DLLL INIIL1kA1ING LNDCML1kICSIS: A1nCGLNLSIS, DIAGNCSIS AND CLINICAL MANAGLMLN1
Cristina Maciel*, Hlder Ferreira**, Rosa Macedo***. nospltol Cetol 5ooto Aotolo, ceotto nospltolot Jo lotto, lotto A8S1kAC1: ueeply lofllttotloq eoJomettlosls (ul) locloJes tectovoqlool lesloos os well os lofllttotlve fotms tbot lovolve vltol sttoctotes socb os bowel, otetets, ooJ bloJJet tbot mlqbt qteotly oltet tbe poollty of llfe becoose of sevete polo ot meosttootloo ooJ lotetcootse, ooJ pose pottlcolotly Jlfflcolt sotqlcol ptoblems. lt ls JloqooseJ lo 20X of womeo wltb eoJomettlosls. 1be vost boJy of evlJeoce ovolloble lo tbe lltetotote sttooqly soppotts tbe lmploototloo tbeoty lo ul potboqeoesls. Altbooqb botmoool tteotmeot ls efflcleot wltb tespect to tbe polo, sotqety ls tbe tteotmeot of tefeteoce lo tbls cootext. 1be complexlty ooJ motblJlty ossocloteJ wltb tbe sotqlcol ptoceJotes most be cooslJetote. Cteot lmpottooce most be qlveo to complete ooJ bolooceJ coooselloq, os owoteoess of tbe teol posslbllltles of Jlffeteot tteotmeots wlll eobooce tbe potleots collobototloo. lt oppeots to be locteosloqly cleot tbot ul wlll be best tteoteJ lo o moltlJlsclpllooty ceotet. _______________________________________________________________________________________________ IN1kCDUC1ICN LndomeLrlosls ls an esLrogen-dependenL lnflammaLory dlsease and lLs deflnlng feaLure ls Lhe presence of endomeLrlum-llke Llssue ln slLes ouLslde Lhe endomeLrlal cavlLy and uLerlne musculaLure. 1hese ecLoplc endomeLrlal lmplanLs are usually locaLed ln pelvls, buL can occur nearly anywhere ln Lhe body. 1he Lhree cllnlcally dlsLlncL forms of endomeLrlosls are endomeLrloLlc lmplanLs on Lhe surface of Lhe pelvlc perlLoneum and ovarles (perlLoneal endomeLrlosls), ovarlan cysLs llned by endomeLrlold mucosa (endomeLrlomas), and deeply lnfllLraLlng endomeLrlosls (ulL). 1 ulL lncludes recLovaglnal leslons as well as lnfllLraLlve forms LhaL lnvolve vlLal sLrucLures such as bowel, ureLers, and bladder, whlch mlghL greaLly alLer Lhe quallLy of llfe because of severe paln aL mensLruaLlon and lnLercourse, and pose parLlcularly dlfflculL surglcal problems. 2 _____________________________________________________________________ * Aluna do 6.ano do Mestrado em Medicina ** Interno Complementar de Ginecologia/Obstetrcia *** Assistente Hospitalar Graduada de Ginecologia e Obstetrcia ln Lhe presenL paper, Lhe anaLomlc and paLhologlc evldence regardlng Lhe paLhogenesls of lnfllLraLlng endomeLrlosls ls descrlbed. 1hls consLlLuLes Lhe basls for Lhe developmenL of a classlflcaLlon of deep dlsease. uesplLe exLenslve research, Lhe opLlmal managemenL of endomeLrlosls ls unclear. 1hls paper wlll revlew medlcal and surglcal opLlons for LreaLlng women wlLh ulL. kLVALLNCL 1he overall Lrue prevalence of endomeLrlosls ls unknown, prlmarlly because surgery ls Lhe only rellable meLhod for dlagnosls and generally ls noL performed on women wlLhouL sympLoms or physlcal flndlngs LhaL sLrongly suggesL Lhe posslblllLy. 1he prevalence of asympLomaLlc endomeLrlosls ls approxlmaLely 4 ln women havlng surgery for elecLlve sLerlllzaLlon. MosL esLlmaLes of prevalence of endomeLrlosls have ranged beLween 3 and 20 among women wlLh pelvlc paln and beLween 20 and 40 among lnferLlle women, a general prevalence ranglng beLween 3 and 10 ln reproducLlve-aged women ls llkely. 3 kLVILW Ak1ICLL 2 ulL ls dlagnosed ln 20 of women wlLh endomeLrlosls. Moreover, bowel endomeLrlosls ls found ln 3 Lo 12 of paLlenLs wlLh endomeLrlosls. 4 A1nCGLNLSIS 1he eLlology and paLhology of Lhe dlsease has been argued abouL slnce Lhe flrsL deLalled paLhologlc descrlpLlon ln 1860 by karl lrelherr von 8oklLansky. uesplLe Lhe passage of Llme and exLenslve lnvesLlgaLlon, Lhe exacL paLhogenesls of Lhls enlgmaLlc dlsorder remalns unknown. 1wo physlopaLhologlcal hypoLheses are Lhe mosL ofLen proposed Lo explaln Lhe paLhogenesls of endomeLrlosls. 1he flrsL Lheory ls LhaL of reLrograde mensLruaLlon ln whlch Lhe leslons would be secondary Lo lmplanLaLlon and prollferaLlon of regurglLaLed endomeLrlal cells ln an ecLoplc slLuaLlon (Sampson, 1927). 1he second Lheory ls LhaL of meLaplasla (Meyer, 1919), elLher of Lhe celomlc meLaplasla or Mullerlan remnanLs meLaplasla. 