{"id":506,"date":"2013-03-14T09:38:25","date_gmt":"2013-03-14T09:38:25","guid":{"rendered":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/?page_id=506"},"modified":"2013-06-12T07:53:24","modified_gmt":"2013-06-12T07:53:24","slug":"11-2","status":"publish","type":"page","link":"https:\/\/eportal.chirurgie.upol.cz\/portal_final\/?page_id=506","title":{"rendered":"11 Neuromuskul\u00e1rn\u00ed poruchy j\u00edcnu"},"content":{"rendered":"

11.1 Definice a historie ezofagokardi\u00e1ln\u00ed achal\u00e1zie<\/h3>\n

Ezofagokardi\u00e1ln\u00ed achal\u00e1zie (EKA) (achal\u00e1zie j\u00edcnu, achal\u00e1zie kardie) je funk\u010dn\u00ed onemocn\u011bn\u00ed cel\u00e9ho j\u00edcnu, charakterizovan\u00e9 \u00fabytkem a\u017e nep\u0159\u00edtomnost\u00ed j\u00edcnov\u00e9 peristaltiky, postupnou dilatac\u00ed j\u00edcnu a sou\u010dasn\u011b neschopnost\u00ed norm\u00e1ln\u00ed relaxace doln\u00edho j\u00edcnov\u00e9ho sv\u011bra\u010de b\u011bhem polykac\u00edho aktu. Jde o nej\u010dast\u011bj\u0161\u00ed a nejzn\u00e1m\u011bj\u0161\u00ed, by\u0165 ne jedinou, neuromuskul\u00e1rn\u00ed poruchu j\u00edcnu. O dal\u0161\u00edch typech t\u011bchto poruch je pojedn\u00e1no v podkapitole 11.7 Diferenci\u00e1ln\u00ed diagnostika.<\/p>\n

Prvn\u00edho nemocn\u00e9ho s dilatac\u00ed j\u00edcnu bez anatomick\u00e9 p\u0159ek\u00e1\u017eky popsal v roce 1874 Willis a podrobn\u011bji onemocn\u011bn\u00ed charakterizovali Purton (1821), Mayo (1828) a Hannay (1833). Rozpornost ve v\u00fdkladu etiopatogeneze onemocn\u011bn\u00ed vedla k u\u017e\u00edv\u00e1n\u00ed cel\u00e9 \u0159ady dnes ji\u017e p\u0159ev\u00e1\u017en\u011b opu\u0161t\u011bn\u00fdch n\u00e1zv\u016f [1]: kardiospazmus \u2013 Huss 1842, Mikulicz 1882, prim\u00e1rn\u00ed muskul\u00e1rn\u00ed atonie j\u00edcnu \u2013 Rosenheim 1899, (kongenit\u00e1ln\u00ed) idiopatick\u00e1 dilatace j\u00edcnu \u2013 Sievers 1903, Langmead 1929, frenospazmus \u2013 Jackson 1922, aperistaltika ezofagu \u2013 Brazil 1955, aganglion\u00e1rn\u00ed nebo amyenterick\u00e1 achal\u00e1zie \u2013 Adams 1964.<\/p>\n

N\u00e1zev achal\u00e1zie navrhl poprv\u00e9 Perry (1913), kdy\u017e Hurst hledal pojem pro vyj\u00e1d\u0159en\u00ed p\u0159\u00ed\u010diny t\u00e9to choroby [2]. P\u0159esto\u017ee i toto ozna\u010den\u00ed postihuje jen jeden z charakteristick\u00fdch znak\u016f onemocn\u011bn\u00ed \u2013 neschopnost relaxace DJS \u2013 n\u00e1zev se v\u017eil a je\u00a0dnes v\u0161eobecn\u011b u\u017e\u00edv\u00e1n.<\/p>\n

11.2 Patologicko-anatomick\u00e9 n\u00e1lezy<\/h3>\n

U nemocn\u00fdch s EKA byly nalezeny morfologick\u00e9 zm\u011bny svaloviny a plexus myentericus j\u00edcnu, vagov\u00fdch nerv\u016f a mozkov\u00e9ho kmene.<\/p>\n

