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10.4 OTHER UROGENITAL CONDITIONS Epispadias and bladder exstr p!

" A congenital defect of the anterior urethra in which the canal terminates on the dorsum of the penis and posterior to its normal opening. Exstrophy of the bladder complete epispadias = 1 in 30 000 live births severest form is commonest! " times more common in #

Embryologically different from hypospadias$ %eflects abnormal development of the cloacal membrane & failure of rupture and mesenchymal penetration & abnormal abdo wall development. 'evelopmental abnormality of abdominal wall. (lassification )enopubic )enile *lanular 'iagnosis and %x (linical features vary from very mild to complete may include Epispadias + dorsal meatus # epispadiac meatal dystopia!$ short penis$ dorsal chordee$ divergent corpora. Epispadias proximal to bladder nec, = incontinence. -hort vagina$ cleft clitoris and wide labia minora in . 'iastasis of recti Absent or widened pubic symphysis /ow set umbilicus Exstrophy of bladder0 Ant wall may be absent$ eversion of bladder$ 1anagement %econstruct genitalia & appearance and function Exstrophy & reconstruct bladder and abdo wall defect %epair & li,e for hypospadias flip flap$ preputial island! 2ladder exstrophy & urinary diversion$ reconstruction of abdo wall etc. Abdo wall$ symphysis$ ant bladder wall$ bladder eversion$ 3 cancer ris,4 untreated 5eonate bladder closure & wee, 1 Epispadias repair & 106 yrs 2ladder nec, reconstruction & 30"yrs 7liac osteotomies to allow closure of symphysis 8ree bladder edges approximate and closed Abdo wall closure may need rectus muscle flap. C n#enital $enile C%r&at%re Abnormal fixation of the penile s,in or 'artos fascia (orporal disproportion )reserved urethral plate. 5eed tunica albuginea plication 9 excision 5esbit! :orsional deformity is always counter0cloc,wise + correct by degloving.

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C! rdee A fibrous remnant of the corpus spongiosum causing ventral penile curvature in assoc. hypospadias 'issection of urethral plate alone will not correct 7n C @0D the chordee is due to ventral s,in shortage + i.e. growth arrest Esually hypospadias but occasionally caused by scar tissue resulting from urethral infection of trauma.

$e"r nie's disease. 1aFority have upward curvature with thic,ening on the dorsal surface of the tunica. "0Ath decade En,nown aetiology (ommon 0 up to <.AD prev -eems to be increasing in young :unica albuginea fibrosis extending into septum between corpora -imilar to 'upuytrenGs %epetitive trauma esp. to semi erect penis :*80H over expression4 )resentation o c;o lump and pain initially o Enstable erection o (hronic inflammation then fibrosis and calcification;ossification o 6A030D will have 'upuytrenGs o 6D family Ix of )eyronieGs$ <D of 'upuytrenGs on mothers side 1edical %x o )ara0aminobenJoic acid$ Kit E for pain!$ (olchicine$ E-$ :amoxifen$ -teroid 7nFection$ Kerapamil. -urgical %x o if unable to have sex$ L"Adeg dorsally usually tolerated$ Kentral usually not tolerated$ but surgery contraindicated if there is erectile dysfunction. o -hortening= 5esbit procedure to plicate the ventral tunica shortens penis! )enile prosthesis is bordering on impotence Ex of plaMue and graft with dermis dura$ fascia lata$ alloderm$ vein or gortex patch have all been reported!$ but can resorb or produce ballooning of the corpora and compromise erection 1odified 5esbit 0 excise ellipse on normal$ longer side 0 popular in EN o /engthening /ue techniMue 0 )laMue incision and grafting with saphenous vein 0 plaMue is not excised 0 will cause erectile dysfunction o )enile 7mplant

A0D spontaneous resolution may occur & no op until at least 1yr.

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Cr"pt spadias 5o fusion defect *rowth deficiency 1ay have congenital fistula )reserved frenulum 'orsal hooding ObliMue raphe :erminal meatus 7nguinal hernia

$enile En!an(e)ent /iposuction of fat pad )artial;complete division of suspensory ligament drops angle of erection! K0P s,in advancement from pubic area 'ermofat onlay grafts around tunica to increase girth -tretching with weights

A)bi#% %s Genitalia 1ust assign sex by 6 yrs 1': 0 -een by geneticist and paeds EnJyme deficiencies or Naryotype problems Androgen + insensitivity & *enetic # appears . (ongenital adrenal hyperplasia & *enetic . ambiguous genitalia AQ reductase$ 1>Q hydroxylase etc 1ixed gonads "AR;"<RP :urners "ARO$ strea, gonads$ . genitalia$ short statue$ webbed nec, Nlinefelters syndrome ">RRP$ small testes$ hypogonadism$ gynaecomastia (ongenital Adrenocortical Iyperplasia& female pseudohermaphroditism Or adrenogenital syndrome *enetic . "<RR! 3 androgen production usually due to deficiency in 610 hydroxylase involved in aldosterone pathway$ precursors diverted into androgen synthesis -alt loosing type may have & S5a$ 3N$ and dehydration at 1w, old. *enitalia = mildly 3 clitoris to a normal penis with terminal meatus. -hould be raised female and can be fertile -urgical correction may be needed.& usually 30<;16$ clitoral recession$ vaginoplasty 1ale )seudo0hermaphroditism *enetic # "<RP! 7nadeMuate masculinisation of external genitalia. 'ue to A0Q reductase deficiency = S testosterone production converts testosterone to dihydrotestosterone in the target tissues!. 1>0Q0hydroxylase def = enJyme reMuired for synthesis of androgens and oestrogens. Or :esticular feminiJation syndrome = absent androgen receptors %aise as . as will always have inadeMuate phallus
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-urgery = orchidectomy prior to puberty$ vaginal reconstruction. *onadal dysgenesis :he testis one side develops normally other testis = strea, gonad. 1ost have "<RP or "<RO ,aryotype. 1ay be mosaic with "AR;"<RP 5ormal testis li,ely to get gonadoblastoma %aise as . -urgery = bilat gonadectomy$ clitoral recession and vaginoplasty.

