reference hipospadia

8/20/2019 Reference Hipospadia

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http://emedicine.medscape.com/article/1015227-overview#showall

http://www.brazjurol.com.br/novembro/baskin !21 !2".pd

http://www.lipteh.com/$tud%-&otes/'ro(enital/$nod(rass)20method.pd

Hypospadias Repair: An Overview of the Actual TechniquesRamnath Subramaniam , F.R.C.S., F.E.A.P.U., 1 Anne Francoise Spinoit , M.D., 2 and Piet Hoebe e , M.D., Ph.D. 2

http://www.ncbi.nlm.nih.(ov/pmc/articles/*+, 121 5/

!ac "round

H#pospadias is an abnorma$it# o% anterior urethra$ and peni$e de&e$opment in 'hich the urethra$ openin" isectopica$$# $ocated on the &entra$ aspect o% the penis pro(ima$ to the tip o% the "$ans penis, 'hich, in thiscondition, is sp$a#ed open. )he urethra$ openin" ma# be $ocated as %ar do'n as in the scrotum or perineum.)he penis is more $i e$# to ha&e associated &entra$ shortenin" and cur&ature, ca$$ed chordee, 'ith morepro(ima$ urethra$ de%ects.

)he ear$iest medica$ te(t describin" h#pospadias dates bac to the second centur# CE and 'as the 'or o%*a$en, the %irst to use the term. Durin" the %irst mi$$ennium, the primar# treatment %or h#pospadias 'asamputation o% the penis dista$ to the meatus. Since that time, man# ha&e contributed to de&e$opment o% modern

http://emedicine.medscape.com/article/1015227-overview#showallhttp://www.brazjurol.com.br/novembro/baskin_621_629.pdfhttp://www.lipteh.com/Study-Notes/Urogenital/Snodgrass%20method.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Subramaniam%20R%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Subramaniam%20R%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Spinoit%20AF%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Hoebeke%20P%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Hoebeke%20P%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC3312185/http://www.brazjurol.com.br/novembro/baskin_621_629.pdfhttp://www.lipteh.com/Study-Notes/Urogenital/Snodgrass%20method.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Subramaniam%20R%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Spinoit%20AF%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pubmed/?term=Hoebeke%20P%5Bauth%5Dhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC3312185/http://emedicine.medscape.com/article/1015227-overview#showall


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h#pospadias repair. More than + di%%erent t#pes o% repairs ha&e been described in the medica$ $iterature. A$thou"h most reports ha&e been in the past - #ears, most basic techni ues 'ere described o&er a centur#a"o.

Modern anesthetic techni ues, %ine instrumentation, sutures, dressin" materia$s, and antibiotics ha&e impro&edc$inica$ outcomes and ha&e, in most cases, a$$o'ed sur"ica$ treatment 'ith a sin"$e/sta"e repair 'ithin the %irst

#ear o% $i%e on an outpatient basis.

Pathoph#sio$o"#

H#pospadias is a con"enita$ de%ect that is thou"ht to occur embr#o$o"ica$$# durin" urethra$ de&e$opment,bet'een 0 and 2 'ee s "estation. )he e(terna$ "enita$ structures are identica$ in ma$es and %ema$es unti$ 0'ee s "estation the "enita$s de&e$op a mascu$ine phenot#pe in ma$es primari$# under the in%$uence o%testosterone. As the pha$$us "ro's, the open urethra$ "roo&e e(tends %rom its base to the $e&e$ o% the corona.

)he c$assic theor# is that the urethra$ %o$ds coa$esce in the mid$ine %rom base to tip, %ormin" a tubu$ari3ed peni$eurethra and median scrota$ raphe. )his accounts %or the posterior and midd$e urethra. )he anterior or "$anu$ar

urethra is thou"ht to de&e$op in a pro(ima$ direction, 'ith an ectoderma$ core %ormin" at the tip o% the "$anspenis, 'hich cana$i3es to 4oin 'ith the more pro(ima$ urethra at the $e&e$ o% the corona. )he hi"her incidence o%subcorona$ h#pospadias supports the &u$nerab$e %ina$ step in this theor# o% de&e$opment.

