{"id":6136,"date":"2020-04-03T22:19:49","date_gmt":"2020-04-03T22:19:49","guid":{"rendered":"http:\/\/www.narkozsuzsunnet.com\/?p=6136"},"modified":"2020-04-03T22:19:49","modified_gmt":"2020-04-03T22:19:49","slug":"konjenital-diafragma-hernisi","status":"publish","type":"post","link":"https:\/\/www.narkozsuzsunnet.com\/index.php\/2020\/04\/03\/konjenital-diafragma-hernisi\/","title":{"rendered":"Konjenital Diafragma hernisi"},"content":{"rendered":"<p><img fetchpriority=\"high\" decoding=\"async\" src=\"http:\/\/www.vipsunnet.com\/wp-content\/uploads\/diafragma-hernisi-vip-2.jpg\" alt=\"Antalya diafram hernisi\" width=\"1200\" height=\"300\" \/><\/p>\n<p>D\u0130AFRAGMA HERN\u0130S\u0130<br \/>\nDiafragmadaki bir defekt nedeniyle kar\u0131n i\u00e7i organlar\u0131n toraks i\u00e7inde yeralmas\u0131d\u0131r.<br \/>\n\u0130nsidans: 4000 canl\u0131 do\u011fumda bir g\u00f6r\u00fcl\u00fcr . Ancak \u00f6l\u00fc do\u011fumlarda dikkate al\u0131nd\u0131\u011f\u0131nda bu oran 1\/2000\u2032 dir. Genellikle sporadik olmakla birlikte; ikiszlerde, ayn\u0131 ailenin 2. Jenerasyonunda yay\u0131nlanm\u0131\u015f vakalar mevcuttur. Serilerin \u00e7o\u011funda herni %85-90 sol tarafta yer al\u0131r. \u0130ki tarafl\u0131 diafragma hernisi \u00e7ok nadir g\u00f6r\u00fcl\u00fcr.<br \/>\nAnatomi; Diafragma 4 k\u0131s\u0131mdan olu\u015fur.<br \/>\n1.\u00d6ndeki merkezi k\u0131s\u0131m (central tendon)<br \/>\n2.Dorsal porsiyon: \u00d6zefagus mezenterinden geli\u015fir.<br \/>\n3.Sirkumferensiyal porsiyon: major kas tabakas\u0131d\u0131r.<br \/>\n4.Dorsal \u00e7ift pleuroperitoneal membranlar: pleuroperitoneal kanallar\u0131n kapanmas\u0131 sonucu ortaya \u00e7\u0131kar.<br \/>\n<img decoding=\"async\" src=\"http:\/\/www.vipsunnet.com\/wp-content\/uploads\/diafragma-hernisi-vip-3.jpg\" alt=\"Antalya diafram hernisi\" width=\"1200\" height=\"300\" \/><\/p>\n<p>Embrioloji; Diafragma olu\u015fumu embriyoda 4-8. Haftalar aras\u0131nda ger\u00e7ekle\u015fir. Perikardiyal, pleural,peritoneal kavitelerin geli\u015fimide ayn\u0131 zaman dilimine rastlar. Ba\u015flang\u0131\u00e7ta perikardiyal kese peritoneal kavite ile 2 kanal arac\u0131l\u0131\u011f\u0131 ile birle\u015fir. Embriyo uzad\u0131k\u00e7a bu kanallar arka lateral b\u00f6lgelere kayarlar. Akci\u011ferler bu kanallar\u0131 invagine ederek pleural kaviteleri olu\u015ftururlar. 8. hafta civar\u0131nda pleuroperitoneal kanal ismini alan bu kanallar aortik,kaval,\u00f6zefageal hiatus d\u0131\u015f\u0131nda oblitere olurlar. Soldaki kanal sa\u011fa g\u00f6re daha az kapan\u0131r.<br \/>\nDiafragma kas\u0131n\u0131n nas\u0131l geli\u015fti\u011fi tart\u0131\u015fmal\u0131d\u0131r. Bu konuda iki teori mevcuttur.<br \/>\nLevis Teorisi: Diafragma kalbin kaudale olan g\u00f6\u00e7\u00fc ba\u015flamadan \u00f6nce servikal b\u00f6lgeden geli\u015fen kas segmentlerinden olu\u015fur. Ve kaudal g\u00f6\u00e7 s\u0131ras\u0131nda diafragma olu\u015fumu tamamlan\u0131r. Bu durum diafragmay\u0131 inerve eden frenik sinir kayna\u011f\u0131n\u0131n C4-C5 d\u00fczeyinde olmas\u0131n\u0131 a\u00e7\u0131klamaktad\u0131r.<br \/>\nBremer teorisi; Geli\u015fmekte olan pleural membran torasik duvar\u0131 olu\u015fturan kas yap\u0131s\u0131n\u0131n i\u00e7 tabakas\u0131n\u0131 ay\u0131r\u0131r. Bu durum abdominal duvar 3 tabaka kas dokusundan olu\u015furken toraks duvar\u0131n\u0131n 2 tabakadan olu\u015fmas\u0131na sebep olur. Pleuran\u0131n geli\u015fim esnas\u0131nda toraks duvar\u0131ndan ay\u0131rd\u0131\u011f\u0131 3. tabakan\u0131n kar\u0131ndaki transversus abdominis kas\u0131n\u0131n homolo\u011fu oldu\u011fu kabul edilmekte ve diaafragmay\u0131 olu\u015fturdu\u011fu iddia edilmektedir.<br \/>\nPleuran\u0131n geli\u015fim esnas\u0131nda toraks duvar\u0131ndan ay\u0131rd\u0131\u011f\u0131 3. tabakan\u0131n kar\u0131ndaki transversus abdominis kas\u0131n\u0131n homolo\u011fu oldu\u011fu kabul edilmekte ve diaafragmay\u0131 olu\u015fturdu\u011fu iddia edilmektedir.<br \/>\nDiafragma geli\u015fiminin tamamland\u0131\u011f\u0131 8 ve 9. Haftalar akci\u011fer geli\u015fiminin en kritik safhas\u0131d\u0131r. Bu d\u00f6nemde diafragma geli\u015fimindeki yetmezlik ve herniasyonun ortaya \u00e7\u0131kmas\u0131 o taraf akci\u011ferin geli\u015fmesine engel olmaktad\u0131r. Ancak kar\u015f\u0131 taraf akci\u011fer dokusunda hipoplazi g\u00f6r\u00fclmesi tart\u0131\u015fmal\u0131d\u0131r. Bu durum geli\u015fmekte olan akci\u011fer dokusundaki primer bozuklu\u011fun diafragma geli\u015fiminide aksatt\u0131\u011f\u0131n\u0131 d\u00fc\u015f\u00fcnd\u00fcrmektedir.<br \/>\nKlinik ve Tan\u0131; Diafragma hernisi olan bebek genellikle do\u011fum sonras\u0131 ba\u015flayan solunum s\u0131k\u0131nt\u0131s\u0131 g\u00f6sterir. Yutulan hava ile geni\u015fleyen barsaklar mediastene ve kar\u015f\u0131 taraf akci\u011fer dokusuna bas\u0131 yaparak solunum s\u0131k\u0131nt\u0131s\u0131n\u0131 art\u0131r\u0131r. Fizik Muayenede kar\u0131n \u00e7\u00f6k\u00fck g\u00f6r\u00fcn\u00fcmdedir. G\u00f6\u011f\u00fcs \u00f6n arka duvar \u00e7ap\u0131 artm\u0131\u015ft\u0131r. Solunum sesleri azd\u0131r. Dinlemekle toraks i\u00e7inde barsak sesleri duyulabilir. kalp tepe at\u0131m\u0131 defektin kar\u015f\u0131 taraf\u0131na yer de\u011fi\u015ftirmi\u015ftir. ta\u015fipne, retraksiyon,siyanoz saptanabilir.<br \/>\nKonjenital diafragma hernisinde \u00e7\u00f6z\u00fclemeyen sorun ilk 24 saat i\u00e7inde belirti veren bebeklerin ya\u015fat\u0131lmas\u0131d\u0131r. 1946 y\u0131l\u0131nda Gross taraf\u0131ndan yay\u0131nlanan 24 saatlik hastada ilk ba\u015far\u0131l\u0131 ameliyattan bu yana ya\u015fam sans\u0131nda artma olmam\u0131\u015ft\u0131r. ECMO,Hiperventilasyon,Vazodilat\u00f6r tedaviler hayal k\u0131r\u0131kl\u0131\u011f\u0131 yaratm\u0131\u015ft\u0131r. Bu y\u00f6nden ara\u015ft\u0131rmalar intrauterin cerrahi y\u00f6n\u00fcnde yo\u011funla\u015fm\u0131\u015ft\u0131r.<br \/>\nAy\u0131r\u0131c\u0131 tan\u0131;<br \/>\n1. Kistik akci\u011fer hastal\u0131klar\u0131<br \/>\n2.Diafragma evanterasyonu<br \/>\n3.Stafilokok pn\u00f6monisi ve pn\u00f6matosel<br \/>\n4.Akci\u011fer hipoplazisi<br \/>\nB\u00fct\u00fcn bu hastal\u0131klarda abdominal gaz paterni normaldir. NG tak\u0131larak \u00e7ekilen r\u00f6ntgenogram mide yerle\u015fimini g\u00f6sterir. Nadiren tan\u0131da baryumlu grafi gereklidir.<br \/>\nEk anomaliler; % 23 hastada major ek anomaliye rastlan\u0131r. En s\u0131k olarak Akci\u011fer hipoplazisi, Malrotasyon ve Kardiyak anomaliler g\u00f6r\u00fcl\u00fcr.<br \/>\nPreoperatif tedavi: Hastaya NG tak\u0131larak gaz distansiyonu azalt\u0131l\u0131r. Gerekirse asiste solunum ve hiperventilasyon yap\u0131larak hasta stabil hale getirilir. Solunum stabilizasyonundan sonra hastan\u0131n opere edilmesi \u00f6nerilmektedir. Genellikle abdominal subkostal insizyon tercih edilir. Diafragman\u0131n \u00f6n ve arka kenarlar\u0131 nonabsorbabl st\u00fcrle dikilir. Bazen arka kenar bulunmayabilir. Bu durumda kostaya yak\u0131n st\u00fcrler konulur. B\u00fcy\u00fck defektlerde M.latissimus dorsiflebi veya Dacron Mesh kullan\u0131labilir.<br \/>\nHastaya Akci\u011fer t\u00fcp\u00fc konulmal\u0131m\u0131d\u0131r. ?<br \/>\nT\u00fcp konulan hastalarda transpulmoner bas\u0131n\u00e7 fark\u0131 artmakta ve barotravma sonucu pn\u00f6motoraks ve anfizem ortaya \u00e7\u0131kmaktad\u0131r. T\u00fcp tak\u0131lmayan hastalarda i\u00e7ere b\u0131rak\u0131lan intrapleural hava akci\u011ferleri a\u015f\u0131r\u0131 ekspansiyondan koruyacak, hafif pozitif bir bas\u0131n\u00e7la yava\u015f yava\u015f ekspanse olmas\u0131n\u0131 sa\u011flayacakt\u0131r. Ameliyat olan hastam\u0131zda Mide perforasyonuna ba\u011fl\u0131 pn\u00f6motoraks geli\u015fmi\u015f oldu\u011fundan t\u00fcp preoperatif stabilizasyon i\u00e7in zorunlu olarak konulmu\u015ftur.<br \/>\n\u00d6l\u00fcm oran\u0131; Yenido\u011fanda ortaya \u00e7\u0131kan Diafragma hernisinde operatif ya\u015fam oran\u0131 % 50 civar\u0131ndad\u0131r. Son yay\u0131nlarda bu oranda s\u0131n\u0131rl\u0131 bir art\u0131\u015f olmu\u015ftur. \u0130lk 24 saat i\u00e7inde operasyon gerektiren hastalarda bu mortalite % 100\u2019e yak\u0131nd\u0131r.