:rue Iermaphroditism Kery rare 1ost "<RR can be "<RP 1ay be familial 2antu pop of - Africa! most but not all are infertile 1ay have ovary on one side and testis on the other or bilat ovotestes %aise as .$ will always have inadeMuate phallus -urgery = remove testis Dis rders * sex%al di**erentiati n +arne 1,,-

-%P gene = -ex determining %egion of P chromosome = testis determining factor -%P absent = no testis = . phenotype /eydig cells in testis & testosterone & Tolffian duct develops -ertoli cells & 1Ullerian inhibiting substance 17-! member of :*8H family! :o get viriliJation of external genitalia need to convert testosterone to dihydrotestosterone via AQ reductase 7ntersex 'isorders o RP female & mutated -%P gene$ AQ red def$ 1>Q hydr def o RR male & resembled Nleinfelters$ -%P translocation. o Ambiguous & (AI$ A Q red$ mixed gonads o RP # V 1Ullerian duct structures & mutations in 17- gene or receptors o RR . absent 1Ullerian structures & %o,itans,y$ 4activating 17gene;rec

1anagement (ounsel parents & defer registering;naming child -trea, gonads & malignant change = remove Androgen insensitivity& @D ris, of seminoma & testis out before 60 phenotype.! 8eminiJing genitoplasty o :iming and necessity4 o (AI & almost always reared as . and are fertile = op reinforces identity

.ar" t"pe "<RP "<RR "ARO ">RRP "ARO;"<RP

$ ssible Dia#n sis Androgen insensitivity$ AQ reductase def$ 1> Q hydroxy def (AI 61 hydroxylase def! :urners -yndrome NlinefelterGs syndrome 1ixed gonadal dysgenesis

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"<RP . "<RR # /a#inal A#enesis

-%P gene mutation -%P gene mutation

Nnown as 1ayer0%o,itans,y0NUster0Iauser syndrome 1 in "000 live . births 8ailure of development of paramesonephric duct A0D assoc abnormal E: Ovaries usually normal 1ay have vestigial vaginal dimple (ases with no uterus = amenorrhoea (ases with vaginal agenesis and normal uterus = haematocolpos.

7nvestigation Examine = vaginal dimple E-- = define ,idneys$ bladder and uterus$ not good for vagina 7K) = exclude other E: abnormality EEA = combined with endoscopy and dye to determine extent of deformity (hromosome analysis -urgical %econstruction )rev delayed until Fust before marriage to ,eep the vagina patent intercourse 5ow done in early childhood )edicled (olon & good method 'ilatation& 8ran, 1@3B$ seMuential glass or plastic moulds$ need a dimple to start /ocal labial flaps *raves 1@B1 --* reconstruction & 1c7ndoe. --* over a mould$ labia minora then oversewn and the mould left in situ for < months$ regular dilatation needed to prevent stenosis. 2ut poor graft ta,e$ visible donor site$ reMuires daily dilatation$ multistage$ neoplasia described$ E shaped incision in fourchette to create shallow poc,et for intercourse Tilliams! 8:* over stent 8laps of labia minora %egional flaps such as gracilis /a#inal Re( nstr%(ti n )athology (ongenital absence %o,itans,y syndrome! -egmental imperforate hymen$ long segment atresia! (ongenital malformation & . hypospadias -urgical ablation & e.g. mid section ex for prolapse %adionecrosis & hostile tissues 8istulae & reMuires flap closure -urgical Options )referred tissues & vagina$ vulva$ s,in$ bowel FeFunum! 'istant flaps o Tei & bilat groin flaps o :%A1& pedicled through pelvis on 7EA o 2owel & but tend to get stenosis at mucocutaneous anastomosis

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Kulval tissue expansion o Appropriate tissue o 5o donor defect o 6 stages o hospitalise for <0> w,s during expansion %adionecrosis o 5eed to import well vascularised tissue with own blood supply :%A1$ Omentum$ *racilis!

/a#inal C nstr%(ti n in Transsex%als Kan 5oort )%- 1@@3! #& . 3x more common than . & # Epithelium lined cavity created between prostate;urethra;seminal vesicles and rectum Erethra cut obliMuely to prevent stenosis :echniMues 7nversion of penis$ or penis and scrotum s,in flaps$ most common$ labia from scrotum --* 1c7ndoe! with stents )erineaW or abdo pedicled s,in flaps 7ntestinal segments )ost Op %egular dilatation $ stent$ vibrator$ intercourse (ompared 6> #&.$ Xust penile or )enile and scrotal s,in. 2oth used get bigger neovagina but hair growth and s,in prolapse a problem. 1ost pts in both groups get orgasm. Ne 0$!all plast" 'avid %alph$ 1anchester 0 6@-ep0< Abdo tubes /ocal flaps 0e.g. 0 abdominal and pubic *racilis 8ree radial forearm 0 various designs 0 %alph uses (hang flap uses inner arm hairless s,in for urethra!$ 5eed 3 or " venous anastomoses. 8:-* from buttoc, creases to donor. 6nd stage to form urethra. 3rd stage for glansplasty and creation of coronal sulcus 0 needs --* to sulcus. )enile implant for erection 0 need to wrap it in 'acron to allow anchoring to pubis :esticular implants

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