5n 2 , !as in proposed a modi%ication o% this theor# in 'hich the urethra$ %o$ds %use to %orm a seam o%epithe$ium, 'hich is then trans%ormed into mesench#me and subse uent$# cana$i3es b# apoptosis orpro"rammed ce$$ resorption. 617Simi$ar$#, this seam theoretica$$# a$so de&e$ops at the "$anu$ar $e&e$, and theendoderm di%%erentiates to ectoderm 'ith subse uent cana$i3ation b# apoptosis.

)he prepuce norma$$# %orms as a rid"e o% s in %rom the corona that "ro's circum%erentia$$#, %usin" 'ith the"$ans. Fai$ure o% %usion o% the urethra$ %o$ds in h#pospadias impedes this process, and a dorsa$ hooded prepuceresu$ts. 8n rare occasions, a "$anu$ar c$e%t 'ith intact prepuce ma# occur, 'hich is termed the me"ameatus

intact prepuce 9M5P: &ariant.

Chordee 9&entra$ cur&ature o% the penis: is o%ten associated 'ith h#pospadias, especia$$# more se&ere %orms.)his is thou"ht to resu$t %rom a "ro'th disparit# bet'een the norma$ dorsa$ tissue o% the corpora$ bodies and theattenuated &entra$ urethra and associated tissues. Rare$#, the aborti&e spon"iosa$ tissue and %ascia dista$ to theurethra$ meatus %orms a tetherin" %ibrous band that contributes to the chordee. 9See the ima"e be$o'.:


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Se&ere peni$e chordee. ;ote e(treme &entra$cur&ature o% peni$e sha%t.

)he $ocation o% the abnorma$ urethra$ meatus c$assi%ies the h#pospadias. A$thou"h se&era$ di%%erentc$assi%ications ha&e been described, most ph#sicians use the one proposed b# !arcat and modi%ied b# Duc ett,'hich describes the $ocation o% the meatus a%ter correction o% an# associated chordee. 62, +7Descripti&e $ocationsdescribed inc$ude the %o$$o'in"<

• Anterior 9"$anu$ar and subcorona$:• Midd$e 9dista$ peni$e, midsha%t, and pro(ima$ peni$e:•

Posterior 9penoscrota$, scrota$, and perinea$:

)he $ocation is anterior in = > o% cases, midd$e in 2 >, and posterior in + > the subcorona$ position is themost common o&era$$. 9See the ima"es be$o'.:


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Pro(ima$ sha%t h#pospadias. ;ote de%icient &entra$ %ores in, b$ind urethra$pit at "$anu$ar $e&e$, and $i"hter pi"mented urethra$ p$ate e(tendin" to true meatus at pro(ima$ sha%t $e&e$.

Pro(ima$ sha%t h#pospadias. ;ote t#pica$ dorsa$hood o% %ores in and &entra$ peni$e s in de%icienc#.


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Penoscrota$ h#pospadias. ;ote associated &entra$ chordee and trueurethra$ meatus $ocated at scrota$ $e&e$.

Epidemio$o"#

Fre uenc#

United States

H#pospadias occurs in appro(imate$# 1 in e&er# 2= ma$e births in the United States. )he incidence doub$ed%rom 1?@ to 1??+. A$thou"h some ha&e su""ested that this doub$in" actua$$# re%$ects increased reportin" o%minor "rades o% h#pospadias, increases in se&ere h#pospadias ha&e a$so been noted. 5ncreasin" sensiti&it# o%sur&ei$$ance s#stems a$one cannot e(p$ain this t'o%o$d increase. Ho'e&er, some reports ha&e $in ed theincreased rate o% h#pospadias in bo#s born premature$# and sma$$ %or "estationa$ a"e and bo#s 'ith $o' birth'ei"ht.

International

5n se&era$ countries, the incidence o% h#pospadias ma# be risin". 5n "enera$, the %re uenc# seems rather

constant, at .2- per 1 $i&e births in Me(ico and Scandina&ia and 2.11 per 1 $i&e births in Hun"ar#.6 7

Morta$it#BMorbidit#

)he treatment %or h#pospadias is sur"ica$ repair. H#pospadias is "enera$$# repaired %or %unctiona$ and cosmeticreasons. )he more pro(ima$$# ectopic the position o% the urethra$ meatus, the more $i e$# the urinar# stream isto be de%$ected do'n'ard, 'hich ma# necessitate urination in a seated position. An# e$ement o% chordee cane(acerbate this abnorma$it#. Ferti$it# ma# be a%%ected. )he abnorma$ de%$ection o% e4acu$ate ma# prec$ude


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e%%ecti&e insemination, and si"ni%icant chordee can prec$ude &a"ina$ insertion o% the penis or can be associated'ith inherent$# pain%u$ erections.

