The Clinical Analysis Of Etiology Of25Cases Of Multiple Cystic Lung Disease In Children

Objective： To explore the etiology, clinical and chest image features,and the diagnosis methods of Multiple cystic lung disease (MCLD) inchildren, so as to improve the awareness of MCLD and the level ofdiagnosis.Methods：Retrospective analysis of the clinical data of25cases ofMCLD in Children’s Hospital Affiliated to Chongqing University ofMedical Sciences from March2004to December2012. analysis andsummary the etiology, the clinical manifestation, the characteristics of chestimage, pulmonary function test, pathological examination.Results：(1) in the25cases, definite causes were made in15cases(60%of the whole group),7cases only had suspected diagnosis (28%),3case (12%) of unknown reason.(2) The causes of MCLD inchildren were:12cases (48%) with Bronchopulmonary dysplasia (BPD),7cases (28%) with Langerhans cell histiocytosis (LCH), including4suspected cases (16%),3cases (12%) with suspected idiopathic pulmonaryfibrosis, and3case of unknown reason.(3)24(96%) out of25cases had the clinical manifestations of respiratory system:22cases (88%) had tachypnea,16cases (64%) had cough,14cases(56%) had sputum,15cases (60%) haddyspnea,17cases (68%) had three concave sign,14cases (56%) had moistrales, Extrapulmonary manifestations were various and specific:3cases ofabdominal distension (12%), ear pus overflow in3cases (12%), head massin2cases (8%),1cases of pneumothorax,6patients with anemia(24%),lymph nodes in5cases (20%),7(28%) cases withhepatosplenomegaly,3cases of skin rash (12%),2cases of jaundice(8%),clubbing in1cases(4%).(4)18cases had chest X-ray,only3casessuggest the multiple cystic lesions,25cases all had Computedtomography(CT) or High-resolution computed tomography(HRCT).thecystic lesions of different diseases varied from the distribution, size, cysticwall and accompanying CT abnormalities.(5)3cases of suspected IPF hadpulmonary function tests, the results suggestive restrictive ventilatorydefect.(6) only3cases had pathological data, most patients can not beimplemented or reject lung biopsy, that lead some disease had unknowncause.Conclusion：1. The causes for children with MCLD in this study were BPD, LCH,IPF.2. The common clinical manifestations of MCLD in children wasrespiratory system, and were unspecific, but extra-pulmonary manifestations were various and specific, they were important value indiagnosis of MCLD.3. The Chest X-ray had less sensitive to the cystic lesions than CT, theCT or HRCT can different features in cystic airspaces.4. The diagnosis of MCLD should combine with the history,extrapulmonary manifestations, pulmonary function tests and chestimage.chest CT or HRCT is an important approach in the diagnosis anddifferential diagnosis of cystic lung disease, By analyze and summarize thefeatures of cystic lesions, it could provide the basis for diagnosis.5. Histopathological examination is a diagnosis tool of some disease,if the disease could not diagnosis through the clinical manifestations andimage features, the pathological biopsy should be performed.