{"id":284,"date":"2013-03-14T09:37:01","date_gmt":"2013-03-14T09:37:01","guid":{"rendered":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/?page_id=284"},"modified":"2013-06-09T13:43:11","modified_gmt":"2013-06-09T13:43:11","slug":"7-2","status":"publish","type":"page","link":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/?page_id=284","title":{"rendered":"7 Vrozen\u00e9 vady j\u00edcnu"},"content":{"rendered":"

Kongenit\u00e1ln\u00ed vady j\u00edcnu jsou z\u00e1va\u017en\u00fdm vrozen\u00fdm posti\u017een\u00edm, kter\u00e9 se vyskytuje bu\u010f samostatn\u011b, nebo v kombinaci s dal\u0161\u00edmi vrozen\u00fdmi vadami (v\u00edce ne\u017e v 50 % b\u00fdvaj\u00ed sou\u010dasn\u011b p\u0159\u00edtomny dal\u0161\u00ed vrozen\u00e9 vady na za\u017e\u00edvac\u00edm traktu, srdci, urogenit\u00e1ln\u00edm syst\u00e9mu a muskuloskelet\u00e1rn\u00edm syst\u00e9mu). Prenat\u00e1ln\u00ed diagnostika t\u011bchto vad je mo\u017en\u00e1 d\u00edky zdokonalen\u00ed ultrazvukov\u00e9ho vy\u0161et\u0159en\u00ed a\u017e v posledn\u00edch desetilet\u00edch. Progn\u00f3za t\u00e9to vady je velmi z\u00e1va\u017en\u00e1, proto\u017ee \u0159ada t\u011bchto d\u011bt\u00ed se rod\u00ed p\u0159ed\u010dasn\u011b a p\u0159es pokroky d\u011btsk\u00e9 chirurgie nen\u00ed jejich \u0159e\u0161en\u00ed v\u017edy \u00fasp\u011b\u0161n\u00e9.<\/p>\n

7.1 Historie a sou\u010dasnost<\/h3>\n

V roce 1697 popsal Thomass Gibson [1] d\u00edt\u011b, kter\u00e9 se p\u0159i polyk\u00e1n\u00ed dusilo a potrava se mu vracela nosem a \u00fasty. V n\u00e1sleduj\u00edc\u00edch letech bylo mnoho ne\u00fasp\u011b\u0161n\u00fdch pokus\u016f o opera\u010dn\u00ed \u0159e\u0161en\u00ed pacient\u016f s ezofage\u00e1ln\u00ed atr\u00e9zi\u00ed a tracheoezofage\u00e1ln\u00ed p\u00ed\u0161t\u011bl\u00ed. Marn\u00e1 snaha chirurg\u016f vedla k z\u00e1v\u011bru, \u017ee posti\u017een\u00e9 d\u011bti t\u011bmito anom\u00e1liemi je nejlep\u0161\u00ed nechat zem\u0159\u00edt. Teprve s rozvojem d\u011btsk\u00e9 chirurgie od poloviny minul\u00e9ho stolet\u00ed doch\u00e1z\u00ed k postupn\u00e9mu zlep\u0161ov\u00e1n\u00ed vyhl\u00eddek u d\u011bt\u00ed posti\u017een\u00fdch touto vrozenou anom\u00e1li\u00ed [2, 3]. Nez\u00e1visle na sob\u011b v roce 1939 Leven [4] a Ladd [3] \u00fasp\u011b\u0161n\u011b operovali atr\u00e9zii j\u00edcnu. Provedli gastrostomii a kr\u010dn\u00ed ezofagostomii, ligovali p\u00ed\u0161t\u011bl a n\u00e1sledn\u011b vytvo\u0159ili antestern\u00e1ln\u00ed neoezofagus s \u00fasp\u011b\u0161n\u00fdm v\u00fdsledkem. Haigh a Towsley provedli \u00fasp\u011b\u0161nou operaci o 2 roky pozd\u011bji. Pou\u017eili levostrann\u00fd extrapleur\u00e1ln\u00ed p\u0159\u00edstup k ligaci p\u00ed\u0161t\u011ble a prim\u00e1rn\u00ed anastom\u00f3zu j\u00edcnu [5]. Tak za\u010dala modern\u00ed \u00e9ra novorozeneck\u00e9 j\u00edcnov\u00e9 chirurgie. Podle \u0160najdaufa [16] prvn\u00ed \u00fasp\u011b\u0161nou anastom\u00f3zu vrozen\u00e9 atr\u00e9zie j\u00edcnu v \u010desk\u00fdch zem\u00edch provedli v roce 1953 Van\u011b\u010dkov\u00e1 a Kafka. Pozd\u011bji se t\u00e9to problematice v\u011bnoval M. Kabelka.<\/p>\n

Chirurgick\u00e1 l\u00e9\u010dba vrozen\u00fdch vad j\u00edcnu byla v na\u0161\u00edch zem\u00edch po roce 1945 p\u0159irozen\u011b v\u00e1z\u00e1na na pracovi\u0161t\u011b, kde se prov\u00e1d\u011bla j\u00edcnov\u00e1 chirurgie, a k takov\u00fdm pat\u0159ilo i olomouck\u00e9 po p\u0159\u00edchodu prof. MUDr. Rapanta na obnovenou Univerzitu Palack\u00e9ho v Olomouci v roce 1946. Z jeho \u017e\u00e1k\u016f se touto problematikou zab\u00fdval p\u0159edev\u0161\u00edm doc. MUDr. Eduard Farga\u0161, CSc., kter\u00fd se v\u011bnoval d\u011btsk\u00e9 chirurgii v cel\u00e9m rozsahu. \u00dasp\u011b\u0161n\u011b operoval tyto vrozen\u00e9 vady i dal\u0161\u00ed Rapant\u016fv \u017e\u00e1k, pozd\u011bj\u0161\u00ed prim\u00e1\u0159 chirurgick\u00e9ho odd\u011blen\u00ed nemocnice ve \u0160ternberku, Miloslav \u010cerm\u00e1k [6, 7]. Jedno z d\u011bt\u00ed operovan\u00fdch M. \u010cerm\u00e1kem jsme m\u011bli mo\u017enost sledovat po dobu tak\u0159ka pades\u00e1ti let.<\/p>\n

Kazuistika \u2013 sledov\u00e1n\u00ed nemocn\u00e9 48 rok\u016f po operaci atr\u00e9zie j\u00edcnu\"\"<\/h6>\n

U hol\u010di\u010dky narozen\u00e9 v roce 1960 byla po narozen\u00ed diagnostikov\u00e1na atr\u00e9zie j\u00edcnu bez p\u00ed\u0161t\u011ble (Vogt II). Prvn\u00ed den po porodu byla operov\u00e1na, provedena prim\u00e1rn\u00ed end-to-end anastom\u00f3za. Poopera\u010dn\u00ed pr\u016fb\u011bh byl bez komplikac\u00ed a rovn\u011b\u017e dal\u0161\u00ed v\u00fdvoj d\u00edt\u011bte a\u017e do dosp\u011blosti byl norm\u00e1ln\u00ed a\u017e na p\u0159\u00edznaky chronick\u00e9ho z\u00e1n\u011btu pr\u016fdu\u0161ek. V 15 letech prod\u011blala z\u00e1pal plic se z\u00e1n\u011btem pohrudnice. V dosp\u011blosti se st\u00e1le v\u00edce projevovaly p\u0159\u00edznaky gastroezofage\u00e1ln\u00edho refluxu s projevy pyr\u00f3zy a ob\u010dasnou regurgitac\u00ed a po t\u0159ic\u00e1t\u00e9m roku v\u011bku i s ob\u010dasnou dysfagi\u00ed. Na z\u00e1klad\u011b rentgenov\u00e9ho a endoskopick\u00e9ho vy\u0161et\u0159en\u00ed byla stanovena diagn\u00f3za fixovan\u00e9 skluzn\u00e9 hi\u00e1tov\u00e9 hernie, brachyezofagu a zn\u00e1mky refluxn\u00ed ezofagitidy. Mezi t\u0159ic\u00e1t\u00fdm a\u017e p\u011btat\u0159ic\u00e1t\u00fdm rokem v\u011bku brala dlouhodob\u011b prokinetika, omeprazol a opakovan\u011b byla prov\u00e1d\u011bna dilatace j\u00edcnu. Dysfagick\u00e9 obt\u00ed\u017ee po t\u00e9to l\u00e9\u010db\u011b vymizely, ale v\u00fdrazn\u00e9 refluxn\u00ed obt\u00ed\u017ee p\u0159etrv\u00e1valy, v\u010detn\u011b regurgitace, zvl\u00e1\u0161t\u011b po vysazen\u00ed medikament\u00f3zn\u00ed terapie. Proto u n\u00ed byla ve 38 letech indikov\u00e1na antirefluxn\u00ed operace. Vzhledem k ireponibiln\u00edmu kr\u00e1tk\u00e9mu j\u00edcnu nebyla mo\u017en\u00e1 obvykl\u00e1 fundoplikace a bylo nutn\u00e9 prov\u00e9st tzv. nep\u0159\u00edm\u00fd v\u00fdkon, tj. resekci \u017ealudku s rekonstrukc\u00ed pas\u00e1\u017ee Rouxovou kli\u010dkou. Refluxn\u00ed obt\u00ed\u017ee po t\u00e9to operaci ustoupily. Po deseti letech byla pro neur\u010dit\u00e9 bolesti b\u0159icha z\u00e1visl\u00e9 na j\u00eddle kompletn\u011b vy\u0161et\u0159ena gastroenterologem. P\u0159i gastroskopii p\u0159etrv\u00e1valy zn\u00e1mky brachyezofagu, Z linie byla 35 cm od \u0159ez\u00e1k\u016f, n\u00e1lez na sliznici j\u00edcnu i v pah\u00fdlu \u017ealudku po resekci byl v\u0161ak klidn\u00fd. P\u0159i ultrazvukov\u00e9m vy\u0161et\u0159en\u00ed b\u0159icha byla prok\u00e1z\u00e1na v\u00edce\u010detn\u00e1 cholecystoliti\u00e1za a pacientka v 48 letech v\u011bku podstoupila nekomplikovanou laparoskopickou cholecystektomii, po kter\u00e9 do\u0161lo k \u00fastupu za\u017e\u00edvac\u00edch obt\u00ed\u017e\u00ed.<\/p>\n