Ji\u017e makroskopicky je patrn\u00e1 dilatace hrudn\u00edho ezofagu se svalovou hypertrofi\u00ed, kter\u00e1 p\u0159ech\u00e1z\u00ed v nezbytn\u011bl\u00fd, n\u011bkolik centimetr\u016f dlouh\u00fd termin\u00e1ln\u00ed \u00fasek j\u00edcnu. Mohou b\u00fdt p\u0159\u00edtomny i zn\u00e1mky stagna\u010dn\u00ed ezofagitidy. Histologick\u00e1 vy\u0161et\u0159en\u00ed st\u011bny j\u00edcnu u nemocn\u00fdch s EKA, proveden\u00e1 na na\u0161em pracovi\u0161ti na po\u010d\u00e1tku 50. let minul\u00e9ho stolet\u00ed, prokazovala n\u00e1sleduj\u00edc\u00ed zm\u011bny [3]: 1. \u00dabytek a\u017e nep\u0159\u00edtomnost gangliov\u00fdch bun\u011bk v intramur\u00e1ln\u00edm myenterick\u00e9m plexu. 2. V\u00fdraznou hypertrofii svaloviny, zvl\u00e1\u0161t\u011b cirkul\u00e1rn\u00ed vrstvy, p\u0159i\u010dem\u017e nepom\u011bru mezi zbytn\u011bn\u00edm vnit\u0159n\u00ed a zevn\u00ed vrstvy ub\u00fdvalo sm\u011brem or\u00e1ln\u00edm. 3. R\u016fzn\u00fd stupe\u0148 fibr\u00f3zy, zvl\u00e1\u0161t\u011b vnit\u0159n\u00edch svalov\u00fdch snopc\u016f, p\u0159i mal\u00e9m zmno\u017een\u00ed vaziva v mezisvalov\u00e9 vrstv\u011b. Tento jev byl pokl\u00e1d\u00e1n za druhotn\u00fd, nast\u00e1vaj\u00edc\u00ed a\u017e ve zbytn\u011bl\u00e9 svalovin\u011b. 4. P\u0159\u00edtomnost tk\u00e1\u0148ov\u00fdch \u017e\u00edrn\u00fdch bun\u011bk jako d\u016fsledek chronicky z\u00e1n\u011btliv\u00fdch zm\u011bn, kter\u00e9 byly v\u017edy v r\u016fzn\u00e9m stupni ve svalovin\u011b nalezeny.<\/p>\n

Na zm\u011bny v Auerbachov\u011b plexu jako prvn\u00ed upozornil Brown a Kelly. Hurstem a Rakem byly pova\u017eov\u00e1ny za p\u0159\u00ed\u010dinu vzniku achal\u00e1zie [2]. Tyto n\u00e1lezy byly potvrzeny i pozd\u011bj\u0161\u00edmi vy\u0161et\u0159en\u00edmi [4]. Opakovan\u011b byla rovn\u011b\u017e pops\u00e1na hypertrofie j\u00edcnov\u00e9 svaloviny a jej\u00ed sou\u010dasn\u00e9 degenerativn\u00ed zm\u011bny [5].<\/p>\n

Ji\u017e v roce 1900 upozornil Rosenheim, \u017ee po\u0161kozen\u00ed nn. vagi m\u016f\u017ee b\u00fdt p\u0159\u00ed\u010dinou vzniku EKA. Prvn\u00edmi, kdo popsali achal\u00e1zii p\u0159i sou\u010dasn\u00e9 degeneraci obou bloudiv\u00fdch nerv\u016f, byli Kraus (1902) a Heyrovsky (1913). Pozd\u011bj\u0161\u00ed Cassellova vy\u0161et\u0159en\u00ed elektronov\u00fdm mikroskopem prok\u00e1zala degenerativn\u00ed zm\u011bny v drobn\u011bj\u0161\u00edch vagov\u00fdch vl\u00e1knech inervuj\u00edc\u00edch j\u00edcen [5]. Rovn\u011b\u017e zji\u0161t\u011bn\u00ed \u010dast\u00e9 poruchy vagov\u00e9 f\u00e1ze \u017ealude\u010dn\u00ed sekrece u nemocn\u00fdch s EKA sv\u011bd\u010d\u00ed pro mo\u017enost po\u0161kozen\u00ed vagov\u00e9 inervace [6]. Kimurou (1929) a Cassell (1964) prok\u00e1zali degenerativn\u00ed zm\u011bny v oblasti vagov\u00fdch jader v prodlou\u017een\u00e9 m\u00ed\u0161e u nemocn\u00fdch s EKA [5].<\/p>\n

