November 2024 Imaging Case of the Month: A Recurring Issue

Michael B. Gotway MD

Department of Radiology

Mayo Clinic, Arizona

Phoenix, Arizona USA

Clinical History: A 65-year-old woman with a history of orthotopic liver transplantation 2 years earlier for non-alcoholic steatohepatitis presented to the Emergency Room for chronic fatigue, malaise, nausea and vomiting, and generalized weakness. She denies shortness of breath, hemoptysis, or productive cough. Her post liver transplant course was complicated by wound infection, biliary stricture requiring ERCP with stent placement, and Clostridium difficile colitis. Prior to her liver transplant the patient had chronic renal insufficiency which has been slowly progressing (creatinine of 2.8 mg/dL, estimated GFR of 17.6 mL) and she was currently undergoing renal transplant evaluation. The patient also has a past medical history of coronary artery disease requiring bypass grafting surgery, hypothyroidism requiring hormone replacement, and type II diabetes not requiring specific therapy. Her past surgical history included cholecystectomy and hysterectomy.

The patient is a lifelong non-smoker, she reports an allergy to penicillin and amoxicillin (hives), and she does not drink alcohol, and denies illicit drug use. Her medications include tacrolimus, mycophenolic acid, allopurinol, calcium, vitamin D, levothyroxine, pantoprazole, sertraline, fluconazole, sulfamethoxazole and trimethoprim, and prednisone.

On physical examination the patient was febrile (39.2º C). Her blood pressure was initially 97/53 mmHg; however, during her stay in the Emergency Department went as low as 71/41 mmHg. Her heart rate remained in the low to mid 90s, her respiratory rate was 12-14 breaths per minute, and her oxygen saturations were 99% on room air. The patient had uniformly decreased breath sounds bilaterally but the lungs were otherwise clear. Her cardiac examination was normal aside from trace bilateral pedal edema. Her abdominal examination was normal. She was neurologically intact.

A complete blood count showed a normal white blood cell count at 6.2 x 10⁹/L (normal, 3.4 – 9.6 x 10⁹/L), with a normal absolute neutrophil count of 3.65 x 10⁹/L (normal, 1.4 – 6.6 x 10⁹/L); the percent distribution of lymphocytes, monocytes, and eosinophils was normal. Her hemoglobin and hematocrit values were 10 gm/dL (normal, 13.2 – 16.6 gm/dL) and 33.7% (normal, 34.9 – 44.5%). The platelet count was normal at 134 x 10⁹/L (normal, 149 – 375 x 10⁹/L). The patient’s serum chemistries and liver function studies were normal, including an albumin level at 4.3 gm/dL (normal, 3.5 – 5 gm/dL), with normal alanine aminotransferase at 42 U/L (normal, 7-45 U/L) and aspartate aminotransferase of 40 U/L (normal, 8-43 U/L); alkaline phosphatase levels, bilirubin, and coagulation studies were normal. SARS-CoV-2 PCR testing was negative.

Frontal chest radiography (Figure 1) was performed.

Figure 1. Frontal (A) and lateral (B) chest radiography obtained in the Emergency Room. To view Figure 1 in a separate enlarged window, click here.

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

1. Frontal chest radiography shows unremarkable findings
2. Frontal chest radiography shows a moderate-to-large right pleural effusion
3. Frontal chest radiography shows mediastinal lymphadenopathy
4. Frontal chest radiography shows pneumothorax
5. Frontal chest radiography shows numerous small nodules

Cite as: Gotway MB. November 2024 Imaging Case of the Month: A Recurring Issue. Southwest J Pulm Crit Care Sleep. 2024;29(6):36-44. doi: https://doi.org/10.13175/swjpccs047-24 PDF

February 2024 Medical Image of the Month: Pulmonary Alveolar Proteinosis in Myelodysplastic Syndrome

Figure 1.  Axial CT images from a contrast-enhanced chest CT performed on the patient at time of admission (A,B) demonstrate a combination of smooth septal line thickening and superimposed ground glass, resulting in a “crazy paving” appearance.  A noncontrast chest CT performed at an outside hospital 2 months earlier also demonstrates “crazy paving”; however, the findings have progressed significantly during the 2 intervening months.

A 56-year-old man presented to our institution with weight loss, fatigue, and worsening dyspnea. A chest CT (Figure 1A,B) showed a patchy, mostly central crazy-paving pattern. An outside hospital CT performed 2 months earlier was also made available for comparison (Figure 1C,D) demonstrating interval progression of these findings. The patient also had mediastinal and hilar adenopathy.  Past workup had primarily focused on the lymphadenopathy with considerations of possible lymphoma, sarcoidosis or coccidiomycosis infection. A past lymph node biopsy had revealed non-caseating granulomas and serum titers were positive for coccidiomycosis. Lymphadenopathy decreased after initiation of coccidiomycosis treatment, but symptoms and crazy paving findings continued to worsen. Further workup revealed a new diagnosis of myelodysplastic syndrome (MDS) and subsequent bronchoalveolar lavage (BAL) and histology results were consistent with secondary PAP, likely due to patient’s underlying hematologic disease.

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of lipoproteinaceous material in the lung alveoli. There are two types of PAP that occur in adults: Idiopathic/autoimmune and Secondary PAP. Idiopathic/autoimmune PAP is more common and is thought to result from antibody production against granulocyte-macrophage-colony-stimulating factor (GM-CSF) that regulates surfactant homeostasis. Secondary PAP results from a precipitating condition, often inhalation exposure, underlying malignancy, or immunocompromise.

The clinical manifestations of PAP are nonspecific and includes dyspnea, nonproductive cough, fatigue, and weight loss. CT may show nonspecific findings of smooth, bilateral interlobular septal thickening superimposed on a background of ground-glass opacification (crazy-paving). Diagnosis is confirmed with BAL and lung biopsy showing accumulation of eosinophilic and periodic acid-Schiff stain (PAS) positive lipoproteinaceous material within alveoli. Treatment involves pulmonary lavage for idiopathic/autoimmune PAP and treating the underlying condition for secondary PAP.

Parker Brown MD, Clint Jokerst MD, Michael Gotway MD, Matthew Stib MD

Department of Radiology

Mayo Clinic Arizona, Scottsdale, AZ

References

1. Frazier AA, Franks TJ, Cooke EO, Mohammed TL, Pugatch RD, Galvin JR. From the archives of the AFIP: pulmonary alveolar proteinosis. Radiographics. 2008 May-Jun;28(3):883-99; quiz 915. [CrossRef][PubMed]
2. Ioachimescu OC, Kavuru MS. Pulmonary alveolar proteinosis. Chron Respir Dis. 2006;3(3):149-59. [CrossRef] [PubMed]
3. Presneill JJ, Nakata K, Inoue Y, Seymour JF. Pulmonary alveolar proteinosis. Clin Chest Med. 2004 Sep;25(3):593-613, viii. [CrossRef] [PubMed]

Cite as: Brown P, Jokerst C, Gotway MB, Stib M. February 2024 Medical Image of the Month: Pulmonary Alveolar Proteinosis in Myelodysplastic Syndrome. Southwest J Pulm Crit Care Sleep. 2024;28(2):18-19. doi: https://doi.org/10.13175/swjpccs004-24 PDF

January 2024 Medical Image of the Month: Polyangiitis Overlap Syndrome (POS) Mimicking Fungal Pneumonia

Figure 1. PA chest radiographs obtained on 9/14/23 (A) and approximately 2.5 months later (B) demonstrates rapidly growing cavitary masses in the upper lungs (arrows). The rapid interval growth is more suggestive of an inflammatory as opposed to malignant process. To view Figure 1 in a separate, enlarged window click here.

Figure 2. Axial reconstructions from an unenhanced chest CT (A,B) demonstrate multiple areas of mass-like consolidation with some areas of cavitation and some internal air bronchograms. As was surmised from the CXRs, the appearance suggests an infections/inflammatory etiology. To view Figure 2 in a separate, enlarged window click here.

Figure 3. H&E (A) and GMS (B) stains of a specimen from biopsy of right upper lobe lesion. There is an organizing inflammatory process with extensive necrosis and no evidence of infectious organism. H&E staining of a renal biopsy (C) demonstrates chronic and active necrotizing and crescentic glomerulosclerosis with diffuse interstitial fibrosis and tubular atrophy. Taken in conjunction with the history and lack of any other findings to suggest infection, histopathological findings were deemed to be consistent with active granulomatosis with polyangiitis.  To view Figure 3 in a separate, enlarged window click here.