<br \/>\nFetal Cerrahi; Harrison ve arkada\u015flar\u0131 insanda ilk fetal diafragma onar\u0131m\u0131n\u0131 yapt\u0131lar. \u00c7al\u0131\u015fmalar experimental d\u00fczeyde olup anne ve fet\u00fcs i\u00e7in risk hen\u00fcz y\u00fcksek d\u00fczeydedir.<br \/>\nKONJEN\u0130TAL D\u0130AFRAGMA HERN\u0130S\u0130 HAKKINDAK\u0130 EN KAPSAMLI MAKALE burada payla\u015f\u0131lm\u0131\u015ft\u0131r.<\/p>\n<p>Mehmet O\u011fuzhan \u00d6zyurtkan, Murat K\u0131l\u0131\u00e7 F\u0131rat \u00dcniversitesi T\u0131p Fak\u00fcltesi, G\u00f6\u011f\u00fcs Cerrahisi Anabilim Dal\u0131, Elaz\u0131\u011f, T\u00fcrkiye e-posta: moozyurtkan@hotmail.com doi:10.5152\/tcb.2012.37<br \/>\n279<br \/>\nTANIM VE SINIFLANDIRMA<br \/>\nKonjenital diyafram hernisi (KDH), diyaframda bir defektin bulundu\u011fu do\u011fumsal bir anomalidir. KDH\u2019nin %95\u2019inden fazlas\u0131n\u0131 olu\u015fturan Bochdalek hernisinde bat\u0131n organlar\u0131n\u0131n toraks kavitesine do\u011fru yer de\u011fi\u015ftirdi\u011fi bir posterolateral herni ve de\u011fi\u015fik derecelerde akci\u011fer hipoplazisi mevcuttur. Posterolateral olmayan herniler \u00fc\u00e7 gruba ayr\u0131l\u0131r: (a) retrosternal veya parasternal herniler (Morgagni- Larrey); (b) santral tendonun \u00f6n k\u0131sm\u0131nda bulunan ve di\u011fer anomalilerle ili\u015fkili olan herniler ve (c) defektin s\u0131kl\u0131kla diyafram\u0131n kas d\u0131\u015f\u0131 santral tendin\u00f6z k\u0131sm\u0131nda oldu\u011fu santral herniler (1).<br \/>\nTAR\u0130H\u00c7E (2-4)<br \/>\n\u0130lk KDH vakas\u0131n\u0131 1679\u2019da Lazarus Riverius, 24 ya\u015f\u0131nda bir erke\u011fin otopsisini takiben sunmu\u015ftur. Bir \u00e7ocuktaki ilk KDH vakas\u0131 ise 1701\u2019de Holta taraf\u0131ndan bildirilmi\u015ftir. Cooper 1827\u2019de KDH\u2019n\u0131n semptomlar\u0131n\u0131, patolojisini ve s\u0131n\u0131fland\u0131rmas\u0131n\u0131 tariflemi\u015ftir. Bowditch 1847 y\u0131l\u0131ndaki Boston Society for Medical Observation toplant\u0131s\u0131nda KDH hastalar\u0131n\u0131 kapsayan ilk seriyi sun- mu\u015ftur. Daha sonra 1848 y\u0131l\u0131nda, Bochdalek, ileride kendi ad\u0131yla an\u0131lacak olan, posterolateral herniyi tarif- lemi\u015ftir. KDH\u2019nin ilk cerrahi d\u00fczeltme giri\u015fimini 1888\u2019de Nauman yapm\u0131\u015f; akut bat\u0131n ve akut solunum s\u0131k\u0131nt\u0131s\u0131<br \/>\n\u00d6zet Konjenital diyafram hernisi (KDH), diyaframda bir defektin bulundu\u011fu do\u011fumsal bir anomalidir. KDH\u2019nin %95\u2019inden fazlas\u0131n\u0131 Bochdalek hernisi, geri kalanlar\u0131n\u0131 Morgagni hernisi ve di\u011fer nadir tipler olu\u015fturur. KDH\u2019nin patolojisinde diyaf- ram hernisi, akci\u011fer hipoplazisi ve bat\u0131n organlar\u0131n\u0131n toraks i\u00e7i yer de\u011fi\u015ftirmesi olmak \u00fczere \u00fc\u00e7 \u00f6\u011fe bulunur. KDH\u2019ye s\u0131k- l\u0131kla, bir sendroma ait olsun veya olmas\u0131n, ba\u015fka bir anomali e\u015flik eder. Hastal\u0131\u011f\u0131n do\u011fum \u00f6ncesi tan\u0131s\u0131 \u00f6zellikle ultrason incelemesiyle m\u00fcmk\u00fcnd\u00fcr. Mortalitenin b\u00fcy\u00fck oranda e\u015flik eden akci\u011fer hipoplazisi ve pulmoner hipertansiyona ba\u011fl\u0131 oldu\u011funun anla\u015f\u0131lmas\u0131, \u00f6zellikle Bochdalek hernisinde, ope- rasyon \u00f6ncesi \u00e7e\u015fitli cerrahi d\u0131\u015f\u0131 y\u00f6ntemlerin kullan\u0131m\u0131n\u0131 \u00f6ne \u00e7\u0131karm\u0131\u015ft\u0131r. KDH\u2019nin cerrahi tedavisinde torakotomi veya laparatomi \u015feklindeki geleneksel yakla\u015f\u0131mlar\u0131n yan\u0131 s\u0131ra minimal invaziv yakla\u015f\u0131mlar (\u00f6zellikle laparaskopik cerrahi) pop\u00fclarite kazanmaktad\u0131r. Anahtar kelimeler: Diyafram hernisi, Akci\u011fer hipoplazisi, Pulmoner hipertansiyon, Cerrahi<br \/>\nAbstract Congenital diaphragmatic hernia (CDH) is a birth anomaly where there is a defect in the diaphragm. Bochdalek hernia accounts for more than 95% of CDH, followed by Morgagni hernia, and other lesser types. The pathology of CDH comp- rises three elements: the diaphragmatic hernia, pulmonary hypoplasia, and herniation of the abdominal organs into the thorax. Associated syndromic or nonsyndromic anomali- es occur commonly in CDH. Prenatal diagnosis might be possible in the majority of the cases by using ultrasound. Several nonsurgical attempts have gained importance in the presurgical stabilization of patients with CDH, mostly with the Bochdalek type, since it became clear that the mortality depends largely on the degree of the pulmonary hypoplasia and pulmonary hypertension. In the surgical treatment of CDH, minimally invasive methods (mostly laparascopic sur- gery) have gained popularity compared with the traditional approaches through thoracotomy or laparatomy. Key words: Diaphragmatic hernia, Pulmonary hypoplasia, Pulmonary hypertension, Surgery<br \/>\nolan 19 ya\u015f\u0131ndaki bir erkek hastaya laparatomi uygu- lam\u0131\u015ft\u0131r. Bunu 1889\u2019da O\u2019Dwyer\u2019\u0131n bir \u00e7ocukta uygu- lad\u0131\u011f\u0131 ilk cerrahi d\u00fczeltme izlemi\u015ftir. \u0130lk ba\u015far\u0131l\u0131 cerrahi tamir ise 1905 y\u0131l\u0131nda yap\u0131lm\u0131\u015ft\u0131r. Heidenhain 9 ya\u015f\u0131n- daki bir \u00e7ocu\u011fun hernisini red\u00fckte etmi\u015f ve diyafram defektini orta hat laparatomi insizyonuyla kapatm\u0131\u015ft\u0131r. Bundan yakla\u015f\u0131k 20 y\u0131l sonra Hedbolm KDH i\u00e7in cer- rahi uygulanan hastalarda %58 mortalite g\u00f6r\u00fcld\u00fc\u011f\u00fcn\u00fc bildirmi\u015ftir. 1940 y\u0131l\u0131nda Ladd ve Gross, KDH tan\u0131s\u0131n\u0131 \u00f6zge\u00e7mi\u015f bilgileri, fizik muayene bulgular\u0131 ve baryum e\u015flik etsin veya etmesin akci\u011ferin radyolojik incelen- mesi sonu\u00e7lar\u0131na dayanarak vermi\u015flerdir. Ayr\u0131ca cerra- hi giri\u015fimin ilk iki g\u00fcn i\u00e7inde yap\u0131lmas\u0131n\u0131 \u00f6nermi\u015flerdir. Gross ayn\u0131 zamanda, zor vakalarda bat\u0131n duvar\u0131n\u0131n iki- a\u015famal\u0131 kapat\u0131lmas\u0131 (cilt ve ciltalt\u0131n\u0131n ilk giri\u015fimde, bat\u0131n duvar\u0131n\u0131n 5-6 g\u00fcn sonra) y\u00f6ntemini tariflemi\u015ftir. 1950 y\u0131l\u0131nda Koop ve Johnson daha direkt bir g\u00f6r\u00fc\u015f alt\u0131nda onar\u0131m\u0131 sa\u011flayan transtorasik yakla\u015f\u0131m\u0131 \u00f6nermi\u015flerdir. Areechon ve Reid 1960\u2019l\u0131 y\u0131llara gelindi\u011finde KDH\u2019nin y\u00fcksek mortalitesinin sebebinin do\u011fum an\u0131n- daki akci\u011fer hipoplazisinin derecesiyle ili\u015fkili oldu\u011fu- nu g\u00f6zlemlemi\u015flerdir. Kal\u0131c\u0131 pulmoner hipertansiyo- nun tedavisinde 1976 y\u0131l\u0131nda ECMO (Extracorporeal Membrane Oxygenation) ilk kez ba\u015far\u0131yla uygulanm\u0131\u015f- t\u0131r. Daha sonra ECMO KDH tedavisinde de yer bul- mu\u015ftur. Son yirmi y\u0131lda KDH\u2019nin patofizyolojisinde pul- moner hipertansiyon ve akci\u011fer hipoplazisinin \u00f6nemi ortaya konmu\u015ftur. Yak\u0131n tarihli \u00e7e\u015fitli \u00e7al\u0131\u015fmalar ise kardiyak geli\u015fim bozuklu\u011funun KDH patofizyolojisini daha komplike hale getirdi\u011fini g\u00f6stermi\u015ftir.