November 2023 Imaging Case of the Month: A Crazy Association

Parker J. Brown MD, Prasad M. Panse MD and Michael B. Gotway MD

Department of Radiology

Mayo Clinic, Arizona

Phoenix, Arizona

HPI: A 55-year-old man presents with a history of cough, poor appetite, low energy, and weight loss over the previous 6-10 months following COVID-19 infection 2 months earlier. 

PMH, SH, FH: The patient’s past medical history was positive for CVOID-19 infection 2 months earlier as well as pneumonia, not specified, in the previous year.

The patient’s past medical history was also remarkable for a 7-unit gastrointestinal hemorrhage approximately one year earlier following polypectomy for benign lesions in the transverse colon. During that hospital admission a complete blood count showed 1% blasts which prompted hematology consultation. The consulting oncologist felt the peripheral blasts were the result of a leukemoid reaction secondary to increased bone marrow stimulation owing to the patient’s acute anemia caused by the gastrointestinal hemorrhage. Macrocytosis and reticulocytosis was also noted and attributed to the same. Repeat complete blood count showed no blasts although some myelocytes, metamyelocytes, and polychromasia was noted for which follow up assessment was recommended. Serum B12 and folate levels were normal.

The patient had no prior surgeries.

The patient was not taking any prescription medications.

The patient is a non-smoker. He has no known allergies and drinks alcohol only socially and denied illicit drug use.

There was no significant family history.

Physical Examination: The patient’s physical examination showed his temperature to be 96.7°F with borderline elevated pulse rate of 95/min, a normal respiratory rate, and blood pressure of 118/67 mmHg. Room air oxygen saturation was 98%.

Initial Laboratory: A complete blood count showed a normal white blood cell count at 5.6 x10⁹/L (normal, 3.4 – 9.6 x10⁹/L), with 75% bands (normal, 50-75%). His hemoglobin and hematocrit values were 10.1 gm/dL (normal, 13.2 – 16.6 gm/dL) and 31.6% (normal, 38.3 – 48.6%). The platelet count was normal at 225 x 10⁹/L (normal, 135 – 317 x 10⁹/L). The patient’s serum chemistries and liver function studies were normal aside from mildly decreased total protein at 5.7 gm/dL (normal, 6.3-.9 gm/dL). The patient had an elevated anti-nuclear antibody titer at 1:320. SARS-CoV-2 PCR testing was positive.  

Radiography: Frontal chest radiography (Figure 1) was performed.

Figure 1. Frontal chest radiography at presentation.

Which of the following statements regarding this chest radiograph is accurate? (Click on the correct answer to be directed to the second of fourteen pages).

1. Frontal chest radiography shows normal findings
2. Frontal chest radiography shows marked cardiomegaly
3. Frontal chest radiography shows mediastinal lymphadenopathy
4. Frontal chest radiography shows pleural effusion
5. Frontal chest radiography shows multifocal consolidation

Cite as: Brown PJ, Panse PM, Gotway MB. November 2023 Imaging Case of the Month: A Crazy Association. Southwest J Pulm Crit Care Sleep. 2023;27(5):47-55. doi: https://doi.org/10.13175/swjpccs046-23 PDF

October 2023 Medical Image of the Month: Swyer-James-MacLeod Syndrome

Figure 1. Frontal (A) and lateral (B) topographic images from a non-contrast chest CT show a relative paucity of lung markings in the right hemithorax. There are at least 2 large, cystic-appearing lesions in the right lung, which appears somewhat hyperinflated. Axial (C) and sagittal (D) reconstructions from the CT confirm unilateral areas of emphysematous appearing hyperinflated lung with surrounding atelectasis. The left lung appears relatively normal. Click here to view Figure 1 in an enlarged, separate window.

Figure 2. Hematoxylin and Eosin stained low-power pathological image from right upper lobectomy (A) demonstrates chronic bronchiolitis with features of subtotal obliterative bronchiolitis associated with mild septal fibrosis and prominent emphysematous/cystic change. Elastic trichrome stain of a small airway (B) demonstrates subtotal bronchiolitis obliterans. Click here to view Figure 2 in an enlarged, separate window.

Pathological slides from a right upper lobectomy specimen obtained at an outside institution were submitted to our Department of Pathology for review. A pre-operative noncontrast chest CT from the outside institution was submitted along with the path slides. The patient was a 27 y/o man who presented to the outside institution with exercise intolerance and increasing shortness of breath following a bout of COVID in early 2023. The patient also related a history of possible chronic myocarditis. A review of the CT demonstrated 2 distinct right-sided areas of hyperinflated, emphysematous lung with a relatively normal appearing left lung (Figure 1). Although congenital lobar emphysema was considered, the multifocal nature of the findings suggested against this, and a diagnosis of Swyer-James-MacLeod Syndrome was entertained. Histopathological analysis (Figure 2) confirmed this suspicion. When questioned further, the patient related a history of neonatal RSV infection requiring 3 weeks of hospitalization. The constellation of historical, radiological, and pathological findings was consistent with Swyer-James- MacLeod Syndrome.

This syndrome was first described in 1949 in a case report of a six-year-old boy from the UK by pediatrician Paul Swyer and radiologist George James (1). The pulmonologist William MacLeod published a review of nine cases in 1954 (2). Also known as “unilateral hyperlucent lung syndrome”, this is a postinfectious form of bronchiolitis obliterans. Severe infection early in life, while the lungs are still developing, is the proposed mechanism. Although different infections organisms are associated with this, respiratory syncytial virus is most implicated. Swyer-James-MacLeod Syndrome usually affects the lungs asymmetrically. On imaging, the most common pattern is that of a unilateral hyperlucent lung which may or may not be associated with hyperinflation and may or may not be associated with bronchiectasis (3). There is reduction in pulmonary blood flow and formation of septal fibrosis which leads to obstruction of pulmonary capillary beds. Alveolar hyperinflation leads to mechanical resistance to pulmonary blood flow and reduced ventilation leads to pulmonary vasoconstriction. Most patients asymptomatic, and many cases discovered incidentally. Treatment is usually conservative and preventative, focused on controlling pulmonary infections. Inhaled corticosteroids may have a limited role in treatment as well (4).

Samantha Moore, MD, PhD¹ and Clinton Jokerst MD²

Department of Laboratory Medicine and Pathology¹ and Department of Radiology²

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Swyer PR, James GC. A case of unilateral pulmonary emphysema. Thorax. 1953 Jun;8(2):133-6. [CrossRef] [PubMed]
2. William Mathieson Macleod. Lancet. 1977 Oct 15;2(8042):833. [PubMed]
3. Lucaya J, Gartner S, García-Peña P, Cobos N, Roca I, Liñan S. Spectrum of manifestations of Swyer-James-MacLeod syndrome. J Comput Assist Tomogr. 1998 Jul-Aug;22(4):592-7. [CrossRef] [PubMed]
4. Mehra S, Basnayake T, Falhammar H, Heraganahally S, Tripathi S. Swyer-James-MacLeod syndrome-a rare diagnosis presented through two adult patients. Respirol Case Rep. 2017 Jun 16;5(5):e00245. [CrossRef] [PubMed]

Cite as: Moore S, Jokerst C. 2023 Medical Image of the Month: Swyer-James-MacLeod Syndrome. Southwest J Pulm Crit Care Sleep. 2023;27(4):45-46. doi: https://doi.org/10.13175/swjpccs042-23 PDF