Answer for BIR CoW 02 Apr 2023
Congenital venolobar syndrome
Findings
Right partial anomalous pulmonary vein draining into IVC –scimitar vein
Right focal eventration of diaphragm
Hypoplastic right superior basal segment of lower lobe with dextro position of heart
Horseshoe lung
Discussion
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. Approximately 67% of patients with partial anomalous pulmonary venous return also have atrial-level defects, most commonly a sinus venosus defect Clinical features : Anomalous connection of one pulmonary vein usually is not clinically apparent in childhood, but these patients may present in the third and fourth decades with cyanosis resulting from increased pulmonary vascular resistance. Patients with scimitar syndrome may present in infancy with pulmonary hypertension from the arterial supply to the right lower lung ,stenosis of the anomalous pulmonary veins and pulmonary infections. Otherwise, this syndrome may be detected in adulthood in persons who do not have significant symptoms. Horseshoe lung is a rare congenital malformation in which an isthmus of pulmonary parenchyma extends from the right lung base across the midline behind the pericardium and joins the posterobasal segments of the lungs In most of the cases, horseshoe lung is associated with hypogenetic lung syndrome Imaging features : Xray : cardiomegaly with right heart enlargement increased pulmonary flow are seen on chest radiography crescent-shaped anomalous pulmonary vein (resembling a Turkish sword or scimitar) paralleling the lower right heart border ) associated hypoplasia of the right lung, a small right pulmonary artery varying degrees of cardiac dextroposition. Cross-sectional imaging goals include : Identifying the anomalous pulmonary to systemic venous connection, locating each pulmonary vein and its drainage relative to the left atrium, and determining the location of venous obstruction, if present Evaluation for the presence and size of either an ASD or sinus venosus defect, is necessary The heart and great vessels are evaluated for other abnormalities, and when scimitar syndrome is present, the upper abdomen should be assessed for anomalous venous drainage and systemic supply to the lung. CT or magnetic resonance angiography is very useful for identifying the relationship between the anomalous pulmonary veins and the left atrium. MRI also is useful for ASD evaluation and for quantification of systemic to pulmonary shunting. CT and MRI are accurate in assessing for postoperative pulmonary vein obstruction or narrowing . Treatment : In patients with scimitar syndrome, pulmonary resection or catheter occlusion of the anomalous arterial blood supply may be necessary. The anomalous vein in patients with scimitar syndrome may be anastomosed directly to the left atrium.
Reference :
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
Findings
Right partial anomalous pulmonary vein draining into IVC –scimitar vein
Right focal eventration of diaphragm
Hypoplastic right superior basal segment of lower lobe with dextro position of heart
Horseshoe lung
Discussion
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. Approximately 67% of patients with partial anomalous pulmonary venous return also have atrial-level defects, most commonly a sinus venosus defect Clinical features : Anomalous connection of one pulmonary vein usually is not clinically apparent in childhood, but these patients may present in the third and fourth decades with cyanosis resulting from increased pulmonary vascular resistance. Patients with scimitar syndrome may present in infancy with pulmonary hypertension from the arterial supply to the right lower lung ,stenosis of the anomalous pulmonary veins and pulmonary infections. Otherwise, this syndrome may be detected in adulthood in persons who do not have significant symptoms. Horseshoe lung is a rare congenital malformation in which an isthmus of pulmonary parenchyma extends from the right lung base across the midline behind the pericardium and joins the posterobasal segments of the lungs In most of the cases, horseshoe lung is associated with hypogenetic lung syndrome Imaging features : Xray : cardiomegaly with right heart enlargement increased pulmonary flow are seen on chest radiography crescent-shaped anomalous pulmonary vein (resembling a Turkish sword or scimitar) paralleling the lower right heart border ) associated hypoplasia of the right lung, a small right pulmonary artery varying degrees of cardiac dextroposition. Cross-sectional imaging goals include : Identifying the anomalous pulmonary to systemic venous connection, locating each pulmonary vein and its drainage relative to the left atrium, and determining the location of venous obstruction, if present Evaluation for the presence and size of either an ASD or sinus venosus defect, is necessary The heart and great vessels are evaluated for other abnormalities, and when scimitar syndrome is present, the upper abdomen should be assessed for anomalous venous drainage and systemic supply to the lung. CT or magnetic resonance angiography is very useful for identifying the relationship between the anomalous pulmonary veins and the left atrium. MRI also is useful for ASD evaluation and for quantification of systemic to pulmonary shunting. CT and MRI are accurate in assessing for postoperative pulmonary vein obstruction or narrowing . Treatment : In patients with scimitar syndrome, pulmonary resection or catheter occlusion of the anomalous arterial blood supply may be necessary. The anomalous vein in patients with scimitar syndrome may be anastomosed directly to the left atrium.
Reference :
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
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!