A 32-year-old woman with a history including hypertension, end-stage renal disease requiring dialysis, asthma, nonischemic cardiomyopathy, and migraines, was directly transferred to our hospital in November 2023 for the evaluation of hemoptysis. The patient reported a two-week history of a nonproductive cough, runny nose, muscle aches, subjective fevers, chills, fatigue, nausea, and decreased appetite. Within the past 2 days the patient had also developed hemoptysis, with 5-6 episodes per day.

Initial investigations, including chest X-ray and CT chest, revealed large biapical pulmonary consolidations with cavitation. Multiple nodular densities were observed throughout both lungs (Figures 1 and 2). The patient denied any recent sick contacts, travel history, and prior tuberculosis infection. She did, however, disclose a period of incarceration from 2011 to 2019.

Upon arrival at our hospital, the patient recounted a relatively normal state of health until January 2023 when she underwent a two-month hospitalization, culminating in the diagnosis of end-stage renal disease by biopsy at an outside facility. She attributed this to anautoimmune disease, for which she did not receive immunosuppressive therapy at the time. Subsequent hospitalization in September 2023 for rhinovirus pneumonia led to the diagnosis of heart failure with a reduced ejection fraction of 15-20%, determined to be of nonischemic origin.

In our ED vital signs revealed a heart rate of 110, blood pressure of 180/90 mmHg, normal respiratory rate, and no hypoxia on room air. Laboratory results were significant for leukocytosis 18.7x10⁹/L with high eosinophils count of 2.32x10⁹/L, elevated potassium 5.7 mmol/L, BUN 51 mg/dL, and creatinine 9.5 mg/dL. Chest X-ray depicted bilateral upper lung consolidations, notably worsened on the right with central cavitation (Figure 1B). Additional nodularity was observed in the left mid-lung, which was new in comparison to a prior chest x-ray done in September 2023 (Figure 1A).

Following her admission, an extensive infectious workup, including TB QuantiFERON testing, lumbar puncture, bronchoscopy with BAL, and blood cultures, was conducted. The results were unremarkable. Transbronchial biopsies from the right upper lobe cavity revealed an organizing inflammatory process with extensive necrosis, negative for neoplasm and infectious staining including GMS & acid-fast bacilli (Figure 3A,3B). An autoimmune panel revealed elevated ESR, CRP, PR3 antibody, and positive c-ANCA, leading to a diagnosis of Polyangiitis overlap syndrome. Treatment commenced with IV methylprednisone, transitioning to oral prednisone (60 mg daily) with a gradual taper over the next eight weeks. Inpatient administration of rituximab was initiated, with plans for three more infusions as part of her induction therapy.

According to the data from the French Vasculitis Study Group Registry (1), among the 795 patients with granulomatosis with polyangiitis (GPA), 354 individuals (44.5%) exhibited elevated blood eosinophil counts. Notably, hypereosinophilia, primarily of mild-to-moderate severity (ranging from 500 to 1500/mm³), was identified in approximately one-quarter of GPA patients at the time of diagnosis. In contrast, severe eosinophilia (>1500/mm3) was observed in only 28 patients (8%). Furthermore, this subset with severe eosinophilia was noted to have worse renal function at the time of presentation. Whereas in a retrospective European multicentre cohort published by Papo et al. (2), ANCA status was accessible for 734 EGPA patients with only 16 patients (2.2%) having PR3-ANCA. Notably, at baseline, PR3-ANCA positive patients, in comparison to those with MPO-ANCA and ANCA-negative individuals, exhibited a lower prevalence of active asthma and peripheral neuropathy. Conversely, they manifested a higher incidence of cutaneous manifestations and pulmonary nodules. Adding to the complexity, EGPA, characterized by peripheral blood eosinophilia, asthma, and chronic rhinosinusitis, contrasts with GPA, which manifests pulmonary nodules without eosinophilic infiltration and usually a more severe renal disease.

Polyangiitis overlap syndrome (POS), previously published by Leavitt and Fauci (3), was defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis or overlaps more than one subtype of vasculitis. Several polyangiitis overlap syndromes have been identified since 1986; however, less than 20 case reports of an overlap syndrome involving both GPA and EGPA have been published so far. As per the literature review performed by Bruno et al. (4), most of the reported POS cases had lung involvement with over half developed alveolar hemorrhage. They noted genetic and clinical heterogeneity in the pathogenesis of polyangiitis overlap syndrome suggesting distinct clinical phenotypes and outcomes to therapy. Notably, treatment strategies in polyangiitis overlap syndrome are usually tailored to the severity of the disease rather than the ANCA phenotype, leading to favorable outcomes in most cases.

John Fanous MD¹, Clint Jokerst MD², Rodrigo Cartin-Ceba MD¹

Division of Pulmonology¹and Department of Radiology²

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Iudici M, Puéchal X, Pagnoux C, et al.; French Vasculitis Study Group. Significance of eosinophilia in granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry. Rheumatology (Oxford). 2022 Mar 2;61(3):1211-1216. [CrossRef] [PubMed]
2. Papo M, Sinico RA, Teixeira V, et al.; French Vasculitis Study Group and the EGPA European Study Group. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Rheumatology (Oxford). 2021 Sep 1;60(9):4355-4360. [CrossRef] [PubMed]
3. Leavitt RY, Fauci AS. Pulmonary vasculitis. Am Rev Respir Dis. 1986 Jul;134(1):149-66. [CrossRef] [PubMed]
4. Bruno L, Mandarano M, Bellezza G, Sidoni A, Gerli R, Bartoloni E, Perricone C. Polyangiitis overlap syndrome: a rare clinical entity. Rheumatol Int. 2023 Mar;43(3):537-543. [CrossRef] [PubMed]

Cite as: Fanous J, Jokerst C, Cartin-Ceba R. January 2024 Medical Image of the Month: Polyangiitis Overlap Syndrome (POS) Mimicking Fungal Pneumonia. Southwest J Pulm Crit Care Sleep. 2024;28(1):5-8. doi: https://doi.org/10.13175/swjpccs052-23 PDF

November 2023 Medical Image of the Month: Obstructive Uropathy Extremis

Figure 1. Video of CT angiography abdomen/pelvis, played caudal to cranial, obtained during assessment in the ED demonstrating obstructive uropathy with bilateral multiloculated urinomas leading to compression of the inferior vena cava. To view Figure 1  video in a separate, enlarged window click here.

Figure 2. A: CT angiography of the abdomen, axial plane, at the level of the renal veins demonstrating compression of the inferior vena cava (yellow arrow). B: CT angiography of the abdomen and pelvis, coronal plane, demonstrating bilateral multiloculated urinomas (blue brackets). To view Figure 2 in a separate, enlarged window click here.

A 71-year-old veteran presented to the emergency department with two-weeks of progressive back pain radiating to the abdomen associated low-grade fever, nausea, and new lower extremity edema. The family reported confusion. His medical history was significant for chronic prostatitis and low-grade prostate cancer on biopsy that was lost to follow-up eleven years ago. His only reported medications were aspirin 81 mg daily and naproxen 500mg up to four times a day for his pain.

Vitals were significant for a temperature of 36.1 C, initial blood pressure of 201/74, heart rate of 128/min, respirations at 18/min with a saturation of 97% on 2L NC. Physical exam demonstrated no difference in blood pressures between arms. No abnormal heart sounds. Clear breath sounds to auscultation bilaterally. Flank tenderness to percussion and significant abdominal tenderness over the epigastric and suprapubic region with 3+ pitting edema of the bilateral lower extremities. Screening labs were notable for critical values of a WBC of 43.5 K/mL and potassium of 7 mEq/L with a creatinine of 6.5 mg/dL. He was started on esmolol and hyperkalemia temporizing therapy with the decision made to obtain an urgent contrast enhanced computed tomography of the chest, abdomen, and pelvis.

Imaging confirmed a diagnosis of severe obstructive uropathy with heterogenous prostate with nonspecific small hypodensities, marked bladder distension, hydronephroureter with ureteral wall prominence, and bilateral perinephric multiloculated fluid collections with extension into the abdominal and retroperitoneal spaces leading to indentation of the inferior vena cava (Figures 1 and 2). A Foley catheter was urgently placed with 2.5L of urine immediately relieved and prompt response in blood pressure to 130/80, and resolution of pain, altered mentation, and nausea. He was started on vancomycin and piperacillin/tazobactam for empiric coverage with three percutaneous drains subsequently placed in the multiloculated fluid collections with purulent discharge expressed. Cultures of the output demonstrated no growth. His post-obstructive diuresis was managed with replacement Lactated Ringers’ solution at 75% of the rate of Foley output. He demonstrated complete improvement in leg swelling, heart rate, and WBC and creatinine normalized to 1.1 mg/dL with discharge to home in seven days with close urology follow-up for his prostatic abnormality.

