EDUCATION EXHIBIT - Continuing Medical Education

Congenital Pulmonary Venolobar Syndrome: Spectrum of Helical CT Findings with Emphasis on Computerized Reformatting

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The term congenital pulmonary venolobar syndrome refers to a wide spectrum of pulmonary developmental anomalies that may appear singly or in combination. The main components of congenital pulmonary venolobar syndrome are hypogenetic lung (including lobar agenesis, aplasia, or hypoplasia), partial anomalous pulmonary venous return, absence of pulmonary artery, pulmonary sequestration, systemic arterialization of lung, absence of inferior vena cava, and accessory diaphragm. The recent introduction of multisection helical computed tomography (CT), combined with use of advanced postprocessing graphic workstations, allows improved noninvasive delineation of complex congenital anomalies. A single fast (5–15-second) CT scan now enables the radiologist to (a) generate angiogram-like images of the anomalous pulmonary arteries and veins; (b) demonstrate tracheobronchial abnormalities by generating simulated bronchographic or bronchoscopic images; and (c) depict associated parenchymal abnormalities on axial, coronal, or sagittal images, which once represented an important advantage of magnetic resonance imaging over CT. Multisection helical CT is a helpful diagnostic tool in the preoperative evaluation of patients with suspected congenital pulmonary venolobar syndrome.

© RSNA, 2003


  • 1 Felson B. Chest roentgenology Philadelphia, Pa: Saunders, 1973; 87-92.
  • 2 Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. RadioGraphics 1994; 14:349-369.
  • 3 Gerle RD, Jaretzki A, III, Ashley CA, Berne AS. Congenital bronchopulmonary-foregut malformation: pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968; 278:1413-1419.
  • 4 Frank JL, Poole CA, Rosas G. Horseshoe lung: clinical, pathologic and radiologic features and a new plain film finding. AJR Am J Roentgenol 1986; 146:217-226.
  • 5 Ellis K. Developmental abnormalities in the systemic blood supply to the lungs. AJR Am J Roentgenol 1991; 156:669-679.
  • 6 Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am 1994; 41:453-472.
  • 7 Yamanaka A, Hirai T, Fujimoto T, Hase M, Noguchi M, Konishi F. Anomalous systemic arterial supply to normal basal segments of the left lower lobe. Ann Thorac Surg 1999; 68:332-338.
  • 8 Godwin JD, Tarver RD. Scimitar syndrome: four new cases examined with CT. Radiology 1986; 159:15-20.
  • 9 Rappaport DC, Herman SJ, Weisbrod GL. Congenital bronchopulmonary diseases in adults: CT findings. AJR Am J Roentgenol 1994; 162:1295-1299.
  • 10 Ikezoe J, Murayama S, Godwin JD, Done SL, Verschakelen JA. Bronchopulmonary sequestration: CT assessment. Radiology 1990; 176:375-379.
  • 11 Hopkins KL, Patrick LE, Simoneaux SF, Bank ER, Parks WJ, Smith SS. Pediatric great vessel anomalies: initial clinical experience with spiral CT angiography. Radiology 1996; 200:811-815.
  • 12 Amitai M, Konen E, Rozenman J, Gerniak A. Preoperative evaluation of pulmonary sequestration by helical CT angiography. AJR Am J Roentgenol 1996; 167:1069-1070.
  • 13 Chung JW, Park JH, Im JG, Chung MJ, Han MC, Ahn H. Spiral CT angiography of the thoracic aorta. RadioGraphics 1996; 16:811-824.
  • 14 Cho SY, Kim HC, Bae SH, et al. Demonstration of blood supply to pulmonary sequestration by MR and CT angiography. J Comput Assist Tomogr 1996; 20:993-995.
  • 15 Frush DP, Donnelly LF. Pulmonary sequestration spectrum: a new spin with helical CT. AJR Am J Roentgenol 1997; 169:679-682.
  • 16 Franco J, Aliaga R, Domingo ML, Plaza P. Diagnosis of pulmonary sequestration by spiral CT angiography. Thorax 1998; 53:1089-1092.
  • 17 Zylak CJ, Eyler WR, Spizarny DL, Stone CH. Developmental lung anomalies in the adult: radiologic-pathologic correlation. RadioGraphics 2002; 22:S25-S43.
  • 18 Felker RE, Tonkin IL. Imaging of pulmonary sequestration. AJR Am J Roentgenol 1990; 154:241- 249.
  • 19 Garcia-Peña P, Lucaya J, Hendry GMA, McAndrew PT, Duran C. Spontaneous involution of pulmonary sequestration in children: a report of two cases and review of the literature. Pediatr Radiol 1998; 28:266-270.
  • 20 Sakai S, Murayama S, Soeda H, et al. Unilateral proximal interruption of the pulmonary artery in adults: CT findings in eight patients. J Comput Assist Tomogr 2002; 26:777-783.
  • 21 Brenner DJ, Elliston CD, Hall EJ, Berdon WE. Estimated risks of radiation-induced fatal cancer from pediatric CT. AJR Am J Roentgenol 2001; 176:289-296.
  • 22 McNitt-Gray MF. AAPM/RSNA Physics Tutorial for Residents: topics in CT—radiation dose in CT. RadioGraphics 2002; 22:1541-1553.

Article History

Published in print: Sept 2003