RESUMO
UNLABELLED: Bronchogenic cyst of the mediastinum, a cause of stridor early in life, is the result of abnormal budding of the ventral segment of the primitive foregut. Bronchogenic cysts are often asymptomatic in older children and adults. However, symptomatic cases usually manifest early in life with cough, stridor or wheezing due to airway compression. We report a female infant aged 4.5 months with a normal full-term pregnancy, who developed respiratory distress with stridor. This stridor was preceded by a history of slowly progressive noisy breathing. Physical examination revealed evidence of bilateral obstructive emphysema. Chest radiograph revealed bilateral overinflation. Fibro-optic bronchoscopy revealed posterior mediastinal compression. Possibility of congenital cystic lung disease (CCLD) was considered, emphasizing the value of computed tomography (CT) chest, which revealed a cyst probably bronchogenic. Surgical excision was performed with evident histological confirmation of bronchogenic cyst. CONCLUSION: we highlight that in any infant, presented with slowly progressive noisy breathing in the first year of life, CCLD should be considered in the differential diagnosis even with normal X-ray chest. CT chest should be performed for exclusion or diagnosis of the case.
Assuntos
Cisto Broncogênico/diagnóstico , Enfisema Pulmonar/diagnóstico , Sons Respiratórios/etiologia , Obstrução das Vias Respiratórias/etiologia , Cisto Broncogênico/complicações , Broncoscopia , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Enfisema Pulmonar/complicações , Insuficiência Respiratória/etiologia , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Aortic insufficiency (AI) associated with ventricular septal defect (VSD) is often repaired using a modification of Trusler's aortic valvuloplasty technique. The purpose of this study was to identify the risk factors for failure of aortic valve (AV) repair in patients who underwent repair of associated VSD. METHODS: A univariate analysis was performed in this retrospective study to identify the possible risk factors for failure of the repair resulting in the need for AV replacement (AVR). RESULTS: The study included 24 patients, 15 (62%) boys and 9 (38%) girls, with a mean age of 9.1 +/- 1.2 (SEM) years. The VSD was perimembranous in 15 (62%) and subarterial in 9 (38%). The prolapsed aortic cusp was the right in 13 (54%), the noncoronary in 6 (25%), and both in 5 (21%). Plication was performed at one end of the free edge of the prolapsed cusp(s) in 12 (50%) and at more than one end in 12 (50%) of the patients. The VSD was closed by use of a patch in 21 (88%) and by direct suture closure in 3 (12%). At the mean follow up of 7.3 +/- 1.3 years, the degree of AI was none in 6 (25%), trivial in 5 (21%), mild in 9 (38%), moderate in 1 (4%), and severe in 3 (12%). The 15-year actuarial freedom from reoperation was 81% +/- 19% (95% confidence limit). By univariate analysis, the possible risk factors for AV repair failure were the degree of AI at hospital discharge (p = 0.004), direct closure of the VSD (p = 0.061), smaller size of the VSD (p = 0.081), and plication of more than one end of the prolapsed cusp(s) (p = 0.095). CONCLUSIONS: Trusler's AV repair is an effective and durable technique for the surgical treatment of patients with VSD-AI syndrome. The adequacy of the initial repair is the most important determinant of the long-term results.