<br \/>\nET\u0130YOLOJ\u0130 VE PATOF\u0130ZYOLOJ\u0130<br \/>\nKDH\u2019n\u0131n patolojisi, diyafram hernisi, pulmoner hipoplazi ve bat\u0131n organlar\u0131n\u0131n toraks i\u00e7ine yer de\u011fi\u015f- tirmesi olmak \u00fczere \u00fc\u00e7 \u00f6\u011feden olu\u015fur. KDH\u2019n\u0131n etiyo- lojisine y\u00f6nelik mekanizmalar halen a\u00e7\u0131k olmamakla birlikte \u00f6ne s\u00fcr\u00fclen \u00e7e\u015fitli teoriler bulunmaktad\u0131r. \u0130lk teori diyaframdaki malformasyonun kom\u015fu akci\u011fer dokusundaki geli\u015fim bozuklu\u011funa sekonder geli\u015fti\u011fi \u00fczerinedir. \u0130kinci teori diyafram\u0131n frenik sinir taraf\u0131ndan beslenmesinin kusurlu meydana gelmesi ve bunun da diyafram kaslar\u0131n\u0131n d\u00fczg\u00fcn geli\u015fimini bozdu\u011fu \u015feklin- dedir. \u00dc\u00e7\u00fcnc\u00fc teori geleneksel olarak en s\u0131k \u00f6nerilen- dir. Buna g\u00f6re pl\u00f6roperitoneal kanal\u0131n kapanmas\u0131nda bir problem mevcuttur. D\u00f6rd\u00fcnc\u00fc teoride \u00fczerinde durulan, herniasyon b\u00f6lgesindeki diyafram liflerinin geli\u015fmedi\u011fi veya \u00e7ok zay\u0131f olduklar\u0131, b\u00f6ylece b\u00fcy\u00fcy\u00fcp geli\u015fen bat\u0131n organlar\u0131 kar\u015f\u0131s\u0131nda y\u0131rt\u0131lmaya m\u00fcsait bir zeminde bulunmalar\u0131d\u0131r (5, 6). T\u00fcm bunlara ek olarak defektin normal diyafram geli\u015fimine y\u00f6n veren bir veya daha fazla genle ili\u015fkisi oldu\u011funa da inan\u0131l\u0131r. A vitamini<br \/>\neksikli\u011fi, anti-epileptik ila\u00e7lar ve talidomid gibi baz\u0131 teratojen fakt\u00f6rler de \u00f6ne s\u00fcr\u00fclmektedir (7-9). Patofizyolojinin hem ayn\u0131 taraftaki hem de kar\u015f\u0131 taraftaki akci\u011ferde hasara sebep olan bir \u00e7ift-etki hipo- tezinden kaynakland\u0131\u011f\u0131 d\u00fc\u015f\u00fcn\u00fclmektedir. \u00d6ncelikle, gestasyonun 5 ila 16 haftal\u0131k erken d\u00f6nemlerinde, geli\u015fen akci\u011fer i\u00e7in yeterli bo\u015flu\u011fun bulunmamas\u0131 bron\u015fiyal dallanmay\u0131 azalt\u0131p daha az alveol geli\u015fimine sebep olur. Daha sonra, geli\u015fmeye \u00e7al\u0131\u015fan akci\u011fere bas\u0131 daha da artar; b\u00f6ylece havayollar\u0131n\u0131n \u00e7ap\u0131 ve alveol say\u0131s\u0131 azal\u0131r, interstisyel alan artar ve alveolar bo\u015fluk ile gaz al\u0131\u015fveri\u015f y\u00fczey miktar\u0131 d\u00fc\u015fer. Buna para- lel olarak damarsal de\u011fi\u015fiklikler ortaya \u00e7\u0131kar. Normal damar say\u0131s\u0131 azal\u0131rken k\u00fc\u00e7\u00fck intraasiner arteriyollerin kas tabakalar\u0131 belirgin kal\u0131nla\u015fma g\u00f6sterir. Daha ciddi vakalarda sol ventrik\u00fcl hipoplazisi e\u015flik eder. Pulmoner damar yata\u011f\u0131n\u0131n kesitsel \u00e7ap\u0131n\u0131n azalmas\u0131 y\u00fcz\u00fcnden kapiller kan ak\u0131m h\u0131z\u0131 d\u00fc\u015fer. Anormal pulmoner vazo- konstriksiyon da tabloya e\u015flik edince kan ak\u0131m\u0131 daha da azal\u0131r. Bu de\u011fi\u015fiklikler do\u011fum sonras\u0131nda kendini pulmoner hipoplazi ve hipertansiyon \u015feklinde g\u00f6sterir- ler. Bu de\u011fi\u015fikliklerin derecesi s\u00fcrviye en s\u0131k etki eden fakt\u00f6rd\u00fcr (10, 11).<br \/>\nEpidemiyoloji ve E\u015flik eden hastal\u0131klar\/anomaliler KDH insidans\u0131 1\/2500\u2019d\u00fcr ve e\u011fer \u00f6l\u00fc do\u011fumlar da eklenirse oran 1\/5000 olmaktad\u0131r (12). KDH\u2019n\u0131n yakla- \u015f\u0131k %85-90\u2019\u0131 solda, %10-15\u2019i sa\u011fda g\u00f6r\u00fcl\u00fcrken, %2\u2019si bilateraldir. Vakalar\u0131n yakla\u015f\u0131k %60\u2019\u0131nda hastal\u0131k tek ba\u015f\u0131nad\u0131r ve ba\u015fka bir konjenital anomali e\u015flik etmez (13). Bir sendromun par\u00e7as\u0131 olmayan anomaliler %16- 53 oran\u0131nda g\u00f6r\u00fcl\u00fcrler ve g\u00f6r\u00fclme s\u0131kl\u0131klar\u0131na g\u00f6re kar- diyovask\u00fcler (%14-27), \u00fcrogenital (%5-18), kas iskelet sistemi (%7-16), merkezi sinir sistemi (%10-13), gast- rointestinal (%6), kraniofasyal (%5) ve pulmoner (%2) defektlerdir. Diyafram defektinin geni\u015f veya bilateral oldu\u011fu hallerde birden fazla e\u015flik eden anomali oldu\u011fu beliritilmi\u015ftir (14). KDH\u2019da e\u015flik eden anomali oran\u0131 %40\u2019lara kadar \u00e7\u0131kabilir; \u00f6l\u00fc do\u011fum ve d\u00fc\u015f\u00fckler de g\u00f6z \u00f6n\u00fcne al\u0131n\u0131rsa bu oran %95\u2019e y\u00fckselir (15). Say\u0131sal kromozom anomalileri (trizomi 18 veya tetrazomi) veya yap\u0131sal kompleks kromozom anomalileri (kromozom 11 ve 22 aras\u0131ndaki dengesiz translokasyonlar; 12q, 4p ve 8p\u2019deki delesyonlar) KDH hastalar\u0131n\u0131n %3 ila 40\u2019\u0131nda g\u00f6r\u00fcl\u00fcr (1). Sitogenetik ve molek\u00fcler genetik \u00e7al\u0131\u015fmalar\u0131 KDH ile kromozom 1q41q42 delesyonu aras\u0131ndaki ili\u015fkiyi g\u00f6stermi\u015ftir (16). \u00c7o\u011fu izole herni vakas\u0131 sporadiktir, ancak d\u00f6llerde, ikizlerde veya iki ku\u015fak boyunca g\u00f6r\u00fclen vakalar mevcuttur (8). \u00d6\u011feleri aras\u0131nda KDH\u2019y\u0131 i\u00e7eren \u00e7ok say\u0131da genetik sendrom bulunur. Bunlar aras\u0131nda Fryns, Brachman de Lange, CHARGE, Goldenhar, Smith-Lemli-Opitz, Simpson-<br \/>\nKONJEN\u0130TAL D\u0130YAFRAM HERN\u0130LER\u0130<br \/>\n280<br \/>\nGolabi-Behmel, Fraser, Beckwich-Weidemann, Denys Drash, Marfan, kraniyofrontonazal sendrom, multipl pterygium, Noonan ve spondilokostal dizostoz say\u0131la- bilir (9, 11). Hastalar\u0131n %10\u2019unda e\u015flik eden kardiyak anomali bulunmaktad\u0131r. Ciddi kardiyak defektlerin ve genetik anomalilerin s\u00fcrvi \u00fczerinde negatif etkisi vard\u0131r. Bu y\u00fczden fetal ekokardiyografiyle tam tan\u0131sal inceleme ve amniyosentez ile do\u011fum \u00f6ncesi karyotip incelemesi olduk\u00e7a \u00f6nem arz etmektedir (17).<br \/>\nTANI<br \/>\nDo\u011fum \u00f6ncesi tan\u0131 Hamilelik d\u00f6neminde tan\u0131da tercih edilen y\u00f6ntem ultrasondur. Y\u00f6ntemin iyonize radyasyon i\u00e7ermemesi, ta\u015f\u0131nabilir olmas\u0131, ger\u00e7ek zamanl\u0131 g\u00f6r\u00fcnt\u00fcleme sa\u011fla- mas\u0131, damar incelemesinde renkli Doppler katk\u0131s\u0131n\u0131n olmas\u0131 ve anne aday\u0131na s\u0131k\u0131nt\u0131 vermemesi \u00f6ne \u00e7\u0131kan \u00f6zelliklerindendir. Ancak yumu\u015fak doku kontrast\u0131n\u0131n s\u0131n\u0131rl\u0131 ve g\u00f6r\u00fcnt\u00fc alan\u0131n\u0131n dar olmas\u0131, oligohidramni- osun, fet\u00fcs\u00fcn pozisyonunun ve kemik dokunun ince- leme alan\u0131n\u0131 g\u00f6lgeleyebilmesi ve annenin a\u015f\u0131r\u0131 kilolu olmas\u0131 y\u00fcz\u00fcnden teknik s\u0131k\u0131nt\u0131lar ya\u015fanmas\u0131 ultrasonun negatif y\u00f6nleridir. Genellikle gestasyonun 24. hafta- s\u0131ndan sonra ultrason incelemesinin KDH\u2019yi yakalama oran\u0131 %59\u2019dur ve bu oran ilerleyen gestasyonel ya\u015f ve e\u015flik eden di\u011fer bir anomali varl\u0131\u011f\u0131nda artar (18). Do\u011fum \u00f6ncesi tan\u0131 24. haftan\u0131n \u00f6ncesinde %22-52 oran\u0131nda koyulabilir. Ancak yine de 24. haftadan sonra bile tan\u0131 konmas\u0131 genelde zor olarak kabul edilir, \u00e7\u00fcnk\u00fc %25 vaka g\u00f6zden ka\u00e7makta ve %11 vaka do\u011fum sonras\u0131 tan\u0131 almaktad\u0131r (19). Midenin bat\u0131ndaki normal pozisyonunda olmamas\u0131 KDH\u2019y\u0131 d\u00fc\u015f\u00fcnd\u00fcren \u00f6nemli bir bulgudur. Tan\u0131y\u0131 netle\u015ftirebilecek direkt bulgular intratorasik b\u00f6lgede karaci\u011fer, kolon veya midenin g\u00f6r\u00fclmesi, indirekt bulgular ise kardiyak ve mediyas- tinal kayma, kay\u0131k kar\u0131n g\u00f6r\u00fcnt\u00fcs\u00fc ve polihidramnios varl\u0131\u011f\u0131d\u0131r (20). Ultrasona tamamlay\u0131c\u0131 olarak fetal magnetik rezo- nans incelemesi faydal\u0131d\u0131r. Yumu\u015fak dokuyu iyi ay\u0131rd edebilmesi ve geni\u015f bir g\u00f6r\u00fcnt\u00fc alan\u0131 sa\u011flamas\u0131 avan- tajl\u0131 \u00f6zelliklerindendir. Ayr\u0131ca fet\u00fcs\u00fcn pozisyonu, kemik yap\u0131lar\u0131n g\u00f6lge olu\u015fturmas\u0131 veya oligohidramnios gibi durumlarda etkilenmez. Ancak teratojen etkisi y\u00fcz\u00fcn- den hamileli\u011fin ilk \u00fc\u00e7 ay\u0131 i\u00e7inde kullan\u0131lmamaktad\u0131r. Magnetik rezonans incelemesi KDH tan\u0131s\u0131n\u0131 netle\u015f- tirmede, detayl\u0131 bir anatomi sunmada ve e\u015flik eden anomalilerin varl\u0131\u011f\u0131n\u0131 g\u00f6stermede yard\u0131mc\u0131 ve faydal\u0131 bir tekniktir (21).