S odchodem doc. MUDr. Farga\u0161e nejprve na zahrani\u010dn\u00ed expertizu a po n\u00e1vratu po p\u0159evzet\u00ed funkce p\u0159ednosty II. chirurgick\u00e9 kliniky do\u0161lo postupn\u011b v Olomouci ke zm\u011bn\u011b profilu d\u011btsk\u00fdch a zejm\u00e9na novorozeneck\u00fdch operac\u00ed, kter\u00e9 se prov\u00e1d\u011bly v men\u0161\u00edm po\u010dtu. Nepochybn\u011b na to m\u011bla vliv i celkov\u00e1 reorganizace p\u00e9\u010de o d\u011bti v \u010cesk\u00e9 republice, kdy\u017e se vytvo\u0159ila dv\u011b centra pro d\u011btskou chirurgii, a to v Praze a v Brn\u011b, kam byla st\u00e1le siln\u011bj\u0161\u00ed tendence specializovan\u00e9 operace koncentrovat [8\u201316]. Operace pro vrozen\u00e9 vady j\u00edcnu se tak v Olomouci od 70. let minul\u00e9ho stolet\u00ed prov\u00e1d\u011bly m\u00e9n\u011b \u010dasto [17]. Mimo centrum d\u011btsk\u00e9 chirurgie v Brn\u011b byla na Morav\u011b Ostrava dal\u0161\u00edm m\u00edstem, kde se novorozeneck\u00e1 chirurgie prov\u00e1d\u011bla a prov\u00e1d\u00ed ve v\u011bt\u0161\u00ed m\u00ed\u0159e. To bylo tak\u00e9 d\u016fvodem, pro\u010d jsem v t\u00e9to kapitole zvolil ke spolupr\u00e1ci toto pracovi\u0161t\u011b.<\/p>\n

Soubor pacient\u016f hospitalizovan\u00fdch na jednotce intenzivn\u00ed a resuscita\u010dn\u00ed p\u00e9\u010de pro novorozence (JIRPN) v Ostrav\u011b<\/h6>\n

Spoluautorka t\u00e9to kapitoly (H. Pode\u0161vov\u00e1) vedla neonatologick\u00e9 odd\u011blen\u00ed ve FN Ostrava v letech 1978 a\u017e 2009. Od roku 1988 do roku 2009 z\u00edskala zku\u0161enosti spolu s d\u011btsk\u00fdmi chirurgy (J. Kopeck\u00fd, V. Richter) s 58 d\u011btmi s vrozenou vadou j\u00edcnu.<\/p>\n

V letech 1988 a\u017e 2009 bylo na Jednotce intenzivn\u00ed a resuscita\u010dn\u00ed p\u00e9\u010de pro novorozence (JIRPN) Krajsk\u00e9, pozd\u011bji Fakultn\u00ed nemocnice v Ostrav\u011b hospitalizov\u00e1no 58 novorozenc\u016f s vrozenou vadou j\u00edcnu. Jednalo se o 33 chlapc\u016f a 25 d\u00edvek (57 : 43 %). U 43 d\u011bt\u00ed se jednalo o malformaci typu Vogt IIIb, po 6 d\u011btech m\u011blo typ Vogt II a izolovanou tracheoezofage\u00e1ln\u00ed (\u201eH\u201c) p\u00ed\u0161t\u011bl, u 3 d\u011bt\u00ed \u0161lo o typ Vogt IIIc. Malformace typu Vogt IIIb se tedy vyskytla u 74 % pacient\u016f, literatura ud\u00e1v\u00e1 v\u00fdskyt t\u00e9to nej\u010dast\u011bj\u0161\u00ed formy a\u017e v 90 % [18].<\/p>\n

Pacienti jsou rozd\u011bleni do dvou obdob\u00ed, kter\u00e1 se li\u0161\u00ed hlavn\u011b vybaven\u00edm JIRPN. Teprve od roku 2000 byla na JIRPN adekv\u00e1tn\u00ed technika k um\u011bl\u00e9 plicn\u00ed ventilaci, kter\u00e1 umo\u017enila \u00fasp\u011b\u0161n\u011b zachra\u0148ovat nedono\u0161en\u00e9 novorozence ni\u017e\u0161\u00edch gesta\u010dn\u00edch t\u00fddn\u016f a ni\u017e\u0161\u00ed porodn\u00ed hmotnosti s uveden\u00fdmi vrozen\u00fdmi vadami j\u00edcnu. V cel\u00e9 skupin\u011b pacient\u016f z obdob\u00ed 1988 a\u017e 2009 bylo z 58 hospitalizovan\u00fdch d\u011bt\u00ed 23 dono\u0161en\u00fdch (narozen\u00fdch ve 38.\u201342. gesta\u010dn\u00edm t\u00fddnu), tj. 39,7 %. Z nich 12 (52 %) m\u011blo p\u0159idru\u017een\u00e9 vrozen\u00e9 v\u00fdvojov\u00e9 vady. Z t\u00e9to skupiny zem\u0159elo pouze 1 d\u00edt\u011b, tzn. p\u0159e\u017eit\u00ed je 95,7 %, co\u017e je v souladu s \u00fadaji zahrani\u010dn\u00edch autor\u016f [19] (tab. 1, 2).<\/p>\n\n\n\n\n\n\n\n
Tab. 1<\/span>
\n Soubor pacient\u016f s atr\u00e9zi\u00ed j\u00edcnu v letech 1988\u20131999<\/span><\/td>\n<\/tr>\n
1988\u20131999<\/td>\nPr\u016fm\u011brn\u00e1
\nporodn\u00ed<\/span>
\nhmotnost<\/span><\/td>\n		Pr\u016fm\u011brn\u00fd
\nt\u00fdden<\/span>
\ngestace<\/span><\/td>\n		P\u0159idru\u017een\u00e9
\nVVV*<\/span><\/td>\n		Po\u010det zem\u0159el\u00fdch<\/td>\n<\/tr>\n
Po\u010det novorozenc\u016f celkem<\/td>\n37<\/td>\n<\/td>\n<\/td>\n20 (54 %)<\/td>\n11 (29,7 %)<\/td>\n<\/tr>\n
Po\u010det operovan\u00fdch novorozenc\u016f<\/td>\n30<\/td>\n2498 g
\n(1600\u20133850 g)<\/span><\/td>\n		36,0 (32\u201341)<\/td>\n15 (50 %)<\/td>\n4 (13,3 %)<\/td>\n<\/tr>\n
Po\u010det neoperovan\u00fdch novorozenc\u016f<\/td>\n7<\/td>\n1186 g
\n(750\u20132100 g)<\/span><\/td>\n		30,6 (28\u201334)<\/td>\n5 (71 %)<\/td>\n7 (100 %)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

* VVV \u2013 vrozen\u00e9 v\u00fdvojov\u00e9 vady<\/em>
\n.<\/span><\/p>\n\n\n\n\n\n\n\n
Tab. 2
\nSoubor pacient\u016f s atr\u00e9zi\u00ed j\u00edcnu v letech 2000\u20132009<\/span><\/td>\n<\/tr>\n
2000\u20132009<\/td>\nPr\u016fm\u011brn\u00e1 porodn\u00ed hmotnost<\/td>\nPr\u016fm\u011brn\u00fd t\u00fdden gestace<\/td>\nP\u0159idru\u017een\u00e9VVV<\/td>\nPo\u010det zem\u0159el\u00fdch<\/td>\n<\/tr>\n
Po\u010det novorozenc\u016f celkem<\/td>\n21<\/td>\n<\/td>\n<\/td>\n12 (57 %)<\/td>\n4 (19,0 %)<\/td>\n<\/tr>\n
Po\u010det operovan\u00fdch novorozenc\u016f<\/td>\n19<\/td>\n2456 g
\n(920\u20133500 g)<\/span><\/td>\n		36,2 (29\u201342)<\/td>\n10 (53 %)<\/td>\n2 (10,5 %)<\/td>\n<\/tr>\n
Po\u010det neoperovan\u00fdch novorozenc\u016f<\/td>\n2<\/td>\n695 g
\n(650\u2013740 g)<\/span><\/td>\n		27,0 (26\u201328)<\/td>\n2 (100 %)<\/td>\n2 (100 %)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

*VVV \u2013 vrozen\u00e9 v\u00fdvojov\u00e9 vady<\/em><\/p>\n

.<\/span><\/p>\n

\"Obr.<\/a>

Obr. 1
Ezofagogram vrozen\u00e9 atr\u00e9zie j\u00edcnu, v p\u0159edozadn\u00ed projekci kontrastn\u00ed n\u00e1pl\u0148 dilatovan\u00e9ho horn\u00edho pah\u00fdlu j\u00edcnu se zavedenou sondou (rtg sn\u00edmek z osobn\u00edho archivu H. Pode\u0161vov\u00e9, zhotoven\u00fd na radiologick\u00e9m odd\u011blen\u00ed FN Ostrava, zve\u0159ejn\u011bn\u00fd se souhlasem prim\u00e1\u0159ky tohoto odd\u011blen\u00ed MUDr. A. Jahodov\u00e9)<\/p><\/div>\n