A$thou"h the most minor %orms o% h#pospadias are insi"ni%icant in ph#sio$o"ic terms, the# too ma# merit repairon the basis o% the potentia$ ps#cho$o"ica$ stress associated 'ith ha&in" a "enita$ anoma$#.

Race

)he incidence o% h#pospadias is hi"her in 'hites than in b$ac s, and the condition is more common in those o%e'ish and 5ta$ian descent. A "enetic component ma# be present in certain %ami$ies the %ami$ia$ rate o%

h#pospadias is about @>.

Histor#

8btain a thorou"h histor# and ph#sica$ e(amination, inc$udin" an# histor# o% a %ami$ia$ pattern o% h#pospadias,an# past medica$ histor# or comorbidit#, and a ph#sica$ assessment %ocusin" on the meata$ $ocation, "$anscon%i"uration, s in co&era"e, and chordee.

A histor# o% in%erti$it# and treatment shou$d a$so be documented in &itro %erti$i3ation 95 F: has been associated'ith a hi"her incidence o% h#pospadias.

Ph#sica$

A$thou"h the dia"nosis o% h#pospadias has been made 'ith both prenata$ %eta$ u$trasono"raph# and ma"neticresonance ima"in" 9MR5:, the dia"nosis is "enera$$# made upon e(amination o% the ne'born in%ant. 6=7

A dorsa$ hood o% %ores in and "$anu$ar "roo&e are e&ident, but upon c$oser inspection, the prepuce isincomp$ete &entra$$# and the urethra$ meatus is noted in a pro(ima$$# ectopic position. Rare$#, the %ores in ma#be comp$ete, and the h#pospadias is re&ea$ed at the time o% circumcision . 5% h#pospadias is encountered durin"neonata$ circumcision, a%ter the dorsa$ s$it has been per%ormed, the procedure shou$d be ha$ted, and the patientshou$d be re%erred %or uro$o"ic e&a$uation.

Chordee ma# be readi$# apparent or ma# be discernib$e on$# durin" erection. Pro(ima$ h#pospadias iscommon$# associated 'ith a bi%id scrotum and penoscrota$ transposition 9see the ima"e be$o':, in 'hich theru"ated scrota$ s in be"ins $atera$ to the penis rather than in its norma$ posterior ori"in.

http://emedicine.medscape.com/article/436829-overviewhttp://emedicine.medscape.com/article/436829-overviewhttp://emedicine.medscape.com/article/1015820-overviewhttp://emedicine.medscape.com/article/1015820-overviewhttp://emedicine.medscape.com/article/436829-overviewhttp://emedicine.medscape.com/article/1015820-overview


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Penoscrota$ transposition. ;ote ru"ated scrota$ s in $atera$ to penis,cepha$ad to its norma$ position.

Causes

Se&era$ etio$o"ies %or h#pospadias ha&e been su""ested, inc$udin" "enetic, endocrine, and en&ironmenta$%actors.

*enetic %actors

A "enetic predisposition has been su""ested b# the ei"ht%o$d increase in incidence o% h#pospadias amon"mono3#"otic t'ins compared 'ith sin"$etons. )his %indin" ma# re$ate to the demand o% t'o %etuses %or humanchorionic "onadotropin 9HC*: produced b# a sin"$e p$acenta, 'ith an inade uate supp$# durin" critica$ periodso% urethra$ de&e$opment.

A %ami$ia$ trend has been noted 'ith h#pospadias. )he pre&a$ence o% h#pospadias in ma$e chi$dren o% %athers'ith h#pospadias has been reported as 0>, and 1 > o% brothers o% chi$dren 'ith h#pospadias are a$soa%%ected. )he inheritance is $i e$# po$#"enic.

Endocrine %actors

A decrease in a&ai$ab$e andro"en or an inabi$it# to use a&ai$ab$e andro"en appropriate$# ma# resu$t inh#pospadias. 5n a 1??@ report b# Aaronson et a$, --> o% bo#s 'ith mi$d h#pospadias and > 'ith se&ereh#pospadias 'ere %ound to ha&e a de%ect in testicu$ar testosterone bios#nthesis. 6-7

Mutations in the =/a$pha reductase en3#me, 'hich con&erts testosterone 9): to the more potentdih#drotestosterone 9DH):, ha&e been associated 'ith h#pospadias. A 1??? report b# Si$&er et a$ %ound near$#1 > o% bo#s 'ith iso$ated h#pospadias had at $east one a%%ected a$$e$e 'ith a =/a$pha reductase mutation.