This case of obstructive uropathy extremis, probable cystocerebral syndrome (hypertension, altered mental status, and bladder distension in the elderly), and bilateral urinomas leading to inferior vena cava syndrome is unique to the literature (1). The rupture of the renal fornices, the most delicate and purported “pressure check valve” of the renal conduits, can precipitate the formation of localized urinomas within the perinephric and retroperitoneal space, most commonly unilateral from ureteral or kidney stones or tumor related obstruction, rarely bilateral from bladder outlet obstruction (2). These urinomas, as seen in this case can exert a progressively escalating pressure on the contiguous inferior vena cava, which has been described as “inferior vena cava syndrome” (3). The implications of this pressure increase are manifold, encompassing the observed clinical manifestations ranging from lower extremity edema, worsening kidney perfusion, to hemodynamic instability (3). Management centers upon decompression of the obstruction, treatment of secondary infection with source control, and compensation for post-obstructive diuresis with generally favorable prognosis if recognized early in the clinical course.

Nathan Walton MD¹, Elizabeth Mata MD¹, Max Hart MD¹, Matthew Borchart MD²and Adnan Abbasi MBBS³

¹Internal Medicine Program, University of Arizona College of Medicine – Tucson

²Department of Medicine, Pulmonary & Critical Care, University of Arizona College of Medicine – Tucson

³Department of Medicine, Pulmonary & Critical Care, Southern Arizona Veterans Affairs Health Care System (SAVAHCS) – Tucson

References

1. Blackburn T, Dunn M. Cystocerebral syndrome. Acute urinary retention presenting as confusion in elderly patients. Arch Intern Med. 1990 Dec;150(12):2577-8. [CrossRef][PubMed]
2. Gershman B, Kulkarni N, Sahani DV, Eisner BH. Causes of renal forniceal rupture. BJU Int. 2011 Dec;108(11):1909-11; discussion 1912. [CrossRef][PubMed]
3. Lawrensia S, Khan YS. Inferior Vena Cava Syndrome. 2023 May 20. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan–. [PubMed]

Cite as: Walton N, Mata E, Hart M,  Borchart M, Abbasi A. November 2023 Medical Image of the Month: Obstructive Uropathy Extremis. Southwest J Pulm Crit Care Sleep. 2023;27(5):56-58. doi: https://doi.org/10.13175/swjpccs043-23 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

September 2023 Medical Image of the Month: Aspergillus Presenting as a Pulmonary Nodule in an Immunocompetent Patient

Figure 1. Chest CT showing 11 x10 mm nodule in the anterior segment of the left upper lobe in the background of emphysematous and basal sub segmental atelectatic changes.

Figure 2. Lung biopsy low power (A) showing chronic inflammatory infiltrate in the interstitium along with a collection of fungus (arrow) (H&E: x40). Fungus with an area of necrosis (B) (H&E: x100). Numerous thin, narrow-angle, and branching hyphae with septa morphologically consistent with Aspergillus (C) (H&E: x400). Collection of Aspergillus (D). (Periodic acid–Schiff stain: x400).

A 32-year-old nonsmoking woman presented with complaints of recurrent hemoptysis for 5 months and dyspnea on exertion for 1 month. She denied any history of fever, cough, or COVID infection. She has hypothyroidism controlled on thyroxine 25mcg. During the evaluation, she was found to have an enhancing solitary pulmonary nodule (11 x 10 x 9mm) in the anterior segment of the left upper lobe (Figure 1). The patient was given a course of oral antibiotics (amoxicillin /clavulanic acid) and supportive treatment for hemoptysis. Sputum for Ziehl–Neelsen stain and cartridge based nucleic acid amplification test (CBNAAT) was negative. CT- guided biopsy of the nodule was performed. Histopathology showed fungal organisms which were thin, septate with acute angle branching and focal necrotic areas, morphologically consistent with Aspergillus (Figure 2). Serum-specific IgG against aspergillus antigen was normal. The patient was started on oral itraconazole 200mg BID. Follow-up after 1 month showed both symptomatic and radiological improvement. Repeat chest CT showed a significant decrease in size of the nodule.

There is a large spectrum of pulmonary aspergillosis. From this spectrum, pulmonary nodules are a less common manifestation of chronic pulmonary aspergillosis (CPA), especially in immunocompetent individuals. Aspergillus nodules are defined as small, round, discrete, and focal opacities on chest imaging. It can be further classified on basis of internal cavitation (i.e., non-cavitary nodules and cavitary nodules). Differentiating these nodules from other lung pathology may be difficult on CT findings alone and may demand further investigation like image-guided needle aspiration cytology or biopsy, blood investigations like serum Aspergillus precipitin IgG antibody and/or serum Aspergillus galactomannan. Delay in diagnoses may lead to persistence of pulmonary symptoms, and cavitation of the nodule. This entity has a favorable prognosis if managed accordingly. Although there is data regarding surgical management of aspergillus nodules, but data regarding the benefits of anti-fungal therapy in the same is limited.

Diagnosing aspergillus nodules in an immunocompetent individual is a challenge to all pulmonologists. Literature shows limited case reports and small case series on CPA presenting as non-cavitating SPN on radiology. Usually, in such cases, the diagnosis is made following removal or biopsy of the nodule(s), presuming it to be malignant. Patients diagnosed with Aspergillus nodules can’t be differentiated from lung malignant conditions based on demographics, which are usually similar. In the largest case series of Aspergillus nodules done by Muldoon EG et al. (6), 33 patients were reviewed constituting less than 10 % of the cohort of patients with CPA.  In a study done by Kang et al. (4) 77% of patients with aspergillus nodules were symptomatic and the most common symptom reported was hemoptysis. Similarly in our case hemoptysis was the chief complaint of the patient. Our patient is a woman and non-smoker similar to previous case reports and series.

In the current guidelines, the detection of serum Aspergillus precipitin IgG antibody is a key diagnostic criterion for CPA. Literature is unclear if the presence of Aspergillus IgG antibody could be considered a supportive finding in the making the diagnosis of Aspergillus nodules. Similarly, in our case also serum specific IgG against Aspergillus fumigatus was negative. Azoles are the primary treatment option in all subtypes of CPA including aspergillus nodule. Our patient also showed disease regression during itraconazole treatment. Another option for management is surgical, though it is associated with significant postoperative complications and recurrence of disease at other sites and must be reserved for selected patients.

Dr. Deependra Kumar Rai, Dr. Priya Sharma, Dr. Vatsal Bhushan Gupta

Department of Pulmonary, Critical Care, and Sleep Medicine

AIIMS Patna, Bihar, India

References

1. Kosmidis C, Denning DW. The clinical spectrum of pulmonary aspergillosis. Thorax. 2015 Mar;70(3):270-7. [CrossRef] [PubMed]
2. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008 Mar;246(3):697-722. [CrossRef] [PubMed]
3. Lee SH, Lee BJ, Jung DY, Kim JH, Sohn DS, Shin JW, Kim JY, Park IW, Choi BW. Clinical manifestations and treatment outcomes of pulmonary aspergilloma. Korean J Intern Med. 2004 Mar;19(1):38-42. [CrossRef] [PubMed]
4. Kang N, Park J, Jhun BW. Clinical Characteristics and Treatment Outcomes of Pathologically Confirmed Aspergillus Nodules. J Clin Med. 2020 Jul 10;9(7):2185. [CrossRef] [PubMed]
5. Yasuda M, Nagashima A, Haro A, Saitoh G. Aspergilloma mimicking a lung cancer. Int J Surg Case Rep. 2013;4(8):690-2. [CrossRef] [PubMed]
6. Muldoon EG, Sharman A, Page I, Bishop P, Denning DW. Aspergillus nodules; another presentation of Chronic Pulmonary Aspergillosis. BMC Pulm Med. 2016 Aug 18;16(1):123. [CrossRef] [PubMed]
7. Denning DW, Cadranel J, Beigelman-Aubry C, et al. Chronic pulmonary aspergillosis: rationale and clinical guidelines for diagnosis and management. Eur Respir J. 2016 Jan;47(1):45-68. [CrossRef] [PubMed]
8. Limper AH, Knox KS, Sarosi GA, et al. An official American Thoracic Society statement: Treatment of fungal infections in adult pulmonary and critical care patients. Am J Respir Crit Care Med. 2011 Jan 1;183(1):96-128. [CrossRef] [PubMed]
9. Godet C, Philippe B, Laurent F, Cadranel J. Chronic pulmonary aspergillosis: an update on diagnosis and treatment. Respiration. 2014;88(2):162-74. [CrossRef] [PubMed]
10. Kousha M, Tadi R, Soubani AO. Pulmonary aspergillosis: a clinical review. Eur Respir Rev. 2011 Sep 1;20(121):156-74. [CrossRef] [PubMed]

Cite as: Rai DK, Sharma P, Gupta VB. September 2023 Medical Image of the Month: Aspergillus Presenting as a Pulmonary Nodule in an Immunocompetent Patient. Southwest J Pulm Crit Care Sleep;27(3):30-32. doi: https://doi.org/10.13175/swjpccs035-23 PDF

August 2023 Medical Image of the Month: Cannonball Metastases from Metastatic Melanoma

Figure 1. Upright PA chest radiograph (A) demonstrates innumerable rounded nodules with a slight lower lung predilection. Coronal reconstruction from a subsequent chest CT with contrast (B) confirms innumerable round, solid and non-calcified pulmonary nodules most consistent with a “cannonball” pattern of pulmonary metastatic disease.

