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The Journal of Cardiovascular Surgery 2000 June;41(3):507-10

Copyright © 2009 EDIZIONI MINERVA MEDICA

language: English

Congenital malformation of the lung

Çay A., Sarihan H.

From the Department of Pediatric Surgery, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey

Con­gen­ital mal­for­ma­tions of the ­lung are ­rare and ­vary ­widely in ­their pres­en­ta­tion and ­severity. How­ever, fre­quently ­they ­present ­with dra­matic symp­toms ­resulting in the ­need for ­prompt diag­nosis and treat­ment. ­Five ­patients ­with con­gen­ital ­lung mal­for­ma­tions ­were ­treated in Karad­eniz Tech­nical Uni­ver­sity ­Farabi Med­ical ­School Pedi­atric Sur­gery Depart­ment ­between ­June 1992 and Jan­uary 1997. ­There ­were one ­girl and ­four ­boys. The ­mean age was 42 ­days (­range 1 to 120 ­days) and the ­mean ­weight was 3.9 kg (­range 2.4 to 5.4 kg). The fol­lowing ­cases ­were ­included; one pul­mo­nary agen­esis, two con­gen­ital ­lobar emphy­sema, one pul­mo­nary seques­tra­tion, and one ­cystic ade­nom­a­toid mal­for­ma­tion. Res­pir­a­tory dis­tress was the ­most ­common pre­senting ­symptom. The diag­nosis was per­formed by ­chest X-ray ­with pos­te­ro-an­terior and lat­eral ­view and tho­racic com­puted tomog­raphy (CT). Asso­ciated con­gen­ital anom­a­lies ­were ­detected in one ­patient and the fol­lowing anom­a­lies ­were ­found; esoph­a­geal ­atresia, tracheo-e­soph­a­geal fis­tula, ­short ­distal esoph­agus, and ­small intra­tho­racic ­stomach. Pos­te­ro­lat­eral thor­a­cotomy was ­used for all ­patients and com­plete sur­gical resec­tion was per­formed in ­four ­patients. Cor­rec­tive sur­gery was ­delayed in one ­patient in ­whom ­extended res­pir­a­tory sup­port was ­required and he ­died on the ­ninth day due to res­pir­a­tory ­failure. In ­four ­patients prog­nosis was excel­lent. In ­this ­article, diag­nosis and treat­ment of con­gen­ital ­lung mal­for­ma­tions are dis­cussed.