M E D iC A L
JO U R N A L
Albany 8raz, M aria C ristina M artoni Andrade
T r a n s s p h in c te r ic a n o r e c ta l
r e c o n s tr u c tio n o f a m b ig u o u s
g e n ita lia : a n in n o v a tiv e a p p r o a c h to n e o v a g in o p la s ty ,
pioneer in Brazil
Intersexuality
Unit of the Children
'sHospital Darcy Vargas
of Sao Paulo - Sp, Brazil
T h e a u th o rs , b a s e d u p o n p re v io u s e x p e rie n c e in th e tre a tm e n t o f te n c h ild re n w ith c lo a c a l a b n o rm a litie s , a m o n g w h ic h tw o w ith fe m a le p s e u d o h e rm a p h ro d itis m w ith c lo a c a , h e re b y p re s e n t a p io n e e r e x p e rie n c e in B ra z il, w ith th e "posterior sagittal transsphinctericanorectalurethrovaginoplasty" a s u rg ic a l a p p ro a c h to n e o v a g in o p la s ty d e s c rib e d b y "C ripps/Pena", w h ic h w a s u ti-liz e d fo r th e re c o n s tru c tio n o f th e g e n ita lia o f th re e in te rs e x u a te d a d o le s c e n ts w ith p ro te c tiv e c o lo s to m y , w h o p re s e n te d a n u ro g e n ita l s in u s , h ig h v a g in a l im p la n ta tio n a n d a n o rm a l re c tu m . O n e p a tie n t w a s a n a d re n a l fe m a le p s e u d o h e rm a p h ro d ite a n d tw o w e re m a le p s e u d o h e rm a p h ro d ite s . T h e a u th o rs p re s e n t a b rie f re p o rt o n th e th re e c a s e s , d e s c rib e th e s u rg ic a l p ro c e d u re , re la te o n th e a n a to m y o f th e u ro g e n ita l s in u s a n d d is c u s s s u rg ic a l in d ic a tio n s , d ia g n o s is a n d re s u lts , c o m p a rin g th e m to th e re v ie w e d in te rn a tio n a l lite ra tu re .
U N IT E R M S : A m b ig u o u s g e n ita lia . P s e u d o h e rm a p h ro d itis m . P o s te rio r s a g itta l tra n s s p h in c te ric a n o re c ta l a p p ro a c h . In te rs e x u a lity . V a g in o p la s ty .
IN T R O D U C T IO N
M
t h e f e m a l ea n a g e m e n t o f t h e a m b i g u o u sg e n d e r w i t h u r o g e n i t a lg e n i t a l i as i n u s ( U G S ) ,( A G ) , o f h i g h v a g i n a l a g e n e s i s a n d n o r m a l r e c t u m , h a sb e e n a m a j o r c h a l l e n g e t o t h e p e d i a t r i c s u r g e o n , f o r w h o m
t h e m a i n o b j e c t i v e i s t o p r o p i t i a t e a n o r m a l s e x u a l f u n c t i o n
t o b e a r e r s o f t h i s t y p e o f g e n i t a l i a , i n c l u d i n g a p o s s i b i l i t y
o f p r o c r e a t i o n , a s w e l l a s r e a l l y u p o n t h e o b j e c t i v e s o f
m a n a g e m e n t r e g a r d i n g c o n t r o l o f d e f e c a t i o n a n d u r i n a t i o n ,
A ddress for correspondence: Albany Braz
R ua C uba tao, 1.209 - Vila M ariana Sao Paulo / SP - Brasil - C EP 04013-044
a n d a l s o o f t h e a n a t o m i c a l a n d e s t h e t i c a l a s p e c t s , w h i c h
a r e v e r y i m p o r t a n t .
A t t h e C h i l d r e n 's H o s p i t a l " D a r c y V a r g a s " ( C R D V ) ,
w e h a d t h e o p p o r t u n i t y t o a c q u i r e a n d c o l l e c t i n t h e s e l a s t
s e v e n t e e n y e a r s ( 2 - 6 , 2 2 , 2 3 ) s o m e e x p e r i e n c e w i t h t h e
d i a g n o s i s a n d c l i n i c a l - s u r g i c a l m a n a g e m e n t o f A G ,
e s p e c i a l l y w i t h t h o s e c a s e s r e q u i r i n g r e c o n s t r u c t i o n o f t h e
e x t e r n a l g e n i t a l i a , t h u s c o m p l e m e n t i n g t h e e x p e r i e n c e w i t h
t h e c o r r e c t i o n o f a n o r e c t a l a b n o r m a l i t i e s a n d w i t h t h a t o f
o u r t e n c a s e s o f c l o a c a l a b n o r m a l i t i e s ( C A ) ( 2 ) w h i c h w e r e
s u b m i t t e d t o a
"posterior sagittal
anorectalurethrovagino-plasty"
( 7 , 1 3 , 1 4 , 1 6 , 1 8 ) ( P S A R U V P ) . I t f u r t h e r i n d u c e du s t o a p p l y t h i s p r o c e d u r e w i t h m o d i f i c a t i o n s ( C r i p p s /
P e n a 's t e c n i q u e ) ( 2 1 ) t o i n t e r s e x u a t e d p a t i e n t s , t o c o r r e c t
t h e U G S w i t h h i g h v a g i n a l i m p l a n t a t i o n ( a g e n e s i s ) a n d
n o r m a l r e c t u m .
