From the Archives of the AFIP: Intralobar Sequestration

Radiologic-Pathologic Correlation

Abstract Introduction Postulated Pathogenesis Clinical Characteristics

Pathologic Features Histologic Features Radiologic Appearance

Therapy And Prognosis Conclusions Source Information References

Clinical Characteristics

Patients with intralobar sequestration typically present in early adulthood with a history of chronic cough, mucopurulent sputum production, and recurrent pneumonia that progresses in severity and is usually caused by pyogenic bacteria (2,8). Half of all patients reach the age of 20 years before the diagnosis is made. Hemoptysis is a common presenting sign, and patients may produce as much as 800 mL of bright red blood. Chest pain, asthma, or pleuritic pain may also be presenting complaints (1,2,9,25,30). A small percentage (15%) of patients may be asymptomatic when the lesion is discovered (1).

Physical findings of intralobar sequestration are nonspecific and sporadic. (In unusual cases, patients may have cyanosis, clubbed fingers, chest wall asymmetry or pectus excavatum) (1,2,31). A rare manifestation of intralobar sequestration in the neonatal period is high output congestive heart failure, a hemodynamic state that has been attributed to left-to-left shunting caused by the lesion (16,18,25,29,32,33). The combination of congestive heart failure and pulmonary consolidation in an infant should raise the suspicion of sequestration (30). A continuous or pansystolic murmur, auscultated over the anomalous feeding artery, may occur in children and young adults (9,17,29,34). Massive, spontaneous, nontraumatic pleural hemorrhage, which is attributed to fibrinoid necrosis of the systemic artery, is an extremely rare but potentially fatal complication (35).

In a review by Savic and colleagues (1) of over 400 cases of intralobar sequestration, male and female subjects were found to be equally affected. Intralobar sequestration is very rare in infants and is infrequently associated with congenital anomalies, including esophagobronchial diverticula, diaphragmatic hernias, skeletal deformities, cardiovascular defects (including tetralogy of Fallot), and renal anomalies (eg, failure of ascent and rotation) (1,2,10,31). Esophagobronchial fistulas and diverticula account for approximately 25% of the anomalies that do occur in association with intralobar sequestration, and these anomalies correlate with the presence of a bronchopulmonary foregut malformation or, as in the case of diverticula, a traction effect on local tissues caused by the chronic inflammatory process (2,10). Another 25% of the associated anomalies are skeletal, including scoliosis, rib abnormalities, and vertebral anomalies. Stocker and Malczak (2,10) suggest that some of these findings are not actually congenital but instead may be related to underlying chronic lung disease that affects the thoracic portion of the axial skeleton and the chest wall.

Abstract Introduction Postulated Pathogenesis Clinical Characteristics

Pathologic Features Histologic Features Radiologic Appearance

Therapy And Prognosis Conclusions Source Information References