Figure 2. Axial susceptibility-weighted (A) and pre-contrast T1-weighted (B) images from a brain MRI centered on one (of many) intracranial lesions. This lesion in the posterior right cerebral hemisphere demonstrates a large amount of surrounding susceptibility artifact (A) consistent with blood product, indicating a hemorrhagic metastasis, common in metastatic melanoma which can be very vascular in nature.  There is elevated T1 signal surrounding the lesion (B), also consistent with hemorrhage. There is T1 signal in the center of the lesion as well (arrow), which could represent hemorrhage but could also represent melanin, which is T1 bright and can suggest the primary tumor histology (melanoma).

A 48-year-old man with a significant smoking history presented with progressive dyspnea, a mildly productive cough with brown-tinged sputum, headache, weight loss, and progressive back pain over a period of 8 weeks. The patient had no known history of lung conditions. As part of their initial evaluation, the patient received a frontal CXR and was found to have innumerable rounded pulmonary nodules throughout the lungs, which were consistent with a “cannonball” pattern of pulmonary metastatic disease (Figure 1A). A subsequent CT confirmed innumerable pulmonary metastases (Figure 1B). An MRI of the brain and spine revealed hemorrhagic brain metastases (Figure 2), spinal metastases, and bone metastases. A biopsy of a lesion in the left iliac bone confirmed the diagnosis of metastatic melanoma.

This case highlights the so-called “cannonball” pattern of pulmonary metastatic disease with innumerable round nodules throughout the lungs with a slight lower lung predilection, consistent with a hematogenous spread of disease to the lungs. Classically, this pattern consists of numerous well-circumscribed relatively large lesions in the lungs with a pattern resembling multiple small "cannonballs". The distribution of nodules is random, and the factors that distinguish a cannonball pattern from a miliary pattern of pulmonary metastatic disease are that cannonball metastases are larger and less numerous compared to a miliary pattern of pulmonary metastatic disease (1,2). Although cannonball pattern of pulmonary metastatic disease is classically described in the setting of metastatic renal cell carcinoma, prostate cancer, choriocarcinoma, and sarcoma, it has been reported for a variety of other primary cancers, including melanoma. Similar cases of late-stage metastatic melanoma presenting as widely metastatic disease of unknown origin has also been reported (3).

Melanoma spreads to the lungs through hematogenous dissemination, resulting in the cannonball (or miliary) lesions observed on imaging. Specifically, when these lesions are small and distributed throughout the lung, they commonly indicate metastatic melanoma. While this classical presentation can be pathognomonic, it is important to consider other potential diagnoses, including malignancy, infectious diseases, and rheumatologic conditions. Malignant causes encompass metastatic colorectal cancer, breast cancer, renal cell carcinoma, choriocarcinoma, prostate carcinoma, and adrenal carcinoma. Additionally, non-cancerous causes include infectious etiologies such as septic emboli, coccidiomycosis, histoplasmosis, miliary tuberculosis, and nocardiosis. It is also important to note that rheumatologic conditions like granulomatosis with polyangiitis (Wegener's granulomatosis) can present with the cannonball sign; however, they typically involve both the upper and lower lungs. In this case, the lesions were determined to be due to metastatic melanoma, which had spread to the lungs via hematogenous dissemination.

For this patient, an oncologist was consulted, who recommended that the patient undergo treatment with ipilimumab and nivolumab. The response rate for this treatment regimen generally exceeds 50%, and more than 50% of individuals continue to survive after 5 years. Frequently, when extensive metastases are observed on imaging, healthcare providers often assume a limited life expectancy and tend to lean towards discussions of hospice care. However, it is important to keep in mind that even in cases of extensive metastatic disease, many cancers can be effectively treated with novel and highly effective immunologic anticancer medications.

Andrew Barsoum MD, Mueez Hussain MD, Ranjit Sivanandham MD, and Sina Bagheri MD.

Southwest Healthcare System

Murrieta, California 

References

1. Ammannagari N, Polu V. 'Cannon ball' pulmonary metastases. BMJ Case Rep. 2013 Jan 8;2013:bcr2012008158. [CrossRef][PubMed]
2. Andreu J, Mauleón S, Pallisa E, Majó J, Martinez-Rodriguez M, Cáceres J. Miliary lung disease revisited. Curr Probl Diagn Radiol. 2002 Sep-Oct;31(5):189-97. [PubMed]
3. Lowe A, Bray JJH. Late-stage melanoma presenting with cannonball metastases. BMJ Case Rep. 2020 Dec 12;13(12):e237969. [CrossRef][PubMed]

Cite as: Barsoum A, Hussain M, Sivanandham R, Bagheri S. August 2023 Medical Image of the Month: Cannonball Metastases from Metastatic Melanoma. Southwest J Pulm Crit Care Sleep. 2023;27(2):23-25. doi: https://doi.org/10.13175/swjpccs025-23 PDF

July 2023 Medical Image of the Month: Primary Tracheal Lymphoma

Figure 1. Frontal (A) and lateral (B) x-rays demonstrate a large density disrupting the tracheal air column (arrows), a reminder of how important it can be to evaluate the trachea on radiographs. The finding arises from the left aspect of the mid trachea, causing severe narrowing (arrowheads). To view Figure 1 in a separate, enlarged window click here.

Figure 2. Axial reconstruction from a contrast-enhanced chest CT (A) demonstrates an exophytic mass arising from the left aspect of the upper to mid trachea, causing severe tracheal stenosis. Centerline (B) and 3D Volume-rendered (C) reconstructions from a subsequent pre-bronchoscopy planning noncontrast CT scan demonstrate a mass arising from the left lateral wall of the trachea (arrows) involving slightly more than 5 cm of tracheal length. To view Figure 2 in a separate, enlarge window click here.

Figure 3. Bronchoscopic images of the trachea prior to (A) and following (B) piecemeal electrocautery for de-bulking of a fungating exophytic mass in the mid trachea. The procedure was performed to alleviate the patient’s severe symptoms related to severe tracheal stenosis. To view Figure 3 in a separate, enlarged window click here.

A 72-year-old woman was referred to our practice from an outside hospital with a history of tracheal mass with impending airway compromise. She originally presented with several days of dyspnea, stridor, and an increasingly muffled voice. She also provided a history of severe shortness of breath, but only when eating. Interestingly, she had no trouble swallowing. CXR findings at the outside hospital (Figure 1) prompted a CT (Figure 2A), which further prompted transfer of patient to our facility. The patient had a history of hypertension, hyperlipidemia, type 2 diabetes and obstructive sleep apnea. Her past surgical history included C-section and hysterectomy. The patient was a never-smoker with no significant drinking or drug usage history. The patient had no recent travel or exposure history. Review of symptoms otherwise negative. Although the patient had no formal diagnosis of asthma, she was on Mometasone/formoterol, 2 puffs bid and also had an albuterol rescue inhaler. The patient was on various other medications for seasonal allergies, hyperlipidemia, hypertension, and diabetes. No significant allergy history.

Vital signs upon presentation were normal, aside from tachycardia, heart rate was 103 bpm. No fever, normal oxygen saturation. On physical exam, the patient had obvious stridor heard throughout the lungs. Physical exam was otherwise unremarkable. Initial lab testing consisted of a basic metabolic panel and a complete blood count, both of which were normal. A repeat CT scan was obtained at our institution for pre-procedural planning. That CT demonstrated an endotracheal mass with a significant intraluminal component causing near-complete obstruction (Figure 2B,C). Of note, slightly more than 5 cm of the tracheal length was involved with tumor. In order to obtain a tissue diagnosis and also due to impending airway compromise, ridged bronchoscopy was performed (Figure 3). A combination of suction and electrocautery were used to debulk the tumor in order to relieve airway obstruction. Histopathology was consistent with diffuse large B-cell lymphoma. A bone marrow biopsy was negative for any marrow involvement. A diagnosis of primary tracheal lymphoma was made. The patient was started on the appropriate chemotherapy and had a good response.

