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Current practice in feminizing surgery for congenital
adrenal hyperplasia; A specialist survey
Francisca Yankovic
a
, Abraham Cherian
a,
*, Lisa Steven
a
, Azad Mathur
b
,
Peter Cuckow
a
a
Department of Paediatric Urology, Great Ormond Street Hospital NHS Foundation Trust, Great Ormond Street,
London WC1N 3JH, UK
b
Department of Paediatric Surgery, Norfolk and Norwich University Hospital, Colney Land, Norwich NR4 7UY, UK
Received 27 September 2012; accepted 25 March 2013
KEYWORDS
Congenital adrenal
hyperplasia;
Specialist survey;
Feminizing
genitoplasty
Abstract Aim: To present the outcome of an online survey of the current practice in femi-
nizing surgery for congenital adrenal hyperplasia (CAH) among the specialists attending the
IVth World Congress of the International Society of Hypospadias and Disorders of the Sex Devel-
opment (ISHID), 2011.
Material and methods: An online survey covered 13 individual questions regarding the manage-
ment and surgical techniques for 46XX CAH patients. All delegates attending the conference
were invited to complete this anonymous survey. The data was analysed by three of the
authors.
Results: A total of 162 delegates had registered for the conference and 60% of them were
paediatric surgeons or paediatric urologists. 65 delegates completed the online survey. Early
surgery, before the age of two years, is preferred by 78% of the surgeons and most of them
would include clitoroplasty, vaginoplasty and labioplasty. The most frequent surgical tech-
nique used for the clitoroplasty is the partial excision of the corpora cavernosa and the skin
flap or “U flap” vaginoplasty. Routine vaginal dilatations after puberty are advocated by 28%
of the delegates. More than 75% report good outcomes.
Conclusions: Within the limitations of the methodology of this survey, this study suggests that
there is agreement in many aspects related with the surgical treatment for 46XX CAH. Self
reported outcomes are satisfactory for most of the respondents.
ª2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
* Corresponding author. Tel.: þ44 20 74059200; fax: þ44 20 78138260.
E-mail address: Abraham.cherian@gosh.nhs.uk (A. Cherian).
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Please cite this article in press as: Yankovic F, et al., Current practice in feminizing surgery for congenital adrenal hyperplasia; A
specialist survey, Journal of Pediatric Urology (2013), http://dx.doi.org/10.1016/j.jpurol.2013.03.013
1477-5131/$36 ª2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.jpurol.2013.03.013
Journal of Pediatric Urology (2013) xx,1e5
Introduction
46 XX congenital adrenal hyperplasia (CAH) is the most
frequent diagnosis in girls who present with genital virili-
zation. In line with the new classification for the disorders
of sex development (DSD) it falls into the group of 46XX,
DSD [1]. Current surgical techniques aim to create normal
looking female external genitalia, promote adequate
bladder emptying, allow unimpeded egress of menstrual
fluid, and ultimately allow a normal reproductive life in
adulthood [2]. Due to the complex nature of this condition,
the best surgical management and timing of intervention
remain the subject of controversy. Some studies have
challenged the approach of early surgery in childhood
reporting high complication and revision rates in adoles-
cence [3,4]. However, other studies have shown good out-
comes [5,6]. The aim of this study is to report the current
practice among specialists who attended the IVth World
Congress of the International Society for Hypospadias and
Disorders of the Sex Development (ISHID) meeting held in
London in 2011.
Material and methods
An online survey was set up by the organizing committee
and all delegates registered for the conference were
invited to complete an anonymous online questionnaire
regarding their practice in feminizing surgery for 46XX CAH.
The survey covered 13 individual questions relating to
clitoral and vaginal surgery, pre-operative care, surgical
technique and outcome. The survey questionnaire may still
be viewed online at www.jotform.com/form/11473104676.
Verbal reminders to complete the questionnaire were given
during the conference and further emails sent to partici-
pants in the months after the conference. The survey
closed at the end of November 2011.
Results
Demographics
A total of 162 delegates registered and attended the IVth
World Congress of ISHID Meeting in London, September
2011. The delegates were from all 5 continents (Fig. 1) and
the majority were paediatric surgeons (30%), and paediatric
urologists (30%). Considering that 52 delegates had a non-
surgical background, including paediatricians, clinical
nurse specialists and psychologists, we estimate that 55% of
the delegates who may be involved in CAH surgery had
answered our survey. These professionals were from all
over the world, but with a major representation from
the UK (28%) and Europe (46%). No regional differences of
significance in responses were observed among the
respondents.
The number of cases done per year was less than 5 for
50% of the surgeons, 36% perform between 6 and 20 cases
per year and 14% operate more than 20 patients per year.
The majority (87%) of respondents adopt a multidisci-
plinary approach in the treatment of CAH patients and
almost 70% would always include psychological assessment.
When surgical aspects were analysed, 61 delegates were
eligible to respond. The majority (46/61) of this group
would include clitoroplasty, labioplasty and vaginoplasty
and a small percentage (4/61) would do vaginal surgery
alone. Regarding the surgical timing (Fig. 2), most support
surgery in the first two years of life (48/61). Of those who
promote early surgery, 28 would perform all the compo-
nents of feminizing surgery at the same time and 20 would
delay some components. Four respondents advocate only
late surgery.
