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Journal of Pediatric Urology (2015) xx, 1e2

Video bank

Complete female epispadia: The case for


perineal approach

Antonio Macedo Jr, Marcela Leal da Cruz, Rodrigo Trivelato,


Federal University of São Paulo, Gilmar Garrone, Bruno Leslie, Diego Estevam Oliveira,
São Paulo, Brazil Geórgia Rubiane Souza, Valdemar Ortiz

Correspondence to:
Summary Results and discussion
M. Leal da Cruz, Rua Maestro
Cardim, 560/215 01323-000 São
The patient had an excellent cosmetic result, and
Paulo, São Paulo, Brazil, Tel.: Introduction reported continence of 3 h period with mild leakage.
þ55 11 32870639; fax: þ55 11 Complete female epispadia (CFE) is a rare congen- The perineal infrapubic approach offers the possi-
32873954 ital anomaly occurring in 1 of 500,000 live births. bility of restoring cosmesis and providing resistance
The goals of CFE management include achieving in one surgery precluding the need for abdominal
ma_celaleal@yahoo.com.br continence, while protecting the kidneys, and bladder neck surgery.
(M. Leal da Cruz) creating functional, cosmetic external genitalia.

Received 19 August 2014 Patient and methods


Accepted 21 August 2014 We demonstrate in this video the steps of the sur-
Available online xxx gery and present midterm follow-up of a 6-year-old
child with CFE.

Introduction to allow further bladder neck and proximal


urethra tightening. We tubularized the mucosal
Complete female epispadia (CFE) is a rare flap excising redundant mucosal tissue adapting
congenital anomaly occurring in 1 of 500,000 the urethra to a 6F catheter. Subsequent over-
live births [1]. CFE results from failure of the lapping and folding was done of adjacent
dorsal urethra to tubularize. Most patients muscular and subcutaneous tissue in two layers.
present with urinary incontinence and a geni- Labia minora plasty and monsplasty were per-
tal deformity. This deformity is characterized formed and bifid clitoris was approximated in
by a bifid clitoris, depressed mons pubis, and the midline. Vulvoplasty was completed and the
ill-defined labia minora, which is covered by distal urethral meatus was anchored cranially
smooth glabrous skin, and terminates anteri- inferiorly to the clitoral hood.
orly with the corresponding half of the bifid
clitoris. The vagina and internal genital organs Results and discussion
are usually normal. The goals of CFE manage-
ment include achieving continence, while The patient had an uneventful postoperative
protecting the kidneys, and creating func- course, and the Foley tube was removed after
tional, cosmetic external genitalia. 7 days. Present follow-up is 1 year, the parents
We aimed to demonstrate a perineal infra- reported urinary continence of 3 h, but the
pubic approach to treat this rare abnormality. patient still has some stress incontinence. A
urodynamic study revealed a VLPP of 20 cmH2O
at 100 mL, with a bladder capacity of 180 mL.
Patient and methods One-stage perineal urethroplasty increases
the urethral and bladder neck resistance,
The patient presented at the age of 6 years. We mainly by elongating the urethral segment and
defined and created a flap of the mucosal ure- folding adjacent muscular and subcutaneous
thral plate located from the epispadic meatus tissue [2]. It is a simple and effective method
up to the anterior supra-pubic area between for development of both continence and
both bifid clitoris. The flap was moved inferiorly cosmesis [2e4], avoiding initial abdominal
and gave access to the pubic bone. A careful surgery that could be reserved for the failure
infra-pubic dissection of the flap was performed cases. A definitive continence procedure can

http://dx.doi.org/10.1016/j.jpurol.2014.08.013
1477-5131/ª 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: Macedo A, et al., Complete female epispadia: The case for perineal approach, Journal of Pediatric
Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2014.08.013
+ MODEL
2 A. Macedo et al.

be performed when BC is later deemed adequate. The use References


of bulking agents could also be an intermediate attempt
before deciding on open surgery. [1] Gearhart JP, Mathews RI. Exstrophyepispadias complex. In:
Wein AJ, Kavoussi LR, Novick AC, et al., editors. Campbell-
Funding Walsh Urology. 10th ed., vol. 4. Philadelphia: Saunders; 2012.
p. 3325e78. chapt 124.
[2] Bhat LA, Bhat M, Sharma R, Saxena G. Single-stage perineal
None. urethroplasty for continence in female epispadias: a pre-
liminary report. Urology 2008;72:300e4.
Conflict of interest [3] Lazarus J, van den Heever A, Kortekaas B, Alexander A. Female
epispadias managed by bladder neck plication via a perineal
approach. J Pediatr Urol 2012;8:244.
None. [4] Kajbafzadeh AM, Talab SS, Elmi A, Tourchi A. Single-stage
subsymphyseal cystoscopic-guided bladder neck plication and
Appendix A. Supplementary data urethrogenitoplasty in female epispadias: presentation of long-
term follow-up. BJU Int 2011;108(7):1200e7.
Supplementary data related to this article can be found at
http://dx.doi.org/10.1016/j.jpurol.2014.08.013.

Please cite this article in press as: Macedo A, et al., Complete female epispadia: The case for perineal approach, Journal of Pediatric
Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2014.08.013

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