<br \/>\nDo\u011fum sonras\u0131 tan\u0131 KDH\u2019l\u0131 bebeklerin %50\u2019ye yak\u0131n\u0131 do\u011fduklar\u0131nda hen\u00fcz tan\u0131s\u0131zd\u0131r. Bu bebeklerde do\u011fumdan sonra<br \/>\nhemen veya 12 saat i\u00e7inde akut solunum s\u0131k\u0131nt\u0131s\u0131n\u0131n geli\u015fmesi klasik bulgudur. Bir akci\u011fer grafisi toraks i\u00e7indeki barsak par\u00e7alar\u0131n\u0131, akci\u011fer hipoplazisini ve mediyastenin di\u011fer tarafa do\u011fru kaym\u0131\u015f oldu\u011funu g\u00f6sterir. Barsaklar\u0131 dekomprese etmek i\u00e7in mideye yerle\u015ftirilen orogastik t\u00fcp\u00fcn toraks i\u00e7inde oldu\u011fu ve bat\u0131ndaki gaz miktar\u0131n\u0131n azald\u0131\u011f\u0131 g\u00f6r\u00fcl\u00fcr (11). Baz\u0131 hastalarda semptom yoktur ve aylar ile y\u0131llar boyunca herni tan\u0131s\u0131 konulmayabilir. Bu hastalarda akci\u011ferler normaldir ve do\u011fum sonras\u0131 adaptasyonda sorun ya\u015fanmam\u0131\u015ft\u0131r. Belli belirsiz semptomlar\u0131n ince- lenmesi esnas\u0131nda veya ba\u015fka bir sebepten yap\u0131lan radyolojik incelemeler sonucunda KDH varl\u0131\u011f\u0131 sapta- n\u0131r. Bu t\u00fcr hastalarda g\u00f6r\u00fcnt\u00fcleme sonu\u00e7lar\u0131 yan\u0131lt\u0131c\u0131 olabilir; \u00e7\u00fcnk\u00fc bulgular kavitasyonlu pn\u00f6moni, plevral ef\u00fczyon, masif pn\u00f6motoraks veya pn\u00f6matosel gibi akut akci\u011fer problemlerini taklit edebilir. Ge\u00e7 tan\u0131 konan KDH\u2019l\u0131 \u00e7ocuklar\u0131n bat\u0131n grafi bulgular\u0131 aras\u0131nda, hemen her zaman diyafram\u0131n sol alt\u0131nda g\u00f6r\u00fclen mide gaz\u0131 g\u00f6r\u00fcnt\u00fcs\u00fc olduk\u00e7a faydal\u0131d\u0131r. Sol taraf\u0131 etkileyen KDH\u2019larda mide gaz\u0131 bat\u0131nda g\u00f6r\u00fclmez. Bat\u0131nda veya toraks i\u00e7inde anormal bir yerde bulunabilir. Bu durum, ge\u00e7 bulgu veren KDH\u2019lar\u0131n erken tan\u0131s\u0131nda olduk\u00e7a faydal\u0131 bir belirtidir (22).<br \/>\nPROGNOST\u0130K FAKT\u00d6RLER<br \/>\nAnatomik fakt\u00f6rler &#8211; Do\u011fum \u00f6ncesi tan\u0131 konmas\u0131: Bu durum tek ba\u015f\u0131na k\u00f6t\u00fc prognoz kriteri de\u011fildir, ancak anne karn\u0131nda tan\u0131s\u0131 konan KDH\u2019l\u0131 fet\u00fcslerde s\u0131kl\u0131kla e\u015flik eden di\u011fer bir anomali mevcuttur ve prognoza k\u00f6t\u00fc etki eden esas bu durumdur (1, 19). &#8211; Polihidramnios k\u00f6t\u00fc bir prognoz kriteridir (1, 11). &#8211; B\u00fcy\u00fck boyutlu bir KDH k\u00f6t\u00fc prognozu g\u00f6sterir (1). &#8211; Mide herniasyonu: Mide e\u011fer toraks\u0131n i\u00e7indeyse prognoz k\u00f6t\u00fcd\u00fcr (11). &#8211; Y\u00f6n: Tarihsel olarak bak\u0131ld\u0131\u011f\u0131nda herninin y\u00f6n\u00fcn\u00fcn prognozla ili\u015fkili oldu\u011fu d\u00fc\u015f\u00fcn\u00fclm\u00fc\u015ft\u00fcr ve sa\u011f taraf tutulumu sola g\u00f6re daha k\u00f6t\u00fc bir prognoz belirteci- dir. Ayr\u0131ca bilateral tutulumun k\u00f6t\u00fc prognoza i\u015faret etti\u011fini g\u00f6steren yay\u0131nlar mevcuttur (11). &#8211; Karaci\u011fer herniasyonu: Sol taraf tutulumlu KDH\u2019larda fetal karaci\u011ferin yeri en iyi prognostik fakt\u00f6rd\u00fcr ve herniasyon k\u00f6t\u00fc prognozu g\u00f6sterir (1, 11). &#8211; Son y\u0131llarda prognoz tahmininde fetal akci\u011fer\/ kafa oran\u0131 (AKO) \u00f6l\u00e7\u00fcm\u00fc pop\u00fclarite kazanm\u0131\u015ft\u0131r. \u0130ki boyutlu ultrason kullan\u0131larak \u00f6nce kalbin d\u00f6rt odal\u0131 g\u00f6r\u00fcnt\u00fcs\u00fcn\u00fcn hizas\u0131ndan kar\u015f\u0131 akci\u011ferin alan\u0131, sonra lateral ventrik\u00fcller seviyesinden kafa \u00e7evresi \u00f6l\u00e7\u00fclerek birbirlerine oranlan\u0131r. E\u011fer oran birin alt\u0131n- daysa mortalite riski yakla\u015f\u0131k %100\u2019d\u00fcr (23).<br \/>\nKONJEN\u0130TAL D\u0130YAFRAM HERN\u0130LER\u0130<br \/>\n281<br \/>\n&#8211; Cannie ve arkada\u015flar\u0131 (24) do\u011fum \u00f6ncesi tan\u0131 konan KDH\u2019l\u0131 hastalar\u0131n prognoz tayininde yeni bir metod olarak magnetik rezonans kullan\u0131m\u0131n\u0131 \u00f6nermi\u015flerdir. Fetal akci\u011fer hacmi ile t\u00fcm v\u00fccut hacmi aras\u0131nda bir orant\u0131sal ili\u015fki kurarak bir g\u00f6zlenen\/tahmin edilen akci\u011fer hacim \u00f6l\u00e7e\u011fi yaratm\u0131\u015flard\u0131r. Buna g\u00f6re ora- n\u0131n %35\u2019in alt\u0131nda olmas\u0131 k\u00f6t\u00fc prognoz kriteridir. &#8211; Do\u011fum a\u011f\u0131rl\u0131\u011f\u0131n\u0131n 2.7 kg\u2019nin alt\u0131nda olmas\u0131 k\u00f6t\u00fc prognozu g\u00f6sterir (1).<br \/>\nFizyolojik fakt\u00f6rler &#8211; Do\u011fum sonras\u0131 mortalite, akci\u011fer hipoplazisi ile pulmoner hipertansiyonun birlikte sebep oldu\u011fu solu- num yetmezli\u011finin sonucudur. Hemen do\u011fum sonras\u0131 kaybedilen infantlardaki akci\u011fer hipoplazisinin derece- si de\u011fi\u015fkenlik g\u00f6sterirken, ya\u015fayanlarda akci\u011ferler her zaman normaldir. Normal olmayan PaCO2, PaO2 ve pH de\u011ferleri k\u00f6t\u00fc prognoz kriterleridir (11). &#8211; Postduktal parsiyel oksijen bas\u0131nc\u0131n\u0131n 100 mmHg\u2019nin alt\u0131nda ve alveolar\/arteriyel oksijen grad- yan\u0131n\u0131n 500\u2019\u00fcn \u00fczerinde olmas\u0131 y\u00fcksek mortalite oran\u0131n\u0131 g\u00f6sterir (11). &#8211; McGoon indeksi [MG\u0130: (SaPA \u00e7ap\u0131+SoPA \u00e7ap\u0131)\/ \u0130A \u00e7ap\u0131] ve pulmoner arter indeksi [PA\u0130: (SaPA alan\u0131+SoPA alan\u0131)\/VA], KDH\u2019l\u0131 hastalar\u0131n s\u00fcrvileriyle ili\u015fkili iki indekstir (SaPA: sa\u011f pulmoner arter, SoPA: sol pulmoner arter, \u0130A: inen aort, VA: v\u00fccut alan\u0131) (25). &#8211; FiO2 (%)\ufffdortalama havayolu bas\u0131nc\u0131 (cmH2O) \u2044PaO2 (kPa) form\u00fcl\u00fcyle hesaplanan birinci g\u00fcn\u00fcn en iyi oksijenasyon indeksinin &gt;82 olmas\u0131 prognozun iyi oldu\u011funu belirtir (1).<br \/>\nDO\u011eUM \u00d6NCES\u0130 G\u0130R\u0130\u015e\u0130MLER<br \/>\nA\u00e7\u0131k anatomik onar\u0131m KDH\u2019n\u0131n do\u011fum \u00f6ncesi cerrahi onar\u0131m\u0131 ilk kez 1986 y\u0131l\u0131nda denenmi\u015ftir. \u00d6nceleri fetal cerrahinin anne i\u00e7in belirgin riskler i\u00e7ermesi y\u00fcz\u00fcnden, sadece olduk\u00e7a ciddi vakalarda (karaci\u011fer herniasyonu olanlara) giri- \u015fim \u00f6nerilmi\u015ftir. Karaci\u011ferin bat\u0131na red\u00fckte edilmesi umbilikal ven\u00f6z d\u00f6n\u00fc\u015f\u00fc bozmakta ve sonucunda bra- dikardiye ve fet\u00fcs\u00fcn kayb\u0131na sebebiyet vermektedir. Ancak karaci\u011fer herniasyonu olmayan vakalarda bu durum g\u00f6r\u00fclmemektedir. \u0130nsan fet\u00fcs\u00fcnde ilk ba\u015fa- r\u0131l\u0131 do\u011fum \u00f6ncesi cerrahi onar\u0131m 1990 y\u0131l\u0131nda rapor edilmi\u015ftir. Onar\u0131m torakotomi ve organlar\u0131 red\u00fckte etmek i\u00e7in subkostal insizyonu kapsayan iki a\u015famal\u0131 bir teknikle yap\u0131lm\u0131\u015ft\u0131r. Buna ra\u011fmen bu infantlarda s\u00fcrvi %75 iken do\u011fum sonras\u0131 tedavi edilenlerde bu oran %86\u2019d\u0131r. Buna ek olarak ECMO ihtiyac\u0131, ventilat\u00f6r s\u00fcresi ve hastanede kal\u0131\u015f s\u00fcresi her iki grupta farkl\u0131l\u0131k<br \/>\ng\u00f6stermemektedir. Karaci\u011fer herniasyonunun e\u015flik etmedi\u011fi KDH vakalar\u0131nda do\u011fum \u00f6ncesi cerrahi ona- r\u0131m mortalite ve morbiditeyi belirgin etkilemedi\u011fi i\u00e7in bu hasta grubu i\u00e7in daha sonra do\u011fum \u00f6ncesi giri\u015fim \u00f6nerilmemi\u015ftir (26).