
was from remote village, antenatal ultrasonography was
not done. On general examination, apparently child
was healthy looking. External genitalia examination
showed prominent and fused labio-scrotal folds with
posteriorly placed clitoris with clitoromegaly and
an orifice of the urogenital sinus [Figure 1]. Urine
analysis showed 4-5 pus cells /high power field. Urine
culture was sterile. The renal function tests and serum
electrolytes were normal. Serum 17-OH progesterone
and urinary ketosteroids were normal. Chromosome
analysis showed 46 XX karyotype. Ultrasonography

Cite this article as: Khemchandani SI. Continent cutaneous diversion and external genitalia reconstruction in a child with severe variety urogenital
sinus and ambiguous genitalia. J Indian Assoc Pediatr Surg 2015;20:84-6.
Source of Support: Nil, Conflict of Interest: None declared.

84 Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2

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Khemchandani: Urogenital sinus and ambiguous genitalia

of abdomen and excretory urography revealed mild

hydroureteronephrosis of right pelvicalyceal system and
ureter and non-visualization of left kidney and urinary
bladder. Genitogram showed severe variety urogenital
sinus with high vesico-vaginal confluence with anterior
hypo-plastic urinary bladder and posterior vagina.

Genitoscopy showed common urogenital sinus opening,

anterior orifice showed hypo-plastic urinary bladder
with severely incompetent bladder outlet and ectopic
ureteric orifice opening at junction of bladder and
vagina, resulting in total urinary incontinence, although
renal function was well-preserved.

Since urinary bladder was hypo-plastic with lack of

Figure 1: External genitalia showing prominent and fused labio-scrotal
capacity and continence mechanism, continent urinary folds with posteriorly placed clitoris with clitoromegaly
diversion was planned. The urinary tract reconstruction
was done with continent ileocecal pouch, the Penn
pouch, using appendix on Mitrofanoff principle,
the stoma was placed at the umbilicus. The external
genitalia reconstruction was done by clitoroplasty,
preserving glans and neurovascular bundles. Two lateral
plates from dorsal split phallic and preputial skin were
used to construct labia minora and labio-scrotal folds
were used to construct labia majora [Figure 2].

Post-operatively, child was asymptomatic; pouchogram

was done at 3 weeks which showed good capacity
neo-bladder, with no evidence of reflux or urinary
leak [Figure 3]. Child was kept on clean intermittent
catheterization (CIC) through umbilical stoma and
dilation of reconstructed vestibule. Child is on CIC with
normal renal functions with asymptomatic bacteriuria Figure 2: Labia minora and labia majora after re-construction and
over four years of follow up. clitoroplasty

DISCUSSION

The urogenital sinus is an embryological anomaly

which consists of a common channel of urethra and
vagina. The major incidence is produced in congenital
adrenal hyperplasia context. In certain occasions it
can be associated with an imperforate anus, and then
the malformation is called a cloacal defect.[1] In the
high narrow urogenital sinus malformation, the vagina
appears to insert into rather masculinized urethra,
occasionally vaginal insertion occurs at a very high
level, even as high as the bladder triagone,[2] as in our
child.

Continent urinary diversion not incorporating the

bladder is seldom used in children. Potential indications Figure 3: Pouchogram showing good capacity neo-bladder, with no
include following cystectomy for genitourinary evidence of reflux or urinary leak
malignancy, and occasionally in cases of bladder
exstrophy, cloacal anomalies, or neurogenic bladder. urogenital sinus with a high confluence between
[3]
The possibility of functional reconstruction as an bladder and vagina and with urinary incontinence is
alternative to urinary diversion in a case of a severe presented by Scott G et al.,[4] Sheldon et al., have cited
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2 85
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Khemchandani: Urogenital sinus and ambiguous genitalia
a case of a tiny hypo-plastic bladder with a severely
incompetent bladder outlet, in which orthotopic gastric
neobladder and orthotopic neourethra was constructed
in a Mitrofanoff fashion.[5] Sheldon have also cited a
case of solitary kidney which drained through ectopic
ureter, resulting in total urinary incontinence. In
this case also, the bladder was not deemed suitable
for reconstruction; a neobladder was constructed.[2]
Our child also had high vesico-vaginal confluence
with urinary incontinence; hence, continent urinary
diversion with Penn pouch with reversed appendix on
Mitrofanoff principle was constructed with cutaneous
stoma at umbilicus. Simultaneously, ambiguous
genitalia were corrected by clitoral fixation to pubis
preserving neurovascular bundles and reconstruction
of labia minora and labia majora. Patient has complete
continence for 4-5 hours and she evacuates urine by
performing CIC through a completely camouflaged
stoma at umbilicus. Although patient requires clean
intermittent catheterization, this seems to be an
acceptable trade off for continence and freedom from
an external appliance. Sheldon et al., have performed
orthotopic continent catheterizable neourethra using
an extension of Mitrofanoff principle.[5] We prefer
86
catheterizable stoma at umbilicus for its ease of
intermittent catheterization and reduced incidence of
infection, especially in female patients.
Her external genitalia now appear to be functionally
and aesthetically pleasing. Complete functional and
cosmetic reconstruction of ambiguous genitalia patient
with urinary tract abnormality is possible in single
stage.
REFERENCES
1. Molina E, Cerda J, Sanchez-Martin R, Romero R, Estelles C,
Aguilar F, et al. New approach in the surgical treatment of the
urogenital sinus. Cir Pediatr 2000;13:54-7.
2. Sheldon CA. Imperforate anus, urogenital sinus and cloaca.
In: Belman AB, King LR, Kramer SA, editors. Clinical Pediatric
Urology. 4th ed. Martin Dunitz Ltd.; 2002. p. 811-57.
3. Bissada NK, Abdallah MM, Aaronson I, Hammouda HM.
Continent cutaneous urinary diversion in children: Experience
with Charleston pouch I. J Urol 2007;177:307-10.
4. Schott G, Schrott KM. Continent reconstruction of an extreme
urogenital sinus--case report of a so-called high vesicovaginal
confluence. Urologe A 1988;27:193-7.
5. Sheldon CA, Welch TR. Total anatomic urinary tract replacement
and renal transplantation: A surgical strategy to correct severe
genitourinary anomalies. J Pediatr Surg 1998;33:635-8.
Journal of Indian Association of Pediatric Surgeons / Apr-Jun 2015 / Vol 20 / Issue 2