U v\u011bt\u0161iny mal\u00fdch pacient\u016f byla atr\u00e9zie j\u00edcnu diagnostikov\u00e1na a\u017e po narozen\u00ed, u n\u011bkter\u00fdch bylo na tuto vadu podez\u0159en\u00ed ji\u017e prenat\u00e1ln\u011b pro polyhydramnion u matky. Centralizac\u00ed t\u011bhotensk\u00fdch patologi\u00ed do Perinatologick\u00e9ho centra se tyto d\u011bti ve velk\u00e9 m\u00ed\u0159e narodily pr\u00e1v\u011b v tomto centru. Hlavn\u00edm \u00fakolem je d\u00edt\u011b s touto anom\u00e1li\u00ed po narozen\u00ed stabilizovat, zvl\u00e1\u0161t\u011b je-li nedono\u0161en\u00e9. D\u00edt\u011b je t\u0159eba ulo\u017eit na prav\u00fd bok se zv\u00fd\u0161enou horn\u00ed polovinou t\u011bla, aby se v p\u0159\u00edpad\u011b p\u0159\u00edtomnosti tracheoezofage\u00e1ln\u00ed p\u00ed\u0161t\u011ble minimalizoval reflux \u017ealude\u010dn\u00edho obsahu do d\u00fdchac\u00edch cest. Do horn\u00ed slep\u00e9 \u010d\u00e1sti j\u00edcnu se zavede sonda, kterou mus\u00ed b\u00fdt velmi frekventn\u011b ods\u00e1v\u00e1ny sliny. V p\u0159\u00edpad\u011b, \u017ee jde o d\u00edt\u011b nezral\u00e9 a vy\u017eaduj\u00edc\u00ed um\u011blou plicn\u00ed ventilaci, je dobr\u00e9 d\u00edt\u011b zaintubovat sp\u00ed\u0161e v\u011bt\u0161\u00ed endotrache\u00e1ln\u00ed kanylou a je nutn\u00e1 ventilace s co nejni\u017e\u0161\u00edmi inspira\u010dn\u00edmi tlaky, abychom minimalizovali \u00fanik plyn\u016f kolem endotrache\u00e1ln\u00ed rourky do \u017ealudku a st\u0159ev, a p\u0159ede\u0161li tak jejich nadm\u011brn\u00e9 distenzi, vysok\u00e9mu stavu br\u00e1nice a zhor\u0161en\u00ed ventila\u010dn\u00edch pom\u011br\u016f. Diagnostiku prov\u00e1d\u00edme na l\u016f\u017eku, kdy ve svisl\u00e9 nebo alespo\u0148 zv\u00fd\u0161en\u00e9 poloze provedeme rtg sn\u00edmek s mal\u00fdm mno\u017estv\u00edm vodn\u00e9 kontrastn\u00ed l\u00e1tky aplikovan\u00e9 sondou, kterou po expozici okam\u017eit\u011b odsajeme (obr. 1). P\u00e1tr\u00e1me po p\u0159idru\u017een\u00fdch malformac\u00edch. Zku\u0161en\u00fd d\u011btsk\u00fd kardiolog prov\u00e1d\u00ed echokardiografick\u00e9 vy\u0161et\u0159en\u00ed a nutn\u00e9 je i ultrazvukov\u00e9 vy\u0161et\u0159en\u00ed b\u0159icha. Po stabilizaci, zvl\u00e1\u0161t\u011b novorozenc\u016f t\u011b\u017ece nedono\u0161en\u00fdch, d\u011bt\u00ed se z\u00e1va\u017enou srde\u010dn\u00ed vadou a d\u011bt\u00ed s aspira\u010dn\u00ed pneumoni\u00ed, spolu s d\u011btsk\u00fdm chirurgem napl\u00e1nujeme opera\u010dn\u00ed \u0159e\u0161en\u00ed, kter\u00e9 v\u011bt\u0161inou snese ur\u010dit\u00fd odklad, nejde o superurgentn\u00ed v\u00fdkon. Po operaci\u00a0je d\u00edt\u011b hospitalizovan\u00e9 na JIRPN, m\u00e1 zavedenou nasogastrickou sondu, u kter\u00e9 je nutn\u00e9 zabr\u00e1nit dislokaci. N\u011bkolik poopera\u010dn\u00edch dn\u016f je novorozenec podp\u016frn\u011b um\u011ble ventilov\u00e1n a \u0161etrn\u011b ods\u00e1v\u00e1n ze zaveden\u00e9ho pravostrann\u00e9ho hrudn\u00edho dr\u00e9nu, kter\u00fd odstra\u0148ujeme po dohod\u011b s chirurgem. Vyh\u00fdb\u00e1me se hyperhydrataci d\u00edt\u011bte zvl\u00e1\u0161t\u011b p\u0159i anastom\u00f3ze j\u00edcnu pod nap\u011bt\u00edm, ihned po obnoven\u00ed peristaltiky za\u010d\u00edn\u00e1me s \u010dasn\u00fdm enter\u00e1ln\u00edm p\u0159\u00edvodem mal\u00fdmi d\u00e1vkami do nasogastrick\u00e9 sondy. Poopera\u010dn\u00ed pr\u016fb\u011bh je samoz\u0159ejm\u011b ovlivn\u011bn p\u0159idru\u017een\u00fdmi vrozen\u00fdmi v\u00fdvojov\u00fdmi vadami, kter\u00e9 \u0159e\u0161\u00edme s p\u0159\u00edslu\u0161n\u00fdmi specialisty (kardiolog, nefrolog apod.).<\/p>\n

7.2 Patogeneze vrozen\u00fdch vad<\/h3>\n

V\u00fdvoj tr\u00e1vic\u00edho a d\u00fdchac\u00edho \u00fastroj\u00ed, tedy i j\u00edcnu, m\u00e1 sv\u016fj p\u016fvod ve farynge\u00e1ln\u00ed membr\u00e1n\u011b, slo\u017een\u00e9 z ektodermu \u00fastn\u00ed jamky a p\u0159edn\u00ed v\u00fdchlipky st\u0159evn\u00ed. Ve t\u0159et\u00edm t\u00fddnu embryon\u00e1ln\u00edho v\u00fdvoje se membr\u00e1na protrhne a dojde ke spojen\u00ed \u00fastn\u00ed dutiny s faryngem. Kaud\u00e1ln\u00edm sm\u011brem se pak farynx n\u00e1levkovit\u011b zu\u017euje a vytv\u00e1\u0159\u00ed z\u00e1klad j\u00edcnu. Pro vznik v\u011bt\u0161iny kongenit\u00e1ln\u00edch odchylek, se kter\u00fdmi se v klinick\u00e9 praxi setk\u00e1v\u00e1me, je rozhoduj\u00edc\u00ed \u010dtvrt\u00fd a\u017e dvan\u00e1ct\u00fd t\u00fdden embryon\u00e1ln\u00edho v\u00fdvoje. Tehdy se spole\u010dn\u00fd z\u00e1klad j\u00edcnu a pr\u016fdu\u0161nice diferencuje na dv\u011b odd\u011blen\u00e9 trubice. Nedojde-li z n\u011bjak\u00e9ho d\u016fvodu k takov\u00e9mu rozd\u011blen\u00ed, vznikaj\u00ed ezofagotrache\u00e1ln\u00ed nebo m\u00e9n\u011b obvykl\u00e9 ezofagobronchi\u00e1ln\u00ed p\u00ed\u0161t\u011ble. Atr\u00e9zie, kter\u00e9 je \u010dasto prov\u00e1zej\u00ed, jsou d\u016fsledkem nedostate\u010dn\u00e9ho rozsahu vakuolizace epitelov\u00e9 v\u00fdstelky prvotn\u00edho j\u00edcnu.<\/p>\n

7.3 V\u00fdskyt vrozen\u00fdch vad j\u00edcnu<\/h3>\n

Velmi podrobn\u00e9 \u00fadaje o v\u00fdskytu vrozen\u00fdch vad j\u00edcnu jsou u n\u00e1s k dispozici d\u00edky pr\u00e1ci A. \u0160\u00edpka a spolupracovn\u00edk\u016f z \u00dastavu pro p\u00e9\u010di o matku a d\u00edt\u011b v Praze-Podol\u00ed, kter\u00fd zpracoval v\u00fdskyt vrozen\u00fdch vad j\u00edcnu v \u010cesk\u00e9 republice za obdob\u00ed 1961\u20132010. S jeho svolen\u00edm zde tato epidemiologick\u00e1 data uv\u00e1d\u00edme [20, 21]. Zdrojem t\u011bchto \u00fadaj\u016f bylo retrospektivn\u00ed zpracov\u00e1n\u00ed materi\u00e1l\u016f evidovan\u00fdch v \u00dastavu pro p\u00e9\u010di o matku a d\u00edt\u011b v Praze-Podol\u00ed a data z \u00dastavu zdravotnick\u00fdch informac\u00ed a statistiky \u010cesk\u00e9 republiky (\u00daZIS). Takto bylo mo\u017eno vyhodnotit v\u00edce jak 5,5 milion\u016f porod\u016f z obdob\u00ed let 1961\u20132000. V tomto obdob\u00ed bylo diagnostikov\u00e1no 804 vrozen\u00fdch vad j\u00edcnu a v uveden\u00e9m obdob\u00ed byl zji\u0161t\u011bn statisticky nev\u00fdznamn\u00fd nev\u00fdrazn\u00fd n\u00e1r\u016fst \u010detnosti. Pr\u016fm\u011brn\u00e1 incidence t\u00e9to vady byla v tomto obdob\u00ed 1,51 na 10 000 \u017eiv\u011b narozen\u00fdch. Podle pohlav\u00ed byli zastoupeni v\u00edce chlapci (58 %). Podle srovn\u00e1n\u00ed proveden\u00e9ho \u0160\u00edpkem se \u010cesk\u00e1 republika \u0159ad\u00ed ke st\u00e1t\u016fm s ni\u017e\u0161\u00edm v\u00fdskytem t\u00e9to vady (tab. 3). V\u00fdskyt vrozen\u00fdch vad j\u00edcnu podle dat \u00daZIS sestaven\u00fd H. Pode\u0161vovou je uveden v grafech 1, 2 a 3.<\/p>\n