11.3 Etiopatogeneze<\/h3>\n
\"Obr.<\/a>

Obr. 1
Schematick\u00e9 zn\u00e1zorn\u011bn\u00ed vagov\u00e9 inervace j\u00edcnu. \u0160ipky ukazuj\u00ed mo\u017en\u00e9 prim\u00e1rn\u00ed po\u0161kozen\u00ed uplat\u0148uj\u00edc\u00ed se v etiopatogenezi vzniku onemocn\u011bn\u00ed.
A \u2013 centr\u00e1ln\u00ed nervov\u00fd syst\u00e9m,
B \u2013 n. vagus,
C \u2013 plexus myentericus a hladk\u00e1 svalovina j\u00edcnu<\/p><\/div>\n

P\u0159\u00ed\u010dina onemocn\u011bn\u00ed je dosud nezn\u00e1m\u00e1. Kongenit\u00e1ln\u00ed a traumatick\u00fd p\u016fvod je m\u00e1lo pravd\u011bpodobn\u00fd. Ojedin\u011ble byl pops\u00e1n famili\u00e1rn\u00ed v\u00fdskyt onemocn\u011bn\u00ed. Nejpravd\u011bpodobn\u011bj\u0161\u00ed je z\u00edskan\u00e1 etiologie n\u00e1sledkem infek\u010dn\u00edho nebo toxick\u00e9ho po\u0161kozen\u00ed svaloviny j\u00edcnu nebo sp\u00ed\u0161e r\u016fzn\u00fdch et\u00e1\u017e\u00ed vagov\u00e9 inervace (obr. 1). Tuto teorii podporuje vznik poruchy funkce j\u00edcnu v pr\u016fb\u011bhu chronick\u00e9ho stadia Chagasovy choroby, kter\u00e1 je velmi \u010dast\u00e1 v Ji\u017en\u00ed Americe. Projevy onemocn\u011bn\u00ed jsou shodn\u00e9 s obrazem achal\u00e1zie. P\u0159\u00ed\u010dinou je po\u0161kozen\u00ed gangliov\u00fdch bun\u011bk plexus myentericus neurotoxinem Trypanosoma cruzi [7, 8, 9]. V na\u0161ich podm\u00ednk\u00e1ch se uva\u017euje o mo\u017enosti toxick\u00e9ho p\u016fsoben\u00ed enterovir\u016f, autoimunn\u00edm procesu, lok\u00e1ln\u00edm ischemick\u00e9m po\u0161kozen\u00ed nervov\u00fdch struktur i o d\u016fsledku polept\u00e1n\u00ed j\u00edcnu \u017e\u00edravinou. Zn\u00e1ma je manifestace choroby po psychick\u00e9m \u010di fyzick\u00e9m stresu. Jde v\u0161ak sp\u00ed\u0161 o psychoneurotick\u00e9 ovlivn\u011bn\u00ed dosud asymptomatick\u00e9ho onemocn\u011bn\u00ed.<\/p>\n

O slo\u017eitosti etiologie EKA sv\u011bd\u010d\u00ed skute\u010dnost, \u017ee se dosud nepoda\u0159ilo vytvo\u0159it dokonal\u00fd experiment\u00e1ln\u00ed model tohoto onemocn\u011bn\u00ed. Byly prov\u00e1d\u011bny experiment\u00e1ln\u00ed z\u00e1sahy do vagov\u00e9 inervace r\u016fzn\u00fdch et\u00e1\u017e\u00ed. K tomu p\u0159istupuj\u00ed klinick\u00e9 zku\u0161enosti s r\u016fzn\u00fdmi typy vagotomie. V\u00fdznam inervace sympatikem se ukazuje pro funkci j\u00edcnu jako m\u00e1lo v\u00fdznamn\u00fd [10]. Teprve vysok\u00e1 cervik\u00e1ln\u00ed vagotomie vede k t\u011b\u017ek\u00e9 poru\u0161e motility j\u00edcnu, av\u0161ak p\u0159i sou\u010dasn\u00e9m sn\u00ed\u017een\u00ed tonusu DJS, co\u017e neodpov\u00edd\u00e1 obrazu achal\u00e1zie [11, 12, 13]. Ur\u010dit\u00e9ho \u00fasp\u011bchu p\u0159i vytvo\u0159en\u00ed experiment\u00e1ln\u00edho modelu bylo dosa\u017eeno jen stereotakticky a elektrolytick\u00fdm z\u00e1sahem do nervov\u00fdch jader vagu v prodlou\u017een\u00e9 m\u00ed\u0161e u ko\u010dek a ps\u016f [14]. Byly prov\u00e1d\u011bny i \u010detn\u00e9 pokusy o lok\u00e1ln\u00ed po\u0161kozen\u00ed myenterick\u00e9ho plexu fyzik\u00e1ln\u00edmi prost\u0159edky [15] nebo m\u00edstn\u00ed ischemizac\u00ed [16]. Interpretace vznikl\u00fdch mikroskopick\u00fdch a funk\u010dn\u00edch zm\u011bn ale nebyla jednozna\u010dn\u00e1 a nepodala p\u0159esv\u011bd\u010div\u00fd d\u016fkaz o v\u00fdznamu Auerbachova plexu p\u0159i vzniku funk\u010dn\u00edch poruch j\u00edcnu. \u0158ada studi\u00ed v\u0161ak potvrdila \u00fabytek, degenerativn\u00ed a z\u00e1n\u011btliv\u00e9 zm\u011bny gangli\u00ed.<\/p>\n