I n t h e c u r r e n t p a p e r w e s h a l l p r e s e n t o u r e x p e r i e n c e
w i t h t h e s u r g i c a l a p p r o a c h
posterior
sagittal
transsphinc-tericanorectalurethrovaginoplasty
( P S T S A R U V P ) f o r1023
T a b le 1
O rig in s a n d a g e s o f p a tie n ts ; d a te s o f s u rg e rie s a n d e v o lu tio n IN T E R S E X U A L IT Y U N IT - C H IL D R E N 'S H O S P IT A L "D A R C Y V A R G A S "
T R A N S S P H IN C T E R IC A N O R E C T A L U R E T H R O V A G IN O P L A S T Y D A T E
C A S E O R IG IN A G E A T S U R G . P S T S A R U V P E V O L U T IO N
S A N T A N A V A R G E M 1 3 Y E A R S 2 /2 /9 3 C O N T IN E N T
(M IN A S G E R A IS ) 0 9 M O N T H S S T O O U U R IN E
2 N O V A R E D E N Q A o 1 3 Y E A R S 4 /2 7 /9 3 C O N T IN E N T
(B A H IA ) 0 6 M O N T H S S T O O U U R IN E
3 P A R A G U A S S U P A U L . 1 5 Y E A R S 7 /8 /9 3 C O N T IN E N T
(s A o
P A U L O ) 1 0 M O N T H S S T O O U U R IN Eneovaginoplasty (N V P) described by
"Cripps/Peiia"
(21),w here the cases and procedures shall also be discussed
com paring and relating them to the w orldw ide incidence.
M A T E R IA L S A N D M E T H O D S
From 1978 to 1995, at the C H D V , 235 patients w ith
abnorm alities of the external genitalia w ere adm itted and
163 (69.5% ) underw ent som e type of surgery, of these in
three cases (1.8% ) the recon truction of U G S w ith high
vaginal agenesia and norm al rectum w as undertaken by
PSTSA R U V P w ith protective colostom y.
Tw o patients cam e from other States of the country
(66.6% ) and one cam e from a city in the interior of the
Sao Paulo State (33,3% ), Table 1.
A t the tim e of surgery the ages of the three patients
w eres respectively, of 13 years and 9 m onths, 13 years
and 6 m onths, 15 years and 10 m onths, w ith a m edian of
14 years and 4 m onths, Table 1.
R egarding race, one w as negro and tw o w ere w hite,
Table 2.
A s for diagnosis, w e had one patient w ith fem ale
pseudoherm aphroditism (FPH ), bearer of adrenal
congenital hyperplasia (A C H ) and
"sadie diabetes"
(saltloosing) resulting from defect of the enzim e
21-hydroxylases.
O ther tw o w ith m ale pseudoherm aphroditism (M PH )
incom plete syndrom e type I, that is, one w as fam ilial M PH
T a b le 2
Id e n tific a tio n a n d d ia g n o s is o f th e p a tie n ts
IN T E R S E X U A L IT Y U N IT - C H IL D R E N 'S H O S P IT A L "D A R C Y V A R G A S " T R A N S S P H IN C T E R IC A N O R E C T A L U R E T H R O V A G IN O P L A S T Y
C A S E R E G IS T E R P A T IE N T R A C E D IA G N O S IS B IR T H D A T E 1s t C O N S
1 2 3
070468
106260
1 4 4 5 5 8TA R N SST F A F
B .
W .
W .FPH
(ACH)
M PH
(TFS)
M P H(TRS)
05/03/79 10/06/79 0 9 /1 7 /7 7
03/16/81 07/04/83 0 5 /1 5 /8 7
B R A Z , A . & A N D R A D E , M .C .M . - T ra n s s p h in c te ric a n o re c ta l re c o n s tru c tio n o f a m b ig u o u s
g e n ita lia : a n in n o v a tiv e a p p ro a c h to n e o v a g in o p la s ty , p io n n e r in B ra z il
1024
F ig u re 1 - P ro n e g e n u p e ito ra l p o s itio n , "o p e n k n ife " fe a tu re a d e q u a te fo r th e p e rfo rm a n c e o f P S T S A R U V P .
d e r i v e d f r o m a p e r i p h e r a l d e f ic ie n c y
o f a n d r o g e n ic r e c e p to r s , a ls o c a lle d
"testicular
feminilization
syndrome"
( T F S ) a n d th e o th e r w a sa M P H w ith a
"testicular
regres-sion syndrome"
( T R S ) T a b le 2 .T h e n e o v a g in o p la s tie s w e r e
u n d e r ta k e n b y o n e o f u s ( A .B .) a f te r
a s s e s s m e n t o f h o r m o n e le v e ls ,
d e te r m in a tio n o f th e k a r y o ty p e , X
-r a y te s ts ( u r o g e n ita lg r a p h y o r
u r in a r y u r e th r o c y s to v a g in o g r a p h y )
a n d e n d o s c o p ic o n e s ( u r e th r o c y s to
-v a g in o s c o p y ) ; p e r f o r m a n c e o f a
s ig m o id o s to m y ; v e s ic a l c a th e te r is m
w ith a
"Foley"
p r o b e th r o u g h th eU G S ; r e c ta l ir r ig a tio n w ith a
s o lu tio n o f " I o d in e p o v id o n e " a n d a
p r o f ila c tic th e r a p y w ith a n tib io tic s
d u r in g th e p r e a n d p o s to p e r a to r y
s ta g e s , ( A m ik a c in a n d M e tr o
-n id a z o le ) w e r e a ls o u n d e r ta k e n .