Clitoral surgery
Most of the surgeons perform a partial excision of the
corporal bodies. 19% prefer a corporal preserving approach,
including surgeons that bury the corpora and those who
perform a split-dismembered clitoroplasty. The self-
reported outcomes from the respondents that perform
clitoral surgery are very good in 57%, good in 26% and 16%
poor (Table 1).
Vaginal surgery
55 surgeons responded to the section on vaginal surgery and
the survey shows that a wide variety of techniques are in
use (Table 2). A group of 16 surgeons (26%) admit to using
only the skin flap for vaginoplasty. The other 39 re-
spondents (64%) use more than one technique for vaginal
reconstruction. The survey infers that skin flap is the most
popular technique, followed by partial and total urogenital
mobilization. Bowel substitution with colon (17%) or ileum
(7%) was also mentioned among the techniques that might
be required in some patients. 28% of the respondents
include vaginal dilatations after puberty following vaginal
surgery. The self reported outcomes for vaginal surgery are
very good or good for 74% of the delegates, disappointing
for 18%, and 8% report several complications and frequent
need of revision surgery (Table 1).
Discussion
Surveys of surgical practice provide useful information
about the current trends in the management of some clin-
ical conditions especially with regard to controversial is-
sues. For patients with 46XX CAH, this can be even more
helpful as single centre experience is usually limited. There
are many problems with this methodology, which include
the relatively small number of respondents and the bias of
the specialties that have chosen to attend this meeting.
Delegates were asked to answer this survey during the
meeting and not from their workplaces. This can also lead
to inaccuracy regarding the number of cases treated per
year and carries a clear risk of a more positive perception
in their results, as this questionnaire only reflects self-
reported outcomes.
The consensus statement on the management of
intersex disorders published in 2006 suggests only surgeons
with expertise in the care of children and specific training
in surgery of DSD should perform these procedures [1].
To achieve standardization in surgical practice, man-
agement of these complex cases should be performed by
experienced professionals in a multi-disciplinary setting.
As stated by Arul et al., in 1998, centralisation improves
2 F. Yankovic et al.
+MODEL
Please cite this article in press as: Yankovic F, et al., Current practice in feminizing surgery for congenital adrenal hyperplasia; A
specialist survey, Journal of Pediatric Urology (2013), http://dx.doi.org/10.1016/j.jpurol.2013.03.013
expertise as well as aiding in developing support services
[7]. In order to increase surgical expertise and outcomes for
the management of patients with DSD, it would be desir-
able to reduce the number of centres that treat these pa-
tients. However, this disorder is rather uncommon and
occurs in approximately 1 in 16,000 births [3], and half of
the respondents perform less than 5 cases per year.
The phenotype of children with 46XX CAH is variable;
therefore surgical treatment should be individualized. The
decision-making process should include a multidisciplinary
team and the family. This concept seems to be widely
accepted by the respondents in our survey.
The classical surgical technique includes three main
steps: clitoroplasty, vaginoplasty, and perineoplasty (labio-
plasty). Clitoral surgery has evolved from clitoral amputa-
tion to more preservative procedures including plication,
concealing, recession and reduction [2]. The majority of the
respondents excise the corpora cavernosa, preserving the
neurovascular bundle. This technique, described first by
Goodwin and later modified by different authors, has gained
popularity due to the theoretical preservation of the
sensitivity of the clitoral tissue [5]. A novel technique
described by the group from Toronto aims to preserve all of
the clitoro-phallic tissue with the potential for reversibility.
The corporeal sparing dismembered clitoroplasty includes
the glans dissection with the neurovascular bundle, division
of the corpora in two and the placement of each of the
hemicorpora inside the labial scrotal folds [10]. A small
group of delegates are using this technique.
Outcomes for clitoroplasty were reported as very good
or good by 84% of the delegates. The experience from Milan
with 82 consecutive cases, managed with partial excision of
the corporal bodies, reports 100% success in terms of size of
the clitoral reduction and cosmesis [6]. However, no in-
formation is provided regarding clitoral sensation or patient
satisfaction after the surgery. Less encouraging outcomes
are reported from long-term follow up by Crouch and col-
leagues, as genital sensitivity and sexual function were
affected after clitoral surgery [3,4]. However comparison
with contemporary surgery is difficult without accurate
information on what the initial surgery entailed [9].
Vaginoplasty techniques have evolved to improve the
cosmetic and functional aspect of female genitalia [5]. The
technique described by Fortunoff using an inverted U flap
from perineal skin to form the posterior wall of the vagina
[11], with some subsequent modifications, is one of the
most popular, and 78% of delegates declare using this
Figure 1 Geographical distribution of delegates completing the CAH survey.
Figure 2 Feminizing genitoplasty, timing of surgery.
Table 1 Self reported outcomes for genitoplasty.