3 ketrograde Menstruat|on lnlLlally proposed by Sampson ln Lhe 1920s, Lhe Lheory of reLrograde mensLruaLlon ls boLh lnLulLlvely aLLracLlve and supporLed by mulLlple llnes of sclenLlflc evldence, alLhough no slngle mechanlsm can explaln all cases of endomeLrlosls. Accordlng Lo Lhls Lheory, ecLoplc endomeLrlum ls sloughed vla paLenL falloplan Lubes lnLo Lhe perlLoneal cavlLy durlng mensLruaLlon. lndeed, Lhe unlversallLy of Lhls phenomenon ls supporLed by Lhe flndlng of mensLrual blood ln Lhe perlLoneal fluld of up Lo 90 of healLhy women wlLh paLenL falloplan Lubes undergolng laparoscopy durlng Lhe perlmensLrual perlod of Lhe cycle. 6 lurLher supporL for Lhls eLlology ls derlved from sLudles of obsLrucLed or compromlsed ouLflow LracLs. ln adolescenL glrls wlLh congenlLal ouLflow obsLrucLlon, Lhe prevalence of endomeLrlosls ls hlgh. 1he vlablllLy of Lhe reLrogradely mensLruaLed endomeLrlal cells ls of paramounL lmporLance for Lhe plauslblllLy of Lhe LransplanLaLlon Lheory. 1hls has been mosL eleganLly addressed by Lhe experlmenLs of 8ldley and Ldwards. 1he anaLomlc dlsLrlbuLlon of endomeLrloLlc leslons also favors Lhe reLrograde mensLruaLlon Lheory. Superflclal lmplanLs are more ofLen locaLed ln Lhe posLerlor comparLmenL of Lhe pelvls and ln Lhe lefL hemlpelvls. rlmary and recurrenL ovarlan endomeLrlomas are slgnlflcanLly more ofLen locaLed on Lhe lefL ovary ln conLrasL Lo Lhe dlsLrlbuLlon of non endomeLrloLlc benlgn ovarlan cysLs, whlch do noL dlsplay a predllecLlon for sldedness. DIL pathogenes|s A large observaLlonal sLudy 3 suggesL LhaL Lhe anaLomlcal dlsLrlbuLlon of pelvlc ulL leslons presenLs a double asymmeLry. elvlc ulL leslons are more frequenLly observed ln Lhe posLerlor pelvlc comparLmenL and are mosL ofLen locaLed on Lhe lefL slde. lurLhermore, abdomlnal ulL leslons are far less frequenL Lhan pelvlc ulL leslons, and unllke Lhese, Lhey are mosL ofLen locaLed ln Lhe rlghL slde of Lhe abdomlnal cavlLy (appendlx and lleocaecum [uncLlon). All Lhese observaLlons plead ln favour of Lhe Lheory of regurglLaLlon and Lhe lmporLance of perlLoneal flow paLLerns ln ulL paLhogenesls. WlLh Lhe paLlenL sLandlng erecL, under Lhe effecL of gravlLy, mensLrual blood reflux accumulaLes ln Lhe boLLom of Lhe ouch of uouglas, whlch ls Lhe mosL dependanL porLlon of Lhe abdomlno-pelvlc cavlLy. 1he effecL of gravlLy also explalns why pelvlc ulL leslons are more frequenLly observed Lhan abdomlnal ulL leslons and why lnLesLlnal ulL leslons are preferenLlally locaLed on Lhe recLum and Lhe recLo-slgmold [uncLlon. 1he far lower frequency of deep bladder endomeLrlosls compared wlLh uLerosacral llgamenL, vaglnal and recLal ulL can be explalned by Lhe anaLomy, because Lhe lower llmlL of Lhe veslco-uLerlne pouch ls locaLed well above Lhe lower llmlL of Lhe pouch of uouglas, whlch lles opposlLe Lhe mlddle Lhlrd of Lhe posLerlor vaglnal wall. 1he more Lhe uLerus ls reLroverLed, whlch makes lL easler for Lhe perlLoneal llquld Lo flow from Lhe anLerlor comparLmenL Lowards Lhe posLerlor comparLmenL, Lhe more Lhe ulL leslons wlll be found posLerlorly. 7 1he anaLomlcal dlfferences beLween lefL and rlghL hemlpelvls, because of Lhe presence of Lhe slgmold colon on Lhe lefL, could explaln why pelvlc ulL leslons (as superflclal and ovarlan leslons) are observed more frequenLly on Lhe lefL pelvlc slde wall. 1he close anaLomlcal relaLlonshlp beLween Lhe slgmold colon and Lhe lefL adnexa forms a barrler Lo pelvlc dlffuslon of mensLrual blood reflux, resulLlng ln an anaLomlcal slLuaLlon LhaL could encourage adheslons and growLh of regurglLaLed endomeLrlal cells on Lhe lefL pelvlc slde wall. 8esulLs of research lnLo Lhe flow of perlLoneal fluld supporL Lhe hypoLhesls LhaL perlLoneal llquld plays a parL, LogeLher wlLh regurglLaLed endomeLrlal cells, ln ulL paLhogenesls.lour predomlnanL slLes have been ldenLlfled for Lhe preferenLlal, repeaLed or arresLed flow of perlLoneal fluld: (l) Lhe pelvlc cavlLy and especlally Lhe ouch of uouglas, (ll) Lhe rlghL lower quadranL aL Lhe LermlnaLlon of Lhe small bowel mesenLery (caecum and lleocaecum [uncLlon), (lll) Lhe superlor aspecL of Lhe slgmold mesocolon, and (lv) Lhe rlghL paracollc guLLer (Meyers, 1973). !usL as for superflclal leslon, Lhe anaLomlcal dlsLrlbuLlon of ulL 3 leslons correlaLes wlLh Lhe paLhways for Lhe perlLoneal fluld flow. 8 lour oLher observaLlons plead ln favour of regurglLaLlon playlng a role for Lhe dlfferenL Lypes of endomeLrloLlc leslons. llrsLly, unllke Lhose paLlenLs presenLlng endomeLrlosls wlLhouL deep leslons, Lhose wlLh ulL have a slgnlflcanLly reduced uouglas pouch depLh. 