F ig u re 2 - P o s te rio r s a g itta l s a c ro p e rin e a l
in c is io n w ith s e p a ra tio n o f th e a n u s a n d o f
th e a n te rio r a n d p o s te rio r w a lls o f th e
re c tu m w h ic h a c q u ire a b iv a lv u la r fe a tu re (tra n s s p h in c te ric a n o re c ta l in c is io n ), w h ic h p e rm it v ie w in g o f th e p e c tin e a l lin e a n d o f th e u ro g e n ita l s in u s .
F ig u re 3 - S a g itta l in c Is Io n o f th e
u ro g e n ita l s in u s p o s te rio r w a ll a n d o f th e v a g in a l o p e n in g n e a r th e ju n c tio n w ith th e u re th ra .
D E S C R IP T IO N
O F T H E
P R O C E D U R E
"Posterior sagittal
transsphincte-ricanorecta lureth ro vaginoplasty"
T h e p a tie n t is s e t in k n e e - c h e s t
( g e n u p e c to r a l) p r o n e p o s itio n " o p e n
ja c k k n if e " f e a tu r e ( F ig u r e I).
S u r g e r y b e g in s w ith a
lo n g itu d in a l p o s te r io r s a g itta l
in c is io n o f th e s k in ( a t th e
in te r g lu te a l m id lin e ) , s ta r tin g f r o m
th e s a c r o c o c c y g e a l r e g io n p a s s in g
th r o u g h th e a n u s e x te n d in g u n til th e
m e a tu s o f th e u r o g e n ita l s in u s .
T h e in c is io n is s a g itta lly
d e e p e n e d
"always
using
the
electrocautery
and keeping
to the
middle
line, so as to avoid nerve
!
injuries
and also
to divide
into
symrnetric halves",
th e a n u s e le v a to rm u s c le ,
"muscle
complex"
a n c ljo in tly w ith th e e x te r n a l a n a l
1025
Figure 4 -
Maneuvres with multiple sutureson the borders of the vagina (sutures 6-0), while separating it from the urethra with the electrocautery.
Figure 6 -
Patient (case 1) with ambiguous genitalia at the time of the firstconsultation, note clitoromegaly and rugated labioscrotal folds.
Figure
5 - Final aspect of reconstructions of the urethra,vagina, anus, rectum and perineum by means of PSTSARUVP with a vulvar introitus corresponding to the width of a N.20 Hegar bougie.
s p h in c te r , th e p o s te r io r a n d a n te r io r w a lls o f th e r e c tu m . B y th is p r o c e d u r e th e r e c tu m . w a lls g a in a b iv a lv u la r f e a tu r e a n d th r o u g h th e m w e p e r f o r m a ll s u r g ic a l d is s e c tio n s ( F ig u r e 2 ) .
T h e in c is io n w ill e x te n d a n d p e n e tr a te d e e p e r u n til it r e a c h e s th e u r o g e n ita l s i n u s , w h ic h is e x p o s e d , a n d o p e n e d s a g itta lly o n its p o s te r io r w a ll, th e n c e th e v a g in a a n d u r e th r a c a n b e v is u a liz e d a n d id e n tif ie d ( F ig u r e 3 ) .
F o r a b e tte r v is u a liz a tio n , id e n tif ic a tio n a n d d is s e c tio n o f a ll th e s e s tr u c tu r e s w e u tiliz e th e " W e itla n d e r " s e p a r a to r a n d th e e le c tr o m io s tim u la to r , f u r th e r , in o r d e r to a v o id in ju r ie s a n d e n h a n c e d is s e c tio n o f th e v a g in a , " 6 -0 " s u tu r e s a r e a p p lie d to its b ~ i'd e r s , w h ile it is b e in g s e p a r a te d f r o m th e u r e th r a w ith th e e le c tr o c a u te r y ( F ig u r e 4 ) . T h e v a g in a is d is s e c te d f r o m th e u r e th r a , w ith th e e le c tr o c a u te r y , u n ti I its f u ll s e p a r a tio n f r o m th e b la d d e r , n e a r to th e p e r ito n e a l r e f le c tio n . .
T h e la te r a l f ix a tio n s tr a n d s o f th e v a g in a a r e p a r te d a n d a f te r f u ll lib e r a tio n , m o b iliz a tio n a n d e lo n g a tio n o f th e v a g i n a o n e p r o c e e d s to th e r e c o n s tr u c tio n o f th e n e o -u r e th r a ( u r e th r o r h a p h y , in c lu d in g th e s p h in c te r m u s c l~ s ) b y tw o p la n e s o f s u tu r e s , in te r r u p te d o v e r th e " F o le y " p r o b e , w ith r e ta r d e d a b s o r p tio n s u tu r e s " 5 - 0 " .
