Dr. Pepe’s Diploma Casebook: CASE 121 – SOLVED!

Dear Friends,

This week’s radiograph belongs to a 45-year-old man with moderate cough.


1. Mediastinal mass
2. RML disease
3. Enlarged right atrium
4. None of the above

Check the images below, leave your thoughts in the comments section, and come back on Friday for the answer.

Click here for the answer

Findings: PA radiograph shows an abnormal mediastinal contour (A, white arrows). In addition, the right lung is smaller than the left and its vasculature is diminished. The clue to the diagnosis lies in identifying a tubular shadow in the right lower lung (A, red arrow), which represents an anomalous venous return (scimitar sign). Coronal CT shows a smaller right lung and the scimitar vein (B, red arrow), typical of this malformation.

Diagnosis: congenital hypogenetic lung syndrome with scimitar vein

This week I intend to present additional signs in chest imaging. In a sense, recognizing signs is a sort of Aunt Minnie approach: once we are certain that a specific sign is present, we expedite the path to the correct diagnosis.

Today I will focus on signs of the lung compartment.
The first one is the scimitar sign.

The scimitar sign is the hallmark of hypogenetic right lung syndrome, in which there is agenesis of one or two pulmonary lobes. Partially anomalous return of the RLL veins is present in 80% of cases. The vessel is seen as a widening tubular shadow, originating the term scimitar syndrome.
The vein is not seen on plain films in half the cases.

Importance of the sign: It helps to diagnose congenital hypoplasia of the lung and avoids confusing this condition with more serious disorders.

Sometimes the scimitar vein is clearly visible (Fig. 1). On other occasions, it is partially hidden by the lung changes and we have to look carefully for the vein to suggest the correct diagnosis (Fig. 2).

Fig. 1. Scimitar sign in two asymptomatic adult patients (A-C, arrows). Both of them have dextrocardia, secondary to the right lung hypoplasia, which attracts the heart towards the right. Air under the diaphragm in the first patient is secondary to unrelated surgery.

Fig.2 47-year-old asymptomatic patient with hypogenetic lung. The shifting of the lung towards the right partially obscures the scimitar vein (A, arrow), which is better seen in the enhanced axial CT image (B, arrow).

QUIZ: 57-year-old man with chest pain and moderate fever.


1. Pneumonia
2. Pulmonary infarct
3. Pleural fluid
4. None of the above

Findings: PA radiograph shows a triangular pulmonary opacity at the right costophrenic angle with an indistinct convex border (A, arrow), a feature known as Hampton’s hump. In the appropriate clinical circumstances, this sign is highly suggestive of pulmonary infarction. Enhanced coronal CT confirms a thrombus in the lower pulmonary artery (B, arrow).

Diagnosis: pulmonary embolism with Hampton´s hump

On plain radiographs, pulmonary infarcts are peripheral in location. When located in the costophrenic angle, they show an indistinct convex border (Hampton’s hump) that differentiates them from pleural fluid, which has a concave border.

Importance of the sign: In the appropriate clinical setting, Hampton’s hump is a reliable sign of pulmonary infarct and indicates enhanced CT to investigate pulmonary emboli.

Fig. 3. PA radiograph shows a triangular pulmonary opacity at the right costophrenic angle, with an indistinct convex border (A, white arrow), with the characteristic appearance of a Hampton’s hump, suggesting a pulmonary infarct. In addition, a round nodule is visible in the lower aspect of the right hilum (A, red arrow). CT confirmed the pulmonary embolism and an RLL mass (B and C, red arrows) that enhanced to 42 H.U. after contrast injection. Diagnosis: adenocarcinoma with associated pulmonary infarct

It is not uncommon for infarcts to occur in the posterior costophrenic sulcus. In these cases, the Hampton’s hump may not be visible in the PA radiograph, but be very obvious in the lateral projection (Fig. 4).

Fig. 4. 52-year-old man with acute chest pain. PA radiograph shows a discrete opacity at the right cardiophrenic angle (A, arrow). Lateral view shows a large posterior Hampton’s hump (B, arrow).