<br \/>\nFetal trakeal okl\u00fczyon (FETO) ile fizyolojik giri\u015fim PLUG stratejisi (\u2018Plug the Lung Until it Grows\u2019- B\u00fcy\u00fcyene kadar t\u0131ka) trakean\u0131n kapat\u0131lmas\u0131 yoluyla pulmoner hiperplazi yaratarak ve h\u00fccre b\u00fcy\u00fcmesini tetikleyen akci\u011fer s\u0131v\u0131s\u0131n\u0131n akci\u011ferde kal\u0131p damarlan- may\u0131 artt\u0131rmas\u0131n\u0131 sa\u011flayarak akci\u011fer geli\u015fimini kontrol etmeyi hedefler. Ancak intra\u00fcterin d\u00f6nemde trakean\u0131n kapat\u0131lmas\u0131 yoluyla d\u00fczeltilen KDH\u2019l\u0131 insan fet\u00fcsleri- nin akci\u011ferlerinin patolojik incelenmesi sonucunda akci\u011ferde b\u00fcy\u00fcme g\u00f6r\u00fcl\u00fcrken parenkimal yap\u0131 veya pulmoner arterlerin kas yap\u0131s\u0131nda belirgin bir geli- \u015fim olmad\u0131\u011f\u0131 saptanm\u0131\u015ft\u0131r. M\u00fcteakip teknik d\u00fczeltme, fetoskop \u00fczerinden bir intratrakeal balon g\u00f6nderilmesi- ni ve b\u00f6ylece ilave cerrahi uygulanmadan s\u00f6nd\u00fcrmeye imkan verilmesini gerektirir; ancak bu y\u00f6ntemin erken do\u011fum riski ta\u015f\u0131mas\u0131 ve do\u011fum sonras\u0131 tedavi sonu\u00e7- lar\u0131yla kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131\u011f\u0131nda neonatal s\u00fcrvide veya komp- likasyon oran\u0131nda bir avantaj sa\u011flamamas\u0131 kullan\u0131m\u0131n\u0131n k\u0131s\u0131tlanmas\u0131na sebep olmu\u015ftur (27). FETO Task Group do\u011fum \u00f6ncesi tan\u0131 konan ciddi izole KDH vakalar\u0131nda (&lt;25 haftadan k\u00fc\u00e7\u00fck olan) do\u011fum \u00f6ncesi giri\u015fimin tercihi \u00fczerine bir algoritma \u00f6nermi\u015flerdir: Buna g\u00f6re karaci\u011fer herniasyonu olan AKO &lt;1 vakalar FETO y\u00f6nteminden fayda g\u00f6r\u00fcrlerken \u2018iyi\u2019veya \u2018orta\u2019vakalar do\u011fum sonras\u0131 tedaviye y\u00f6n- lendirilmelidir. Bu yakla\u015f\u0131m\u0131n avantajlar\u0131 hala kan\u0131tlan- ma ihtiyac\u0131 g\u00f6stermektedir, \u00e7\u00fcnk\u00fc s\u00fcrviler randomize \u00e7al\u0131\u015fmalarda de\u011fil anl\u0131k kontrol vakalar\u0131nda kar\u015f\u0131la\u015ft\u0131- r\u0131lm\u0131\u015ft\u0131r (28, 29).<br \/>\nDO\u011eUM SONRASI G\u0130R\u0130\u015e\u0130MLER<br \/>\nKDH 1980\u2019li y\u0131llara kadar acil cerrahi gerektiren bir durum olarak g\u00f6r\u00fclmekteydi. Herniye olan bat\u0131n organlar\u0131n\u0131n hastal\u0131\u011f\u0131n esas patolojik \u00f6zelli\u011fi oldu\u011fu, akci\u011fere ve mediyastene bas\u0131 uygulayarak solunum- sal ve hemodinamik bozukluklara sebebiyet verdikleri d\u00fc\u015f\u00fcn\u00fclmekteydi (30). Bu fakt\u00f6rler aras\u0131nda strese ba\u011fl\u0131 pulmoner vazospazm geli\u015fmesi, herniye olan organlar\u0131n red\u00fcksiyonu sonras\u0131 bat\u0131n i\u00e7i bas\u0131nc\u0131n\u0131n artmas\u0131 ve anormal solunum mekanikleri say\u0131labi- lir. Tek merkezli bir retrospektif derlemede, kontrol vakalar\u0131na g\u00f6re s\u00fcrvide art\u0131\u015f g\u00f6sterildi\u011finden dolay\u0131 ge\u00e7 cerrahi uygulanmas\u0131n\u0131n do\u011frulu\u011funu g\u00f6stermi\u015ftir (31). Kesinle\u015ftirici prospektif veriler bulunmamas\u0131na<br \/>\nKONJEN\u0130TAL D\u0130YAFRAM HERN\u0130LER\u0130<br \/>\n282<br \/>\nra\u011fmen, y\u00fcksek hasta potansiyeli olan tersiyer mer- kezlerden gelen birikmi\u015f veriler, erken stabilizasyon sonras\u0131 ge\u00e7 cerrahi uygulanmas\u0131n\u0131n genel olarak kabul edilmesini sa\u011flam\u0131\u015ft\u0131r (28, 32). 2010 y\u0131l\u0131nda yay\u0131nlanan KDH EURO Konsorsiyum konsensusuna g\u00f6re do\u011fum sonras\u0131 tedavide en \u00f6nemli maddeler a\u015fa\u011f\u0131daki gibi \u00f6zetlenmi\u015ftir (33)<br \/>\nDo\u011fum odas\u0131ndaki Maskeyle solutma yasak tedavi Erken ent\u00fcbasyon Tepe bas\u0131nc\u0131 &lt; 25 cmH2O Nazogastrik t\u00fcp tak\u0131lmas\u0131 Yo\u011fun bak\u0131m Preduktal sat\u00fcrasyon %85-95 \u00fcnitesindeki tedavi aras\u0131 olacak \u015fekilde ventilasyonun sa\u011flanmas\u0131 pH &gt; 7.2, laktat 3-5 mmol\/L Konvansiyonel ventilasyon veya y\u00fcksek-frekansl\u0131 osilasyon Gestasyonel ya\u015fa g\u00f6re uygun kan bas\u0131nc\u0131n\u0131 sa\u011flayacak tedavi \u0130notropik destek Pulmoner hipertansiyon Ekokardiyografi tedavisi \u0130nhale nitrik oksit Fosfodiesteraz inhibit\u00f6rleri, endotelin antagonistleri, tirozin kinaz inhibit\u00f6rleri ECMO E\u011fer hasta pred\u00fcktal sat\u00fcrasyon de\u011feri olarak &gt; %85\u2019i yakalayabiliyorsa ba\u015flanmal\u0131 Pred\u00fcktal sat\u00fcrasyonun &gt; %85 seviyesinde tutulamamas\u0131 Respiratuar asidoz Yetersiz oksijen ta\u015f\u0131nmas\u0131 (laktat &gt; 5mmol\/L) Tedaviye diren\u00e7li hipertansiyon Cerrahi onar\u0131m FiO2 &lt; 0.5 Gestasyonel ya\u015fa g\u00f6re normal ortalama kan bas\u0131nc\u0131 \u0130drar \u00e7\u0131k\u0131\u015f\u0131 &gt; 2mL kg-1h-1 Kal\u0131c\u0131 pulmoner hipertansiyon bulgusu olmamas\u0131<br \/>\nKDH, geleneksel olarak herninin oldu\u011fu tarafa yap\u0131- lan bir subkostal insizyonla onar\u0131l\u0131r. Cerrahinin ama\u00e7lar\u0131 herni muhteviyat\u0131n\u0131 bat\u0131na geri g\u00f6ndermek, herni kese- sini kesmek ve diyafram defektini kapatmakt\u0131r. Kapatma tekni\u011fi defektin boyuna g\u00f6re de\u011fi\u015fir. K\u00fc\u00e7\u00fck defektler emilemeyen s\u00fct\u00fcrlerle primer kapat\u0131l\u0131rlar. B\u00f6ylece onar\u0131- lan b\u00f6lge hasta ile birlikte b\u00fcy\u00fcr. E\u011fer diyafram kenarlar\u0131 yetersizse s\u00fct\u00fcrler g\u00f6\u011f\u00fcs kafesine veya bat\u0131n duvar\u0131na sabitlenir. Rek\u00fcrens ihtimalini d\u00fc\u015f\u00fcrmek ve diyafram\u0131n normal hareketini sa\u011flamak i\u00e7in onar\u0131m\u0131n a\u015f\u0131r\u0131 gergin olmas\u0131ndan ka\u00e7\u0131n\u0131lmas\u0131 \u00f6nemlidir (32).<br \/>\nGeni\u015f defektler yama kullan\u0131m\u0131n\u0131 gerektirir. Bu ama\u00e7la \u00e7e\u015fitli t\u00fcrde prostetik materyeller ve dokular kullan\u0131lmaktad\u0131r (32). Yak\u0131n tarihli bir \u00e7al\u0131\u015fmada, yama kullan\u0131m\u0131yla kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131\u011f\u0131nda split bat\u0131n duvar\u0131 kas flebiyle geni\u015f bir KDH onar\u0131m\u0131n\u0131n daha d\u00fc\u015f\u00fck n\u00fcks ora- n\u0131yla ili\u015fkili oldu\u011fu g\u00f6sterilmi\u015ftir (34). Di\u011fer bir \u00e7al\u0131\u015fma ise yama ile onar\u0131m\u0131n daha y\u00fcksek bir uzun d\u00f6nem morbiditeyle ba\u011flant\u0131s\u0131 ortaya konmu\u015ftur. \u00d6zellikle ilk alt\u0131 ay i\u00e7inde b\u00fcy\u00fcme ve geli\u015fmenin daha yava\u015f oldu- \u011fu ve gastro\u00f6zofajiyal refl\u00fc oran\u0131n\u0131n artt\u0131\u011f\u0131 takiplerde g\u00f6zlenmi\u015ftir. Di\u011fer taraftan yama ile onar\u0131m ilk iki y\u0131lda g\u00f6\u011f\u00fcs duvar\u0131 deformitelerini etkilememektedir (35). Cerrahi sonras\u0131 komplikasyonlar enfeksiyon, yama- n\u0131n kom\u015fu dokular\u0131 a\u015f\u0131nd\u0131rmas\u0131, g\u00f6\u011f\u00fcs duvar\u0131 defor- mitesi, restriktif akci\u011fer hastal\u0131\u011f\u0131 ve n\u00fcks\u00fc kapsar. Primer onar\u0131m yap\u0131lanlar\u0131n yakla\u015f\u0131k %7\u2019sinde s\u0131kl\u0131kla ilk bir y\u0131l i\u00e7inde n\u00fcks g\u00f6r\u00fcl\u00fcr. Prostetik materyeller hasta ile birlikte b\u00fcy\u00fcyemediklerinden bu t\u00fcr bir ona- r\u0131m\u0131n zaman i\u00e7erisinde y\u00fcksek n\u00fcks ihtimali (%40\u2019a kadar) vard\u0131r (36). Yama kullan\u0131m\u0131n\u0131n y\u00fcksek n\u00fcks oran\u0131 ta\u015f\u0131mas\u0131 ve ilerleyici g\u00f6\u011f\u00fcs duvar\u0131 deformitesine sebep olabilmesi y\u00fcz\u00fcnden latissimus dorsi veya rektus abdominis kas flebi veya serbest fasya lata gibi otolog dokularla onar\u0131m yap\u0131lmas\u0131 \u00f6nerilmektedir (37, 38). Cerrahi sonras\u0131nda rutin toraks dreni kullan\u0131m\u0131, enfeksiyon riskinden ve intratorasik bas\u0131n\u00e7taki belir- gin dalgalanmalardan sak\u0131nmak i\u00e7in terk edilmi\u015ftir. Operasyonu takiben biriken plevral s\u0131v\u0131 akci\u011fer geni\u015f- ledik\u00e7e emilir. Solunumu etkileyecek miktardaki belir- gin s\u0131v\u0131lar torasentezle bo\u015falt\u0131l\u0131r. Geni\u015f bir KDH\u2019n\u0131n onar\u0131m\u0131 sonras\u0131nda bat\u0131n insizyonunun kapat\u0131lmas\u0131 se\u00e7enekleri aras\u0131nda sadece cildin kapat\u0131lmas\u0131, fasya kenarlar\u0131na dikilen d\u00f6\u015feyici bir yama kullan\u0131lmas\u0131 ve bir silo yarat\u0131lmas\u0131 say\u0131labilir (32). Minimal invaziv cerrahi a\u00e7\u0131s\u0131ndan bak\u0131l\u0131rsa, KDH onar\u0131m\u0131nda torakoskopi kullan\u0131labilir. Hemitoraksa d\u00fc\u015f\u00fck bas\u0131n\u00e7l\u0131 karbondioksit verilmesi bat\u0131n organla- r\u0131n\u0131n red\u00fcksiyonunu kolayla\u015ft\u0131rabilir, ancak bu durum operasyon esnas\u0131nda respiratuar asidozu artt\u0131rabilir. K\u00fc\u00e7\u00fck boyutlu hernisi olan ve ventilat\u00f6r ayarlar\u0131 d\u00fc\u015f\u00fck seviyede olan infantlar torakoskopik yakla\u015f\u0131m i\u00e7in tercih edilirler (32). A\u00e7\u0131k cerrahiyle kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131\u011f\u0131nda torakos- kopik cerrahinin hastane i\u00e7i n\u00fcks oran\u0131 daha y\u00fcksektir. N\u00fcksler torakoskopik olarak da onar\u0131labilirler (39).