Incidence je m\u00edrn\u011b vy\u0161\u0161\u00ed u chlapc\u016f a u d\u011bt\u00ed star\u0161\u00edch a diabetick\u00fdch matek [22]. V p\u0159\u00ed\u010dinn\u00e9 souvislosti s vadou b\u00fdvaj\u00ed uv\u00e1d\u011bny kontraceptiva, progesteron, estrogen \u010di thalidomid a jednozna\u010dn\u011b diabetes mellitus. A\u010dkoli jde o anom\u00e1lii sporadickou, byl zji\u0161t\u011bn i famili\u00e1rn\u00ed v\u00fdskyt t\u00e9to vrozen\u00e9 vady. D\u00edt\u011b narozen\u00e9 takto posti\u017een\u00e9mu rodi\u010di m\u00e1 3\u20134 % riziko, d\u00edt\u011b maj\u00edc\u00ed takto posti\u017een\u00e9ho sourozence m\u00e1 0,5\u20132 % riziko a riziko roste a\u017e na 20 %, jestli\u017ee m\u00e1 takto posti\u017een\u00e9 dva sourozence [23].<\/p>\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
Tab. 3:
\n<\/strong>Vrozen\u00e9 vady j\u00edcnu na 10 000 \u017eiv\u011b narozen\u00fdch v r\u016fzn\u00fdch st\u00e1tek sv\u011bta z obdob\u00ed 1974 a\u017e 1998. P\u0159ehled zpracov\u00e1n A. \u0160\u00edpkem a zve\u0159ejn\u011bn s jeho svolen\u00edm, zkratky uveden\u00e9 u jednotliv\u00fdch st\u00e1t\u016f jsou ozna\u010den\u00edm n\u00e1rodn\u00edch registr\u016f vrozen\u00fdch v\u00fdvojov\u00fdch vad<\/span><\/td>\n<\/tr>\n
St\u00e1t\/oblast<\/td>\n1974\u20131988<\/td>\n1989\u20131994<\/td>\n1998<\/td>\n<\/tr>\n
Austr\u00e1lie<\/td>\n3,1<\/td>\n2,9<\/td>\n2,4<\/td>\n<\/tr>\n
\u010cesk\u00e1 republika<\/td>\n1,4<\/td>\n1,9<\/td>\n2,8<\/td>\n<\/tr>\n
D\u00e1nsko<\/td>\n2,2<\/td>\n0,6<\/td>\n\u2013<\/td>\n<\/tr>\n
Anglie a Wales<\/td>\n1,6<\/td>\n0,9<\/td>\n0,8<\/td>\n<\/tr>\n
Finsko<\/td>\n1,5<\/td>\n2,1<\/td>\n0,9<\/td>\n<\/tr>\n
Francie \u2013 CE Registr<\/td>\n2,6<\/td>\n2,7<\/td>\n3,5<\/td>\n<\/tr>\n
Francie \u2013 Pa\u0159\u00ed\u017e<\/td>\n2,5<\/td>\n3,8<\/td>\n2,2<\/td>\n<\/tr>\n
Francie \u2013 \u0160trasburk<\/td>\n2,3<\/td>\n3,1<\/td>\n1,5<\/td>\n<\/tr>\n
Holandsko<\/td>\n\u2013<\/td>\n1,3<\/td>\n0,5<\/td>\n<\/tr>\n
Izrael<\/td>\n2,0<\/td>\n3,3<\/td>\n1,9<\/td>\n<\/tr>\n
It\u00e1lie \u2013 IPIMC<\/td>\n2,9<\/td>\n2,9<\/td>\n1,8<\/td>\n<\/tr>\n
It\u00e1lie \u2013 IMER<\/td>\n3,6<\/td>\n4,0<\/td>\n1,2<\/td>\n<\/tr>\n
Japonsko \u2013 JAMW<\/td>\n1,4<\/td>\n2,1<\/td>\n3,3<\/td>\n<\/tr>\n
Jihoafrick\u00e1 republika<\/td>\n\u2013<\/td>\n0,9<\/td>\n\u2013<\/td>\n<\/tr>\n
Ji\u017en\u00ed Amerika\u2013 ECLAMC<\/td>\n2,4<\/td>\n2,9<\/td>\n4,1<\/td>\n<\/tr>\n
Ma\u010farsko<\/td>\n1,8<\/td>\n1,5<\/td>\n0,4<\/td>\n<\/tr>\n
Mexiko<\/td>\n1,4<\/td>\n2,5<\/td>\n3,2<\/td>\n<\/tr>\n
Nov\u00fd Z\u00e9land<\/td>\n1,9<\/td>\n2,3<\/td>\n2,3<\/td>\n<\/tr>\n
Norsko<\/td>\n2,0<\/td>\n2,4<\/td>\n1,9<\/td>\n<\/tr>\n
\u0160pan\u011blsko<\/td>\n1,8<\/td>\n2,3<\/td>\n0,6<\/td>\n<\/tr>\n
\u0160v\u00e9dsko<\/td>\n2,7<\/td>\n1,7<\/td>\n0,7<\/td>\n<\/tr>\n
USA \u2013 Atlanta<\/td>\n2,3<\/td>\n1,9<\/td>\n0,7<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

.<\/span><\/p>\n

\"Graf<\/a>

Graf 1
V\u00fdskyt VVV j\u00edcnu na 10 000 \u017eiv\u011b narozen\u00fdch v \u010cesk\u00e9 republice v letech 1994\u20132009<\/p><\/div>\n

\"Graf<\/a>

Graf 2
Pr \u016fm\u011brn\u00fd v\u00fdskyt VVV j\u00edcnu na 10 000 \u017eiv\u011b narozen\u00fdch v \u010cR v p\u011btilet\u00fdch inter valech v letech 1980\u20132009<\/p><\/div>\n

\"Graf<\/a>

Graf 3
Srovn\u00e1 n\u00ed v\u00fdskytu VVV j\u00edcnu na 10 000 \u017eiv\u011b narozen\u00fdch v \u010c esk\u00e9 republice (\u010cR), Moravskoslezsk\u00e9m regionu (MSR) a Olomouck\u00e9m regionu (OLR) v letech 2000\u20132009<\/p><\/div>\n

7.4 Klasifikace vrozen\u00fdch vad j\u00edcnu<\/h3>\n

Existuje n\u011bkolik klasifika\u010dn\u00edch sch\u00e9mat popisuj\u00edc\u00edch anatomick\u00e9 varianty vrozen\u00fdch vad j\u00edcnu, mezi n\u011b\u017e pat\u0159\u00ed klasifikace Laddova a Grossova [2, 3]. U n\u00e1s se nej\u010dast\u011bji u\u017e\u00edv\u00e1 klasifikace podle Vogta z roku 1929 (obr. 2).<\/p>\n\n\n\n
\n

Obr. 2
\nVogtova klasifikace vrozen\u00fdch vad j\u00edcnu<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

.<\/span><\/p>\n

    \n
  • Typ I \u2013 kr\u00e1tk\u00fd horn\u00ed i doln\u00ed segment, mezi nimi dlouh\u00fd atretick\u00fd \u00fasek<\/li>\n
  • Typ II \u2013 horn\u00ed i doln\u00ed slep\u00fd vak bez ezofagotrach\u00e1ln\u00ed p\u00ed\u0161t\u011ble<\/li>\n
  • Typ IIIa \u2013 horn\u00ed a doln\u00ed slep\u00fd vak s horn\u00ed ezofagotrach\u00e1ln\u00ed p\u00ed\u0161t\u011bl\u00ed<\/li>\n
  • Typ IIIb \u2013 horn\u00ed a doln\u00ed slep\u00fd vak s doln\u00ed ezofagotrach\u00e1ln\u00ed p\u00ed\u0161t\u011bl\u00ed
    \n(nej\u010dast\u011bj\u0161\u00ed forma \u2013 a\u017e 90 %)<\/li>\n
  • Typ IIIc \u2013 horn\u00ed i doln\u00ed ezofagotrach\u00e1ln\u00ed p\u00ed\u0161t\u011bl<\/li>\n
  • Typ IV \u2013 pr\u016fchoz\u00ed ezofagus, ezofagotrach\u00e1ln\u00ed p\u00ed\u0161t\u011bl \u2013 H-p\u00ed\u0161t\u011bl<\/li>\n
  • Typ V \u2013 sten\u00f3za ezofagu<\/li>\n<\/ul>\n

    .<\/span><\/p>\n

    7.4.1 Atr\u00e9zie a p\u00ed\u0161t\u011ble<\/h5>\n

    Frekvence v\u00fdskytu atr\u00e9zie j\u00edcnu se uv\u00e1d\u00ed 1 na 2500 a\u017e 3000 porod\u016f. Atr\u00e9zie a\u017e ageneze j\u00edcnu se m\u016f\u017ee vyskytovat bez p\u00ed\u0161t\u011ble (typ Vogt I a II) nebo s tracheoezofage\u00e1ln\u00ed p\u00ed\u0161t\u011bl\u00ed (Vogt IIIa, IIIb a IIIc) \u010di jako samostatn\u00e1 p\u00ed\u0161t\u011bl bez atr\u00e9zie (Vogt IV neboli H-p\u00ed\u0161t\u011bl).<\/p>\n

