Clinical analysis of 234 cases with congenital malformations of respiratory system / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore clinical characteristics, radiographic findings and diagnostic methods of patients with congenital malformations of respiratory system for enhancing the diagnosis of congenital malformations of respiratory system in children.</p><p><b>METHOD</b>Totally 234 patients with congenital malformations of respiratory system were chosen from the inpatient department of Yuying Children's Hospital Affiliated to Wenzhou Medical College from July 2003 to June 2008. The clinical presentations and radiographic findings of these children were analyzed.</p><p><b>RESULT</b>Of the 234 patients with congenital malformations of respiratory system, the age at diagnosis was between the first day and 14 years of age, mean age was 1.12 years. The main symptoms were persistent laryngeal stridor, recurrent wheezing, recurrent respiratory tract infections and dyspnea. Through the use of chest X-ray, spiral CT 3D reconstructions, fiberoptic bronchoscopy and other laboratory techniques, 213 cases were diagnosed as having single malformation and 21 cases were found to have multiple malformations. Of the 213 cases with single malformation, 97 cases had laryngeal malformation (congenital laryngeal stridor in 90 cases, congenital laryngeal webs in 5 cases and congenital laryngeal cyst in 2 cases), 35 cases had tracheal-bronchial malformation (congenital tracheobronchial stenosis in 17 cases, congenital abnormal bronchial origin in 7 cases, tracheobronchomalacia in 10 cases and tracheoesophageal fistula in 1 case), 43 cases had lung malformation (pulmonary sequestration in 5 cases, congenital lung cysts in 22 cases, congenital lobar emphysema in 1 case, agenesis of lung and hypoplasia of lung in 8 cases and congenital cystic adenomatoid malformation in 7 cases), 38 cases had diaphragm malformation, 28 cases had congenital tracheal-bronchial stenosis as confirmed by spiral CT 3D reconstructions and fiberoptic bronchoscopy. Ten cases with congenital abnormal bronchial origin were diagnosed with spiral CT 3D reconstructions. Laryngeal stridor and tracheobronchomalacia were diagnosed by fiberoptic laryngoscope and fiberoptic bronchoscopy. The accuracy rates of preoperative diagnosis through clinical and radiographic examinations of 37 cases with lung malformation and 36 cases with diaphragm malformation were 83.78% and 91.67%.</p><p><b>CONCLUSION</b>Congenital malformations of respiratory system are a group of diseases that are important for pediatric respiratory clinicians. Congenital malformations of respiratory system should be considered in children with persistent laryngeal stridor, recurrent wheezing, recurrent respiratory tract infections and dyspnea. The radiographic examination and respiratory endoscope play important roles in the diagnosis of congenital malformations of respiratory system.</p>

Tuberous sclerosis complex:skeletal CT findings / 中华放射学杂志

Objective To describe the skeletal CT imaging manifestations in patients with tuberous sclerosis complex(TSC),and to analyze their diagnostic value so as to establish an adequate skeletal change imaging data for the diagnosis of TSC.Methods Thirteen patients fulfilling TSC diagnostic criteria were examined with CT of the brain (n=13)and abdomen (n=7).Examinations from January,2004 to July, 2006 were retrospectively analyzed.Results There were three forms of lesions being demonstrated on CT: (1)Multiple sclerosing nodule (n=13):numerous,ovoid and circular,homogeneous,small and well- defined loci and symmetrical lesions were revealed in all cases in the central marrow portion of the bones, which could mimic blastic metastases.Follow-up CT imaging showed no change in both size and number. The lesions measured approximately 2-10mm.(2)Local sclerosing bone dysplasia with little bone expansion (n=7).Symmetrical and irregular density in the radix of the posterior arch of the vertebral body (n = 5 ).(3 )The spherical periosteal proliferation demonstrating as a cortex double line sign (n=2 ),and cortical thickening of metatarsals (n = 3 ).The appearance of the skeletal manifestation was as that in adulthood.Conclusion CT imaging of the skeletal system in TSC has some characteristics,by which the diagnosis of TSC could be made if combined with other main clinical diagnostic criteria. We suggest that those particular findings can be added as primary diagnostic features in the clinical diagnosis of TSC.