A f te r w a r d s w e u n d e r ta k e th e s u tu r e f r o m th e v a g in a l o s tiu m to th e p e r in e u m , q u ite c lo s e to th e u r e th r a l n e o -m e a tu s .
R e c o n s tr u c tio n o f th e p e r in e a l b o d y a n d o f a ll m u s c le s ( a n u s e le v a to r m u s c le s a n d th o s e o f th e
"muscle
complex")
is p e r f o r m e d , a id e d b y e le c tr o m io s tim u la tio nBRAZ, A. & ANDRADE, M.C.M. - Transsphinctericanorectal reconstruction of ambiguous genitalia: an innovative approach to neovaginoplasty, pionner in Brazil
1026
~ r~ t1W ~ iID iW ;~ :t*~ % ii~ :B :!~ :~ :.r::1= ~ ,*~ :~ w ~ ~ ~ :::;~ ;t~ t~ ~ t;m .ri~ = W U ~ ;:;~ ~ :lli:m m zW ::W :;~ ~ w ili~ ~ ~ *~ ~ ~ :~ ~ w ;~ t:~ ~ :rm ~ ~ "*;:~ :i~ ~ ;~ f:~ *:"*~ ~ ~ :~ i'ID '~ *:r~ :lK r:ill~ ~ K ~ % ~ :~ £:£rlm & J~ ~ ~ t:,*~ ~ 1~ m = Z W '*~ w ~ ~ ~ :~ 1~ ti*~ ~ ~ t~ f::'Jm t;;;-~ U ~ 1tt~ ;~ M :::;~ :;~ m w ~ r$"~ -ill:~ j~ ~ tt:ti.~ ~ ~ llt~ ~ ~ $~ :J
T a b le 3
S u rg e rie s u n d e rta k e n o n p a tie n t, c a s e n .1
S U R G E R IE S O N C A S E N . 1
(T.A.R.)
T a b le 4
S u rg e rie s u n d e rta k e n o n p a tie n t, c a s e n .2
S U R G E R IE S O N C A S E N . 2
(N.S.S. T.)
1 C L iT O R O P L A S T Y
2 S IG M O ID O S T O M Y
3 N E O V A G IN O P L A S T Y
(PSTSARUVP)
4 C L O S U R E O F T H E C O L O S T O M Y
(1 0 /4 /8 2 )
(1 /2 1 /9 3 )
(2 /2 /9 3 )
(7 /2 5 /9 3 )
1 C L iT O R O P L A S T Y
2 G O N A D E C T O M Y
(TESTICLE)
3 S IG M O ID O S T O M Y
4 N E O V A G IN O P L A S T Y
(PSTSARUVP)
5 C L O S U R E O F T H E C O L O S T O M Y
(1 /1 6 /8 4 )
(4 /1 5 /8 6 )
(2 /9 /9 3 )
(4 /2 7 /9 3 )
(6 /1 /9 3 )
and utilization of interrupted sutures w ith the sam e "5-0" thread. T he rectum posterior and anterior w alls, w hich had been divided, are also brought together by continuous sutures w ith "4-0" thread, and finally the circular fibers of the external anal sphincter are brought closer together using "3-0" thread and the skin sutured by continuous interderm ic stitch using "5-0" thread, w hich shall be protected by special adhesi ve tape.
T he "F oley" probe is w ithdraw n betw een the eight and tenth day post surgery, and the enlargem ent of the vagina w ith
"Hegar"
bougies until the vagina reaches bougie gauge n. 20 is initiated (F igure 5), at this point the colostom y w ill be closed.C A S E R E P O R T S
Case 1
T .A .R ., w as adm itted at C H D V w ith A G (F igure 6) w hen one year and ten m onths old, and w as assigned to the m ale gender (D .A .R .), having been subm itted to diagnostic investigation, and w as proven to be F P H w ith A C H , T able 2.
A t clinical exam ination exhibited clitorom egaly, urethral m eatus at the penile shaft, labioscrotal fusion, rugated labioscrotal folds of increased volum e, deprived of pubogenital hair grow th; inguino-scrotal gonads w ere 'not palpable, but at rectal exam ination the w om b w as
palpable.
L aboratory assessm ents show ed presence of B aar corpuscles, karyotype 46X X , high plasm a level of the 17a-hydroxyprogesterone (over 5ug/m l), the bone m aturation corresponded to 28 m onths and urogenital X -ray and urethrovaginoscopy disclosed presence ofU G S and w om b.
S he w as reassigned to the fem ale gender.
S urgical interventions for the correction of the external genitalia are reported on T able 3.
A t 15 years of age she is continent for stool and urine, continues recei vi ng 9a-fl uorhydrocorti sone and prednisone; she has regular m enses, norm al breasts and pubo-genital hair grow th (F igure 7). Is using a vaginal inflatable stent.
Case
2N .S .S .T ., w hen she w as 3 years and nine m onth old w as adm itted at the C H D V w ith A G and w as assigned to
F ig u re 7 - F in a l a s p e c t o f p a tie n t g e n ita lia
(c a s e 1 ) a t im m e d ia te p o s to p e ra to ry b y
P S T S A R U V P , o b s e rv e th e u re th ra l p ro b e , th e v u lv a r in tro itu s a n d th e a n u s w ith th e ir s u tu re s .