Dne\u0161n\u00ed znalosti sv\u011bd\u010d\u00ed pro to, \u017ee EKA je neuromuskul\u00e1rn\u00ed poruchou funkce j\u00edcnu, lokalizovanou na n\u011bkter\u00e9 et\u00e1\u017ei vagov\u00e9 inervace nebo ve svalovin\u011b j\u00edcnu, jej\u00ed\u017e etiologii zat\u00edm nezn\u00e1me (obr. 1).<\/p>\n

Nov\u00e9 pohledy na etiopatogenezi achal\u00e1zie p\u0159inesly poznatky o vlivu gastrinu na funkci DJS. Z \u010detn\u00fdch teori\u00ed o patogenezi achal\u00e1zie byl nejobecn\u011bji p\u0159ijat v\u00fdklad Hurst\u016fv [2], kter\u00fd hlavn\u00ed p\u0159\u00ed\u010dinu onemocn\u011bn\u00ed vid\u011bl v neschopnosti relaxace DJS v d\u016fsledku degenerativn\u00edch zm\u011bn a\u017e vymizen\u00ed gangliov\u00fdch bun\u011bk j\u00edcnu. Dnes v\u00edme, \u017ee nejde jen o tuto poruchu, ale \u017ee nemoc postihuje cel\u00fd ezofagus a inerva\u010dn\u00ed oblast nn. vagi. Zm\u011bny myenterick\u00e9ho plexu mohou b\u00fdt n\u011bkdy omezeny jen na \u010d\u00e1st j\u00edcnu nad DJS a jsou z\u00e1visl\u00e9 na d\u00e9lce trv\u00e1n\u00ed onemocn\u011bn\u00ed, co\u017e p\u0159ipou\u0161t\u00ed v\u00fdklad a\u017e druhotn\u00e9ho po\u0161kozen\u00ed gangliov\u00fdch bun\u011bk.<\/p>\n