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6@7 A$thou"h andro"en receptor de%icits, uantitati&e or ua$itati&e, ha&e been sho'n to resu$t in h#pospadias,this is thou"ht to be re$ati&e$# uncommon, and other %actors are more common$# imp$icated.

A hi"her incidence o% h#pospadias in 'inter conceptions has a$so been proposed. )heoretica$$#, this ma# bere$ated to the e%%ect o% da#$i"ht on pituitar# %unction, 'hich, in turn, a%%ects the materna$ and %eta$ hormona$mi$ieu ho'e&er, other authors ha&e not noticed this association.

A %i&e%o$d increased ris o% h#pospadias appears to e(ist in ma$es born throu"h 5 F in comparison 'ith acontro$ "roup. )his ma# re%$ect materna$ e(posure to pro"esterone, 'hich is common$# administered in 5 Fprotoco$s. Pro"esterone is a substrate %or =/a$pha reductase and acts as a competiti&e inhibitor o% the )/to/DH)con&ersion.

8ther %actors that contribute to in%erti$it#, such as under$#in" endocrinopathies or %eta$ endocrine abnorma$ities,ma# p$a# a ro$e.

En&ironmenta$ %actors

Endocrine disruption b# en&ironmenta$ a"ents is "ainin" popu$arit# as a possib$e etio$o"# %or h#pospadias andas an e(p$anation %or its increasin" incidence.

Estro"ens ha&e been imp$icated in abnorma$ peni$e de&e$opment in man# anima$ mode$s. En&ironmenta$substances 'ith si"ni%icant estro"enic acti&it# are ubi uitous in industria$i3ed societ# and are in"ested aspesticides on %ruits and &e"etab$es, endo"enous p$ant estro"ens, in mi$ %rom $actatin" pre"nant dair# co's,%rom p$astic $inin"s in meta$ cans, and in pharmaceutica$s.

A stud# b# Had3ise$imo&ic reported increased estradio$ concentration in p$acenta$ basa$ s#nc#tiotrophob$asts o%bo#s 'ith undescended testes as compared 'ith a contro$ popu$ation .607Undescended testes and h#pospadiasha&e been associated, but increased estradio$ concentration has not been imp$icated in h#pospadias per se.)his ma# support the association o% h#pospadias 'ith increasin" parit#, increasin" materna$ a"e, and $o' birth

'ei"ht noted in some studies in re$ation to $i%e$on" e(posure to en&ironmenta$ disruptors and a possib$ecumu$ati&e e%%ect.

Combination theor#

A "ro'in" bod# o% e&idence su""ests that the de&e$opment o% h#pospadias has a t'o/hit etio$o"# in&o$&in" a"enetic predisposition coup$ed 'ith %eta$ e(posure to an en&ironmenta$ disruptor .6?, 1 7

Dia"nostic Considerations

Undescended testes and in"uina$ hernias are the anoma$ies most common$# associated 'ith h#pospadias $oo%or undescended testes and in"uina$ hernias durin" the re&ie' o% s#mptoms and ph#sica$ e(amination. 5n 1?01,a re&ie' b# huri o% more than 1 patients 'ith h#pospadias reported that the incidence o% undescendedtestes and in"uina$ hernias 'as ?> %or each . 6117 ith more se&ere %orms o% h#pospadias, the incidence o%undescended testes e(ceeded + >, and the incidence o% in"uina$ hernias approached 2 >.

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)he combination o% h#pospadias and undescended testis can be an indicator o% an under$#in" disorder o%se(ua$ de&e$opment 9DSD:. 5n a 1??? stud# b# ae%er et a$, DSD states 'ere identi%ied in appro(imate$# + >o% patients 'ith uni$atera$ or bi$atera$ undescended testes and h#pospadias, and more pro(ima$ meata$ $ocationcarried a hi"her association o% DSD states than more dista$ meata$ $ocation. 61275% an# "onad 'as nonpa$pab$e,the incidence rose to = > ho'e&er, i% both "onads 'ere pa$pab$e, the incidence 'as on$# 1=>.