    Atr\u00e9zie j\u00edcnu<\/h6>\n

    Podez\u0159en\u00ed na atr\u00e9zii j\u00edcnu m\u016f\u017ee zjistit ve II. trimestru prov\u00e1d\u011bn\u00e9 ultrazvukov\u00e9 vy\u0161et\u0159en\u00ed plodu, kdy\u017e nen\u00ed viditeln\u00e1 \u017ealude\u010dn\u00ed bublina [24]. V posledn\u00edm trimestru gravidity na tuto vadu m\u016f\u017ee upozornit polyhydramnion, jen\u017e je vyvol\u00e1n poruchou polyk\u00e1n\u00ed plodov\u00e9 vody. U atr\u00e9zi\u00ed bez p\u00ed\u0161t\u011ble se polyhydramnion vyskytuje asi v 60%, u atr\u00e9zi\u00ed s doln\u00ed p\u00ed\u0161t\u011bl\u00ed u 30% p\u0159\u00edpad\u016f. Novorozenci se \u010dasto rod\u00ed p\u0159ed\u010dasn\u011b, u 40% novorozenc\u016f je porodn\u00ed hmotnost < 2500g [25]. Atr\u00e9zie j\u00edcnu b\u00fdv\u00e1 velmi \u010dasto sdru\u017eena s dal\u0161\u00edmi malformacemi (tab. 4). V 10% b\u00fdvaj\u00ed malformace mnoho\u010detn\u00e9. Proto\u017ee opera\u010dn\u00ed a poopera\u010dn\u00ed postupy tracheoezofage\u00e1ln\u00edch posti\u017een\u00ed se v posledn\u00edch desetilet\u00edch dramaticky zdokonalily, p\u0159idru\u017een\u00e9 anom\u00e1lie se st\u00e1vaj\u00ed st\u00e1le d\u016fle\u017eit\u011bj\u0161\u00edmi faktory v progn\u00f3ze t\u011bchto pacient\u016f. Za mortalitu operovan\u00fdch d\u011bt\u00ed ji\u017e nejsou zodpov\u011bdn\u00e9 respira\u010dn\u00ed probl\u00e9my \u010di sepse, ale p\u0159\u00edtomnost z\u00e1va\u017en\u00fdch vrozen\u00fdch vad, zvl\u00e1\u0161t\u011b srde\u010dn\u00edch, kter\u00e9 se mohou st\u00e1t hlavn\u00ed p\u0159\u00ed\u010dinou smrti [26]. Incidence p\u0159idru\u017een\u00fdch anom\u00e1li\u00ed je n\u011bkolikr\u00e1t vy\u0161\u0161\u00ed u d\u011bt\u00ed s porodn\u00ed hmotnost\u00ed\u00a0< 2000g, o n\u011b\u017e zejm\u00e9na je p\u00e9\u010de problematick\u00e1. Pe\u010dliv\u00e9 zhodnocen\u00ed p\u0159idru\u017een\u00fdch anom\u00e1li\u00ed je rozhoduj\u00edc\u00ed pro kone\u010dnou progn\u00f3zu t\u011bchto d\u011bt\u00ed [25, 30\u201334].<\/p>\n\n\n\n\n\n\n\n\n
    Tab. 4<\/span>
    \n P\u0159idru\u017een\u00e9 vrozen\u00e9 vady u pacient\u016f s atr\u00e9zi\u00ed j\u00edcnu [26\u201329]<\/span><\/td>\n<\/tr>\n
    Posti\u017een\u00fd syst\u00e9m <\/strong><\/td>\nPotenci\u00e1ln\u00ed anom\u00e1lie <\/strong><\/td>\n<\/tr>\n
    Kardiovaskul\u00e1rn\u00ed [26]
    \n<\/strong>(asi ve 20\u201330 %)<\/span><\/td>\n    		Defekt komorov\u00e9ho septa a s\u00ed\u0148ov\u00e9ho septa, perzistuj\u00edc\u00ed Botallova du\u010dej, Fallotova tetralogie, anom\u00e1lie aort\u00e1ln\u00edho oblouku, ageneze pupe\u010dn\u00ed arterie<\/td>\n<\/tr>\n
    Gastrointestin\u00e1ln\u00ed [27]
    \n<\/strong>(asi ve 25 %)<\/span><\/td>\n    		Nej\u010dast\u011bji (asi ve 42 %) anorekt\u00e1ln\u00ed atr\u00e9zie, duoden\u00e1ln\u00ed atr\u00e9zie, atr\u00e9zie ilea, malrotace, Meckel\u016fv divertikl, anul\u00e1rn\u00ed pankreas a pylorosten\u00f3za<\/td>\n<\/tr>\n
    Urogenit\u00e1ln\u00ed [28]
    \n<\/strong>(asi ve 24 %)<\/span><\/td>\n    		Jednostrann\u00e1 \u010di oboustrann\u00e1 ageneze nebo dysplazie ledvin, podkovovit\u00e9 ledviny, polycystick\u00e9 ledviny, vezikoureter\u00e1ln\u00ed reflux, hypospadi<\/td>\n<\/tr>\n
    Nervov\u00fd a muskuloskelet\u00e1rn\u00ed [29]
    \n<\/strong>(asi v 10 %)<\/span><\/td>\n    		Defekty neur\u00e1ln\u00ed trubice, hydrocefalus, skoli\u00f3zy, hemivertebrae, dysplazie radia, amelie, polydaktylie, syndaktylie, anom\u00e1lie \u017eeber, vady doln\u00edch kon\u010detin<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

    .<\/span><\/p>\n

    Klinick\u00e9 p\u0159\u00edznaky<\/strong>
    \nU novorozence se atr\u00e9zie projevuje excesivn\u00edm slin\u011bn\u00edm s tvorbou jez\u00edrka v zadn\u00edm\u00a0faryngu. To \u010dasto vede k aspiraci, paroxysm\u00e1ln\u00edmu ka\u0161li, du\u0161en\u00ed, cyan\u00f3ze a respira\u010dn\u00edmu distresu. V p\u0159\u00edpad\u011b aspirace doch\u00e1z\u00ed k apnoi, bradykardii, a dokonce m\u016f\u017ee\u00a0n\u00e1sledovat i \u00famrt\u00ed. Velmi z\u00e1hy se rozv\u00edj\u00ed aspira\u010dn\u00ed pneumonie, kter\u00e1 zt\u011b\u017euje opera\u010dn\u00ed v\u00fdkon a m\u016f\u017ee ohrozit jeho v\u00fdsledek. Jestli\u017ee nen\u00ed p\u0159\u00edtomna ezofagotrache\u00e1ln\u00ed p\u00ed\u0161t\u011bl, m\u00e1 novorozenec \u010dlunkovit\u011b vpadl\u00e9 b\u0159icho. P\u0159\u00edtomnost doln\u00ed p\u00ed\u0161t\u011ble distenduje\u00a0st\u0159evn\u00ed kli\u010dky a m\u016f\u017ee v\u00e9st k refluxu \u017ealude\u010dn\u00edho obsahu do tracheobronchi\u00e1ln\u00edho\u00a0stromu a vyvolat pneumonitidu s mo\u017enost\u00ed sepse.<\/p>\n

    Diagnostika<\/h6>\n

    K zji\u0161t\u011bn\u00ed atr\u00e9zie vede nemo\u017enost zav\u00e9st c\u00e9vku do \u017ealudku p\u0159i ods\u00e1v\u00e1n\u00ed novorozence.
    \nNa prost\u00e9m sn\u00edmku hrudn\u00edku a b\u0159icha nal\u00e9z\u00e1me bu\u010f norm\u00e1ln\u00ed st\u0159evn\u00ed plynatost,\u00a0je-li p\u0159\u00edtomna doln\u00ed p\u00ed\u0161t\u011bl, nebo plyn v tr\u00e1vic\u00ed trubici chyb\u00ed, pokud p\u00ed\u0161t\u011bl nen\u00ed p\u0159\u00edtomna. Pro velk\u00e9 riziko aspirace po aplikaci kontrastn\u00ed l\u00e1tky do horn\u00edho slep\u00e9ho pah\u00fdlu se doporu\u010duje jeho lokalizaci up\u0159esnit zaveden\u00edm kontrastn\u00ed c\u00e9vky nebo aplikac\u00ed vzduchu.<\/p>\n

    H-p\u00ed\u0161t\u011ble bez atr\u00e9zie<\/h6>\n

    Jsou lokalizov\u00e1ny v oblasti kr\u010dn\u00ed nebo horn\u00ed hrudn\u00ed krajiny a tvo\u0159\u00ed asi 3% a\u017e 5% p\u0159\u00edpad\u016f vrozen\u00e9ho posti\u017een\u00ed j\u00edcnu.<\/p>\n

    Klinick\u00e9 p\u0159\u00edznaky
    \n<\/strong>Projevuj\u00ed se paroxysm\u00e1ln\u00edm ka\u0161lem, zvl\u00e1\u0161t\u011b p\u0159i j\u00eddle, refluxem \u017ealude\u010dn\u00edch \u0161\u0165\u00e1v\u00a0do trachey, co\u017e zp\u016fsobuje tracheobronchi\u00e1ln\u00ed pneumonitidu, \u010dasto oboustrannou a recidivuj\u00edc\u00ed. \u0160t\u011bkav\u00fd ka\u0161el je pro d\u011bti s tracheoezofage\u00e1ln\u00ed p\u00ed\u0161t\u011bl\u00ed typick\u00fd, je\u00a0sekund\u00e1rn\u00ed v souvislosti s tracheomalaci\u00ed. Struktura trachey m\u016f\u017ee b\u00fdt abnorm\u00e1ln\u00ed,\u00a0s neuzav\u0159en\u00fdmi chrupav\u010dit\u00fdmi prstenci a v\u00fdrazn\u011b nadb\u00fdvaj\u00edc\u00ed muk\u00f3zou vytv\u00e1\u0159ej\u00edc\u00ed\u00a0vrstven\u00ed na p\u0159edn\u00ed i zadn\u00ed st\u011bn\u011b a zp\u016fsobuj\u00edc\u00ed \u0161t\u011bkav\u00fd ka\u0161el [35]. Dal\u0161\u00edm v\u00fdznamn\u00fdm p\u0159\u00edznakem je nadm\u011brn\u00e9 rozepnut\u00ed \u017ealudku a st\u0159ev plynem. Z\u00e1vis\u00ed na velikosti\u00a0p\u00ed\u0161t\u011ble, zda se projev\u00ed ji\u017e v novorozeneck\u00e9m obdob\u00ed, nebo a\u017e pozd\u011bji s r\u016fstem d\u00edt\u011bte.<\/p>\n

    Diagnostika
    \n<\/strong>Orienta\u010dn\u00edm vy\u0161et\u0159en\u00edm, kter\u00e9 nez\u0159\u00eddka potvrd\u00ed diagn\u00f3zu, je Koop\u016fv test, p\u0159i kter\u00e9m se do \u017ealudku zavede sonda a jej\u00ed\u017e druh\u00fd konec se pono\u0159\u00ed pod vodn\u00ed hladinu.\u00a0Sonda se pak pozvolna povytahuje j\u00edcnem. V m\u00edst\u011b komunikace pr\u016fdu\u0161nice s j\u00edcnem\u00a0pozorujeme \u00fanik vzduchu pod vodn\u00ed hladinu. K up\u0159esn\u011bn\u00ed diagn\u00f3zy se pou\u017e\u00edv\u00e1\u00a0endoskopick\u00e9 vy\u0161et\u0159en\u00ed trachey, p\u0159\u00edpadn\u011b j\u00edcnu. Rentgenov\u00e9 vy\u0161et\u0159en\u00ed vodn\u00edm kontrastem je spojen\u00e9 s rizikem aspirace.<\/p>\n