1 0 2 7
Figure 8 - Patient (case 2) with am biguous genitalia post bilateral gonadectom y and clitoroglanuloplasty.
th e fe m a le g e n d e r, h a v in g b e e n su b m itte d to d ia g n o stic
in v e stig a tio n , a n d p ro v e n to b e
M P H
(S T F ), T a b le 2 .A t c lin ic a l e x a m in a tio n , p re se n te d c lito ro m e g a ly ,
u re th ra l m e a tu s a t th e p e n ile sh a ft, la b io sc ro ta l fu sio n ;
n o rm a l la b ia m a jo ra w ith n o fo ld s, n o p u b o g e n ita l h a ir
g ro w th ; in g u in a l g o n a d s w e re p a lp a b le , b u t a t re c ta l
e x a m in a tio n n o w o m b w a s p a lp a b le (F ig u re 8 ).
A t la b o ra to ry a sse ssm e n ts, B a a l' c o rp u sc le s w e re
a b se n t, k a ry o ty p e w a s 4 6 X Y , a n d p la sm a le v e ls o f 1 7 a
-h y d ro x y p ro g e ste ro n e w e re n o rm a l, b o n e m a tu ra tio n w a s
p ro p o rtio n a l to th e c h ro n o lo g ic a l a g e a n d u ri n a ry
u re th ro c y sto g ra p h y a n d u re th ro v a g in o sc o p y c o n firm e d th e
p re se n c e o f U G S , w ith o u t w o m b .
T h e u n d e rta k e n su rg e rie s a re re p o rte d o n T a b le 4 .
A t 1 5 y e a rs o f a g e is c o n tin e n t fo r sto o l a n d u rin e ,
still re c e iv in g e stro g e n th e ra p y " P re m a rin " h a s n o m e n se s
b u t h a s n o rm a l b re a sts a n d h a ir g ro w th (F ig u re s 5 a n d 9 ).
Is u sin g a v a g in a l in fla ta b le ste n t.
Figure 9 - Final aspect of the patient genitalia (case 2) after reconstruction by the PSTSARUVP approach, notice the urethra, vagina and anus in their typical positions (arrows).
Case 3
F .A .F ., a t 9 y e a rs a n d 8 m o n th s o f a g e w a s a d m itte d
a t th e C H D V w ith a d ia g n o sis o f v a g in a l a g e n e sia , a n d
h a v e b e e n su b m itte d to d ia g n o stic in v e stig a tio n ,
a sc e rta in in g th a t sh e w a s
M P H
(T R S ), T a b le 2 .Table 5
Surgeries undertaken on patient, case n.3
SURG ERIES O N CASE N. 3
(T.A.R.)
1
2
3
4
5
LAPARO TO M Y
(GONADAL
AGENESIS)
SIG M O IDO STO M Y
NEO VAG INO PLASTY
(PSTSARUVP)
NEO VAG INO SIG M O IDPLASTY
(PSTSARUVSP)
CLO SURE O F THE CO LO STO M Y(1/4/88)
(6/29/93)
(7/8/93)
(11/16/93)
(2/17/94)
BRAZ, A. & ANDRADE, M .C.M . - Transsphinctericanorectal reconstruction of am biguous genitalia: an innovative approach to neovaginoplasty, pionner in Brazil
1028
F ig u r e 1 0 - P a tie n t ( c a s e 3 ) w ith a m b ig u o u s g e n ita lia b e fo r e P S T S A R U V P , n o tic e th e r u g a te d la b ia m a jo r a a n d th e p e r in e a l s c a r o f a p o s s ib le a tte m p t to n e o v a g in o p la s ty ( a r r o w ) .
At
clinical
examination
presented
normal
clitoris,
"hypospadic"
urethral
meatus,
a
scar
at the
labioscrotal
fusion,
rugated
labia
majora,
no-pubogenital
hair
growth,
groin
gonads
were not palpable,
nor, at
rectal examination
was the womb.
Laboratory
assessments,
showed
absence
of
Baar
. corpuscles,
karyotype
46XY,
. plasma
levels
of
T7a-hydro-xyprogesterone
were
normal,
bone maturation
was proportional
to
the
chronological
age
and
urinary
urethrocystography
and
urethrovaginoscopy
confirmed
that she was a M PH (TRS), Table 2.
The undertaken
surgeries
are
reported
on Table
S.
At
18 years
of age
she
is
recei vi ng
estrogen
therapy,
"Premarin ",
and she has normal
breasts,
and
pubogenital
hair
growth,
(Figure
10), however
has
no menses.
She is urine
and stool continent.
Due to a
retraction of the vagina, she has undergone
another surgery
and a neovagina
has been constructed
with a loop of the
sigmoid,
Table
S.
R E S U L T S
In our casuistry,
one patient
(33%) was reassigned
to the female gender (Case
1) (Figures
6 and 7).
Clitoris
of patients
(cases
1 and 2) submitted
to
clitoroplasty
have retained
their full sensitivity.
Secondary
sexual characteristics
are well developed
in all patients,
as a result of the estrogen
therapy
in the
case of two patients
with M PH
(cases 2 and 3). In case N.
1 with
ACH,
administration
of prednisone
and
9u-fluorhydrocortisone
is still continued.