A prostatic utric$e is occasiona$$# noted 'hen catheteri3ation o% the urethra is attempted in patients 'ithh#pospadias.

Di%%erentia$ Dia"noses

• Circumcision

• Disorders o% Se(ua$ De&e$opment

• *enita$ Anoma$ies

ma"in" Studies

Upper urinar# tract anoma$ies are rare$# associated 'ith h#pospadias and do not 4usti%# routine ima"in" inthese patients un$ess other or"an s#stem anoma$ies are present. 8ther associated %indin"s are more common9e", en$ar"ed prostatic utric$e, $o'/"rade &esicouretera$ re%$u( : but are o% $itt$e conse uence c$inica$$# un$essother s#mptoms merit e&a$uation. 61+7

Medica$ Care

Minor cases o% h#pospadias, in 'hich the meatus is $ocated up to'ard the tip o% the "$ans, ma# not re uiresur"ica$ repair and ma# simp$# be mana"ed 'ith obser&ation.

Sur"ica$ Care

)he "oa$s o% sur"ica$ treatment o% h#pospadias are as %o$$o's<

• )o create a strai"ht penis b# repairin" an# cur&ature 9orthop$ast#:•

)o create a urethra 'ith its meatus at the tip o% the penis 9urethrop$ast#:• )o re/%orm the "$ans into a more natura$ conica$ con%i"uration 9"$ansp$ast#:• )o achie&e cosmetica$$# acceptab$e peni$e s in co&era"e• )o create a norma$/appearin" scrotum

)he resu$tin" penis shou$d be suitab$e %or %uture se(ua$ intercourse, shou$d enab$e the patient to &oid 'hi$estandin", and shou$d present an acceptab$e cosmetic appearance.

)imin" o% sur"er#

http://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1016681-overviewhttp://emedicine.medscape.com/article/1016439-overviewhttp://emedicine.medscape.com/article/1016439-overviewhttp://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1016681-overviewhttp://emedicine.medscape.com/article/1016439-overview


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!e%ore 1?0 , h#pospadias repair 'as per%ormed in chi$dren o$der than + #ears because o% the $ar"er si3e o% thepha$$us and a technica$$# easier procedure ho'e&er, "enita$ sur"er# at this a"e 9"enita$ a'areness occurs atabout a"e 10 months: can be associated 'ith si"ni%icant ps#cho$o"ica$ morbidit#, inc$udin" abnorma$ beha&ior,"ui$t, and "ender identit# con%usion.

Current$#, most ph#sicians attempt to repair h#pospadias 'hen the chi$d is a"ed /10 months, 'ith a trend

to'ard ear$ier inter&ention. )his has been associated 'ith an impro&ed emotiona$ and ps#cho$o"ica$ resu$t. Abene%it in 'ound hea$in" 'ith ear$ier repair has a$so been percei&ed and ma# ha&e a basis in the reducedproin%$ammator# c#to ine production noted at #oun"er a"es. 61 7

Gate h#pospadias repair, in the puberta$ and postpuberta$ period, is associated 'ith comp$ications, primari$#urethrocutaneous %istu$a, in near$# ha$% o% patients. 61=7Some reports cite a hi"her rate o% comp$ication in =/#ear/o$d patients than in 1/#ear/o$d patients, su""estin" that ear$ier repair is "enera$$# better .61-7

)#pes o% repair

)he speci%ic techni ues %or h#pospadias repair are be#ond the scope o% this artic$e 9see Uro"enita$Reconstruction, Peni$e H#pospadias : ho'e&er, the t#pes o% repairs can be "enerica$$# "rouped, and theapproach to the repair is re$ati&e$# standard.

A%ter a %u$$ assessment o% the peni$e anatom#, the sha%t s in o% the penis is de"$o&ed to e$iminate an# s intetherin", and an arti%icia$ erection is per%ormed to ru$e out an# cur&ature. Mi$d/to/moderate chordee ma# berepaired b# e(cisin" an# &entra$ %ibrous tetherin" tissue or b# p$icatin" the dorsa$ tunics o% the corpora$ bodies,compensatin" %or an# &entra$/to/dorsa$ disproportion.