    L\u00e9\u010dba
    \n<\/strong>Chirurgick\u00e9 l\u00e9\u010den\u00ed vrozen\u00fdch vad j\u00edcnu je dnes dom\u00e9nou specializovan\u00fdch d\u011btsk\u00fdch\u00a0chirurg\u016f v centrech novorozeneck\u00e9 chirurgie. Pokud nen\u00ed vzd\u00e1lenost obou slep\u00fdch\u00a0konc\u016f j\u00edcnu p\u0159\u00edli\u0161 velk\u00e1, prov\u00e1d\u00ed se prim\u00e1rn\u00ed end-to-end anastom\u00f3za. P\u0159i v\u011bt\u0161\u00ed vzd\u00e1lenosti existuje cel\u00e1 \u0159ada metod k elongaci j\u00edcnu, p\u0159\u00edpadn\u011b je t\u0159eba chyb\u011bj\u00edc\u00ed \u00fasek\u00a0j\u00edcnu nahradit. U atr\u00e9zi\u00ed spojen\u00fdch s p\u00ed\u0161t\u011bl\u00ed je t\u0159eba fistulu resekovat a d\u00fdchac\u00ed cesty\u00a0uzav\u0159\u00edt. U komplikovan\u00fdch stav\u016f je nutno operovat dvouf\u00e1zov\u011b [8, 10, 12]. S rozvojem novorozeneck\u00e9 chirurgie a intenzivn\u00ed p\u00e9\u010de o novorozence se procento \u00fasp\u011b\u0161n\u011b\u00a0operovan\u00fdch novorozenc\u016f, a\u0165 u\u017e prim\u00e1rn\u011b \u010di dvouf\u00e1zov\u011b, st\u00e1le zvy\u0161uje [13\u201315].<\/p>\n

    Komplikace
    \n<\/strong>Z bezprost\u0159edn\u00edch komplikac\u00ed je nejz\u00e1va\u017en\u011bj\u0161\u00ed insuficience anastom\u00f3zy. Jej\u00ed v\u00fdskyt se\u00a0uv\u00e1d\u00ed a\u017e v 15%, z toho ve dvou t\u0159etin\u00e1ch je men\u0161\u00edho rozsahu. Dehiscence anastom\u00f3zy jsou v\u00fdznamnou p\u0159\u00ed\u010dinou poopera\u010dn\u00ed morbidity a letality [16]. Asi v 10% doch\u00e1z\u00ed k recidiv\u011b tracheoezofage\u00e1ln\u00ed p\u00ed\u0161t\u011ble. K pozdn\u00edm komplikac\u00edm pat\u0159\u00ed striktura v m\u00edst\u011b anastom\u00f3zy (a\u017e ve 30%), kter\u00e1 dob\u0159e reaguje na dilataci [9, 11], a gastroezofage\u00e1ln\u00ed reflux (u 20-60% operovan\u00fdch). V\u011bt\u0161ina d\u011bt\u00ed m\u00e1 r\u016fzn\u00fd stupe\u0148 tracheomalacie, kter\u00e1 je p\u0159\u00ed\u010dinou respira\u010dn\u00edch komplikac\u00ed. Jde o anatomickou a funk\u010dn\u00ed chabost trachey, kter\u00e1 zp\u016fsobuje obstrukci. B\u00fdv\u00e1 v oblasti p\u016fvodn\u00ed tracheoezofage\u00e1ln\u00ed p\u00ed\u0161t\u011ble v horn\u00ed \u010d\u00e1sti hrudn\u00edho j\u00edcnu. Jej\u00edm n\u00e1sledkem je \u0161t\u011bkav\u00fd ka\u0161el, kter\u00fd se s v\u011bkem zm\u00edr\u0148uje.<\/p>\n

    V\u00fdsledky
    \n<\/strong>Z\u00e1vis\u00ed zejm\u00e9na na p\u0159\u00edtomnosti \u010di nep\u0159\u00edtomnosti p\u0159idru\u017een\u00fdch anom\u00e1li\u00ed. Je uv\u00e1d\u011bno\u00a0p\u0159e\u017eit\u00ed 95% u dono\u0161en\u00fdch novorozenc\u016f s izolovanou atr\u00e9zi\u00ed j\u00edcnu nebo tracheoezofege\u00e1ln\u00ed p\u00ed\u0161t\u011bl\u00ed, ale kles\u00e1 ke 20% u novorozenc\u016f s velmi n\u00edzkou porodn\u00ed hmotnost\u00ed\u00a0a z\u00e1va\u017enou srde\u010dn\u00ed vadou [19].<\/p>\n

    Laryngo-tracheoezofage\u00e1ln\u00ed roz\u0161t\u011bp<\/h6>\n

    Je vz\u00e1cn\u00e1 kongenit\u00e1ln\u00ed anom\u00e1lie charakterizovan\u00e1 rozs\u00e1hlou komunikac\u00ed mezi zadn\u00ed st\u011bnou laryngu a embran\u00f3zn\u00ed tracheou a p\u0159edn\u00ed st\u011bnou j\u00edcnu.<\/p>\n

    Nejz\u00e1va\u017en\u011bj\u0161\u00edm typem p\u00ed\u0161t\u011ble je laryngo-tracheoezofage\u00e1ln\u00ed roz\u0161t\u011bp (tracheoezofagus, ezofagotrachea). Tento \u00a0efekt b\u00fdv\u00e1 prov\u00e1zen t\u011b\u017ek\u00fdmi anom\u00e1liemi kardiovaskul\u00e1rn\u00edho, gastrointestin\u00e1ln\u00edho a urogenit\u00e1ln\u00edho syst\u00e9mu [36]. Roz\u0161t\u011bp je rozd\u011blen do 4 podtyp\u016f podle d\u00e9lky defektu a stupn\u011b z\u00e1va\u017enosti operace [37\u201340].<\/p>\n

      \n
    • Typ I \u2013 roz\u0161t\u011bp je limitov\u00e1n na hrtan a m\u016f\u017ee pokra\u010dovat na prsten\u010ditou chrupavku (asi 1\/3 p\u0159\u00edpad\u016f).<\/span><\/li>\n
    • Typ II \u2013 roz\u0161t\u011bp se \u0161\u00ed\u0159\u00ed p\u0159es prsten\u010dit\u00e9 chrupavky kr\u010dn\u00ed trachey.<\/span><\/li>\n
    • Typ III \u2013 roz\u0161t\u011bp sah\u00e1 ke karin\u011b.<\/span><\/li>\n
    • Typ IV \u2013 roz\u0161t\u011bp zahrnuje jeden nebo oba hlavn\u00ed bronchy.<\/span><\/li>\n<\/ul>\n

      Klinick\u00e9 p\u0159\u00edznaky
      \n<\/strong>Na rozsahu roz\u0161t\u011bpu z\u00e1vis\u00ed t\u00ed\u017ee klinick\u00fdch symptom\u016f, jako je du\u0161en\u00ed a cyan\u00f3za p\u0159i\u00a0krmen\u00ed. Tyto d\u011bti maj\u00ed tak\u00e9 charakteristick\u00fd afonick\u00fd pl\u00e1\u010d. Aspira\u010dn\u00ed pneumonie\u00a0zp\u016fsoben\u00e1 poruchou funkce posti\u017een\u00e9ho hrtanu se objevuje brzy a obvykle kon\u010d\u00ed\u00a0smrt\u00ed novorozence. Dechov\u00e9 pot\u00ed\u017ee se neda\u0159\u00ed zvl\u00e1dnout ani intubac\u00ed a um\u011blou plicn\u00ed\u00a0ventilac\u00ed.<\/p>\n

      Diagnostika
      \n<\/strong>Je nutn\u00e9 prov\u00e9st endoskopick\u00e9 vy\u0161et\u0159en\u00ed laryngu a trachey.<\/p>\n

      L\u00e9\u010dba
      \n<\/strong>Chirurgick\u00e1 l\u00e9\u010dba t\u00e9to anom\u00e1lie je obt\u00ed\u017en\u00e1. Je spojena s vysokou \u00famrtnost\u00ed a velik\u00fdm po\u010dtem n\u00e1sledn\u00fdch pot\u00ed\u017e\u00ed. Tato anom\u00e1lie, zejm\u00e9na typ IV roz\u0161t\u011bpu, je \u010dasto\u00a0komplikov\u00e1na t\u011b\u017ekou tracheomalaci\u00ed a hypertrofickou sliznic\u00ed. D\u011bti vy\u017eaduj\u00ed dlouhodobou um\u011blou plicn\u00ed ventilaci k prevenci intermitentn\u00edho kolapsu trachey. Prevenc\u00ed kolapsu poddajn\u00fdch d\u00fdchac\u00edch cest je proveden\u00ed tracheostomie a propu\u0161t\u011bn\u00ed d\u011bt\u00ed se zavedenou tracheostomickou kanylou [40].<\/p>\n

      7.4.2 Cysty, duplikace (duplikatury) a vrozen\u00e9 divertikly<\/h5>\n
      Cysty<\/h6>\n

      Cysta byla poprv\u00e9 pops\u00e1na Blasiem v roce 1674 [41]. V\u00edce ne\u017e o 100 let pozd\u011bji\u00a0Roth v roce 1881 popsal cystu mediastina adheruj\u00edc\u00ed k p\u00e1te\u0159i, nyn\u00ed zn\u00e1mou jako\u00a0neuroenterogenn\u00ed cysta. Enter\u00e1ln\u00ed cysty nad br\u00e1nic\u00ed tvo\u0159\u00ed 18 % v\u0161ech st\u0159evn\u00edch cyst\u00a0[42]. Vznikaj\u00ed p\u0159i v\u00fdvoji z divertiklu p\u0159edn\u00edho st\u0159eva. B\u00fdvaj\u00ed um\u00edst\u011bny na lev\u00e9 stran\u011b j\u00edcnu, v\u011bt\u0161inou vzadu, a jsou vystl\u00e1ny zpravidla cylindrick\u00fdm epitelem. Pouze 12 %\u00a0z velk\u00e9 \u0159ady mediastin\u00e1ln\u00edch cyst bylo ur\u010deno jako cysty j\u00edcnu [43]. U d\u011bt\u00ed nejm\u00e9n\u011b\u00a030 % rezistenc\u00ed v mediastinu m\u00e1 p\u016fvod v p\u0159edn\u00edm st\u0159evu a nakonec se vyv\u00edj\u00ed jako j\u00edcnov\u00e1, neurogenn\u00ed, bronchogenn\u00ed nebo izolovan\u00e1 cysta [44]. A\u010dkoliv se j\u00edcnov\u00e9 cysty pova\u017euj\u00ed za benign\u00ed, byl zaznamen\u00e1n i malign\u00ed zvrat [45, 46].<\/p>\n