W ith respect to menstruation
only case 1 (FPH)
had
regular
menses
(33%),
while the other two have uterine
agenesis.
All patients
have urinary
and fecal continence.
Regarding
to the enlargement
of the vagina, in all of
the cases
the opening
permits
the passage
of a N. 20
Hegar's
bougie, (Figure
5),
but at night these patients
are
using a vaginal inflatable
stent to avoid
"for lack of usage"
stenosis or retraction
of the introitus.
Finally,
the esthetic
features
of the genitalia
are
extraordinary
(Figures
7 and 9), but up to now, no patient
has undertaken
sexual intercourse
(Figures
5,
7, 9 and 10)
and there is no record
of death,
nor interruption
of the
follow-up.
On Table 6, is recorded
the statistical
summary
of
results.
D IS C U S S IO N
The urogenital
sinus associated
to a normal
rectum
is usually seen in patients
with AG, and is more frequent
in cases of FPH
with or without
ACH
and in a shorter
degree in M PH
or other forms of intersexuality,
such as
in
mixed
gonadal
dysgenesia
or
even
in
true
hermaphroditism.
There is a large spectrum
in the constitution
of the
urogenital
sinus, ranging
from
a vagina
communicating
with the urethra close to the perineum,
whose treatment
is
much
simpler
(7,13,14,16,18,21),
to those
in higher
1029
T a b le 6
S ta tis tic a l s u m m a ry o f th e e v o lu tio n a n d re s u lts
IN T E R S E X U A L IT Y U N IT - C H IL D R E N 'S H O S P IT A L "D A R C Y V A R G A S "
T R A N S S P H IN C T E R IC A N O R E C T A L U R E T H R O V A G I N O P L A S T Y
C A S U IS T R Y (3 P A T IE N T S )
N . O F C A S E S S U R G E R IE S - E V O L U T IO N A N D R E S U L T S
1 S E X U A L R E A S S IG N M E N T
1 M E N S E S
1 U R E T H R O V A G IN A L F IS T U L A
1 R E T R A C T IO N O F T H E V A G IN A
2 C L iT O R O P L A S T Y
3 F E C A L C O N T IN E N C E
3 U R IN A R Y C O N T IN E N C E
3 N E O V A G IN O P L A S T Y
(PSTSARUVP)
1
NEOVAGINOSIGMOIDPLASTY(PSTSARUVSP)
P E R C E N T A G E
3 3 .3 %
3 3 .3 %
3 3 .3 %
3 3 .3 %
6 6 .6 %
100.0%
100.0%
100.0%
3 3 .3 %positions and nearer to the vesicular cervix, w here they
require m ore careful dissection and m ore difficult
approaches to neovaginoplasty (19,21).
For the repair of such virilized external genitalia of
the fem ale gender w ith U G S and norm al rectum , a
reductive clitoroglanuloplasty (4) and aN V P are required.
Furtherm ore, this last one, w hen vaginal im plantation w as
in the upper urethra, had a good indication for som e type
of abdom inal-perineal approach. H ow ever, this tends to be changed after the em ergence of the posterior sagittal
sacroperineoplasty (PSSP), a technique updated in 1982
by Pena (8), to redress rectoanal abnorm alities, w ho also used it for the correction of cloacal persistences (20) and
for post-surgical fecal incontinence (15). O n the other
hand, a m edian vaginal atresia w ith anal im perforation (9) and a penile agenesis (24) w ere respectively corrected by
the
"Kraske"
and"Cripps"
approaches. A ll of thes~ approaches gained such a m om entum that they w ereindicated for children bearing perineal disorders requiring
further surgeries (17), in the com plex abnorm alities of fem ale genitalia (11,12), for recto-vaginal (1),
rectoperineal (19) and urethral (10,19) traum a, in the
ressection of rectum tum ors and repeated surgeries of the
H irschsprung disease (19) and currently reached a peak w ith the l:lse of the PSTSA R U V P, in cases of intersexuality
w ith U G S and norm al rectum (19,21), thus bringing forth
a com plete m odification of the surgical panoram a
regarding the m anagem ent of the perineal region and to the external genitalia, m oreover, often avoiding
laparotom y.
Pena (19,21,25) points out that to utilize the
"Posterior
sagittal
transsphinctericanorectalplasty"
(PSTSA R P), a fecal deviation by m eans of a
sigm oidostom y in separate openings is required, to avoid
com plications or severe consequences. Such requirem ent
is confirm ed by the author him self (19) in his bibliographic
survey of 228 cases, subm itted to PSTSA R P w ith no
colostom y. In this group there w as a 13% contam ination
w hereas those operated w ith protective colostom y did not
exhibit such com plications.
In the literature review ed by Pena (19), only 20
cases subm itted to PSTSA R P w ith colostom y w ere
recorded, and to those w e add our results (cases 1, 2 and 3) sum m ing up 23 reported cases. It is perceivable that
the association ofPSTSA R P w ith colostom y m eets som e
reluctance. N evertheless, w e agree w ith Pena (19), that
13% of com plications is a high rate and as an alternative
to avoid colostom y w e suggest a
J
0days long parenteral
nutrition, however associated
to a careful cleaning
of the
whole bowel.