9 1hls obllLeraLlon of Lhe pouch of uouglas ls secondary Lo Lhe lnflammaLory process subsequenL Lo perlLoneal lmplanLaLlon of regurglLaLed endomeLrlal cells, whlch glves Lhe false lmpresslon LhaL deep leslons are of reLroperlLoneal orlgln. MagneLlc resonance lmaglng clearly shows LhaL ulL orlglnaLes from Lhe reLrocervlcal area and noL from Lhe recLovaglnal sepLum. 10 llrsLly, Lhe Lerm 'recLovaglnal sepLum endomeLrlosls' ls lncorrecL ln Lhe anaLomlcal sense as ulL leslons are lnlLlally locaLed above Lhe upper border of Lhe recLovaglnal sepLum. Secondly, no perlLoneal and/or ovarlan endomeLrloLlc leslons are seen afLer Lubal llgaLlon, and Lhe raLe of endomeLrlosls recurrence aL 24 monLhs ls slgnlflcanLly less lmporLanL when endomeLrlal ablaLlon ls assoclaLed wlLh laparoscoplc LreaLmenL of endomeLrlosls. 1hlrdly, perlLoneal fluld represenLs a speclflc mlcroenvlronmenL LhaL could play a role ln Lhe paLhogenesls of endomeLrlosls. Several sLudles have shown LhaL ovulaLlon ls more frequenL on Lhe rlghL slde. CreaLer exposure Lo progesLerone ln Lhe rlghL hemlpelvls could conLrlbuLe ln Lhls parL of Lhe pelvls Lo seL up an lnapproprlaLe mlcroenvlronmenL for lmplanLaLlon of endomeLrloLlc cells and for developlng ulL. lourLhly, Lhe asymmeLrlc naLure of pelvlc vascularlzaLlon, because of Lhe enLrance of Lhe lefL ovarlan veln lnLo Lhe homolaLeral renal veln raLher Lhan lnLo Lhe vena cava, explalns why varlcocele occurs more frequenLly on Lhe lefL slde. 1hls venous sLasls on Lhe lefL slde could glve rlse Lo local varlaLlon ln blood sLream facLors LhaL would affecL Lhe developmenL of ulL, whlch ls more dependenL on plasma levels Lhan Lhe lnfluence of Lhe perlLoneal llquld. ln concluslon, Lhe vasL body of evldence avallable ln Lhe llLeraLure, sLrongly supporL Lhe lmplanLaLlon Lheory ln ulL paLhogenesls and LhaL Lhe role of Lhe perlLoneal llquld ls essenLlal for undersLandlng Lhe anaLomlcal dlsLrlbuLlon of ulL leslons. 3 erlLoneal, ovarlan, and deep endomeLrlosls may be dlverse manlfesLaLlons of a dlsease wlLh a slngle orlgln l.e., regurglLaLed endomeLrlum. 6 Mo|ecu|ar mechan|sms 1hough reLrograde mensLruaLlon explalns Lhe physlcal dlsplacemenL of endomeLrlal fragmenLs lnLo Lhe perlLoneal cavlLy, addlLlonal sLeps are necessary for Lhe developmenL of lmplanLs. lL ls Lhe propenslLy for lmplanLaLlon LhaL besL accounLs for Lhe dlsparlLy beLween Lhe 90 prevalence of reLrograde mensLruaLlon and Lhe nearly 10 prevalence of Lhe dlsease. Cne of Lwo mechanlsms could explaln Lhe successful lmplanLaLlon of refluxed endomeLrlum onLo Lhe perlLoneal surface: molecular defecLs or lmmunologlc abnormallLles (or boLh). ln endomeLrlosls, Lhe euLoplc endomeLrlum exhlblLs mulLlple subLle buL blologlcally lmporLanL molecular abnormallLles, lncludlng Lhe acLlvaLlon of oncogenlc paLhways (e.g., Lhe Wlngless-Lype MM1v lnLegraLlon slLe famlly member wot or Lhe raL sarcoma vlral oncogene homologue kos) or blosynLheLlc cascades favorlng lncreased producLlon of esLrogen, cyLoklnes, prosLaglandlns, and meLalloproLelnases. When Lhe euLoplc endomeLrlum, blologlcally dlsLlncL Llssue, aLLaches Lo mesoLhellal cells, Lhe magnlLude of Lhe molecular abnormallLles ls ampllfled drasLlcally, enhanclng Lhe survlval of Lhe lmplanL. A posslble second mechanlsm of lmplanL survlval enLalls a fallure of Lhe lmmune sysLem Lo clear lmplanLs from Lhe perlLoneal surface. 8oLh mechanlsms may conLrlbuLe Lo Lhe developmenL of endomeLrlosls. 1 CLASSIIICA1ICN CI DLL LNDCML1kICSIS 8ased on dlfferenL paLhogeneLlc hypoLheses, several schemes have been proposed Lo classlfy deep endomeLrlosls, buL furLher daLa are needed Lo demonsLraLe Lhelr valldlLy and rellablllLy. lL seems lnsLlncLlve Lo lnclude deep endomeLrlosls ln sLage 4 of Lhe revlsed Amerlcan SocleLy for 8eproducLlve Medlclne (AS8M) classlflcaLlon. Powever, lnfllLraLlve forms are noL speclflcally addressed ln Lhe AS8M scheme, whlch was devlsed malnly wlLh Lhe ob[ecL of sLraLlfylng paLlenLs wlLh dlfferenL reproducLlve prognoses. konlnckx and MarLln were Lhe flrsL Lo deflne deep endomeLrlosls. 1hey dlsLlngulshed posLerlor cul- de-sac and recLovaglnal leslons ln Lhree dlfferenL subgroups: Lype l, conlcally shaped, developed from lnfllLraLlon, Lype ll, deeply locaLed, covered by exLenslve adheslons, probably formed by reLracLlon, and Lype lll, Lhe mosL severe, one or more spherlcal nodules locaLed ln Lhe recLovaglnal sepLum wlLh Lhe largesL dlmenslon under Lhe perlLoneum, posslbly Lo be consldered as adenomyosls exLerna. 