      J\u00edcnov\u00e9 cysty byly pops\u00e1ny ve v\u0161ech lokalit\u00e1ch pod\u00e9l j\u00edcnu [47\u201349]. Jsou m\u00e9n\u011b obvykl\u00e9 v kr\u010dn\u00ed oblasti [50]. J\u00edcnov\u00e9 cysty mohou b\u00fdt intramur\u00e1ln\u00ed nebo kompletn\u011b separovan\u00e9 od j\u00edcnu. A\u010dkoliv z\u0159\u00eddka komunikuj\u00ed s lumen j\u00edcnu, propojen\u00ed mezi cystou\u00a0a j\u00edcnem bylo pops\u00e1no [51]. Hrudn\u00ed cysta maj\u00edc\u00ed p\u016fvod v p\u0159edn\u00edm st\u0159evu m\u016f\u017ee komunikovat s jin\u00fdmi b\u0159i\u0161n\u00edmi org\u00e1ny, jako je st\u0159evo, \u017elu\u010dov\u00e9 cesty, nebo dokonce\u00a0v\u00fdvod pankreatu [52, 53]. V\u011bt\u0161ina cyst je solit\u00e1rn\u00edch, ale byly pops\u00e1ny i mnoho\u010detn\u00e9\u00a0cysty [54, 55]. Zejm\u00e9na zadn\u00ed j\u00edcnov\u00e9 cysty b\u00fdvaj\u00ed mnoho\u010detn\u00e9 [56]. Tlust\u00e9 cysty,\u00a0v\u011bt\u0161inou vypln\u011bn\u00e9 visk\u00f3zn\u00ed tekutinou, ale i nekrotick\u00fdmi a z\u00e1n\u011btliv\u00fdmi bu\u0148kami a starou krv\u00ed, mohou b\u00fdt komplikovan\u00e9 ulcerac\u00ed, infekc\u00ed nebo krv\u00e1cen\u00edm.<\/p>\n

      Duplikace<\/strong> (duplikatury)
      \nDuplikace j\u00edcnu prob\u00edhaj\u00ed paraleln\u011b s j\u00edcnem a chovaj\u00ed se jako cysty. Jde v podsta<\/span>t\u011b o intramur\u00e1ln\u00ed j\u00edcnov\u00e9 cysty vystlan\u00e9 dla\u017edicov\u00fdm nebo cylindrick\u00fdm epitelem\u00a0a jsou pova\u017eov\u00e1ny za aberaci ve vakuolizaci primitivn\u00edho j\u00edcnu.<\/p>\n

      Divertikl<\/strong>
      \nJe ulo\u017een obvykle vpravo a vyklenuje se dozadu a do stran jako kr\u00e1tk\u00fd, trubicovit\u00fd\u00a0\u00fatvar. Ob\u011b formy z\u00edskan\u00fdch divertikl\u016f, pulzn\u00ed i trak\u010dn\u00ed, vznikaj\u00ed herniac\u00ed submuk\u00f3zy\u00a0a muk\u00f3zy defektem svalov\u00e9 vrstvy. Prav\u00fd kongenit\u00e1ln\u00ed divertikl naopak obsahuje\u00a0v\u0161echny vrstvy st\u011bny j\u00edcnu (muk\u00f3zu, submuk\u00f3zu a svalovinu) uvnit\u0159 v\u00fdchlipky [57].\u00a0Kongenit\u00e1ln\u00ed divertikly jsou extr\u00e9mn\u011b vz\u00e1cn\u00e9 a \u010dast\u011bji b\u00fdvaj\u00ed posti\u017eeni chlapci. V\u011bt\u0161inou se nach\u00e1zej\u00ed v kr\u010dn\u00ed \u010d\u00e1sti j\u00edcnu okolo faryngoezofage\u00e1ln\u00edho spojen\u00ed stejn\u011b jako\u00a0z\u00edskan\u00e9, ale mohou b\u00fdt i ve st\u0159edn\u00ed \u010d\u00e1sti j\u00edcnu.<\/p>\n

      Klinick\u00e9 p\u0159\u00edznaky
      \n<\/strong>V\u00fdskyt t\u011bchto anom\u00e1li\u00ed je velmi vz\u00e1cn\u00fd. Jedna t\u0159etina pacient\u016f s j\u00edcnov\u00fdmi cystami\u00a0je v d\u011btstv\u00ed asymptomatick\u00e1 a cysty jsou n\u00e1hodn\u011b objeveny p\u0159i rentgenov\u00e9m vy\u0161et\u0159en\u00ed hrudn\u00edku. V\u011bt\u0161ina dal\u0161\u00edch pacient\u016f m\u016f\u017ee m\u00edt minim\u00e1ln\u00ed a\u017e m\u00edrnou dysfagii.\u00a0Velmi velk\u00e9 j\u00edcnov\u00e9 cysty mohly m\u00edt za n\u00e1sledek syndrom horn\u00ed dut\u00e9 \u017e\u00edly [58] nebo\u00a0v p\u0159\u00edpad\u011b velk\u00fdch dist\u00e1ln\u00edch cyst se mohou prezentovat jako b\u0159i\u0161n\u00ed masy [49]. Nejdramati\u010dt\u011bj\u0161\u00ed p\u0159\u00edznaky vypl\u00fdvaj\u00ed ze zt\u00ed\u017een\u00e9 ventilace u mlad\u00fdch pacient\u016f. Symptomy\u00a0mohou vznikat z redukce samotn\u00e9ho dechov\u00e9ho objemu, z mechanick\u00e9 komprese trachey [59] nebo ze zevn\u00edho tlaku na velk\u00e9 bronchy zp\u016fsobuj\u00edc\u00ed emfyz\u00e9m (air\u00a0trapping a atelekt\u00e1zy) nebo konsolidaci (kompletn\u00ed kolaps pl\u00edce) [60, 61]. \u010cast\u011bj\u0161\u00ed,\u00a0ale m\u00e9n\u011b dramatick\u00e9 formy respira\u010dn\u00edch pot\u00ed\u017e\u00ed u pacient\u016f s men\u0161\u00edmi j\u00edcnov\u00fdmi\u00a0cystami zahrnuj\u00ed stridor, perzistuj\u00edc\u00ed ka\u0161el nebo rekurentn\u00ed pneumonie [42]. Z\u00e1va\u017en\u00e9 malformace vedou k \u00famrt\u00ed v ran\u00e9m v\u011bku, m\u00edrn\u011bj\u0161\u00ed formy jsou slu\u010diteln\u00e9 se\u00a0\u017eivotem. Dosp\u011bl\u00ed jedinci jsou bu\u010f asymptomati\u010dt\u00ed, nebo trp\u00ed dysfagi\u00ed, pak je nutn\u00e9\u00a0prov\u00e9st exstirpaci.<\/p>\n

      Novorozenci s j\u00edcnov\u00fdmi divertikly se mohou projevovat excesivn\u00edm slin\u011bn\u00edm a mohou tak simulovat atr\u00e9zii j\u00edcnu. V p\u0159\u00edpad\u011b velk\u00e9ho divertiklu mohou m\u00edt pacienti\u00a0b\u011bhem krmen\u00ed zn\u00e1mky respira\u010dn\u00ed obstrukce a jsou ohro\u017eeni aspirac\u00ed.<\/p>\n

      Sten\u00f3za j\u00edcnu<\/h6>\n

      Nejstar\u0161\u00ed zm\u00ednky o sten\u00f3ze j\u00edcnu u novorozenc\u016f l\u00e9\u010den\u00fdch dilatac\u00ed jsou z let 1911 [62] a 1917 [63]. V\u011bt\u0161\u00ed soubor 50 p\u0159\u00edpad\u016f je z roku 1928 [64]. Rezidua tracheobronchi\u00e1ln\u00ed tk\u00e1n\u011b v j\u00edcnu byla pops\u00e1na v roce 1936 [65] a hypertrofick\u00e1 sten\u00f3za o 23 let pozd\u011bji [66]. P\u0159esto\u017ee je dilatace nejb\u011b\u017en\u011bj\u0161\u00ed terapi\u00ed, byly u\u017e v minulosti prov\u00e1d\u011bny i resekce [67, 68].<\/p>\n

      Kongenit\u00e1ln\u00ed j\u00edcnov\u00e1 sten\u00f3za je definov\u00e1na jako z\u00fa\u017een\u00ed j\u00edcnu projevuj\u00edc\u00ed se po narozen\u00ed a je vrozenou malformac\u00ed struktury jeho st\u011bny [69]. Incidence t\u00e9to vady je ud\u00e1v\u00e1na 1 : 25 000\u201350 000 \u017eiv\u011b narozen\u00fdch [69, 70] s vy\u0161\u0161\u00edm v\u00fdskytem v Japonsku [71]. Ve sv\u011btov\u00e9 literatu\u0159e bylo nalezeno pouze 500 popsan\u00fdch p\u0159\u00edpad\u016f [72].<\/p>\n

      V 17 a\u017e 33% je sten\u00f3za spojena s dal\u0161\u00edmi vrozen\u00fdmi v\u00fdvojov\u00fdmi vadami \u2013 atr\u00e9zi\u00ed st\u0159eva, malrotac\u00ed, anorekt\u00e1ln\u00ed malformac\u00ed, srde\u010dn\u00ed vadou, hypospadi\u00ed, chromozom\u00e1ln\u00ed aberac\u00ed a malformacemi hlavy, obli\u010deje a kon\u010detin.<\/p>\n