11.4 Patofyziologick\u00e1 charakteristika<\/h3>\n

I kdy\u017e poruchu j\u00edcnov\u00e9 motility je mo\u017eno registrovat i rentgenologick\u00fdm a rentgenokinematografick\u00fdm vy\u0161et\u0159en\u00edm, umo\u017enila detailn\u00ed posouzen\u00ed funk\u010dn\u00edch poruch j\u00edcnu a\u017e j\u00edcnov\u00e1 manometrie. U EKA je mimo deglutinaci tlak v j\u00edcnu m\u00edrn\u011b zv\u00fd\u0161en, co\u017e se p\u0159i\u010d\u00edt\u00e1 svalov\u00e9 hypertrofii a zbytk\u016fm potravy v ezofagu. B\u011bhem polykac\u00edho aktu je registrov\u00e1na zna\u010dn\u011b ni\u017e\u0161\u00ed tlakov\u00e1 amplituda peristaltick\u00fdch vln, kter\u00e1 se sni\u017euje \u00fam\u011brn\u011b s nar\u016fstaj\u00edc\u00ed dilatac\u00ed j\u00edcnu. Odpov\u00edd\u00e1 to postupn\u00e9mu \u00fabytku prim\u00e1rn\u00ed a sekund\u00e1rn\u00ed peristaltiky. Naproti tomu se mno\u017e\u00ed simult\u00e1nn\u00ed, nepropulzivn\u00ed, tzv. terci\u00e1rn\u00ed kontrakce neovliv\u0148uj\u00edc\u00ed posun potravy, kter\u00e9 vznikaj\u00ed zvl\u00e1\u0161t\u011b v doln\u00ed polovin\u011b j\u00edcnu. Tyto stahy za\u010d\u00ednaj\u00ed \u010dasto sou\u010dasn\u011b na v\u00edce m\u00edstech j\u00edcnu najednou v n\u00e1vaznosti na polknut\u00ed nebo spont\u00e1nn\u011b (repetitivn\u00ed kontrakce). Doba jejich trv\u00e1n\u00ed je a\u017e dvojn\u00e1sobn\u00e1 proti norm\u00e1ln\u00ed peristaltick\u00e9 vln\u011b a je zn\u00e1m\u00e1 rentgenolog\u016fm jako pilovit\u00e9 pseudodivertikul\u00f3zn\u00ed z\u00e1\u0159ezy na j\u00edcnu. Dal\u0161\u00edm charakteristick\u00fdm znakem achal\u00e1zie je zv\u00fd\u0161en\u00ed klidov\u00e9ho tonu DJS proti zdrav\u00fdm jedinc\u016fm. Nen\u00ed v\u0161ak bez zaj\u00edmavosti, \u017ee u n\u011bkter\u00fdch nemocn\u00fdch s typick\u00fdm obrazem onemocn\u011bn\u00ed mohou b\u00fdt tlakov\u00e9 hodnoty norm\u00e1ln\u00ed [20, 21] <\/b>(viz kapitola 6 Diagnostika, tab. 1 a obr. 1). B\u011bhem polykac\u00edho aktu nedoch\u00e1z\u00ed k \u00fapln\u00e9 a koordinovan\u00e9 relaxaci DJS [22]. Vyu\u017eit\u00ed modern\u00edch manometrick\u00fdch postup\u016f [23, 24] umo\u017e\u0148uje maxim\u00e1ln\u011b zp\u0159esnit diagnostiku achal\u00e1zie a rozli\u0161it i ur\u010dit\u00e9 subtypy dle manometrick\u00e9 charakteristiky. Manometrie se rovn\u011b\u017e v\u00fdznamn\u011b uplat\u0148uje p\u0159i hodnocen\u00ed proveden\u00e9 l\u00e9\u010dby [25].<\/p>\n

Ji\u017e \u0159adu let je zn\u00e1mo a v radiodiagnostice vyu\u017e\u00edv\u00e1no zv\u00fd\u0161en\u00ed kontrakc\u00ed j\u00edcnu po aplikaci cholinergn\u00edho mecholilu. V\u00fdzkumy p\u0159inesly poznatky o hormon\u00e1ln\u00edm a farmakologick\u00e9m ovlivn\u011bn\u00ed funkce DJS a bylo zkoum\u00e1no i jejich p\u016fsoben\u00ed u EKA (tab. 1).<\/p>\n\n\n\n\n\n
Tab. 1<\/span>
\nVliv r\u016fzn\u00fdch farmak na klidov\u00fd tonus DJS u EKA podle Wienbecka [26]<\/span><\/span><\/td>\n<\/tr>\n
Zvy\u0161uj\u00edc\u00ed<\/strong><\/td>\nSni\u017euj\u00edc\u00ed<\/strong><\/td>\nBez \u00fa\u010dinku<\/strong><\/td>\n<\/tr>\n
\n
    \n
  • Cholinergica<\/span>!<\/span><\/sup><\/li>\n
  • Gastrin<\/span>?<\/span><\/sup><\/li>\n
  • Motilin<\/span>?<\/span><\/sup><\/li>\n
  • Prostaglandin F<\/span><\/li>\n<\/ul>\n<\/td>\n
\n
    \n
  • Anticholinergica<\/span><\/li>\n
  • Beta-adrenergica<\/span>?<\/span><\/sup><\/li>\n
  • Energetick\u00e9 fosf\u00e1ty<\/span>?<\/span><\/sup><\/li>\n
  • Cholecystokinin<\/span><\/li>\n
  • Caerulein<\/span><\/li>\n
  • Sekretin<\/span><\/li>\n
  • Glucagon<\/span><\/li>\n<\/ul>\n<\/td>\n
\n