11 Adamyan classlfled speclflcally reLrocervlcal endomeLrlosls ln four sLages accordlng Lo exLenL of Lhe dlsease. AL sLage l, endomeLrloLlc leslons are conflned Lo Lhe recLovaglnal cellular Llssue ln Lhe area of Lhe vaglnal vaulL. AL sLage ll, endomeLrloLlc Llssue 4 lnvades Lhe cervlx and peneLraLes Lhe vaglnal wall, causlng flbrosls and small cysL formaLlon. AL sLage lll, leslons spread lnLo Lhe uLerosacral llgamenLs and Lhe recLal serosa. AL sLage lv, Lhe recLal wall, recLoslgmold zone, and recLouLerlne perlLoneum are compleLely lnvolved, and Lhe recLouLerlne pouch ls LoLally obllLeraLed. MarLln and 8aLL dlfferenLlaLed among reLrocervlcal, recLovaglnal pouch, and recLovaglnal sepLum endomeLrlosls. 8eLrocervlcal endomeLrlosls lncludes leslons ln Lhe anLerlor aspecL of Lhe uouglas pouch, posLerlor vaglnal fornlx, and reLroperlLoneal area behlnd or beneaLh Lhe cervlx wlLh no recLal lnvolvemenL. ln recLovaglnal endomeLrlosls, recLal and vaglnal walls, as well as boLh vaglnal and recLal aspecLs of Lhe posLerlor cul-de-sac are lnvolved. 8ecLovaglnal sepLum endomeLrlosls refers Lo lsolaLed, Lrue subperlLoneal leslons wlLhouL conLlnulLy wlLh uouglas pouch leslons. Accordlng Lo Lhe proponenLs of Lhls scheme, lL ls dlfflculL Lo deLermlne Lrue recLovaglnal sepLum lnvolvemenL, also because recLovaglnal and recLovaglnal sepLum leslons may be assoclaLed. MosL recenLly a classlflcaLlon sysLem for deep endomeLrlosls based on Lhe anaLomlcal locaLlon of Lhe leslon wlLh a recommended preferred surglcal LreaLmenL for each locaLlon has been developed. 12-13 DIL: A1nCLCG AND SI1LS CI INVCLVLMLN1 MarLln eL al., (1989) and laLer Cornlllle eL al. (1990) suggesLed LhaL endomeLrlosls should be classlfled accordlng Lo Lhe depLh of lnvaslon lnLo superflclal (<1 mm), lnLermedlaLe (24 mm), deep (>3 mm) and very deep (>10 mm). 12 ulL ls deflned by Lhe presence of endomeLrlal lmplanLs, flbrosls and muscular hyperplasla under Lhe perlLoneum, and can lnvolve, ln descendlng order of frequency,Lhe uLerosacral llgamenLs, Lhe recLoslgmold colon, Lhe vaglna and Lhe bladder. 7 8ecLovaglnal endomeLrlosls accounLs for 3 Lo 10 of women wlLh endomeLrlosls and ls characLerlsed by Lhe presence of palpable endomeLrloLlc nodules deep ln Lhe connecLlve Llssue of Lhe pelvls, whlch show profound flbrosls and flbromuscular hyperplasla. 14 MulLlfocallLy ls a ma[or characLerlsLlc of ulL leslons. 13 kLC1CVAGINAL LNDCML1kICSIS: CCNIUSICN A8CU1 NAMLS Cullen's orlglnal name for deep pelvlc dlsease was adenomyoma of Lhe recLovaglnal sepLum (Cullen, 1920). 12 lL ls now recognlzed LhaL endomeLrlosls only rarely lnvolves Lhe Lrue recLovaglnal sepLumand much more frequenLly lnvolves Lhe overlylng recLovaglnal pouch. 9 . A varleLy of oLher descrlpLlons has been used Lo descrlbe Lhe anaLomlcal locaLlon of Lhese leslons and lncludes cul-de sac endomeLrlosls (MarLln eL al., 1989), obllLeraLlon of Lhe cul-de-sac (8edwlne, 1991) and recLocervlcal endomeLrlosls (Adamyan eL al., 1993, erry eL al., 1993), 1hese Lhree Lerms were comblned Lo produce an anaLomlcally preclse buL hardly conclse cul-de-sac obllLeraLlon secondary Lo reLrocervlcal deep flbroLlc endomeLrlosls (8elch eL al., 1991). CLher Lerms LhaL have also been used lnclude pelvlc wall-lnfllLraLlng endomeLrlosls (khare eL al., 1996), recLovaglnal sepLum adenomyoLlc nodules (uonnez eL al., 1997), reLroperlLoneal adenomyosls (uonnez, 2001) and recLovaglnal endomeLrlosls (kavallarls eL al., 2003).lrom Lhls revlew lL ls noL lmmedlaLely obvlous whaL Lhese leslons should be called or how we should descrlbe Lhelr locaLlons. 12 CLINICAL ILA1UkLS CI DIL LndomeLrlosls exhlblLs a broad specLrum of cllnlcal manlfesLaLlons: can be asympLomaLlc, belng lncldenLally dlscovered aL laparoscopy or posLmorLem examlnaLlon, 7 or a dlsorder of such severlLy LhaL Lhe sufferers quallLy of llve ls desLroyed. 12 ulL ls known as a severe and palnful modallLy of dlsease. 13 A causal assoclaLlon beLween severe dysmenorrhoea and endomeLrlosls ls very probable. 1hls assoclaLlon ls lndependenL of Lhe macroscoplc Lype of Lhe leslons (superflclal endomeLrlosls, cysLlc ovarlan endomeLrlosls or ulL) or Lhelr anaLomlcal locaLlons and may be relaLed Lo recurrenL cycllc mlcro-bleedlng ln Lhe lmplanLs. LndomeLrlosls-relaLed adheslons may also cause severe dysmenorrhoea. Powever, pelvlc paln may be more common ln women wlLh deep, lnfllLraLlng lmplanLs. 