Primary tumors of the trachea are rare and make up less than 0.4% of all tumors, producing 0.1% of cancer deaths worldwide. Primary malignant tumors typically arise from surface epithelium or salivary glands (1). As such, squamous cell carcinoma and adenoid cystic carcinoma account for the 1st and 2nd most common primary malignant tumors of the trachea. Mucoepidermoid carcinoma, a salivary gland type tumor, also occurs in the trachea. Given the presence of APUD cells in the trachea, carcinoid tumors can also occur, although these are rare in the central airways, and more common in the segmental and subsegmental bronchi. Given the presence of cartilage in the wall of the trachea, chondrosarcoma of the trachea can also occur. While most of these tumor histologies have a relatively nonspecific appearance, chondrosarcomas can produce chondroid matrix, which, when present, allows for some specificity when imaged with CT. Malignant involvement of the trachea can also result from direct invasion by tumors arising from adjacent structures such as the thyroid, lung, esophagus, and larynx, or by hematogenous metastasis from distant organs such as kidney, breast, colon, and melanoma (1).

Non-Hodgkin lymphoma (NHL) involving the central airway is uncommon, even though the disease frequently involves extranodal organs. Primary tracheobronchial NHL occurs in less than 1% of all NHL patients (2). Involvement of central airways in NHL can cause respiratory failure (3). A case very similar to our own was reported recently (4). In that case a patient with primary tracheobronchial NHL suffered rare, life-threatening obstruction of the central airway that prevented chemotherapy. To alleviate the airway obstruction, interventional bronchoscopy involving cryotherapy, electrocautery and argon plasma coagulation was performed, allowing administration of chemotherapy.

Clinton Jokerst MD, Matthew Stib MD, Carlos Rojas MD, Prasad Panse MD, Kris Cummings MD, Eric Jensen MD and Michael Gotway MD

Department of Radiology

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Bedayat A, Yang E, Ghandili S, Galera P, Chalian H, Ansari-Gilani K, Guo HH. Tracheobronchial Tumors: Radiologic-Pathologic Correlation of Tumors and Mimics. Curr Probl Diagn Radiol. 2020 Jul-Aug;49(4):275-284. [CrossRef] [PubMed]
2. Solomonov A, Zuckerman T, Goralnik L, Ben-Arieh Y, Rowe JM, Yigla M. Non-Hodgkin's lymphoma presenting as an endobronchial tumor: report of eight cases and literature review. Am J Hematol. 2008 May;83(5):416-9. [CrossRef] [PubMed]
3. Tan DS, Eng PC, Lim ST, Tao M. Primary tracheal lymphoma causing respiratory failure. J Thorac Oncol. 2008 Aug;3(8):929-30. [CrossRef] [PubMed]
4. Yang FF, Gao R, Miao Y, Yan XJ, Hou G, Li Y, Wang QY, Kang J. Primary tracheobronchial non-Hodgkin lymphoma causing life-threatening airway obstruction: a case report. J Thorac Dis. 2015 Dec;7(12):E667-71. [CrossRef] [PubMed]

Cite as: Jokerst C, Stib M, Rojas C, Panse P, Cummings K, Jensen E, Gotway MB. July 2023 Medical Image of the Month: Primary Tracheal Lymphoma. Southwest J Pulm Crit Care. 2023;27(1):4-6. doi: https://doi.org/10.13175/swjpccs026-23 PDF

April 2023 Medical Image of the Month: Atrial Myxoma in the setting of Raynaud’s Phenomenon: Early Echocardiography and Management of Thrombotic Disease

Figure 1. ECG demonstrating sinus bradycardia and T-wave inversion in lead III and aVF.

Figure 2.  Transthoracic echo apical four-chamber view (zoomed) demonstrating 2.3 x 2.6 cm echogenic mass of the left atrium. LV = left ventricle. RA = right atrium. LA = left atrium.

Figure 3.  Transesophageal echo, midesophageal long axis view demonstrating 2.5 x 1.71 cm echogenic left atrial mass attached to upper dome of the left atrium. LA = left atrium. LV = left ventricle.

A 43-year-old woman presents to the Emergency Department (ED) with right-sided weakness and numbness for several hours. Medical history is significant for Raynaud’s Phenomenon (RP), initially presenting six months prior to presentation, manifesting as intermittent episodes of painless discoloration of multiple fingers.  Cardiac exam was unremarkable with regular rhythm and no discernable murmur. Neurological exam demonstrated right arm pronator drift. Other examination findings were unremarkable. Labs demonstrated a troponin of 0.00 ng/mL, C-reactive protein of 2.28 mg/dL, and an erythrocyte sedimentation rate of 40 mm/hr. The electrocardiogram (ECG) demonstrated sinus bradycardia and notable for T-wave inversion in lead III and aVF, but without any ST-segment deviations (Figure 1).  Magnetic Resonance Imagining (MRI) of the brain demonstrated acute ischemic left frontal, left parietal, and right parietal infarcts along with mild subcortical left parietal infarct, concerning for venous or watershed distal embolic arterial infarct. MRI Angiogram of the brain showing diminutive bilateral, lateral transverse dural venous sinuses, consistent with thrombus. The patient’s neurological deficits resolved within five hours of ED arrival. Given the background diagnoses of RP and new thrombosis, a complete autoimmune and hypercoagulability workup was pursued and was otherwise negative.

As part of acute stroke work-up, the patient also underwent transthoracic echocardiogram (TTE) with a bubble study, which was significant for left atrial (LA) echogenic intracardiac structure attached to the superior part of the LA (Figure 2). Transesophageal Echocardiogram (TEE) was performed which demonstrated a large, 2.5 x 1.71 cm mass, consistent with an atrial myxoma, not appearing to involve the interatrial septum but instead thought to originate from the upper dome of the atrium immediately adjacent to the pulmonary veins (Figure 3). Patient was also evaluated by neurology and started on anticoagulation with parental continuous unfractionated heparin infusion given the dural venous sinus thrombosis and a possible hypercoagulable state due to the underlying myxoma. Patient underwent surgical resection of the atrial mass Histopathological examination of the resected mass was consistent with the diagnosis of atrial myxoma.

Although atrial myxomas are the most common primary cardiac tumor, clinical presentation ranges from incidental imaging findings to profound life-threading cardiovascular manifestations (1). This range of presentation is closely associated with size, mobility, and location (2). Pinede et al. studied 112 cases of atrial myxomas and reported that signs of cardiac obstruction were the primary manifestation of LA myxoma. Approximately, 67% of patients presented with signs of cardiac obstruction, such as heart failure, syncope, or myocardial infarction, while embolic signs were only present in 29% of patients. Systemic signs including fever and weight loss were only reported in 34% of patients with only 5% of patients having associated connective tissue disease (3). Rarely, RP has been described as the primary presenting symptom of atrial myxoma (4,5), underscoring the utility of maintaining a high degree of suspicion when symptomatology coexists.

RP is a vascular response to stress or cold temperature that appears as color changes in the digits (6). Although primary RP has no known underlying etiology, it is more commonly seen in female patients with a history of smoking, migraine headaches, and cardiovascular disease (6). This is in contrast to secondary RP, which presents in patient with an underlying autoimmune rheumatic disease including, but not limited, to Systemic Sclerosis, Mixed Connective Tissue Disease, Systemic Lupus Erythematosus, Sjogren’s Syndrome, and hematologic disorders such as Cryoglobulinemia, Cold Agglutinins Disease, and Paraproteinemia (7).

Atrial myxoma may rarely make its initial appearance under the guise of RP (4). This phenomenon is likely attributable to overproduction of IL-6 by the myxoma (9-11). Our patient presented with RP six months prior to her presentation to the ED with right-sided weakness and numbness and a complete autoimmune and hypercoagulability workup was negative; this may suggest that the underlying pathophysiology of her RP is the associated overproduction of IL-6 by the atrial myxoma.

TTE may be considered in the initial diagnostic evaluation of a patient presenting with RP without additional findings suggestive of secondary etiologies. Given that myxomas are typically localized within the atrial lumen, transthoracic echocardiography is a highly sensitive modality for diagnosis, whereas CT and MRI may also help in diagnostics in uncertain cases. Once suspicion of a cardiac myxoma has been supported by imaging modalities, surgical removal of the tumor should be performed as soon as possible due to the risk of myxoma associated embolic episodes (5).  Post intervention, long term prognosis is excellent with an approximated 5% rate of recurrence (3). Long-term follow-up with serial TTE are recommended, particularly in younger patients (3) but there is no specific guideline regarding the frequency of TTE surveillance post atrial myxoma resection.

