Total Urogenital Sinus Mobilization in the Repair of Cloacal
Anomalies and Congenital Adrenal Hyperplasia
By Alaa F. Hamza, Hesham A. Soliman, Sameh A. Abdel Hay, Ashraf A. Kabesh, and Mossad M. Elbehery
Cairo, Egypt
Purpose: Urogenital sinus mobilization has facilitated mark-
edly the vaginal reconstruction in cases of cloacal anomalies.
Application of the same technique in cases of congenital
adrenal hyperplasia has resulted in cosmetic and functional
improvement.
Methods: Total urogenital sinus mobilization was used as a
part of the repair in 9 patients: 6 with congenital adrenal
hyperplasia (4 high and 2 mid vaginal confluence), and 3 with
cloacal anomalies; sinus mobilization to the level of the
symphesis pubis allowed the vagina to reach the perineum
in all cases. Their age ranged from 6 to 8 months, and
follow-up ranged from 6 months to 2 years. In the former
group, the mobilized sinus was split dorsally and used as an
anterior vaginal flap. Cases of cloaca needed weekly dilata-
tion early postoperatively.
P EÑA HAS DESCRIBED the technique of urogenital
sinus mobilization in cases of cloaca to avoid
separation of the vagina from the urethra, thus reducing
the operating time and avoiding vaginal ischemia and
stenosis.1 This was applied by Richard Rink who ex-
tended its use in patients with urogenital sinus anomalies
with high or mid vaginal confluence.2
Since his description, total urogenital mobilization
(TUM) was used in various surgical procedures includ-
ing exstrophy and penile agenesis.3
We used this technique for cases of cloaca and con-
genital adrenal hyperplasia (CAH). Our results in cor-
rection at age 6 to 8 months were encouraging with better
cosmetic outcome and fewer complications than other
techniques.
MATERIALS AND METHODS
This study included 9 patients, 3 with cloaca and 6 with urogenital
sinus anomaly caused by congenital adrenal hyperplasia (CAH) in
which the vagina was high (supra-sphincteric) in 4 cases and in mid
position in 2 cases.
The age of correction was between 6 and 8 months, and preoperative
From Ain Shams University, Cairo, Egypt.
Presented at the 34th Annual Meeting of the Pacific Association of
Pediatric Surgeons, Kyoto, Japan, April 4-8, 2001.
Address reprint requests to Alaa F. Hamza MD, FRCS, 45, Ramsis
St, 11341 Heliopolis, Cairo, Egypt.
Copyright © 2001 by W.B. Saunders Company
0022-3468/01/3611-0013$35.00/0
doi:10.1053/jpsu.2001.27943
1656 Journal of Pediatric Surgery, Vol 36, No 11 (November), 2001: pp 1656-1658
Results: All patients are below 3 years of age, so urinary
control assessment is not yet objective; however, all mothers
reported dry intervals. Examination 6 months postopera-
tively showed wide vagina and excellent cosmetic appear-
ance in all cases.
Conclusions: Total urogenital mobilization provides an eas-
ier way for vaginal reconstruction. The improved cosmetic
appearance and the absence of vaginal stenosis provided by
this technique is a major advantage in the management of
these difficult surgical situations.
J Pediatr Surg 36:1656-1658. Copyright © 2001 by W.B.
Saunders Company.
INDEX WORDS: Urogenital sinus mobilization, cloaca, con-
genital adrenal hyperplasia.
assessment included identification of the sinus length radiologically
(genitogram in CAH and distal loopogram in cloaca) and cystoscopi-
cally before surgery. All patients had rectal washes before surgery; patients
with CAH received the necessary medications (eg, corticosteroids).
Patients with urogenital sinus anomaly had a Fogarty catheter placed
in the vagina for easier identification. In patients with cloaca, the prone
position was used after total body preparation, but in patients with
CAH, the lithotomy position was used in all patients. (Rink used the
prone position to separate the vagina form the urethra,2,4 but we found
it not necessary even in high confluence cases.) In cloaca patients TUM
mobilization starts after separation of the rectum as previously de-
scribed.5 In patients with urogenital syndrome we start by the ordinary
incisions previously described by Hendren (Fig 1).6
Clitoral reduction is performed using Kogan’s technique of subtu-
nical resection7: degloving the phallic skin to the bifurcation of the
corpora, applying 2 transfixing sutures to each corpora, 2 lateral
incisions of the tunica to avoid neurovascular damage, and then the
erectile tissue is resected. A glans reduction is performed if needed; the
glans is sutured to the undersurface of the pubis, and the phallic skin is
split dorsally to be used as labia minora. A strip of urethral plate is not
left attached because it is divided during mobilization of the sinus. A
posterior inverted wide U-shaped flap is raised, and the rectum is
dissected carefully posteriorly in the midline (sometimes a finger is
inserted into the rectum to facilitate dissection) after creation of an
ample space posteriorly; mobilization of the sinus is started by applying
silk 5-0 traction sutures. In cloacal cases, the rectovaginal space is
wide, and mobilization is easy, but in CAH posterior dissection is
performed first to facilitate the location of the vagina. The incision is
performed around the urogenital sinus, and dissection is carried out
carefully in the retro pubic space until the sinus is felt easily mobile, at
this moment the circular muscles of the sphincter could be seen, and
dissection should not injure these muscles (Fig 2).