More se&ere chordee ma# re uire "ra%tin" o% the &entra$ corpora$ bodies usin" s#nthetic, anima$ 9sma$$intestina$ subunit:, cada&eric, or auto$o"ous tissues 9tunica &a"ina$is or derma$ "ra%ts: to a&oid e(cessi&eshortenin" o% peni$e $en"th. 8n rare occasion, the urethra$ p$ate ma# be tethered and transection o% the p$atema# be re uired, prec$udin" the use o% nati&e urethra$ tissues %or urethrop$ast#.

)he urethra ma# be e(tended b# usin" &arious techni ues. )hese techni ues are "enera$$# cate"ori3ed asprimar# tubu$ari3ations, $oca$ pedic$ed s in %$aps, tissue "ra%tin" techni ues, or meata$ ad&ancementprocedures.

)he tubu$ari3ed incised p$ate 9)5P: repair has become the most common$# used repair %or both dista$ andmidsha%t h#pospadias. )his techni ue is a primar# tubu$ari3ation o% the urethra$ p$ate, 'ith incision o% theposterior 'a$$ o% the p$ate, 'hich a$$o's it to hin"e %or'ard 9see the ima"e be$o':. )his creates a "reaterdiameter $umen than 'ou$d other'ise be possib$e, ob&iatin" the routine use o% a %$ap or "ra%t to brid"e a shortnarro' se"ment o% urethra$ p$ate.

)he )5P repair has pro&ed adaptab$e to &arious settin"s, and current sur&e#s indicate that it is the procedure o%

choice %or most repairs b# most uro$o"ists.

arious sutures ha&e been used in the repair o% h#pospadias, but po$#"$#co$ic acid based sutures ma# o%%er thebest ba$ance o% resi$ience 'hen e(posed to urine, 'ithout e(cessi&e time to absorption resu$tin" in a %orei"nbod# reaction. 61@79See the ima"e be$o'.:

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)ubu$ari3ed incised p$ate 9)5P: techni ue. Urethra$ p$ate has beenincised in dorsa$ mid$ine this e(pands 'idth o% p$ate and a$$o's it to hin"e %or'ard %or tubu$ari3ation.

Studies support the "enera$ concept that increasin" the $a#ers o% tissue bet'een the urethra and o&er$#in" s inco&era"e ma es subse uent de&e$opment o% urethrocutaneous %istu$a $ess $i e$#. 6107)emporar# urethra$ stentsare a common ad4unct to h#pospadias repair and are %e$t to decrease the $i e$ihood o% %istu$a %ormation. ariousdraina"e tubes ha&e been uti$i3ed %or this purpose. 61?7)o stent or not to stent is an on"oin" contro&ers#,ba$ancin" the ris o% irritati&e s#mptoms and urinar# tract in%ection 'ith the ris o% urinar# retention. 62 7

5n a retrospecti&e/prospecti&e obser&ationa$ stud# o% 10? patients that compared 1 'ee o% transurethra$b$adder catheteri3ation a%ter h#pospadias repair 'ith + 'ee s o% catheteri3ation, Daher et a$ %ound the $on"ercatheteri3ation period to be associated 'ith better outcomes and %e'er comp$ications. 6217

5n the settin" o% repeat repair a%ter unsuccess%u$ sur"er# %or h#pospadias 'hen $oca$ tissues are una&ai$ab$e,bucca$ mucosa has been used %or urethra$ "ra%tin". )his tissue is 'e$$ suited %or this purpose because o% itsa&ai$abi$it#, characteristics that %a&or "ra%t success, and resi$ience to a moist en&ironment. Urethra$ stents are"enera$$# used %or b$adder draina"e 'hi$e hea$in" occurs in a$$ but the most dista$ h#pospadias repairs.

Steps o% repair

*$ans %$aps are "enera$$# mobi$i3ed to co&er the dista$ urethra$ repair, brin"in" the di&er"ent &entra$components to the mid$ine and creatin" a more conica$ con%i"uration. )he e(cess dorsa$ s in is mobi$i3ed to thede%icient &entra$ aspect o% the penis %or %ina$ s in co&era"e.

)he repair o% penoscrota$ transposition is o%ten per%ormed as a sta"ed procedure because the necessar#incisions ma# compromise the &ascu$ar pedic$e to s in %$aps used in the primar# urethrop$ast#. )he repair o%penoscrota$ transposition is usua$$# de%erred at $east - months to a$$o' %or ade uate %ormation o% co$$atera$b$ood supp$#.