Ali A. Mahdi MD, Chris Allahverdian MD, Vishal Patel MD, Serap Sobnosky MD

Dignity Health, St. Mary Medical Center, Department of Internal Medicine, Long Beach, CA

References

1. Roberts WC. Primary and secondary neoplasms of the heart. Am J Cardiol. 1997 Sep 1;80(5):671-82. [CrossRef] [PubMed]
2. Zaher MF, Bajaj S, Habib M, Doss E, Habib M, Bikkina M, Shamoon F, Hoyek WN. A giant left atrial myxoma. Case Rep Med. 2014;2014:819052. [CrossRef]
3. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore). 2001 May;80(3):159-72. [CrossRef] [PubMed]
4. Skanse B, Berg No, Westfelt L. Atrial myxoma with Raynaud's phenomenon as the initial symptom. Acta Med Scand. 1959 Jul 25;164:321-4. [CrossRef] [PubMed]
5. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996 Jan 1;77(1):107. [CrossRef] [PubMed]
6. Abdulla, M. C., & Alungal, J. (2015). Atrial myxoma in a primigravida presenting as Raynaud’s phenomenon. Rheumatology Reports, 7(1). [CrossRef]
7. Prete M, Favoino E, Giacomelli R, et al. Evaluation of the influence of social, demographic, environmental, work-related factors and/or lifestyle habits on Raynaud's phenomenon: a case-control study. Clin Exp Med. 2020 Feb;20(1):31-37. [CrossRef] [PubMed]
8. Khouri C, Blaise S, Carpentier P, Villier C, Cracowski JL, Roustit M. Drug-induced Raynaud's phenomenon: beyond β-adrenoceptor blockers. Br J Clin Pharmacol. 2016 Jul;82(1):6-16. [CrossRef] [PubMed]
9. Jourdan M, Bataille R, Seguin J, Zhang XG, Chaptal PA, Klein B. Constitutive production of interleukin-6 and immunologic features in cardiac myxomas. Arthritis Rheum. 1990 Mar;33(3):398-402. [CrossRef] [PubMed]
10. Saji T, Yanagawa E, Matsuura H, Yamamoto S, Ishikita T, Matsuo N, Yoshirwara K, Takanashi Y. Increased serum interleukin-6 in cardiac myxoma. Am Heart J. 1991 Aug;122(2):579-80. [CrossRef] [PubMed]
11. Parissis JT, Mentzikof D, Georgopoulou M, Gikopoulos M, Kanapitsas A, Merkouris K, Kefalas C. Correlation of interleukin-6 gene expression to immunologic features in patients with cardiac myxomas. J Interferon Cytokine Res. 1996 Aug;16(8):589-93. [CrossRef] [PubMed]

Cite as: Mahdi AA, Allahverdian C, Patel V, Sobnosky S. April 2023 Medical Image of the Month: Atrial Myxoma in the setting of Raynaud’s Phenomenon: Early Echocardiography and Management of Thrombotic Disease. Southwest J Pulm Crit Care Sleep. 2023;26(4):56-58. doi:https://doi.org/10.13175/swjpccs006-23 PDF

April 2023 Imaging Case of the Month: Large Impact from a Small Lesion

Michael B. Gotway MD

Department of Radiology

Mayo Clinic, Arizona

Phoenix, Arizona USA

History of Present Illness: A 65-year-old woman with a history of diabetes mellitus complained of worsening fatigue with a 20 lbs. weight gain over the last year as well as shortness of breath. The patient also complained of bruising without recalling specific injury and complained her complexion had changed recently, becoming “ruddier”, accompanied by increasing growth of facial hair. Her past medical history was remarkable for hypertension, including a previous hospitalization for a hypertensive emergency. The patient’s diabetes had become more difficult to control in recent months, with labile blood glucose levels requiring escalating insulin doses. The patient denied recent changes in sleep, worsening anxiety or depression, or changes in mood.

PMH, SH, FH: The patient’s past medical history was also notable for diastolic dysfunction and hyperlipidemia, and she required oxygen use at night. Her past surgical history was significant for a previous hysterectomy and a knee arthroplasty. Her family history was unremarkable.

Medications: Her medications included insulin, pravastatin, lisinopril, metformin, aspirin, furosemide, felodipine, citalopram, and potassium supplementation.

Physical Examination: The patient’s physical examination showed her to be afebrile with pulse rate and blood pressure within the normal range at 128/75 mmHg. She was obese (113 kg) and her facial complexion was indeed ruddy with a rounded appearance. The patient’s skin appeared somewhat thin and several bruises were noted over her extremities. Her lungs were clear and her cardiovascular examination

was normal.

Laboratory Evaluation:  A complete blood count showed normal findings. The patient’s plasma glucose was elevated at 171 mg/dL (normal, 65-95 mg/dL) Her hemoglobin A1c was 9.4% (normal, 4-5.6%). The white blood cell count was normal with no left shift and her liver function studies were entirely normal. Serum chemistries were completely within normal limits aside from a borderline elevated blood urea nitrogen level of 20 mg/dL (normal, 6-20 mg/dL) serum creatinine was normal.

Radiologic Evaluation: Frontal chest radiography (Figure 1) was performed.

Figure 1. Frontal chest radiography.

Which of the following statements regarding this chest radiograph is accurate? (Click on the correct answer to be directed to the next page)

1. Frontal chest radiography shows normal findings
2. Frontal chest radiography shows cardiomegaly
3. Frontal chest radiography shows mediastinal lymphadenopathy
4. Frontal chest radiography shows pleural effusion
5. Frontal chest radiography shows several nodules

Cite as: Gotway MB. April 2023 Imaging Case of the Month: Large Impact from a Small Lesion. Southwest J Pulm Crit Care Sleep. 2023;26(4):48-55. doi: https://doi.org/10.13175/swjpccs014-23 PDF

December 2022 Medical Image of the Month: Bronchoesophageal Fistula in the Setting of Pulmonary Actinomycosis

Figure 1. Axial (A) and sagittal (B) reconstructions from a contrast-enhanced chest CT demonstrates an ill-defined low-attenuation subcarinal mass (*) which causes deformity of the left mainstem bronchus (LMSB) (arrow). Axial reconstruction from a repeat contrast-enhanced CT performed 6 days later (C) demonstrates a gas-filled fistulous tract between the LMSB and esophagus through the mass (arrowheads).  An esophogram (D) performed 24 hours after esophageal stent placement demonstrates occlusion of the fistula.

A 65-year-old woman, never smoker with hypothyroidism, hypertension, anxiety, and depression disorders, initially presented to the emergency department with progressive nonspecific chest discomfort for two days. She had CT Angio, which was negative for PE but showed a 4.6 cm subcarinal centrally necrotic nodal mass (Figure 1A-B). She was subsequently advised to follow up with her primary care physician. A week later, she attended our emergency department again with a new intermittent cough and one episode of non-bloody emesis. She reported a sensation of drowning with the intake of liquids and subsequent intractable coughing. Otherwise, she did not have other associated symptoms such as shortness of breath, abdominal pain, fever, sweats, or chills.

Vital signs and physical exam were unremarkable. A repeat chest CT was performed, which demonstrated internal cavitation of the subcarinal mass with fistulous communication between the lumen of the midthoracic esophagus and the proximal left mainstem bronchus posteriorly, suggestive of broncho-esophageal fistula (Figure 1C). She subsequently underwent bronchoscopy, which revealed areas of friable bronchial mucosal nodularity along the posterior membrane of the mid to distal left mainstem bronchus. Despite a thorough airway inspection, no clear fistula was observed, and no gastric or bilious material was seen within the airway. She underwent endobronchial ultrasound (EBUS) with transbronchial nodal aspiration (TBNA) of the mediastinal lymphadenopathy, which showed extensive necrotic debris and granulomatous inflammation; however, Giemsa stain was negative and no sulfur granules were observed. An upper endoscopy was performed in tandem with the bronchoscopy. The EGD identified a cratered esophageal ulcer in the mid esophagus, which was biopsied. As well, a 25 mm fistulous track was found within the ulcerated region, and thus, an esophageal stent was placed. An esophagogram performed the next day showed no evidence of a leak (Figure 1D), which is suggestive of successful occlusion of the fistula. The esophageal biopsy was negative for malignancy though it also revealed ulcerated squamous mucosa with marked acute and chronic inflammation with reactive granulation tissue.