1here are hlsLologlcal and physlopaLhologlcal argumenLs for Lhe responslblllLy of ulL ln severe chronlc pelvlc paln sympLoms. ulL-relaLed paln may be ln relaLlon wlLh compresslon or lnfllLraLlon of nerves ln Lhe sub- perlLoneal pelvlc space by Lhe lmplanLs. 16 1he lnLenslLy of paln ln woman wlLh ulL correlaLes well wlLh Lhe depLh and volume of lnfllLraLlon. 3 1he palnful sympLoms caused by ulL presenL parLlcular characLerlsLlcs, belng speclflc Lo lnvolvemenL of preclse anaLomlcal locaLlons (severe deep dyspareunla, palnful defecaLlon) or organs (funcLlonal urlnary LracL slgns, bowel slgns). 1hey can Lhus be descrlbed as locotloo loJlcotloq polo. 1here ls a clear-cuL relaLlonshlp beLween posLerlor ulL and deep dyspareunla, palnful defecaLlon durlng menses wlLh lnvolvemenL of Lhe posLerlor wall of Lhe vaglna, non-cycllc pelvlc paln and funcLlonal bowel slgns wlLh bowel lnvolvemenL and funcLlonal urlnary LracL slgns wlLh lnvolvemenL of Lhe 3 bladder. A prospecLlve sLudy, based on paLlenLs operaLed by laparoscopy for chronlc pelvlc paln, demonsLraLed LhaL palnful defecaLlon durlng menses and severe dyspareunla were speclflcally connecLed Lo ulL lnvolvlng Lhe posLerlor area compared Lo Lhe oLher dlagnoses (oLher macroscoplc Lype of endomeLrlosls or non-endomeLrlosls dlagnosls). 16 DIL and deep dyspareun|a When dyspareunla ls referred Lo Lhe recLum or lower sacrococcygeal area, lL suggesLs recLovaglnal or uLerosacral llgamenL lnvolvemenL. ln parLlcular, several sLudles correlaLed deep dyspareunla wlLh Lhe presence of endomeLrlosls of Lhe uLerosacral llgamenLs. 1hls correlaLlon ls conslsLenL wlLh Lhe presence of a conslderable amounL of nerve Llssue wlLhln Lhe uLerosacral llgamenLs, dyspareunla may be relaLed Lo Lhe sLlmulaLlon of paln flbers by LracLlon of scarred lnelasLlc Llssues and by pressure on endomeLrloLlc nodules embedded ln flbroLlc Llssues. uyspareunla has been assoclaLed wlLh a negaLlve aLLlLude Loward sexuallLy, anxleLy and avoldance of lnLercourse. Women wlLh dyspareunla, noL surprlslngly, have lower frequency of lnLercourse and lower levels of deslre and arousal and experlence fewer orgasms. 17 Gastro|ntest|na| |nvo|vement |n DIL 8owel endomeLrlosls ls found ln 3 Lo 12 of paLlenLs wlLh endomeLrlosls and colorecLum represenLs 90 of all bowel locaLlons. 4 SympLoms range from recLal bleedlng, urgency, pelvlc paln, severe dyschezla, bowel cramplng and alLeraLlon ln bowel hablL from dlarrhea Lo obsLrucLlon of Lhe colon. 18 SympLoms usually occur cycllcally aL or abouL Lhe Llme of mensLruaLlon. lnLesLlnal endomeLrlosls should be suspecLed ln women of chlldbearlng age who presenL wlLh gasLrolnLesLlnal sympLoms and a hlsLory of endomeLrlosls. 6 MallgnanL change ln colonlc endomeLrlosls has been documenLed. 1he raLe of progresslon ls unknown buL ls uncommon. 12 Gen|tour|nary |nvo|vement |n DIL 1he urlnary LracL ls lnvolved ln 1 Lo 4 of women wlLh endomeLrlosls of whlch around 90 lnvolve Lhe bladder. aLlenL presenLaLlon ln veslcal endomeLrlosls ls qulLe varlable, and sympLoms may conslsL of suprapublc dlscomforL, pelvlc paln, dysmenorrheal, dysurla, urlnary frequency, urgency, mlcroscoplc hemaLurla, and even cycllcal gross hemaLurla. 6 MallgnanL LransformaLlon of bladder endomeLrlosls alLhough rare has been descrlbed repeaLedly and musL represenL a rlsk lf bladder leslons remaln unLreaLed. 12 ureLeral endomeLrlosls ls uncommon, wlLh an lncldence of less Lhan 0.1 of all cases of endomeLrlosls. CbsLrucLlve uropaLhy LhaL may lead Lo renal corLlcal aLrophy and severe loss of renal funcLlon can occur wlLh Lhls Lype of leslon. 6 DIAGNCSIS CI DIL LndomeLrlosls remalns dlfflculL Lo dlagnose, wlLh a delay of 8-11 years beLween flrsL reporL of sympLoms and recognlLlon of dlsease. 19 unforLunaLely, Lhe subsLanclal advances ln our undersLandlng of Lhe paLhogenesls of endomeLrlosls have noL yeL provlded any rellable nonlnvaslve alLernaLlve Lo laparoscopy for dlagnosls of Lhe dlsease. 3 hys|ca| exam|nat|on ln endomeLrlosls physlcal examlnaLlon of Lhe exLernal genlLalla ls Lyplcally normal. Cccaslonally, speculum examlnaLlon may reveal Lyplcal blue-colored lmplanLs or red prollferaLlve leslons LhaL bleed on conLacL, boLh usually ln Lhe posLerlor fornlx. Whereas dlsease ln women wlLh ulL lnvolvlng Lhe recLovaglnal sepLum ls frequenLly palpable, lL ls much less ofLen vlslble, and ln many Lhere ls no remarkable flndlngs. 