      Vyskytuj\u00ed se 3 druhy sten\u00f3zy:<\/p>\n

        \n
      1. Fibromuskul\u00e1rn\u00ed ztlu\u0161t\u011bn\u00ed, tak\u00e9 naz\u00fdvan\u00e9 idiopatick\u00e1 muskul\u00e1rn\u00ed hypertrofie\u00a0nebo fibromuskul\u00e1rn\u00ed sten\u00f3za \u2013 tj. difuzn\u00ed fibr\u00f3za st\u011bny se segment\u00e1rn\u00ed hypertrofi\u00ed svaloviny a submuk\u00f3zy. Hypertrofick\u00e9 segmenty b\u00fdvaj\u00ed v dist\u00e1ln\u00edm j\u00edcnu,\u00a0m\u00e9n\u011b \u010dasto v jeho st\u0159edn\u00ed t\u0159etin\u011b [73]. Tyto l\u00e9ze jsou dlouh\u00e9 a ostr\u00e9 se zna\u010dnou\u00a0variabilitou ve stupni sten\u00f3zy. Tento typ je nej\u010dast\u011bji spojen s atr\u00e9zi\u00ed j\u00edcnu [17].<\/li>\n
      2. Rezidua tracheobronchi\u00e1ln\u00ed tk\u00e1n\u011b tvo\u0159ena chrupavkou, hlenov\u00fdmi \u017el\u00e1zkami nebo\u00a0\u0159asinkov\u00fdm epitelem, p\u016fsob\u00ed rigidn\u00ed z\u00fa\u017een\u00ed nej\u010dast\u011bji v dist\u00e1ln\u00edm j\u00edcnu [65, 74].\u00a0B\u00fdvaj\u00ed spojena s atr\u00e9zi\u00ed j\u00edcnu a tracheoezofage\u00e1ln\u00edmi fistulami [71, 75].<\/li>\n
      3. Kongenit\u00e1ln\u00ed membran\u00f3zn\u00ed v\u00fdchlipky (nebo diafragma), nejvz\u00e1cn\u011bj\u0161\u00ed typ kongenit\u00e1ln\u00ed sten\u00f3zy. Mohou se nach\u00e1zet na v\u0161ech \u00farovn\u00edch j\u00edcnu [62, 72], nej\u010dast\u011bji v jeho horn\u00ed polovin\u011b.<\/li>\n<\/ol>\n

        Klinick\u00fd obraz a diagnostika
        \n<\/strong>V\u0161echny formy se projevuj\u00ed opakovan\u00fdm zvracen\u00edm, \u010dasto a\u017e v kojeneck\u00e9m v\u011bku\u00a0po p\u0159echodu na tu\u017e\u0161\u00ed stravu. Diagnostikuj\u00ed se kontrastn\u00edm rentgenov\u00fdm vy\u0161et\u0159en\u00edm\u00a0a endoskopi\u00ed.<\/p>\n

        Terapie
        \n<\/strong>Z\u00e1le\u017e\u00ed na rozsahu posti\u017een\u00ed j\u00edcnu. Membran\u00f3zn\u00ed v\u00fdchlipky se opakovan\u011b dilatuj\u00ed\u00a0balonkov\u00fdm kat\u00e9trem. P\u0159i ne\u00fasp\u011b\u0161n\u00e9 dilataci je mo\u017en\u00e1 discize nebo resekce \u010d\u00e1sti\u00a0j\u00edcnu s anastom\u00f3zou. Chrupav\u010dit\u00e9 z\u00fa\u017een\u00ed v\u011bt\u0161inou nelze dilatovat. Segment\u00e1rn\u00ed hypertrofie svaloviny men\u0161\u00edho rozsahu se rovn\u011b\u017e l\u00e9\u010d\u00ed dilatac\u00ed. N\u011bkdy m\u016f\u017ee hypertrofie\u00a0postihnout j\u00edcen v cel\u00e9m rozsahu a po ne\u00fasp\u011b\u0161n\u00e9m pokusu o dilataci je nutn\u00e1 jeho\u00a0n\u00e1hrada.<\/p>\n

        7.4.3 Dysphagia lusoria<\/h5>\n

        Je to horn\u00ed typ \u201edysfagie rozmarn\u00e9ho pr\u016fb\u011bhu\u201c (odtud jm\u00e9no), zp\u016fsoben\u00fd vrozenou anom\u00e1li\u00ed oblouku aorty a jej\u00edch v\u011btv\u00ed. V roce 1945 provedl Gross prvn\u00ed \u00fasp\u011b\u0161nou operaci c\u00e9vn\u00edho prstence u ro\u010dn\u00edho chlapce, rozd\u011blen\u00ed zdvojen\u00e9ho oblouku aorty [76].<\/p>\n

        Patologick\u00e9 n\u00e1lezy
        \n<\/strong>V \u0161ir\u0161\u00edm smyslu se t\u00edmto term\u00ednem ozna\u010duje dysfagie u v\u0161ech vrozen\u00fdch vad aort\u00e1ln\u00edho oblouku, v u\u017e\u0161\u00edm pak aberantn\u00ed odstup pravostrann\u00e9 arteria suclavia (a. lusoria), kter\u00e1 odstupuje z aort\u00e1ln\u00edho oblouku jako posledn\u00ed a prob\u00edh\u00e1 v\u011bt\u0161inou za j\u00edcnem. Vznik\u00e1 c\u00e9vn\u00ed prstenec, ve kter\u00e9m jsou utla\u010dov\u00e1ny j\u00edcen a trachea.<\/p>\n

        Klinick\u00e9 p\u0159\u00edznaky
        \n<\/strong>Z\u00e1vis\u00ed na stupni komprese. V\u011bt\u0161inou se klinicky neprojevuje. T\u011bsn\u00e1 komprese je\u00a0charakterizov\u00e1na ji\u017e v \u010dasn\u00e9m kojeneck\u00e9m v\u011bku ka\u0161lem, respira\u010dn\u00edmi infekty nebo\u00a0polykac\u00edmi pot\u00ed\u017eemi n\u011bkdy teprve tuh\u00e9 stravy. St\u00e1le hroz\u00ed nebezpe\u010d\u00ed aspirace. V\u00fdrazn\u00fd stridor se nemus\u00ed projevit v klidu, n\u00fdbr\u017e a\u017e po n\u00e1maze (p\u0159i krmen\u00ed, k\u0159iku apod.).<\/p>\n

        U men\u0161iny posti\u017een\u00fdch vznikaj\u00ed pot\u00ed\u017ee ve st\u0159edn\u00edm v\u011bku, kdy se c\u00e9vn\u00ed st\u011bny skleroticky m\u011bn\u00ed a st\u00e1vaj\u00ed se rigidn\u00edmi.<\/p>\n

        Diagnostika<\/strong>
        \nVelmi cenn\u00fdm vy\u0161et\u0159en\u00edm je ezofagografie, p\u0159i n\u00ed\u017e je patrn\u00fd m\u00edrn\u00fd z\u00e1\u0159ez na zadn\u00ed\u00a0st\u011bn\u011b j\u00edcnu ve v\u00fd\u0161i 3.\u20134. hrudn\u00edho obratle. Trachea v\u011bt\u0161inou neb\u00fdv\u00e1 komprimov\u00e1na\u00a0[77].<\/p>\n

        P\u0159i ezofagoskopii se zjist\u00ed na zadn\u00ed st\u011bn\u011b j\u00edcnu pulzuj\u00edc\u00ed val. Pokud prstenec tvo\u0159\u00ed a. subclavia dx., pak po kompresi pulzuj\u00edc\u00edho valu ezofagoskopem vymiz\u00ed pulz\u00a0na prav\u00e9 horn\u00ed kon\u010detin\u011b. Bronchoskopie je d\u016fle\u017eit\u00e1 ke zji\u0161t\u011bn\u00ed souvisej\u00edc\u00ed trache\u00e1ln\u00ed\u00a0sten\u00f3zy [78].<\/p>\n

        L\u00e9\u010dba<\/strong>
        \nP\u0159i dysfagii v d\u011btstv\u00ed je nutn\u00e9 operovat co nejd\u0159\u00edve, v dosp\u011blosti teprve tehdy, je-li\u00a0dysfagie \u00faporn\u00e1 a je-li ohro\u017eena v\u00fd\u017eiva.<\/p>\n

        Literatura<\/h3>\n
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          Kongenit\u00e1ln\u00ed vady j\u00edcnu jsou z\u00e1va\u017en\u00fdm vrozen\u00fdm posti\u017een\u00edm, kter\u00e9 se vyskytuje bu\u010f samostatn\u011b, nebo v kombinaci s dal\u0161\u00edmi vrozen\u00fdmi vadami (v\u00edce ne\u017e v 50 % b\u00fdvaj\u00ed sou\u010dasn\u011b p\u0159\u00edtomny dal\u0161\u00ed vrozen\u00e9 vady na za\u017e\u00edvac\u00edm traktu, srdci, urogenit\u00e1ln\u00edm syst\u00e9mu a muskuloskelet\u00e1rn\u00edm syst\u00e9mu). Prenat\u00e1ln\u00ed diagnostika t\u011bchto vad je mo\u017en\u00e1 d\u00edky zdokonalen\u00ed ultrazvukov\u00e9ho vy\u0161et\u0159en\u00ed a\u017e v posledn\u00edch desetilet\u00edch. Progn\u00f3za t\u00e9to […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":65,"menu_order":35,"comment_status":"closed","ping_status":"open","template":"","meta":{"footnotes":"","_links_to":"","_links_to_target":""},"class_list":["post-284","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=\/wp\/v2\/pages\/284","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=284"}],"version-history":[{"count":25,"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=\/wp\/v2\/pages\/284\/revisions"}],"predecessor-version":[{"id":3553,"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=\/wp\/v2\/pages\/284\/revisions\/3553"}],"up":[{"embeddable":true,"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=\/wp\/v2\/pages\/65"}],"wp:attachment":[{"href":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=284"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}