PSTSA R P, know n as the
"Cripps"
or"Kraske"
(19) approach, had been essentially perform ed in adults until D im ler (9), Stealer (24) and Pena (19, 21) began to
utilize it in children to achieve a better access to the vagina
or to U G S, as w ell as to the inferior urinary tract.
The evolution of the posterior sagittal pathw ay has an interesting history: beginning in 1835, w hen
"A M U SSA T" perform ed for the first tim e the posterior
sagittal incision, follow ed by "V ER N EU IL" em ployed the coccygectom y in 1873 for the first tim e, and "C R IPPS"
B R A Z , A . & A N D R A D E , M .C .M . - T ra n s s p h in c te ric a n o re c ta l re c o n s tru c tio n o f a m b ig u o u s g e n ita lia : a n in n o v a tiv e a p p ro a c h to n e o v a g in o p la s ty , p io n n e r in B ra z il
1030
~{~~W~~(t.~~~ti;W;t':f%mJ~~~:~:;*7t:'n:~Wi1r&<.m:r~¥J!W:i::;:lillrZ.:W.ID:~t~j~m~t?:i*~~~:~?#;~t*;t~g*~r::*$~'i:illi:lli::"1fir:t:~tr:f:~&"f"iWlliWimtWi~~~~:z]:~*~~;mti&::~~i~&~~~tr~:$.~~¥@,~~~:if~~~~~*l~~*~i1.r:::~i;::~rZ~:!~:~~*~~~~kn*~!m::m~':.;'Wm~~~~~1:&.fi*J~*1~:~
who in
1876
employed the PSTSARP
for the first time
(19). This PSTSARP
has been adapted by a series of
authors like Kraske
(19)
in
1885,
Bevan in
1917,
Dimler
in
1986 (9)
and Pena
1992 (19).
Now, when for the first
time in Brazil, we are utilizingPSTSARPforintersexuated
girls, Table 7.
The PSTSARP
access
pathway
represents
an
extraordinary
and marvellous
approach,
with integral
preservation of the defecation and urinary controls, because
the whole procedure is performed precisely on the midline,
without nerve impairment
(21).
Such pathway enhances
further separation of the
vagina from the urethra
and bladder,
affords
a better
mobilization
from
the vagina
to the perineum,
and
moreover,
avoids the utilization
of perineal skin flaps.
Therefore there is an almost total absence of stenosis of
the vulvar introitus
(21).
On the other hand, Pena
(21)
does not recommend
it for UGS
with low vaginal
implantation.
Regarding intersexuated
patients, only three cases
of FPH operated by PSTSARP
are reported. One was an
ACH
(19,21),
and the others were non adrenal FPH
(21).
Our patient (case 1) was a FPH, bearer of A CH
with
"salt loosing"
and the others (cases 2 and 3) were
MPH type I, incomplete syndrome; One was TFS and the
other TRS.
The ages of the three patients with FPH quoted by
Pena
(19, 21)
were
16,17
and
24
months. Conversely, our
cases are adolescents with
13, 14
and
15
years of age.
We believe that currently
puberty
is the ideal age
to perform this type of surgery, the NVP, because of the
socioeconomic shortcomings inherent to our environment,
as well as because of the effect of the estrogenic
action,
benefical
for the development
of the vagina
and
cicatrization
of the vulvar plasty (18). Although,
at this
age, as a reslt of the growth of the pelvis, the distance
from the beginning of the UGS Uunction of the vagina
with the urethra) to the perineum (common channel of the
urogenital
sinus) becomes
longer,
thus turning
more
difficult the mobility from the vagina to the perineum as
compared to that of a child operated at two years of age.
In the last one obviously, the common channel is shorter,
therefore
surgery
is easier;
However,
when girls are
submitted
to an early
surgery
they are in danger
of
T a b le 7
H is to ry o f th e p o s te rio r s a g itta l in c is io n
IN T E R S E X U A L IT Y U N IT - C H IL D R E N 'S H O S P IT A L "D A R C Y V A R G A S " T R A N S S P H IN C T E R IC A N O R E C T A L U R E T H R O V A G IN O P L A S T Y
H IS T O R Y
A U T H O R S Y E A R S S U R G E R IE S C O U N T R IE S
A M U S S A T M A T A S P E N A C R IP P S K R A S K E B E V A N D IM L E R P E N A B R A Z B R A Z
1835
1897
1982
1907
1916
1917
1986
1992
1993
1994
"PSARP/ARA"
"PSARP/ARA"
"PSARP/ARAC'
"PSTSARP/RCA"
"PSTSARP/ RCA"
"PSTSARP/RCA"
"PSTSARP/VAC'
"PSTSARP/AGC'
"PSTSARP/AGC'
"PSTSARUVSP/AGC'
F R A N C E
U N IT E D S T A T E S M E X IC O
E N G L A N D G E R M A N Y U N IT E D S T A T E S U N IT E D S T A T E S U N IT E D S T A T E S B R A Z IL
B R A Z IL
(PSARP)
(ARA)
(ARAC)
(PSTSARP)
(RCA)
(VAC)
(AGC)
(PSTSARUVSP)
P O S T E R IO R S A G IT IA L A N O R E C T A L P L A S T Y
A N O R E C T A L A B N O R M A L IT Y
A N O R E C T A L A B N O R M A L IT Y IN C H IL D H O O D
P O S T E R IO R S A G IT IA L T R A N S S P H IN C T E R IC A N O R E C T A L P L A S T Y
R E C T U M C A N C E R IN T H E A D U L T
V A G IN A L A T R E S IA IN C H IL D H O O D
A M B IG U O U S G E N IT A L IA IN C H IL D H O O D
P O S T E R IO R S A G IT IA L T R A N S S P H IN C T E R IC A N O R E C T A L U R E T H R O V A G IN O S IG M O ID P L A S T Y
1031
presenting
a stenosis of the vulvar introitus due to lack of
vaginal
enlargement
and will probably
be submitted
to
another surgery in puberty (2,18). Even though Pena (21)
states that
"widening
is not required and that the vaginal
opening has shown to be adequate
2years after surgelY".