3 Analysls of Lhe flndlngs on vaglnal examlnaLlon ln sympLomaLlc paLlenLs wlLh presumed endomeLrlosls suggesLs LhaL palpable lnduraLlon or nodules ln Lhe posLerlor vaglnal fornlx and/or along Lhe uLerosacral llgamenLs appears Lo be paLhognomonlc slgns of deep endomeLrlosls. 12 1hese leslons are Lender and pressure reproduces sympLoms. 1he predlcLlve poslLlve value of such Lenderness predlcLlng endomeLrlosls ls beLween 76 and 79 and Lhls rlses Lo 83 wlLh a speclflclLy of 92 lf focal Lenderness ls locaLed only ln Lhe uLerosacral llgamenLs and cul de sac. 1he accuracy of Lhese flndlngs ls furLher lncreased lf Lhe examlnaLlon ls performed durlng mensLruaLlon. ueep lnvaslve endomeLrlosls ls frequenLly assoclaLed wlLh ovarlan endomeLrlosls. 1he co-exlsLence of Lhese Lwo condlLlons furLher lmproves Lhe rellablllLy of Lhe cllnlcal dlagnosls. ln summary, Lhe presence or absence of palpable nodular or lnfllLraLlve leslons ls relaLed Lo Lhe locaLlon and depLh of Lhe leslons and reflecLs Lhe severlLy of Lhe paln and Lhe rlsks of severe compllcaLlons. 12 Imag|ng stud|es hyslcal examlnaLlon has a llmlLed capaclLy Lo 6 dlagnose and quanLlfy ulL and Lransvaglnal, LransrecLal or recLal endoscoplc sonography as well as magneLlc resonance lmaglng (M8l) has all been recommended for lLs dlagnosls and for deLermlnlng lLs locaLlon. 20 1he slgnlflcanL role of M8l ln Lhe dlagnosls of endomeLrlosls ls relaLed Lo Lhe ldenLlflcaLlon of lnLermlngled leslons ln Lhe presence of adheslons, and also Lhe demonsLraLlon and evaluaLlon of sub- perlLoneal leslons exLenL ln cases where Lhese leslon cannoL be vlsuallzed by laparoscopy, wlLh accuracy, senslLlvlLy and speclflclLy > 90 for deep endomeLrlosls. ln paLlenLs wlLh deep pelvlc endomeLrlosls, cllnlcal and sonographlc resulLs may be normal or poorly elucldaLlve, dlfflculLlng Lhe dlagnosls deLermlnaLlon. ln Lhese cases, M8l ls essenLlal for an accuraLe dlfferenLlal dlagnosls. 8ecause of lLs mulLlplanar capaclLy and excellenL Llssue characLerlzaLlon, M8l plays an essenLlal role ln Lhe preoperaLlve evaluaLlon of paLlenLs wlLh deep pelvlc endomeLrlosls. 21 Serum CA 12S AlLhough women wlLh endomeLrlosls ofLen have hlgh (greaLer Lhan 33 lu/mL) serum CA 123 concenLraLlons, serum CA 123 ls noL a senslLlve lndlcaLor of Lhe dlsease. Powever, knowledge of an elevaLed preoperaLlve CA 123 concenLraLlon may be useful for selecLlng women who are aL hlgh rlsk for bowel ln[ury because of dense pelvlc adheslons and Lhus mosL llkely Lo beneflL from preoperaLlve bowel preparaLlon. 3,7 Laparoscopy Laparoscopy ls Lhe preferred Lechnlque for dlagnosls slnce endomeLrlosls ls locaLed prlmarlly on Lhe pelvlc organs. 1he opLlmal Llmlng of laparoscopy aL a speclflc Llme ln Lhe mensLrual cycle ls unclear, buL lL should noL be performed durlng or wlLhln Lhree monLhs of hormonal LreaLmenL so as Lo avold under- dlagnosls. ldeally, lf surgery ls performed for dlagnosls, consenL ls obLalned for surglcal resecLlon/ablaLlon of endomeLrlosls aL Lhe same Llme. 3,7 1kLA1MLN1 CI DIL Med|ca| treatment of DIL lL ls well esLabllshed LhaL hormonal drugs do noL cure endomeLrlosls buL only lnduce Lemporary qulescence of acLlve leslons and LhaL ln mosL cases of advanced dlsease, surgery ls Lhe flnal soluLlon. Powever, Lhere are several slLuaLlons ln whlch medlcal LreaLmenLs are sLlll useful. Some women who have already undergone several operaLlons mlghL prefer Lo avold furLher surgery buL need paln rellef, and oLhers may wanL only Lo posLpone surgery because of sLudy, work, or famlly problems. lurLhermore, drugs may be chosen as an alLernaLlve Lo surgery ln Lhe rare very dlfflculL cases ln whlch Lhe rlsks of morbldlLy and compllcaLlons ouLwelgh Lhe beneflLs of a radlcal operaLlon. Long-Lerm paln rellef ls Lhe maln ob[ecLlve, and greaL care should be pald Lo Lhe cholce of drug. ulL has been LreaLed successfully wlLh danazol, gonadoLropln-releaslng hormone analogs, progesLlns, and esLrogen-progesLln comblnaLlons. rogesLlns should be consldered as flrsL-llne medlcal LreaLmenL for Lemporary paln rellef. 22 Conservat|ve surg|ca| treatment of DIL ln mosL cases of severely lnfllLraLlng dlsease, surgery ls Lhe LreaLmenL of reference. 13 Surglcal LreaLmenL ls effecLlve for rellevlng pelvlc paln, dyspareunla, and palnful defecaLlon. 2 ln general, deep endomeLrloLlc leslons should noL always be LreaLed [usL because Lhey are Lhere. lnLesLlnal and ureLeral focl LhaL cause progresslve sLenosls consLlLuLe lndlspuLable reasons for operaLlng. CLherwlse, surgery for asympLomaLlc ulL should noL be consldered mandaLory ln all cases. 