Infectious workup included Legionella urinary antigen, Streptococcus pneumoniae urinary antigen, MRSA nasal screen, serum Aspergillus antigen, coccidiomycosis  IgG/IgM (by EIA and CF/ID), QuantiFERON TB gold, and beta-D-glucan, all of which were negative. Histoplasma urinary antigen, Histoplasma and Blastomyces serum antibodies were also negative. Anaerobic cultures from lymph node aspirate later grew Actinomycetes.

Infectious disease was consulted, and the patient was started on ceftriaxone 2 g IV daily for three weeks, for pulmonary actinomyces infection, with a plan to transition to oral amoxicillin 750 mg three times a day for six months. She had a clinic follow-up appointment in eight weeks, in which she reported complete resolution of her symptoms.

Actinomycetes are branching gram-positive anaerobic bacteria and rarely cause infection, with only about 1 in 300,000 cases reported per year (1). Infections can involve any organ system, with pulmonary actinomycosis being the third most common location, representing around 15 % of the total disease cases (2). Actinomyces species are part of normal flora found in the mouth and gastrointestinal tract; therefore, it is hypothesized that pulmonary actinomycosis is caused by aspiration (3).

Diagnosis by clinical features alone can be challenging as it shares many symptoms associated with chronic infections like a low-grade fever, sputum production, cough and malaise. Therefore, it may be wrongfully diagnosed as tuberculosis, lung abscess and fungal infection. It can also often be confused with malignancy. Mabeza et al. (4) reported that around a quarter of cases with thoracic actinomyces were initially thought to have carcinoma.

Image findings of pulmonary actinomyces are also quite diverse. A retrospective study of 94 patients diagnosed with pulmonary actinomycosis pathologically over ten years in Korea revealed that the most common chest CT finding was consolidation (74.5%), mediastinal or hilar lymph node enlargement (29.8%), atelectasis (28.7%), cavitation (23.4%), ground-glass opacity (14.9%), and pleural effusion (9.6%) (5). Actinomyces can spread from the lung to the pleura, mediastinum, and chest wall. It is hypothesized that the mechanism behind their ability to travel through these anatomical barriers is due to their ability to produce proteolytic enzymes (6). Given its indolent presentation, proper diagnosis and treatment may be delayed leading to the involvement of adjacent structures and potentially life-threatening complications, including massive hemoptysis or bronchoesophageal fistula formation.

Detection of ‘sulfur’ granules histologically has been previously described as the hallmark for the diagnosis; however, they can also be found in other infections like nocardiosis (7), and they are only observed in 50% of cases; therefore, their absence does not exclude actinomycosis. Culture confirmation is typically clinically difficult because of inadequate anaerobic conditions, prior antibiotic therapy, or overgrowth of concomitant organisms (2). 

The principal treatment for pulmonary actinomycosis has been penicillin; however, there are no well-established guidelines regarding the duration of antibiotic therapy. High-dose intravenous penicillin is usually used for four to six weeks, followed by six to twelve months of oral amoxicillin in most cases (9). Surgery is typically reserved for pulmonary actinomycosis complicated by abscesses, empyemas, discharging fistulas and sinuses, life-threatening hemoptysis, exclusion of malignancy, and for patients who do not respond to antibiotic therapies (10).

John Fanous MD¹, Nikita Ashcherkin MD², Michael Gotway MD³, Kenneth Sakata, MD¹ and Clinton Jokerst MD³

Division of Pulmonology¹, Department of Internal Medicine², and Department of Radiology³

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Gajdács M, Urbán E, Terhes G. Microbiological and Clinical Aspects of Cervicofacial Actinomyces Infections: An Overview. Dent J (Basel). 2019 Sep 1;7(3):85. [CrossRef] [PubMed]
2. Han JY, Lee KN, Lee et al. An overview of thoracic actinomycosis: CT features. Insights Imaging. 2013 Apr;4(2):245-52. [CrossRef] [PubMed]
3. Park HJ, Park KH, Kim SH, Sung H, Choi SH, Kim YS, Woo JH, Lee SO. A Case of Disseminated Infection due to Actinomyces meyeri Involving Lung and Brain. Infect Chemother. 2014 Dec;46(4):269-73. [CrossRef] [PubMed]
4. Mabeza GF, Macfarlane J. Pulmonary actinomycosis. Eur Respir J. 2003 Mar;21(3):545-51. [CrossRef] [PubMed]
5. Kim SR, Jung LY, Oh IJ, et al. Pulmonary actinomycosis during the first decade of 21st century: cases of 94 patients. BMC Infect Dis. 2013 May 14;13:216. [CrossRef] [PubMed]
6. Heo SH, Shin SS, Kim JW, Lim HS, Seon HJ, Jung SI, Jeong YY, Kang HK. Imaging of actinomycosis in various organs: a comprehensive review. Radiographics. 2014 Jan-Feb;34(1):19-33. [CrossRef] [PubMed]
7. Brown JR. Human actinomycosis. A study of 181 subjects. Hum Pathol. 1973 Sep;4(3):319-30. [CrossRef] [PubMed]
8. Zhang AN, Guss D, Mohanty SR. Esophageal Stricture Caused by Actinomyces in a Patient with No Apparent Predisposing Factors. Case Rep Gastrointest Med. 2019 Jan 2;2019:7182976. [CrossRef] [PubMed]
9. Valour F, Sénéchal A, Dupieux C, et al. Actinomycosis: etiology, clinical features, diagnosis, treatment, and management. Infect Drug Resist. 2014 Jul 5;7:183-97. [CrossRef] [PubMed]
10. LoCicero J 3rd, Shaw JP, Lazzaro RS. Surgery for other pulmonary fungal infections, Actinomyces, and Nocardia. Thorac Surg Clin. 2012 Aug;22(3):363-74. [CrossRef] [PubMed]

Cite as: Fanous J, Ashcherkin N, Gotway M, Sakata K, Jokerst C. December 2022 Medical Image of the Month: Bronchoesophageal fistula in the Setting of Pulmonary Actinomycosis. Southwest J Pulm Crit Care Sleep. 2022;25(6):97-100. doi: https://doi.org/10.13175/swjpccs047-22 PDF 

September 2022 Medical Image of the Month: Epiglottic Calcification

Figure 1. Lateral (A) and frontal (B) topogram from a neck CT demonstrates linear calcifications in the expected location of the epiglottis (black arrows).  Sagittal multiplanar reconstructions demonstrate prominent calcification of the epiglottis (white arrow).

In consideration of dysphagia, most cases predominate in the oropharyngeal region with the remainder caused primary by esophageal causes. Lesser known and studied is the development of dysphagia and globus sensation from epiglottic pathology, namely epiglottic calcification. With less than a dozen published cases in literature, very little data exists on identification, diagnosis, and treatment of this known cause of morbidity. Here we present a case of oropharyngeal dysphagia arising from a rare cause, epiglottic calcification.

An 81-year-old man with a history of aortic stenosis and carotid artery stenosis presented with worsening dysphagia over the course of one month. The patient reported significant dysphagia, initially to solids and subsequently to liquids causing a weight loss of over 50 pounds. Physical exam of the oropharynx and neck were unremarkable. A bedside swallow evaluation suggested mildly decreased hyolaryngeal movement, but no other significant abnormalities.  A barium swallow study revealed incomplete epiglottic excursion during the pharyngeal phase of swallowing. The patient then underwent evaluation with a contrast-enhanced esophagogram, which showed severe esophageal dysmotility and gastroesophageal reflux. A CT of the neck demonstrated calcification of the epiglottis without epiglottal enlargement. ENT was consulted, the patient underwent flexible fiberoptic laryngoscopy and also EGD with biopsy.  No other esophageal or gastric pathology were identified other than the epiglottic calcification. As no effective treatment is known at this time, the patient was changed to a modified diet with ongoing speech and swallow therapy as an outpatient.

Epiglottic calcification is a rare cause of dysphagia that is poorly understood in its etiology, clinical course and outcome (1). This case demonstrates that despite consultant team recommendations, no clear evaluation pathway or treatment currently exists. Currently, diagnosis can be accomplished with radiologic evaluation along with exclusion of other causes; however, no definitive treatments are available for this rare condition. Although the condition itself is rare, epiglottic calcification should be considered when other more common causes of significant dysphagia are ruled out.  