<br \/>\nPROGNOZ VE UZUN D\u00d6NEM SEKELLER<br \/>\nKDH\u2019l\u0131 \u00e7ocuklar\u0131n s\u00fcrvisi %50 ila 80 aras\u0131nda de\u011fi- \u015fir. Bu geni\u015f aral\u0131\u011f\u0131 a\u00e7\u0131klayan birka\u00e7 a\u00e7\u0131klama bulun- maktad\u0131r. Bunlar \u015fu \u015fekildedir: a) Hasta pop\u00fclasyonu, b) Patolojinin ciddiyetinin ger\u00e7ek durumu, mesela di\u011fer bir \u00f6l\u00fcmc\u00fcl malformasyonla birlikte g\u00f6r\u00fclen KDH\u2019da<br \/>\nKONJEN\u0130TAL D\u0130YAFRAM HERN\u0130LER\u0130<br \/>\n283<br \/>\noldu\u011fu gibi, c) KDH\u2019ye \u00f6zg\u00fc di\u011fer fakt\u00f6rler, mesela bilateral olmas\u0131 gibi ve d) Do\u011fum sonras\u0131 bak\u0131m (11). Her ne kadar %25 kadar\u0131 ileriki ya\u015fam d\u00f6nemle- rinde KDH ile ilgili semptomlar g\u00f6sterseler de, KDH\u2019l\u0131 bebeklerin \u00e7o\u011fu belirgin \u015fekilde normal akci\u011fer fonk- siyonlar\u0131yla ya\u015farlar. Gastro\u00f6zofajiyal refl\u00fc KDH\u2019n\u0131n b\u00fcy\u00fcme gerili\u011fine yol a\u00e7an s\u0131k g\u00f6r\u00fclen bir komplikas- yonudur. Refl\u00fc s\u0131kl\u0131kla yama ile onar\u0131m yap\u0131lanlarda g\u00f6r\u00fcl\u00fcr. Yama onar\u0131m\u0131n\u0131 takiben profilaktik fundopli- kasyon uygulanmas\u0131, b\u00fcy\u00fcme gerili\u011fi g\u00f6r\u00fclmeyen daha iyi bir s\u00fcrvi ile ili\u015fkilidir. Bu y\u00fczden diyafram onar\u0131m\u0131 ge\u00e7irecek stabil hastalarda profilaktik fundoplikasyon bir se\u00e7enektir, ama yine de bunun geni\u015f \u015fekilde kabul g\u00f6rmesi i\u00e7in ileri ara\u015ft\u0131rmalara ihtiya\u00e7 vard\u0131r (32, 40). Solunumsal \u015fikayetler primer akci\u011fer hipoplazisi, neonatal ventilasyonun sekelleri ve gastro\u00f6zofajiyal refl\u00fcn\u00fcn etkileriyle ili\u015fkilidir. Baz\u0131 \u00e7ocuklar, t\u0131pk\u0131 pre- mat\u00fcr infantlarda g\u00f6r\u00fcld\u00fc\u011f\u00fc gibi, kronik akci\u011fer has- tal\u0131\u011f\u0131 geli\u015ftirirler ve uzam\u0131\u015f oksijen tedavisine ihtiya\u00e7 duyarlar. Di\u011fer baz\u0131lar\u0131nda inspiratuar kas g\u00fcc\u00fcnde azalma ve belli derecede k\u00fc\u00e7\u00fck havayolu t\u0131kan\u0131kl\u0131\u011f\u0131 g\u00f6r\u00fcl\u00fcr. Son olarak, az bir k\u0131s\u0131m hastada uzun d\u00f6nem pulmoner vazodilat\u00f6r tedavi gerektiren rezid\u00fcel kronik pulmoner hipertansiyon geli\u015fir (11). KDH\u2019n\u0131n ve tedavisinin en s\u0131k ve potansiyel olarak rahats\u0131zl\u0131k verici sonucu n\u00f6rolojik geli\u015fim bozuklu\u011fu- dur. Bozuklu\u011fun ciddiyeti ve patofizyolojik problemler n\u00f6rolojik gerili\u011fin ciddiyetiyle alakal\u0131d\u0131r. KDH yap\u0131sal beyin anormallikleriyle ili\u015fkili olabilir (41). ECMO d\u0131\u015f\u0131 bir tedavi g\u00f6ren ve sonucunda ya\u015fayan KDH\u2019l\u0131larda n\u00f6rolojik geli\u015fim problemleri daha s\u0131k g\u00f6r\u00fcl\u00fcr (42). KDH\u2019l\u0131 hastalarda g\u00f6r\u00fclen di\u011fer uzun d\u00f6nem problem- ler pektus ekskavatum ve skolyozu kapsayan omurga ve g\u00f6\u011f\u00fcs duvar\u0131 deformiteleridir (11).<br \/>\n\u00c7OCUKLARDAK\u0130 D\u0130\u011eER NAD\u0130R D\u0130YAFRAM HERN\u0130LER\u0130<br \/>\nForamen Morgagni Hernisi Sternumun her iki yan\u0131nda, i\u00e7lerinden internal mam- mariyan arterlerin ge\u00e7ti\u011fi ve s\u00fcperior epigastrik arterler ad\u0131yla devam etti\u011fi, foramen Morgagni veya Larrey bo\u015flu\u011fu ad\u0131 verilen potansiyel birer bo\u015fluk bulun- maktad\u0131r. \u00dc\u00e7gen yap\u0131daki bu bo\u015fluk ksifoid ve kostal marjinden k\u00f6ken alan ve diyafram\u0131n santral tendonuna ba\u011flanan kas liflerinin aras\u0131nda kal\u0131r (43). Anterior kon- jenital diyafram hernisini ilk olarak Giovanni Battista Morgagni 1769 y\u0131l\u0131nda tariflemi\u015ftir. Bu herni diyafram\u0131n sternal ve kostal k\u0131s\u0131mlar\u0131n\u0131n birle\u015fmesinin yetersiz olmas\u0131ndan kaynaklan\u0131r. Sternokostal hiyatusun sol yan\u0131ndaysa Larrey hernisi, sa\u011f yan\u0131ndaysa Morgagni hernisi (MH) olarak adland\u0131r\u0131l\u0131r. E\u011fer a\u00e7\u0131kl\u0131k her iki taraf\u0131<br \/>\nkapsayacak kadar geni\u015fse buna da Morgagni-Larrey hernisi denir (44). MH cerrahi olarak d\u00fczeltilen t\u00fcm diyafram hernilerinin %1 ila 6\u2019s\u0131n\u0131 olu\u015fturur. Kalp ve sol yandaki perikardiyal ba\u011flant\u0131lar y\u00fcz\u00fcnden \u00e7o\u011funlukla (%90) sa\u011f taraftad\u0131r. Sol taraf %8 hastada etkilenirken %2 hastada herni bilateraldir. Vakalar\u0131n %95\u2019inde bir herni kesesi bulunur. Kesenin i\u00e7inde en s\u0131k bulunan organlar omentum ve\/veya transvers kolondur (45). Pediyatrik ya\u015f grubunda MH\u2019n\u0131n bulgular\u0131 de\u011fi\u015f- kendir. \u0130nfant ve k\u00fc\u00e7\u00fck \u00e7ocuklarda k\u00fc\u00e7\u00fck bir MH s\u0131kl\u0131kla asemptomatiktir ve tesad\u00fcfen saptan\u0131r. Daha geni\u015f olanlar\u0131n i\u00e7inde bat\u0131n organlar\u0131 bulundu\u011fundan solunum semptomlar\u0131 yaratabilirler. Klinik g\u00f6r\u00fcnt\u00fc Bochdalek hernisindekine benzerdir (43). Bochdalek hernisi ve genetik mutasyonlar aras\u0131nda baz\u0131 kan\u0131tlar bulunmu\u015f olsa da, MH i\u00e7in b\u00f6yle bir rapor bulunma- maktad\u0131r (46). MH\u2019nin di\u011fer ilgin\u00e7 bir \u00f6zelli\u011fi Down, Turner veya Noonan sendromlar\u0131, Cantrell pentalojisi, pektus deformiteleri, barsak malrotasyonu ve genito\u00fc- riner malformasyonlar gibi di\u011fer anomalilerle %34 ila 50 aras\u0131nda de\u011fi\u015fen oranlarda birlikte bulunabilmesi- dir (43). Geni\u015f serilerde MH ile Down sendromunun yakla\u015f\u0131k %20 oran\u0131nda birlikte g\u00f6r\u00fcld\u00fc\u011f\u00fc bildirilmi\u015ftir (47, 48). Toraks\u0131n radyolojik incelemeleri kalbin sa\u011f veya sol yan\u0131na kom\u015fu, solid veya hava i\u00e7eren bir yo\u011funluk art\u0131\u015f\u0131n\u0131 g\u00f6sterir. Toraks bilgisayarl\u0131 tomografisi tan\u0131ya gitmeye en fazla yard\u0131m eden y\u00f6ntemdir. Bazen ultra- son ve magnetik rezonans incelemesi de de\u011ferli ola- bilir. Kal\u0131n barsaklar\u0131n ve \u00fcst gastrointestinal traktusun kontrastl\u0131 incelemeleri baz\u0131 hastalar\u0131n incelenmesinde gerekli olabilir (49). Asemptomatik bir MH\u2019nin tedavisi hala tart\u0131\u015fmal\u0131d\u0131r. Buna kar\u015f\u0131l\u0131k, semptomatik hastalarda MH\u2019nin cerrahi olarak tedavi edilmesi genel bir g\u00f6r\u00fc\u015ft\u00fcr. Cerrahi ona- r\u0131m geleneksel olarak bat\u0131n veya toraks yoluyla a\u00e7\u0131k yakla\u015f\u0131mlarla veya yak\u0131n d\u00f6nemlerin g\u00f6r\u00fc\u015f\u00fc olarak minimal invaziv cerrahiyle yap\u0131labilir (48, 50). Toraks i\u00e7inden yakla\u015f\u0131ld\u0131\u011f\u0131nda herni kesesi kolayl\u0131kla bulunur ve \u00e7\u0131kart\u0131l\u0131r. Buna ek olarak, perikard, akci\u011ferler ve kese aras\u0131nda yap\u0131\u015f\u0131kl\u0131klar olmas\u0131 halinde torask i\u00e7i yakla\u015f\u0131m daha avantajl\u0131d\u0131r. Di\u011fer taraftan bu yakla- \u015f\u0131m\u0131n dezavantajlar\u0131 bilateral onar\u0131m\u0131n ve varsa bar- sak