Reported outcomes
for clitoral surgery
(percentage of
surgeons)
Reported outcomes
for vaginal surgery
(percentage of
surgeons)
Very
Good
58% 52%
Good 26% 22%
Poor 16% 26%
Current practice in feminizing surgery for CAH 3
+MODEL
Please cite this article in press as: Yankovic F, et al., Current practice in feminizing surgery for congenital adrenal hyperplasia; A
specialist survey, Journal of Pediatric Urology (2013), http://dx.doi.org/10.1016/j.jpurol.2013.03.013
technique. However, 64% admit to using more than one
surgical technique. This could reflect the nature of this
condition, in which some cases will present with a vaginal
opening near the perineal skin surface, whereas others may
have a higher confluence. For the ones with a higher
confluence, some authors believe that a partial or total
urogenital mobilization (TUM) will achieve better out-
comes. The TUM, first described by Pen
˜a for cloacal mal-
formations, can provide better mobilization; however it has
also been associated with higher risk of sphincter damage
and incontinence [2,8]. It is concerning to report that 24%
of delegates considered bowel substitution as an option
for vaginal reconstruction in patients with 46XX CAH. This
does not reflect the standard practice and perhaps only
reproduces a bias in the structure of our survey, where
delegates could not explain the situations in which they
would use a specific technique.
The long-term outcomes of vaginal reconstruction,
published in 2006 by Wilcox and colleagues, show compli-
cation rates as high as 73%, with revision surgery in 11%
[12]. However, excluding vaginal discharge and the neces-
sity of vaginal dilation, the complication rate falls to 18%.
Another study has demonstrated that around 77% of the
patients who had vaginal reconstruction in childhood
require further surgery to permit penetrative intercourse
[3]. In our survey, 74% of the respondents consider their
outcomes as very good or good. Nevertheless, nearly a third
of them will use routine vaginal dilatations after puberty.
The main controversies surrounding feminizing surgery
for patients with 46XX CAH include: long-term clitoral
sensitivity, potential reversibility to male phenotype after
early surgery, substitutive tissues to replace or improve the
deficient vagina, and the timing of surgery [2,3,8,10].
Many authors have advocated early surgical treatment,
between 2 and 6 months of age. Potential benefits of per-
forming early surgery include better quality of genital tis-
sues, better vascularization secondary to postnatal
maternal oestrogens, and possibly reduction in the anxiety
of parents and children regarding the appearance of their
external genitalia [2,8]. Detractors of early surgery base
their approach on the concern that there is insufficient
evidence that early surgery benefits gender identity, po-
tential impairment in clitoral sensitivity and high rates of
revision for vaginoplasties. Despite several publications in
this area, there is a lack of evidence to suggest that the late
approach can achieve better outcomes, and this should
be balanced against the good results reported by Hutson’s
group from Melbourne with early surgery [13]. Long-term
follow up literature is limited and conclusions cannot be
drawn until that evidence becomes available.
Summary
Within the limitations of the methodology of this survey,
this study suggests that there is agreement in many aspects
related to the surgical treatment for 46XX CAH patients
among the delegates attending the ISHID 2011 conference.
More than three quarters perform surgery before the age of
two years. Considering the risk of a positive bias in the
perception of surgical outcomes, the self-reported results
are considered good and this correlates with most of the
surgical series published.
Conflict of interest statement
None.
Funding source
None.
Ethical approval
Institutional approval obtained.
References
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Table 2 Surgical technique used for vaginoplasty.
Only one surgical
technique (number
of delegates,
NZ16)
More than one
surgical technique
(number of
delegates, NZ45)
V flap (skin)
vaginoplasty
840
Partial/total UG
mobilization
530
Ileal substitution 0 4
Colonic
substitution
38
4 F. Yankovic et al.
+MODEL
Please cite this article in press as: Yankovic F, et al., Current practice in feminizing surgery for congenital adrenal hyperplasia; A
specialist survey, Journal of Pediatric Urology (2013), http://dx.doi.org/10.1016/j.jpurol.2013.03.013
[10] PippiSalle JL, Braga LP, Macedo N, Rosito N, Bagli D. Corporeal
sparing dismembered clitoroplasty: an alternative technique
for feminizing genitoplasty. J Urol 2007;178(4 pt 2):1796e800.
[11] Fortunoff S, Lattimer JK, Edson M. Vaginoplasty technique for
female pseudohermaphrodites. Surg Gynecol Obstet 1964;
118:545e8.
[12] Burgu B, Duffy PG, Cuckow P, Ransley P, Wilcox DT. Long-term
outcome of vaginal reconstruction: comparing techniques and
timing. J Pediatr Urol 2007;3(4):316e20.
[13] Lean WL, Deshpande A, Hutson J, Grover SR. Cosmetic and
anatomic outcomes after feminizing surgery for ambiguous
genitalia. J Pediatr Surg 2005 Dec;40(12):1856e60.
Current practice in feminizing surgery for CAH 5
+MODEL
Please cite this article in press as: Yankovic F, et al., Current practice in feminizing surgery for congenital adrenal hyperplasia; A
specialist survey, Journal of Pediatric Urology (2013), http://dx.doi.org/10.1016/j.jpurol.2013.03.013