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)he repair o% h#pospadias is "enera$$# p$anned as a sin"$e/sta"e procedure, but e(cessi&e chordee 9especia$$#i% transection o% the urethra$ p$ate is re uired:, poor s in a&ai$abi$it#, and sma$$ pha$$ic si3e ma# be betterapproached in a sta"ed manner. )he chordee is repaired and the s in is mobi$i3ed to the &entra$ peni$e sha%tdurin" the %irst sta"e, and the urethrop$ast# and "$ansp$ast# are repaired a%ter the %irst sta"e has comp$ete$#hea$ed.

Ad4u&ant hormona$ therap#

A$thou"h no correcti&e medica$ therap# %or h#pospadias is no'n, hormona$ therap# has been used as anad4u&ant %or in%ants 'ith e(ceptiona$$# sma$$ pha$$ic si3e. Preoperati&e treatment 'ith testosterone in4ections orcreams, as 'e$$ as human chorionic "onadotropin 9HC*: in4ections, has been used to promote peni$e "ro'thsome ha&e reported impro&ement in chordee 'ith $essenin" in the se&erit# o% h#pospadias. )hat prepuberta$andro"en therap# ma# $imit norma$ "enita$ "ro'th at pubert# is a concern, but this has not been con%irmedc$inica$$#.

5n a stud# o% 102 chi$dren 'ith midsha%t or dista$ h#pospadias 9mean a"e, + months: 'ho under'ent )5Prepair %or h#pospadias, As"ari et a$ %ound preoperati&e parentera$ testosterone administration to be bene%icia$ indecreasin" comp$ication rates 9%rom 1+.10> to =. =>:. 6227

Consu$tations

Consu$tation 'ith a pediatric endocrino$o"ist is indicated in cases in 'hich a chi$d ma# be suspected o% ha&in"a disorder o% se(ua$ de&e$opment .

Comp$ications

5t is c$ear that repairs that are more pro(ima$ are associated 'ith a "reater incidence o% comp$ications. 8$dera"e at sur"er# and $o' sur"ica$ e(perience ha&e a$so been associated 'ith poorer outcomes. ith $on"er%o$$o'/up, it is apparent that $ate comp$ications can occur and most ad&ocate continued e&a$uation throu"hpubert# .62+, 2 , 2=, 2-7

5mmediate postoperati&e concerns

Goca$ edema and b$ood spottin" can be e(pected ear$# a%ter repair and "enera$$# do not cause a si"ni%icantprob$em.

Postoperati&e b$eedin" rare$# occurs and is usua$$# contro$$ed 'ith a compressi&e dressin". 5n%re uent$#,ree(p$oration ma# be re uired to e&acuate a hematoma and to identi%# and treat the source o% b$eedin".

5n%ection is a rare comp$ication o% h#pospadias repair in the modern era. S in preparation and perioperati&eantibiotics are "enera$$# used. Patients are o%ten maintained on an antibiotic course unti$ an# stents areremo&ed, thou"h this has not c$ear$# been sho'n to be bene%icia$. 62@7

Gon"/term issues

http://emedicine.medscape.com/article/1015520-overviewhttp://emedicine.medscape.com/article/1015520-overview


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Urethrocutaneous %istu$i3ation is a ma4or concern in h#pospadias repair. )he rate o% %istu$a %ormation is"enera$$# $ess than 1 > %or most sin"$e/sta"e repairs but rises 'ith the se&erit# o% h#pospadias, approachin"

> 'ith comp$e( reoperati&e e%%orts. Fistu$as rare$# c$ose spontaneous$# and are repaired b# usin" amu$ti$a#ered c$osure 'ith $oca$ s in %$aps - months a%ter the initia$ repair. A%ter repair, %istu$as recur inappro(imate$# 1 > o% patients. 9See the ima"e be$o'.:

Urethrocutaneous %istu$a has appeared a%ter h#pospadiasrepair. ;ote one stream %rom true urethra$ meatus and second stream throu"h more pro(ima$ %istu$a.

Meata$ stenosis , or narro'in" o% the urethra$ meatus, can occur. A urethra$ stent pre&ents an# prob$ems initia$$#,but a %ine/spra#in" urinar# stream that is associated 'ith strainin" to &oid $i e$# re uires operati&e meata$re&ision.

