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Case study NT Hùng • M, 51 • Symptoms: - Chest pain CT Scan Definitive diagnosis Pulmonary sequestration Pulmonary sequestration Definition • Pulmonary sequestration is an embryonic mass of lung tissue that has no identifiable bronchial communication and that receives its blood supply from one or more anomalous systemic arteries • Classification: - Extralobar sequestration (ELS) - Intralobar sequestration (ILS) Extralobar sequestration (ELS) • Present in infancy (respiratory distress and chronic cough) • Called extralobar because the mass lies outside of the normal investment of visceral pleura (may also lie outside of the thorax in a subdiaphragmatic position , 10%) • Arterial supply: - Systemic arteries (95%), commonly branches of the thoracic aorta or the abdominal aorta (80%) - Pulmonary arteries (5%) Extralobar sequestration • Venous drainage: - Systemic veins (75%), inferior vena cava [IVC] or azygos or portal veins - Pulmonary veins (25%) • Association with other congenital abnormalities (65%) - Diaphragmatic hernias (20%) - Congenital cystic adenomatoid malformation - Bronchogenic cysts - Foregut malfomations Intralobar sequestration (ILS) • ILS may contain air via the pores of Kohn or a connection to normal small bronchi • Diagnosed later in childhood or adulthood (infection: pneumonia) • Incorporated within the normal visceral pleura of the lung • Arterial supply: - Descending thoracic aorta (73%) - Abdominal aorta or celiac axis artery (21%) - Intercostal arteries (4%) Intralobar sequestration • Venous drainage: - Pulmonary veins (95%) - The IVC, the superior vena cava (SVC), the azygos systems, or the intercostal veins (5%) * Not commonly associated with other congenital anomalies Intralobar pulmonary sequestration Aortogram in an 8-yearold patient who presented with signs of an acute chest infection A chest radiograph showed a left lower lobe consolidation (not shown) After appropriate medical treatment, the child improved clinically, but an opacity in the left lower lobe persisted A sequestrated lung segment was suspected because of a history of several previous respiratory infections from age years and older This aortogram shows contrast material injected within the upper abdominal aorta An anomalous artery is arising from the infradiaphragmatic portion of the aorta (bottom, shorter arrow) and is supplying a supradiaphragmatic mass in the left lower lobe (top, longer arrow) Intralobar pulmonary sequestration An 8-year-old patient presented with signs of an acute lung infection The venous phase of aortogram shows pulmonary venous drainage into the left atrium (arrow) Extralobar Pulmonary sequestration A solid mass in the posterior mediastinum (arrow) in a 55-year-old patient who smokes A bronchogeni c neoplasm was suspected Extralobar pulmonary sequestration, a 55-year-old patient who smokes This image shows a nonspecific mass in the posterior mediastinum At thoracotomy, the mass was seen to be attached to the paravertebral region by a feeding artery originating from the descending thoracic aorta Histologic examination confirmed an extralobar sequestration Supine chest radiograph in an infant This image shows a large opacity at the left costophrenic angle, which can be followed upward to below the diaphragm Contrast-enhanced portal venous phase transaxial computed tomography scan through the liver and base of the left lung This image shows a large mass of mixed attenuation with an arterial supply from the aorta and an enlarged hemiazygos vein from venous return Contrast enhanced computed tomography angiogram in a 34-year-old female with an extralobar pulmonary sequestration This image shows a 5- x 2-cm subpulmonic mass with punctuate calcification (confirmed on unenhanced CT scan) with an arterial supply from the celiac axis (white arrows) and venous drainage via the left renal vein (red arrow) Chúc mừng ngày nhà giáo Việt Nam 20-11