1he resulLs of LreaLmenL for a benlgn condlLlon such as endomeLrlosls are funcLlonal, and surglcal lndlcaLlons should be based malnly on sympLoms and nonresponse Lo medlcal Lheraples. 22 1he locaLlon of Lhe ulL leslons musL dlcLaLe Lhe cholce of operaLlng Lechnlque. ln mulLlfocal cases, several surglcal procedures musL be assoclaLed. lor bladder ulL, Lhe sLandard LreaLmenL ls parLlal cysLecLomy whlch can be performed by operaLlve laparoscopy. lor ulL lnfllLraLlng Lhe uLerosacral llgamenLs, lL has been shown LhaL laparoscoplc surglcal resecLlon ls efflclenL. lor vaglnal ulL, numerous auLhors have demonsLraLed LhaL operaLlve laparoscopy ls efflclenL uslng varlous Lechnlques (elecLrosurgery, sharp dlssecLlon or laser CC2, excluslvely laparoscoplc procedure or laparoscoplcally asslsLed vaglnal surgery). 1he dlfferenL Lopographlcal locaLlon of uLerosacral llgamenLs and vaglnal ulL requlres speclflc operaLlng Lechnlques. Leslons sLrlcLly locaLed on Lhe uLerosacral llgamenLs requlre, ln Lhe ma[orlLy of paLlenLs, ureLerolysls wlLhouL assoclaLed exeresls of Lhe upper parL of Lhe posLerlor vaglnal wall. ln cases of lsolaLed vaglnal ulL, dlssecLlon of Lhe laLero-recLal fossae ls necessary ln >80 of cases and exeresls of Lhe upper parL of Lhe posLerlor vaglnal wall ls 7 essenLlal. 1he reason for Lhls ls LhaL vaglnal ulL mosL ofLen does noL lnfllLraLe Lhe recLovaglnal sepLum whlch ls locaLed lower down buL raLher Lhe upper Lhlrd of Lhe posLerlor vaglnal wall. 13 1he quesLlon of operaLlve Lechnlque ls far more complex when Lhe dlgesLlve LracL ls lnvolved. Surgery for recLovaglnal endomeLrlosls can be complex and challenglng and ofLen lnvolves a mulLldlsclpllnary Leam. 1here are several approaches Lo be consldered, from shavlng Lhe dlsease off Lhe recLal wall Lo carrylng ouL an exclslon of Lhe anLerlor recLal wall or a segmenLal exclslon of Lhe recLum, none of whlch has been accepLed as besL" pracLlce. All Lhese procedures can be carrled ouL by elLher a laparoscoplc, comblned or open approach. 18 ln women wlLh advanced sympLomaLlc dlsease ln whom medlcal and conservaLlve surglcal LreaLmenL falls, radlcal surglcal LreaLmenL (hysLerecLomy and bllaLeral salplngo-oophorecLomy) merlLs serlous conslderaLlon and dlscusslon. 3 DIL comp||cat|ons and recurrences Surgery for deep endomeLrlosls ls assoclaLed wlLh a relaLlvely hlgh rlsk of posLoperaLlve compllcaLlons, such as de-novo or worsenlng voldlng dysfuncLlon and recLovaglnal flsLula. 2 LndomeLrlosls ls a nonmallgnanL dlsease LhaL usually affecLs young women wlLh hlgh expecLaLlons ln Lerms of concepLlon and quallLy of llfe. ln Lhese clrcumsLances, lnLraoperaLlve and posLoperaLlve compllcaLlons are percelved and LoleraLed wlLh dlfflculLy, and lncapaclLaLlng paln recurrence and perslsLenL lnferLlllLy are parLlcularly frusLraLlng. A Lhorough preoperaLlve dlagnosLlc lnvesLlgaLlon and careful deLalled counsellng are of ma[or lmporLance. lnvolvemenL of Lhe lnLesLlnal and urologlc sysLems should be known ln advance, Lo schedule lnLraoperaLlve consulLaLlon, lf necessary, and Lo lnform Lhe woman abouL Lhe Lype of surgery requlred and lLs poLenLlal sequelae.1hls wlll also help paLlenLs and Lhelr famllles undersLand Lhe cllnlcal severlLy of Lhe condlLlon, and balance Lhe rlsks and beneflLs of Lhe proposed LreaLmenLs. ln parLlcular, as Lhe chances of pregnancy afLer surgery may be llmlLed, an alLernaLlve soluLlon mlghL be chosen such as ln vlLro ferLlllzaLlon or adopLlon. Awareness of Lhe real posslblllLles of dlfferenL LreaLmenLs wlll enhance Lhe paLlenL's collaboraLlon. 22 8ecurrence or perslsLence of endomeLrlosls afLer LreaLmenL ls one of Lhe mosL vexlng problems of gynecology and ls based on Lhe known unpredlcLablllLy of Lhe dlsease. LndomeLrlosls recurrence raLes vary from 2 Lo 47. 1he hlghesL recurrence raLe ls documenLed for deep lnfllLraLlve endomeLrlosls, based on dlfflculLles ln esLlmaLlng Lhe real borders of Lhe lnfllLraLe, as well as Lhe consclous re[ecLlon of more aggresslve approaches Lo removal of Lhe leslons, whlch are locaLed near vlLal organs. 23 1he surgeon should be aware of Lhe blo- psychosoclal naLure of Lhe dlsease: Lhe surgeon who only Lhlnks, for lnsLance, Lo eradlcaLe Lhe leslons of endomeLrlosls, and who does noL conslder all llvlng of Lhe paLlenL wlll ofLen have few successes and many recurrences. 