Urethra$ strictures ma# de&e$op as a $on"/term comp$ication o% h#pospadias repair. )hese are "enera$$#repaired operati&e$# and ma# re uire incision, e(cision 'ith reanastomosis, or patchin" 'ith a "ra%t or pedic$eds in %$ap.

Urethra$ di&erticu$a ma# a$so %orm and are e&idenced b# ba$$oonin" o% the urethra 'hi$e &oidin". A dista$stricture ma# cause out%$o' obstruction and ma# resu$t in a urethra$ di&erticu$um. Di&erticu$a can %orm in theabsence o% dista$ obstruction and are "enera$$# associated 'ith "ra%t/ or %$ap/t#pe h#pospadias repairs, 'hich

$ac the subcutaneous and muscu$ar support o% nati&e urethra$ tissue. )he redundant urethra$ tissue is"enera$$# e(cised, and the urethra is tapered to an appropriate ca$iber.

Hair/bearin" s in is a&oided in h#pospadias reconstruction but 'as used in the past. hen incorporated intothe urethra, it ma# be prob$ematic and can resu$t in urinar# tract in%ection or stone %ormation at the time o%pubert#. )his "enera$$# re uires c#stoscopic depi$ation usin" a $aser or cauter# de&ice or, i% se&ere, e(cision o%hair/bearin" s in and repeat h#pospadias repair.

http://emedicine.medscape.com/article/1016016-overviewhttp://emedicine.medscape.com/article/450903-overviewhttp://emedicine.medscape.com/article/443296-overviewhttp://emedicine.medscape.com/article/443296-overviewhttp://emedicine.medscape.com/article/969643-overviewhttp://emedicine.medscape.com/article/1016016-overviewhttp://emedicine.medscape.com/article/450903-overviewhttp://emedicine.medscape.com/article/443296-overviewhttp://emedicine.medscape.com/article/969643-overview


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;e'er sur&e#s ha&e su""ested mi$der %orms o% erecti$e d#s%unction ma# be more common 'ith more pro(ima$h#pospadias repairs. 6207

Pro"nosis

Current prognosis

ith modern anesthetics, instruments, sutures, dressin" materia$s, and antibiotics, h#pospadias repair hasbecome uite success%u$. Gon"/term studies on the outcomes o% h#pospadias usin" current practices are$imited. A$thou"h some ear$ier studies ha&e been discoura"in", these re%$ect an era 'ith poorer technica$outcomes, increased number o% operations, and a $ac o% appreciation %or the ps#cho$o"ica$ morbidit#associated 'ith inter&ention at an o$der a"e.

Subse uent $on"/term studies ha&e su""ested that despite ha&in" decreased satis%action 'ith their "enita$appearance, patients ha&in" under"one h#pospadias repair are more satis%ied 'ith their se( $i&es compared tohea$th# contro$s. 62?7;e'er scorin" s#stems to $end an ob4ecti&e measure to $on"/term outcomes are becomin"more popu$ar .6+ , +17

Future pro"nosis

A$thou"h the techni ues o% h#pospadias repair continue to e&o$&e, the broader %uture o% h#pospadias treatmentis tru$# promisin". ;ontraditiona$ tissue adherence techni ues are bein" de&e$oped, inc$udin" tissue "$ues and$aser/acti&ated so$derin" techni ues that ha&e been sho'n to impro&e 'ound hea$in" and to reduce %istu$a%ormation.

Urethra$ substitutes, 'hich ma# ob&iate the di%%icu$ties associated 'ith se&ere h#pospadias and poor tissuea&ai$abi$it#, are current$# under in&esti"ation. )hese substitutes are "enera$$# ace$$u$ar s#nthetic or natura$matrices that can incorporate the patient s norma$ urethra$ ce$$u$ar components.

)he embr#o$o"# o% h#pospadias is bein" e$ucidated, and the understandin" o% its causes is impro&in" 'ith ne'in%ormation, an e(citin" ne' paradi"m shi%t to h#pospadias pre&ention or antenata$ inter&ention ma# occur.

Patient Education

!ecause most patients 'ith h#pospadias are sur"ica$$# treated at a &er# #oun" a"e, parenta$ teachin" andreassurance is &er# important to ensure a satis%actor# e(perience %or the %ami$ies o% patients 'ith h#pospadias.E&idence su""ests that on$ine support "roups can p$a# an important ro$e in ho' parents and patients cope 'iththis condition. 6+27

reference hipospadia

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