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A5614 - A Rare Intralobar Pulmonary Sequestration of the Right Upper Lobe in an Infant: Case Report
Author Block: C. L. Grizales1, M. L. Perez2, L. Fernandez3, C. S. Leib4, B; 1Pediatric Pulmonology, Fundacion Valle del Lili, Universidad Icesi, Cali, Colombia, 2Pediatric Resident, Fundacion Valle del Lili, Universidad Icesi, Cali, Colombia, 3Interventional Pulmonology, Fundacion Valle del Lili, Universidad Icesi, Cali, Colombia, 4Medical Research, Fundacion Valle del Lili, Universidad Icesi, Cali, Colombia.
INTRODUCTION Pulmonary sequestration is a rare congenital anomaly consisting of ectopic nonfunctional lung tissue with their own systemic arterial blood supply and without communication with the tracheobronchial tree. Are divided in two types on the basis of their pleural covering, intralobar sequestration (ILS) located within the visceral pleura and surrounded by normal lung, and extralobar sequestration which have a separate pleural covering. Most of intralobar sequestrations were located in lower lobes. Upper lobe intralobar sequestrations are very rare. CASE REPORT A 7-month-old female patient with a history of Down Syndrome and complete atrioventricular septal defect with pulmonary artery cerclage, who presented with multiorgan dysfunction secondary to pneumonia. Chest x-ray shows alveolar-type opacities that erase right heart silhouette with signs of volume loss, which have persisted over time. She is taken to bronchoscopy where bronchial agenesis of the right upper lobe is found. Then a thorax angiography is performed, which shows pulmonary sequestration of the right upper lobe, whereby is carried to cardiac catheterization where the right superior lobar nutritional artery is embolized. A control chest CT scan show a pulmonary infarction, nevertheless there was not abscess formation, whence continues to expectant management, with satisfactory evolution so it does not require lobectomy. DISCUSSION Pulmonary sequestration is the second most common congenital lung anomaly. Intralobar pulmonary sequestration is a rare disease, with an incidence rate of 0.15-1.7% among all congenital lung diseases and is characteristically found in the lower left lobe. There are symptoms such as recurrent chest infection, respiratory distress or congestive cardiac failure without an obvious cardiac cause. In the chest-x-ray the parenchymal changes are variable but persistent. CT angiography is an effective means of diagnosing both forms of pulmonary sequestration as in most cases it can demonstrate the origin and course of the feeding artery. Multiplanar reconstructions are useful to help delineate the pattern of venous drainage which helps define the lesion. Conventional treatment for both forms of pulmonary sequestration is surgical resection, but there is an evidence to support the use of transcatheter arterial coil embolization as an effective definitive treatment option for extralobar sequestration with several case reports and case series in the paediatric literature describing good long term clinical outcomes, there are few reports of coil embolization in intralobar sequestration as a definitive treatment option.