In cloacal cases, this dissection usually is enough, and the vagina
could be pulled to the perineum, so the ventral part of the sinus is
trimmed, and closure of muscles is continued as described previously.1
TOTAL UROGENITAL SINUS MOBILIZATION
Fig 1. Skin incisions.
In CAH after adequate mobilization (depending on the length of the
sinus and the vagina), in mid cases, about 2 cm length usually is
needed; in high cases, further dissection is performed until the Fogarty
catheter is felt posteriorly with adequate vision. The posterior vaginal
wall then is opened, and the anterior vaginal wall is separated from the
urethra, which then is closed in 2 layers.
In cases of mid confluence (2 cases), the sinus is split laterally, the
ventral part is used to make a mucosal-lined vestibule, and the dorsal
one is used to form the anterior vaginal wall after trimming. In high
confluence cases (7 cases), the sinus is split dorsally, and the flap is
used as an anterior vaginal wall flap (Passerini flap8,9). The posterior
vaginal wall is incised, and the U flap then is sutured to it allowing a
wide vaginal opening (Fig 2).
The phallic skin is brought down forming the labia minora, and its
ends are sutured to the sides of the vagina. Labia majora flaps as formed
from the initial V-Y incisions and are brought posteriorly to form a
normal-looking vestibule. A Foley catheter is left in the bladder for 1
week, and a vaginal tube is left as a drain for 5 days.
Patients with cloaca had a routine weekly vaginal dilatation for 3
months, and in CAH cases examination under general anesthesia is
done 3 moths postoperatively.
RESULTS
Cosmetic appearance is excellent in all cases, one case
required labia minora trimming 1 year after surgery. All
patients with CAH are continent to urine with dry inter-
vals. A 3-year-old cloaca patient has dry intervals with
Fig 2. Surgical technique. (A)
Fogarty catheter is placed in the va-
gina, dotted line shows the area of
dissection. (B) Mobilization of the
urogenital sinus up to the undersur-
face of the pubis. (C) Separation of
the vagina. (D) Closure of the urethra.
(E) Dorsal slitting of the sinus to be
used as an anterior vaginal flap. (E)
Flaps sutured to the vagina.
1657
normal voiding pattern (normal sacrum); the other 2
(abnormal sacrum) are still too young for judgement.
Voiding cystourethrogram (VCUG) was done for 1 pa-
tient with CAH and the continent cloaca patient to study
urethral and bladder neck anatomy; the result was com-
parable with normal children with similar age group.
Vaginal examination 3 months postoperatively showed a
wide orifice and normal mucosal lining without urethro-
vaginal fistulae in any case.
DISCUSSION
Various techniques are used to repair cases of urogen-
ital sinus and cloacal anomalies.5,6,10 In anomalies with
high or mid vaginal confluence, separation of the vagina
usually was difficult because of a narrow operative field,
deep position of the vagina, and its close relation to the
external urethral sphincter. The cosmetic results were not
always acceptable because of the appearance of vaginal
orifice not exactly in the vestibule. Added to this, vaginal
stenosis, urethrovesical fistulae, and pooling of urine in
the vagina were troublesome in some cases.11
The ingenious contribution by Alberto Peña using
TUM as a part of the cloacal repair has provided an
easier technique to approach these anomalies.1 This pro-
cedure has been applied by Rink et al2,9 in repairing cases
of urogenital sinus anomalies with several advantages:
shorter operating time, easier reconstruction of the ure-
thra and vagina, and excellent cosmetic outcome. The
principle of TUM was applied in several anomalies,
including female exstrophy, with good results.3,12,13
We used this principle successfully in 9 cases of
cloaca and CAH. The advantages of using this technique
were wide operative field, easier separation of the va-
gina, and accurate closure of the urethra. Follow-up
1658
examinations showed no vaginal stenosis or urethrovag-
inal fistula in any of our patients.
In spite of using this technique, some cases still may
require vaginal replacement,11 however, non of our pa-
tients required replacement of the vagina. Critical issues
were raised regarding urethral length after TUM with the
subsequent possibility of urinary incontinence.14,15 In our
cases as in others,9 dry intervals were noted in all cases
with no sacral anomalies. Assessment of long-term con-
tinence will need further follow-up.
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mobilization: Expanded applications. Br J Urol 83:820-822, 1999
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urogenital sinus: Early Perineal prone approach without division of the
rectum. J Urol 158:1293-1297, 1997
5. Peña A: Anorectal malformations. Semin Pediatr Surg 4:35-47,
1995
6. Hendren WH, Donahoe PK: Correction of congenital abnormal-
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plasty: A safe modification of existing techniques. J Urol 130:746-748,
1983
8. Passerini-Glazel G: A new 1-stage procedure for clitorovagino-
HAMZA ET AL
With regard to urethral length, because of excessive
virilization of cases of CAH, the length of the urogenital
sinus usually is much longer than the normal urethra. We
usually slit dorsally the distal part of the urogenital sinus
leaving a normal proximal urethral length. This was
confirmed by cystoscopy and VCUG in some of our
cases. Although longer follow-up is needed for the as-
sessment of continence in those cases, we recommend
this technique of TUM as a part of treatment of these
technically demanding anomalies.
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anomalies. Dial Pediatr Urol 23:1-2, 2000
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