Nevertheless
we can state that upon reaching puberty
they
shall not have sufficient
width of the vulvar introitus to
achieve
adequate
sexual intercourse
and will require a
vulvar plastic surgery.
C O N C L U S IO N S
This is an original presentation
as it reports on the
first time a PSTSARUVP
was performed
in the country,
on intersexuated
patients.
W e present the first case to be published
in global
literature of a girl submitted
to PSTSARUVP, bearer of
FPH
with ACH that was reassigned for the female gender
as well as the fourth patient with ACH to be submitted to
this approach
(case
I ) .In the reviewed
literature no mention was found
on
MPH,
type I incomplete
form (TFS or TRS) operated
by PSTSARUVP,
therefore
they
will
be the
first
PSTSARUVP for
MPH
to be published worldwide (cases
2 and 3).
Excluding
repair
of the rectoanal
and cloacal
malformations,
there
is no Brazilian
or maybe
Latin-American
recorded
report
on
the
utilization
of
PSTSARUVP
with protective
sigmoidostomy
for the
correction of AG
(intersexuated
patients)
with UGS, high
vaginal implantation
(agenesis)
and normal rectum, be it
for
FPH
or any form of M PH.
Finally, these patients, unfortunately
born with some
abnormality of the genitalia can nowadays
to be available
with a management
that will hopefully
warrant
a better
quality
of life (with less morbidity,
etc.) and is mainly
related
to sexual
intercourse
and to the possibility
of
procreation,
thanks to the approach known as the "Cripps
or Kraske" procedure
(19), and reintroduced
by Pena as
the
PSTSARUVP,
improved
by the
utilization
of
electrocautery
and electromiostimulation
devices,
thus
shortening
time
of surgery
and
enhancing
results,
principally
if performed
by surgeons
with a reasonable
experience
on the posterior sagittal pathway.
A K N O W L E D G M E N T S
To Dr. Zan Mustachi
pediatrician
and geneticist
of
the CHDV, who during these 17 years has devoted himself
to the care and aided in the guidance of the patients quoted
in this and other published papers. To Drs. Jose Carn~vale
and Roberto Dias for the endoscopic
assays, attendance
and dedication
to the patients.
RESUMO
In tr o d u c ;a o : O s a u to r e s , b a s e a d o s n u m a p r e v ia e x p e r ie n c ia n o tr a ta m e n to d e d e z c r ia n 9 a s c o m a n o m a lia s d e c lo a c a , d e n tr e e s ta s , d u a s c o m p s e u d o - h e r m a fr o d itis m o fe m in in o c o m c lo a c a , a p r e s e n ta m a g o r a u m a e x p e r ie n c ia c o m a
"transesfincteroano-retouretrovaginoplastia
sagital posterior"
c o m c o lo s to m ia p r o te to r a , te c n ic a d e n e o v a g in o p la s tia d e s c r ita p o r"Cripps/Pena",
q u e fo i a p lic a d a n a c o r r e 9 a o d a s g e n ita lia s d e tr e s a d o le s c e n te s in te r s e x u a d a s q u e a p r e s e n ta v a m"sinus"
u r o - g e n ita l, im p la n ta 9 a o v a g in a l a lta e r e to n o r m a l. M a te r ia l e M ( H o d o s : U m a p a c ie n te e r a p o r ta d o r a d e p s e u d o - h e r m a fr o d itis m o fe m in in o a d r e n a l e d u a s e r a m p s e u d o - h e r m a fr o d ita s m a s c u lin o , tip o I s fn d r o m e in c o m p le ta . A p r e s e n ta m u m b r e v e r e la to d o s tr e s c a s o s , d e s c r e v e m a te c n ic a c ir u r g ic a u tiliz a d a , d is c o r r e m s o b r e a a n a to m ia d o"sinus'
u r o - g e n ita l e d is c u te m a s in d ic a c ;:6 e s c ir u r g ic a s , o s d ia g n 6 s tic o s e o s r e s u lta d o s , c o m p a r a n d o - o s a o s d a lite r a tu r a m u n d ia l p e s q u is a d a .B R A Z , A . & A N D R A D E , M .C .M . - T r a n s s p h in c te r ic a n o r e c ta l r e c o n s tr u c tio n o f a m b ig u o u s g e n ita lia : a n in n o v a tiv e a p p r o a c h to n e o v a g in o p la s ty , p io n n e r in B r a z il
1032
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