1he key Lo Laklng good charge ls Lherefore Lo acL on boLh aspecLs: psychologlcal and physlcal" (8odolphe Maheux, World LndomeLrlosls AssoclaLlon). 24 1nL IU1UkL. new developmenLs ln flber opLlc endoscopes wlLh dlameLers less Lhan 1 mm have enabled a gynecologlsL Lo dlagnose and sLage Lhe dlsease ln an offlce seLLlng under local anesLhesla, posslbly aL earller sLages. 23 1he developmenL of CenLers of Lxcellence" for Lhe overall managemenL of severe endomeLrlosls has been advocaLed ln recenL publlcaLlons. Worklng ln con[uncLlon wlLh colorecLal and urologlcal surgeons and Lhrough Lhe use of mulLldlsclpllnary Leam meeLlngs approprlaLe case selecLlon can be made Lo ensure LhaL Lhe correcL sklll base ls presenL for curaLlve surgery. 14 kLILkLNCLS 1. 8ulun,SL. LndomeLrlosls. Mechanlsms of dlsease. n Lngl ! Med 2009,360:268-79. 2. Schenken, 8S. Cvervlew of Lhe LreaLmenL of endomeLrlosls. up- Lo-daLe. 2008. 3. Speroff L, lrlLz MA. LndomeLrlosls. ln: Cllnlcal Cynecologlc Lndocrlnology and lnferLlllLy (7 Lh LdlLlon). LlpplncoLL Wllllams &Wllklns, 2003:1103-133. 4. uaral L, eL al. LndomeLrlose recLal eL ferLlllLe. Cynecologle CbsLeLrlque and lerLlllLe. 2008, 36 3. Chapron C, Chopln n, 8orghese 8 eL al. ueeply lnfllLraLlng endomeLrlosls: paLhogeneLlc lmpllcaLlons of Lhe anaLomlcal dlsLrlbuLlon. Pum 8eprod 2006, 21:1839-843. 6. 8urney 8C, Cludlce LC. 1he aLhogenesls of LndomeLrlosls. ln: nezaLs CperaLlve Cynecologlc Laparoscopy and PysLeroscopy (3 Lh LdlLlon). nezhaL C, nezhaL l, nezhaL C (eds). Cambrldge unlverslLy ress, 2008:231-37. 7. Schenken 8S. aLhogenesls, cllnlcal feaLure, and dlagnosls of endomeLrlosls. up-Lo-daLe. 2008 8. !enklns S, Cllve uL, Paney Al. LndomeLrlosls: paLhogeneLlc lmpllcaLlons of Lhe anaLomlc dlsLrlbuLlon. CbsLeL Cynecol 1986,67:333-38. 9. vercelllnl , Alml C, anazza S, vlncenLl S, lacreLa A , Croslgnanl C. ueep endomeLrlosls conundrum: evldence ln favor of a perlLoneal orlgln. lerLll SLerll 2000,73:1043-046. 10. Chapron C, Llaras L, layeL , Poeffel C et ol. MagneLlc resonance lmaglng and endomeLrlosls: deeply lnfllLraLlng endomeLrlosls does noL orlglnaLe from Lhe recLovaglnal sepLum. Cynecol CbsLeL lnvesL 2002, 33:204-08. 8 11. Abro MS, neme 8M, Averbach M. LndomeLrlose do sepLo reLovaglnal: doena de dlagnsLlco e LraLamenLo especlflco.Arq CasLroenLerol 2003. 40:192-97. 12. Carry 8. 1he endomeLrlosls syndromes: a cllnlcal classlflcaLlon ln Lhe presence of aeLlologlcal confuslon and LherapeuLlc anarchy. Pum 8eprod 2004,19:760-8. 13. Chapron C, lauconnler A, vlelra M eL al. AnaLomlcal dlsLrlbuLlon of deeply lnfllLraLlng endomeLrlosls: surglcal lmpllcaLlons and proposlLlon for a classlflcaLlon. Pum 8eprod 2003,18:137-61. 14. Slack A, Chlld 1, kennedy S eL al. urologlcal and colorecLal compllcaLlons followlng surgery for recLovaglnal endomeLrlosls. 8!CC 2007,114:1278-282. 13. 8lbelro A, 8odrlgues lC, kehdl l eL al. Laparoscoplc resecLlon of lnLesLlnal endomeLrlosls: a 3-year experlence. ! Mlnlm lnvaslve Cynecol.2006,13:442-446. 16. lauconler A, Chapron C. LndomeLrlosls and pelvlc paln: epldemlologlcal evldence of Lhe relaLlonshlp and lmpllcaLlons. Pum 8eprod 2003, 11:393-606. 17. lerrero S, AbbamonLe LP, Clordano M eL al. ueep dyspareunla and sex llfe afLer laparoscoplc exclslon of endomeLrlosls. Pum reprod 2007,22:1142-148. 18. 8rouwer 8, Woods 8!. 8ecLal endomeLrlosls: resulLs of radlcal exclslon and revlew of publlshed work. AnZ !. Surg. 2007, 77: 362- 371 19. PudellsL C, kecksLeln !, WrlghL !1. 1he mlgraLlng adenomyoma: pasL vlews on Lhe eLlology of adenomyosls and endomeLrlosls. lerLll SLerll 2008. 20. 8azoL M, Malzy , CorLez A eL al. Accuracy of Lransvaglnal sonography and recLal endoscoplc sonography ln Lhe dlagnosls of deep lnfllLraLlng endomeLrlosls.ulLrasound CbsLeL Cynecol 2007, 30: 994-1001. 21. !unlor AC, Llma CM, CouLlnho L eL al. MagneLlc resonance lmaglng ln deep pelvlc endomeLrlosls: lconographlc essay.8adlol 8ras 2008, 41:129-34 22. vercelllnl , lronLlno C, leLrlpaolo C eL al. ueep endomeLrlosls: deflnlLlon, paLhogenesls and cllnlcal managemenL. ! Am Assoc Cynecolo Laparosc 2004, 11(2):133-61. 23. 8erker 8, Psu 1S, Lee kL eL al. Laparoscoplc LreaLmenL of endomeLrlosls. ln: nezaLs CperaLlve Cynecologlc Laparoscopy and PysLeroscopy (3 Lh LdlLlon). nezhaL C, nezhaL l, nezhaL C (eds). Cambrldge unlverslLy ress, 2008:231-37. 24. 8elalsch !. 8eflecLlons on Lhe medlcal LreaLmenL of endomeLrlosls: an lmporLanL conLrlbuLlon whlch could be more wldely used. 178-184