Enhanced CT confirms the infarct in the posterior costophrenic sinus (C and D, white arrows). A thrombus is seen in the distal artery (D, red arrow).

In asymptomatic patients, diaphragmatic hernias may simulate a Hampton’s hump and should be ruled out (Fig. 5). Remember that the diaphragm is a frontier organ, and lesions arising from below may mimic chest lesions.

Fig. 5. Blunting of the left cardiophrenic sinus in an asymptomatic patient, simulating a Hampton’s hump (A, arrow). Unenhanced CT shows herniated abdominal fat (B, arrow).

QUIZ: 56-year-old man with cough and fever.

What is the probability of malignancy?

1. Low
2. 50/50
3. High
4. Can’t tell

Findings: PA radiograph shows a segmental infiltrate in the RUL (A, arrow). The lateral view shows concavity of the minor fissure (B, white arrow), with convexity at the hilar level (B, red arrow), creating a Golden sign. Malignant disease is very likely.

CT confirms a central mass occluding the RUL bronchus (C and D, arrows). PET/CT shows marked uptake of the mass (E, arrow).

Diagnosis: carcinoma of RUL with Golden sign

The Golden sign is usually seen on posteroanterior chest radiographs. It occurs when a large central mass produces partial collapse of the right upper lobe. As the lobe collapses, the minor fissure is visible as a concave triangular opacity that represents the collapsed lobe (A and B, white arrows). The central mass forms a convex bulge at the origin of the lobe (A and B, red arrows). The combination of these findings resembles a reverse S shape.
 Although initially described in right upper lobe collapse, the Golden sign can be applicable to atelectasis involving any lobe.

Importance of this sign: In adults, it is highly suggestive of bronchial carcinoma.

The Golden sign is usually indicative of carcinoma (Fig. 6), although sometimes it results from non-malignant enlarged lymph nodes (Fig. 7).

Fig. 6. PA chest radiograph shows the typical appearance of RUL collapse, with uniform opacity of the RUL and elevation and convexity of the minor fissure (A, white arrow). The medial aspect of the fissure is convex (A, red arrow), indicating a hilar mass (Golden sign). There is a right pleural effusion. Enhanced axial CT confirms a large hilar mass (B, arrow). Final diagnosis: central carcinoma with RUL collapse and Golden sign

Fig. 7. Primary TB in a 20-year-old male with RUL collapse and a Golden sign (A, arrow). Three weeks earlier, there was a large right hilar lymph node (B, arrow) without collapse. Compression on the bronchus has caused the RUL collapse seen in A. This case nicely explains the Golden sign.

Although the Golden sign is usually found in the RUL, it may also occur in other lobes (Fig. 8).

Fig. 8. 75-year-old woman with chronic cough. PA radiograph depicts air-space disease in the RML (A, arrow). The lateral view shows the typical triangular appearance of RML collapse (B, white arrows), with widening of the proximal aspect (Golden sign), suggesting a central mass (B, red arrow). There are also multiple compression fractures of the spine.

The Golden sign was overlooked and the patient was treated for pneumonia. Five months later the proximal mass was larger (C, arrow). Unenhanced CT shows the mass (D, arrow), confirmed to be malignant at bronchoscopy.

Follow Dr. Pepe’s advice:

1. Discovering a scimitar sign helps to diagnose congenital hypogenetic lung and exclude other significant disease.

2. Hampton’s hump is a reliable sign of pulmonary infarction in the adequate clinical setting.

3. The Golden sign is a good indicator of malignancy.

14 thoughts on “Dr. Pepe’s Diploma Casebook: CASE 121 – SOLVED!

  1. Loss of volume of the right hemithorax.
    Widening of the right paratracheal line with a well defined opacity proyected over right cardiac border (sillhoutte sign-medium lobe)
    There is a vascular structue proyected over the rght hemidiagphragm

    Pulmonary sequestration?

      1. Scimitar syndrome/ PAPVR
        Also the borders of left sided aortic arch (characteristic knob) make me think of aortic coarctation as coexisting problem.

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