t\u0131kan\u0131kl\u0131\u011f\u0131n\u0131n tan\u0131s\u0131n\u0131n konmas\u0131n\u0131n zor olmas\u0131d\u0131r. Toraks i\u00e7ine yer de\u011fi\u015ftiren organlar\u0131n normal yerlerine g\u00f6nderilmeleri bat\u0131n yoluyla yakla\u015f\u0131ld\u0131\u011f\u0131nda kolayd\u0131r. \u00d6zellikle bilateral ve santral hernilerde ve e\u015flik eden bat\u0131n anomalilerinin varl\u0131\u011f\u0131nda bat\u0131n yoluyla yakla\u015f\u0131m kullan\u0131lmal\u0131d\u0131r (50). Minimal invaziv cerrahide son y\u0131llarda g\u00f6r\u00fclen ilerlemeler, \u00e7ocuklarda MH\u2019nin laparaskopik onar\u0131m\u0131- n\u0131 g\u00fcvenli ve etkili k\u0131lm\u0131\u015ft\u0131r. Baz\u0131 raporlara g\u00f6re MH<br \/>\nKONJEN\u0130TAL D\u0130YAFRAM HERN\u0130LER\u0130<br \/>\n284<br \/>\nlaparaskopiyle ba\u015far\u0131yla tedavi edilmektedir (51, 52). Laparaskopik onar\u0131mla ilgili olarak literat\u00fcrde, arala- r\u0131nda kontin\u00fc s\u00fct\u00fcrlerle primer onar\u0131m, separe s\u00fct\u00fcr onar\u0131m\u0131 veya yama kullan\u0131lmas\u0131n\u0131n bulundu\u011fu \u00e7e\u015fitli teknikler bildirilmi\u015ftir (53). Tart\u0131\u015fmal\u0131 olan konu herni kesesinin \u00e7\u0131kar\u0131l\u0131p \u00e7\u0131kar\u0131lmamas\u0131d\u0131r. Baz\u0131 cerrahlar defektin fasyal s\u0131n\u0131rlar\u0131n\u0131 belirlemek i\u00e7in keseyi \u00e7\u0131kar\u0131r- larken (51), di\u011ferleri perikard ve plevrada perforasyon riski olmas\u0131 y\u00fcz\u00fcnden bunu \u00f6nermemektedirler (54). Herni kesesinin plikasyon yap\u0131larak yerinde b\u0131rak\u0131lma- s\u0131n\u0131n k\u00f6t\u00fc bir etki yaratmad\u0131\u011f\u0131 bildirilmi\u015ftir (44, 45, 55). Robot yard\u0131ml\u0131 laparaskopik MH onar\u0131m\u0131n\u0131n \u00e7ocuk- larda uygun ve g\u00fcvenilir oldu\u011fu bildirilmi\u015ftir, ancak kurulum s\u00fcresi i\u015flemi uzatmakta ve operasyon s\u00fcresi laparaskopik onar\u0131mla kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131\u011f\u0131nda daha uzun s\u00fcrmektedir (56).<br \/>\n\u0130NTRAPER\u0130KARD\u0130YAL D\u0130YAFRAM HERN\u0130S\u0130<br \/>\n\u0130ntraperikardiyal diyafram hernisi bat\u0131n organlar\u0131n\u0131n perikard i\u00e7ine f\u0131t\u0131kla\u015ft\u0131\u011f\u0131 \u00e7ok nadir bir diyafram hernisi t\u00fcr\u00fcd\u00fcr. Peritoneoperikardiyal herni olarak da adland\u0131- r\u0131l\u0131r. Bu herni t\u00fcr\u00fc, \u00e7ocuklarda b\u00fcy\u00fck oranda konjenital, yeti\u015fkinlerde ise s\u0131kl\u0131kla travmatiktir. \u0130ngiliz literat\u00fcr\u00fcn- de sadece 15 benzer vakan\u0131n varl\u0131\u011f\u0131 bildirilmi\u015ftir (57). Diyafram geli\u015fimi esnas\u0131nda septum transversumun pars sternalis k\u0131sm\u0131n\u0131n birle\u015fmesinin yetersiz olmas\u0131na ba\u011fl\u0131 geli\u015fti\u011fi d\u00fc\u015f\u00fcn\u00fclmektedir (58). Klinik sunum de\u011fi\u015fkenlik g\u00f6sterir. Herni, do\u011fum \u00f6ncesi d\u00f6nemde kendini masif perikardiyal ef\u00fczyon veya perikardiyal kitle \u015feklinde g\u00f6sterebilir. Etkilenen \u00e7o\u011fu yenido\u011fan do\u011fum sonras\u0131 hemen erken d\u00f6nem- de pulmoner hipoplaziye veya masif plevral ef\u00fczyona ba\u011fl\u0131 geli\u015fen ciddi solunum s\u0131k\u0131nt\u0131s\u0131 ve siyanoz bulgu- lar\u0131 verirler (59, 60). Cerrahi onar\u0131m bat\u0131n veya toraks yoluyla yap\u0131l\u0131r. Bat\u0131ndan yakla\u015fmak s\u0131kl\u0131kla tercih edilir ve minimal inva- ziv tekniklerle onar\u0131m ger\u00e7ekle\u015ftirilebilir. Defekt primer s\u00fct\u00fcre edilebilir. Ancak primer kapat\u0131lamayacak kadar b\u00fcy\u00fck bir defekt varl\u0131\u011f\u0131nda m\u00fcsk\u00fclofasyal flep kullan\u0131- m\u0131 \u00f6nerilir. \u0130ntraperikardiyal diyafram hernisi tamirinde prostetik yamalar\u0131n kullan\u0131m\u0131 hakk\u0131nda bir rapor hen\u00fcz yay\u0131nlanmam\u0131\u015ft\u0131r, ancak konvansiyonel y\u00f6ntemler ba\u015fa- r\u0131 sa\u011flamazsa bu y\u00f6ntem denenebilir (57).<br \/>\nKAYNAKLAR<br \/>\n1. Grisaru-Granosvky S, Rabinowitz R, Ioscovich A, et al. Congenital diaphragmatic hernia: review of the literature in reflection of unresolved dilemmas. Acta Pediatrica 2009;98:1874-81. [CrossRef] 2. Golombek SG. The history of congenital diaphragmatic hernia from 1850s to the present. J Perinatol 2002;22:242-6. [CrossRef]<br \/>\n3. Irish MS, Holm BA, Glick PL. Congenital diaphragmatic hernia: a historical review. Clin Perinatol 1996;23:625-53. 4. Puri P, Wester T. Historical aspects of congenital diaphragmtic hernia. Pediatr Surg Int 1997;12:95-100. [CrossRef] 5. Iritani I. Experimental study on embryogenesis of conge- nital diaphragmatic hernia. Anat Embryol 1984;169:133-9. [CrossRef] 6. Allan DW, Greer JJ. Pathogenesis of nitrofen-induced congenital diaphragmatic hernia in fetal rats. J Appl Physiol 1997;83:338-47. 7. Major D, Cadenas M, Fournier L, et al. Retinol status of newborn infants with congenital diaphragmatic hernia. Pediatr Surg Int 1998;13:547-9. [CrossRef] 8. Pober BR. Genetic aspects of human congenital diaph- ragmatic hernia. Clin Genet 2008;74:1-15. [CrossRef] 9. Waag KL, Loff S, Zahn K, et al. Congenital diaphragma- tic hernia: a modern day approch. Semin Pediatr Surg 2008;17:244-54. [CrossRef] 10. Keijzer R, Liu J, Deimling J, et al. Dual-hit hypoplasia in the nitrofen model of congenital diaphragmatic hernia. Am J Pathology 2000;156:1299-306. [CrossRef] 11. Corbett HJ, Losty PD. Congenital diaphragmatic hernia. In Parikh DH, Crabbe DCG, Auldist AW, Rothenberg SS (eds): Pediatric thoracic surgery. Springer-Verlag London 2009, pp. 483-99. [CrossRef] 12. Deprest JA, Gratacos E, Nicolaides K, et al. Changing perspectives on the perinatal management of isolated congenital diaphragmatic hernia in Europe. Clin Perinatol 2009;36:329-47. [CrossRef] 13. Kline-Fath BM. Congenital diaphragmatic hernia. Pediatr Radiol 2012;42 Suppl 1:S74-90. [CrossRef] 14. Stoll C, Alembik Y, Dott B, Roth MP. Associated malfor- mations in cases with congenital diaphragmatic hernia. Genet Couns 2008;19:331-9. 15. Puri P, Gorman F. Lethal nonpulmonary anomalies asso- ciated with congenital diaphragmatic hernia: implications for early intrauterine surgery. J Pediatr Surg 1984;19:29- 32. [CrossRef] 16. Kantarc\u0131 S, Ackerman KG, Russell MK, et al. Characterization of the chromosome 1q41q42.12 region, and the Candidate gene DISP1, in patients with CDH. Am J Med Genet 2010;152A:2493-504. [CrossRef] 17. Graziano JN. Cardiac anomalies in patients with conge- nital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 2005;40:1045-50. [CrossRef] 18. Garne E, Haeusler M, Barisic I, et al. Congenital diaph- ragmatic hernia: evaluation of prenatal diagnosis in 20 European regions. Ultrasound Obstet Gynecol 2002;19:329-33. [CrossRef] 19. Gallot D, Coste K, Francannet C, et al. Antenatal detec- tion and impact on outcome of congenital diaphragmatic hernia: a 12-year experience in Auvergne, France. Eur J Obstet Gynecol Reprod Biol 2006;125:202-5. [CrossRef] 20. Claus F, Sandaite I, Dekoninck P, et al. Prenatal anato- mical imaging in fetuses with congenital diaphragmatic hernia. Fetal Diagn Therapy 2011;29:88-100. [CrossRef] 21. Coackley FV, Glenn OA, Qayyum A, et al. Fetal MRI: a developing technique fort he developing patient. AJR Am J Roentgenol 2004;182:243-52. 22. Muzzafar S, Swischuk LE, Jadhav SP. Radiographic fin- dings in late-presenting congenital diaphragmatic hernia: helpful imaging findings. Pediatr Radiol 2012;42:337-42. [CrossRef]<br \/>\nKONJEN\u0130TAL D\u0130YAFRAM HERN\u0130LER\u0130<br \/>\n285<br \/>\n23. Deprest J, Jani J, Cannie M, et al. Prenatal intervention for isolated congenital diaphragmatic hernia. Curr Opin Obstet Gynecol 2006;18:355-67. [CrossRef] 24. Cannie M, Jani JC, De Keyzer F, et al. Fetal body volume: use at MR imaging to quantify relative lung volume in fetu- ses suspected of having pulmonary hypoplasia. Radiology 2006;241:847-53. [CrossRef] 25. Takahashi S, Oishi Y, Ito N, et al. Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices. J Ped Surg 2009;44:2101-6. [CrossRef] 26. Grethel EJ, Nobuhara KK. Fetal surgery for congenital diaph- ragmatic hernia. J Paediatr Child Health 2006;42:79-85. [CrossRef] 27. Harrison MR, Keller RL, Hawgood SB, et al. A randomi- zed trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. N Engl J Med 2003;349:1916-24. [CrossRef] 28. Deprest J, De Coppi P. Antenatal management of isolated congenital diaphragmatic hernia today and tomorrow: ongoing collaborative research and development. J Ped Surg 2012;47:282-90. [CrossRef] 29. DeKoninck P, Gratacos E, Van Mieghem T, et al. Results of fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia and the set up of the randomized controlled TOTAL trial. Early Hum Dev 2011;87:619-24. [CrossRef] 30. Ladd WE, Gross RE. Congenital diaphragmatic hernia. N Enj J Med 1940;223:917-25. [CrossRef] 31. Breaux Jr CW, Rouse TM, Cain WS, Georgeson KE. Improvement in survival of patients with congenital diaph- ragmatic hernia utilizing a strategy of delayed repair after medical and\/or extracorporeal membrane oxygenation stabilization. J Pediatr Surg 1991;26:333-6. [CrossRef] 32. Kholdebarin R, Iwasiow BM, Keijzer R. Pulmonary development considerations in the surgical manage- ment of congenital diaphragmatic hernia. Early Hum Dev 2011;87:755-8. [CrossRef] 33. Reiss I, Schaible T, van den Hout L, et al. Standardized postnatal management of infants with congenital diaph- ragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology 2010;98:354-64. [CrossRef] 34. Barnhart DC, Jacques E, Scaife ER, et al. Split abdominal wall muscle flap repair vs patch repair of large congeni- tal diaphragmatic hernias. J Pediatr Surg 2012;47:81-6. [CrossRef] 35. Valfre L, Braguglia A, Confrotti A, et al. Long term follow- up in high-risk congenital diaphragmatic hernia survivors: patching the diaphragm affects the outcome. J Pediatr Surg 2011;46:52-6. [CrossRef] 36. Moss RL, Chen CM, Harisson MR. Prosthetic patch durability in congenital diaphragmatic hernia: a long- term follow-up study. J Pediatr Surg 2001;36:152-4. [CrossRef] 37. Masumoto K, Nagata K, Souzaki R, et al. Effectiveness of diaphragmatic repair using an abdominal muscle flap in patients with recurrent congenital diaphragmatic hernia. J Pediatr Surg 2007;42:2007-11. [CrossRef] 38. Sugiyama A, Fukumoto K, Fukuzawa H, et al. Free fascia lata repair for a second recurrent congenital diaphragma- tic hernia. J Pediatr Surg 2011;46:1838-41. [CrossRef] 39. Tsao K, Lally PA, Lally KP. Minimally invasive repa- ir of congenital diaphragmatic hernia. J Pediatr Surg 2011;46:1158-64. [CrossRef] 40. Di Pace MR, Caruso AM, Farina F, et al. Evaluation of esophageal motility and reflux in children treated for congenital diaphragmatic hernia with use of combined multichannel intraluminal impedance and pH monitoring. J Pediatr Surg 2011;46:1881-6. [CrossRef]<br \/>\n41. Danzer E, Hedrick HL. Neurodevelopmental and neuro- functional outcomes in children with congenital diaphrag- matic hernia. Early Hum Dev 2011;87:625-32. [CrossRef] 42. Frisk V, Jakobson LS, Unger S, et al. Long-term neurode- velopmental outcomes of congenital diaphragmatic hernia survivors not treated with extracorporeal membrane oxy- genation. J Pediatr Surg 2011;46:1309-18. [CrossRef] 43. Pokorney WJ, McGill CW, Herberg FJ. Morgagni hernia during infancy: presentation and associated anomalies. J Pediatr Surg 1984;19:394-7. [CrossRef] 44. Mallick MS, Alqahtani A. Laparascopic-assisted repair of Morgagni hernia in children. J Pediatr Surg 2009;44:1621-4. [CrossRef] 45. Akb\u0131y\u0131k F, Tiryaki TH, \u015eenel E, et al. Is hernial sac removal necessary? Retrospective evaluation of eight patients with Morgagni hernia in 5 years. Pediatr Surg Int 2006;22:825-7. [CrossRef] 46. Slavotinec AM. The genetics of congenital diaphragmatic hernia. Semin Perinatol 2005;29:77-85. [CrossRef] 47. \u00c7i\u011fdem MK, \u00d6nen A, Okur H, Ot\u00e7u S. Associated malformations in Morgagni hernia. Pediatr Surg Int 2007;23:1101-3. [CrossRef] 48. Al-Salem AH, Nawaz A, Matta H, Jacobs A. Herniation through the foramen of Morgagni: early diagnosis and treatment. Pediatr Surg Int 2002;18:93-7. [CrossRef] 49. Zaleska-Dorobisz U, Bag\u0142aj M, Soko\u0142owska B, et al. Late presenting diaphragmatic hernia: clinical and diagnostic aspects. Med Sci Monit 2007;13(Suppl 1):137-46. 50. K\u0131l\u0131\u00e7 D, Nadir A, D\u00f6ner E, et al. Transthoracic appro- ach in surgical management of Morgagni hernia. Eur J Cardiothorac Surg 2001;20:1016-9. [CrossRef] 51. Arca MJ, Barnhart DC, Lelli JL, et al. Early experience with minimally invasive repair of congenital diaphragma- tic hernias: results and lessons learned. J Pediatr Surg 2003;38:1563-8. [CrossRef] 52. Shah SR, Wishnew J, Barsness K, et al. Minimally invasive congenital diaphragmatic hernia repair: a 7-year review of one institution\u2019s experience. Surg Endosc 2009;23:1265-71. [CrossRef] 53. Dutta S, Albanese CT. Use of a prosthetic patch for laparoscopic repair of Morgagni diaphragmatic hernia in children. J Laparoendosc Adv Surg Tech A 2007;17:391-4. [CrossRef] 54. De Vogelaere K. Laparoscopic repair of Morgagni diaph- ragmatic hernia. Surg Laparosc Endosc Percutan Tech 2003;13:401-3. [CrossRef] 55. Alqahtani A, Al-Salem AH. Laparascopic-assisted versus open repair of Morgagni hernia in infants and children. Surg Laparosc Endosc Percutan Tech 2011;21:46-9. [CrossRef] 56. Anderberg M, Kockum CC, Arnbjornsson E. Morgagni hernia repair in a small child using da Vinci robotic instruments-a case report. Eur J Pediatr Surg 2009;19:110-2. [CrossRef] 57. Jain V, Choudhury SR, Chadha R, Puri A. Intrapericardial diaphragmatic hernia: a rare type of congenital diaphrag- matic hernia. J Pediatr Surg 2011;46:E29-E31. [CrossRef] 58. Milne LW, Morosin AM, Campbell JR, Harrison MW. Pars sternalis diaphragmatic hernia with omphalocele: a report of two cases. J Pediatr Surg 1990;25:726-730. [CrossRef] 59. Davies MR, Oksenberg T, Da Fonseca JM. Massive foetal pericardiomegaly causing pulmonary hypoplasia, asso- ciated with intra-pericardial herniation of the liver. Eur J Pediatr Surg 1993;3:343-7. [CrossRef] 60. Hara K, Kikuchi A, Takagi K, et al. Massive pericardial effusion in an early gestational fetus having intrapericardial diaphragmatic hernia. J Obstet Gynaecol Res 2007;33:561-5.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>D\u0130AFRAGMA HERN\u0130S\u0130 Diafragmadaki bir defekt nedeniyle kar\u0131n i\u00e7i organlar\u0131n toraks i\u00e7inde yeralmas\u0131d\u0131r. \u0130nsidans: 4000 canl\u0131 do\u011fumda bir g\u00f6r\u00fcl\u00fcr . Ancak \u00f6l\u00fc do\u011fumlarda dikkate al\u0131nd\u0131\u011f\u0131nda bu oran 1\/2000\u2032 dir. Genellikle sporadik olmakla birlikte; ikiszlerde, ayn\u0131 ailenin 2. Jenerasyonunda yay\u0131nlanm\u0131\u015f vakalar mevcuttur. Serilerin \u00e7o\u011funda herni %85-90 sol tarafta yer al\u0131r. \u0130ki tarafl\u0131 diafragma hernisi \u00e7ok nadir g\u00f6r\u00fcl\u00fcr. [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":8524,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[11],"tags":[],"class_list":["post-6136","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-sunnet"],"blocksy_meta":[],"_links":{"self":[{"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/posts\/6136","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/comments?post=6136"}],"version-history":[{"count":0,"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/posts\/6136\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/"}],"wp:attachment":[{"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/media?parent=6136"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/categories?post=6136"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.narkozsuzsunnet.com\/index.php\/wp-json\/wp\/v2\/tags?post=6136"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}