Shil Punatar DO¹, Dayoung Song MD¹, Azkaa Zaman DO¹, Benjamin Jiao DO², and Tilemahos Spyratos DO^1,3

¹Department of Internal Medicine, Franciscan Health, Olympia Fields, IL

²Department of Radiology, Franciscan Health, Olympia Fields, IL

³Department of Gastroenterology, Franciscan Health, Olympia Fields, IL

Reference

1. Günbey HP, Günbey E, Sayit AT. A rare cause of abnormal epiglottic mobility and dyspagia: calcification of the epiglottis. J Craniofac Surg. 2014 Nov;25(6):e519-21. [CrossRef[[PubMed]

Cite as: Punatar S, Song D, Zaman A, Jiao B, Spyratos T. September 2022 Medical Image of the Month: Epiglottic Calcification. Southwest J Pulm Crit Care Sleep. 2022;25(3):41-42. doi: https//doi.org/10.13175/swjpccs031-22 PDF

Medical Image of the Month: An Unexpected Cause of Chronic Cough

Figure 1. Axial image from a contrast-enhanced CT demonstrates a hollow, calcified structure in the bronchus intermedius with thickening suggesting inflammation in the surrounding bronchial wall (arrow).

 

Figure 2. Photograph of chicken bone fragment retrieved from bronchus intermedius during flexible bronchoscopy (A).  In retrospect, this fragment of bone is visible on the topogram from the chest CT (B) and is circled. Note the prominent notch that is visible on CT and on the actual bone fragment (arrowheads).

Sometimes it is as simple as it looks!  A previously healthy nonsmoking 40 years old man presented with a 7-month history of dry cough which was misdiagnosed as asthma. He had persistent cough despite appropriate asthma treatment including empiric PPIs. This patient had undergone extensive lab work up and evaluation; from negative viral and fungal panel, repeated pulmonary function tests which were within normal limits, chest x-rays, and CT scans which had shown small local calcification in the bronchus intermedius with significant thickening of the surrounding bronchial wall (Figure 1). The decision was made to proceed with flexible bronchoscopy, which yielded a chicken bone fragment with surrounding granulation tissue as shown in Figure 2A. In retrospect the bone is visible within the  bronchus intermedius on the topogram from the CT scan, see Figure 2B.

Foreign body aspiration in adults reported in low rates (0.66 per 100 000) (1). Despite being uncommon, neurological disorders, alcohol abuse, advanced age and altered level of consciousness all found to be the main underlying cause of foreign body aspiration in adults (2). Still, 10% of adult patients with foreign body aspiration have no known risk factors (3). Usually, diagnosis of foreign body aspiration in adults is straightforward only if the patient’s history involves aspiration or choking event. But, if the initial event goes unnoticed, the clinical picture maybe similar to obstructive lung diseases such as COPD or asthma.

Yazan Khair ¹, Hussam Al-Jawaldeh², Ayah AL Mufleh³ , Maxim Abu Joudeh⁴, Emad Hammode⁵  

¹Pulmonary department, Royal Medical Services (RMS), Amman, Jordan

²Internal Medicine Resident, Canyon Vista Medical Center, Sierra Vista, AZ USA                

³Internal Medicine transitional program, King Hussein Cancer Center, Amman, Jordan

⁴Internal Medicine Resident, Canyon Vista Medical Center, Sierra Vista, AZ USA

⁵Program Director of Canyon Vista Medical Center Internal Medicine program, Sierra Vista, AZ USA

References

1. Lund, ME. Foreign body removal in: Ernst A, Herth, FJF eds. Principles and Practice of Interventional Pulmonolgy. New York, NY: Springer; 2013:477-488.
2. Singh A, Kaur M. Recurrent pneumonitis due to tracheobronchial foreign body in an adult. JIACM, 2007:8:242-44.
3. Mise K, Jurcev Savicevic A, Pavlov N, Jankovic S. Removal of tracheobronchial foreign bodies in adults using flexible bronchoscopy: experience 1995-2006. Surg Endosc. 2009 Jun;23(6):1360-4. [CrossRef] [PubMed]

Cite as: Khair Y, Al-Jawaldeh H, Mufleh A, Joudeh M, Hammode E. Medical Image of the Month: An Unexpected Cause of Chronic Cough. Southwest J Pulm, Crit Care & Sleep. 2022;25(2):23-24. doi: https://doi.org/10.13175/swjpccs032-22 PDF 

July 2022 Medical Image of the Month: Pulmonary Nodule in the Setting of Pyoderma Gangrenosum (PG)

Figure 1. Unenhanced lung window chest CT images in the axial (A) and sagittal (B) planes show a solid, non-calcified irregular left upper lobe mass (arrow) with spiculated margins. The nodule demonstrates enhancement on soft tissue windows (C) with associated mediastinal adenopathy (arrowhead). The mass and adenopathy are FDG-avid on axial fused PET-CT image (D).

Figure 2.  (A) Photograph of one of the patient’s skin lesions.  (B) Hematoxylin and Eosin stained low-power pathological image of a biopsy specimen from a skin lesion demonstrates dense mixed neutrophilic dermal inflammation. Extensive infectious and neoplastic workup was negative. The histopathologic diagnosis was consistent with pyoderma gangrenosum.

A 70-year-old man presented with persistent cough productive of clear sputum which had persisted approximately 12 months after COVID-19 infection.  The patient reported a more recent history of night sweats and had also recently developed what he described as “blisters” on his chest wall and right shoulder starting 4 weeks prior to presentation that “opened up” giving off a bloody discharge.  The patient had been treated with trimethoprim-sulfamethoxazole and doxycycline without improvement and reported a 10-pound weight loss over the past several months.  The patient was a never-smoker with no significant travel history and a past medical history of asthma, GERD, gout, and chronic rhinitis. He had no history of autoimmune/inflammatory diseases or malignancy.

Vital signs and physical exam were normal, except for a 1 cm open wound in the center of the patient’s chest [Figure 2A].  A chest CT performed as part of the patient’s workup demonstrated a spiculated mass in the left upper lobe with adjacent mediastinal adenopathy [Figure 1A-C].  This prompted an FDG PET-CT, which demonstrated some increased uptake in the mass and adjacent lymph nodes [Figure 1D].  The mass was biopsied via bronchoscopy, pathology was nondiagnostic with rare groups of benign-appearing bronchial epithelial cells and blood.  The skin lesion was biopsied next demonstrating dense mixed neutrophilic dermal inflammation  [Figure 2B].  The diagnosis of pyoderma gangrenosum was made and the patient was treated with NSAIDs and a systemic glucocorticoid (40 mg/day, tapered over 10 weeks).steroid taper, The pulmonary mass , mediastinal lymph nodes and skin lesions all resolved over time.

Pyoderma gangrenous (PG) is a misnomer in every sense as it is neither infectious nor gangrenous.  It is a rare (3-10 cases/million/year) disorder of skin characterized by neutrophilic dermatosis which usually presents as a with inflammatory and ulcerative disorder of the skin lesions and is usually a diagnosis of exclusion (1).  PG has no pathognomonic clinical or histological findings. Majority of the cases have an underlying systemic disease, commonly inflammatory bowel disease (41%), inflammatory arthritis (20.5%) and oncologic or hematologic disorders (17.2%). While it can in any age group including children, the peak age of onset is 40-60 years. There is a slight female preponderance (2). The most common presentation is inflammatory papule or pustule that progress to a painful ulcer with violaceous undermined borders and a purulent base. The lesions commonly occur in surgical wounds within 2 weeks of surgery, a phenomenon known as pathergy, and often lead to wound dehiscence (3). The lesions may also be peristomal in patients with IBD. Extracutaneous lesions have been reported in liver, intestine, spleen, cornea, bones, muscles, CNS and rarely, in the lungs (4-6).

There have been <50 cases of pulmonary PG ever described in literature (7,8). The patients may present with non-specific symptoms of cough, dyspnea, fever, weight-loss, malaise and occasionally hemoptysis. Chest imaging may show cavitary infiltrates. The diagnosis is established by cutaneous or extracutaneous lesion biopsy of the ulcer edge showing neutrophilic infiltrate. Extensive testing should be performed , extensive testing to rule out alternative causes including infection, and malignancy, in setting of underlying inflammatory bowel disease or inflammatory arthritisautoimmune and inflammatory conditions associated with PG. Presence of pathergy and response to anti-inflammatory therapy also support the diagnosis (9). Treatment includes systemic or intralesional glucocorticoids and/or calcineurin inhibitors (3). Use of TNF alpha inhibitor, infliximab and anti-neutrophil antimicrobial dapsone has also been described in case reports (10). Most patients achieve remission within 6 months to 3 years.

Umesh Goswami MD¹, Michael Gotway MD², Carlos Rojas MD², Prasad Panse MD², Kris Cummings MD², Eric Jensen MD², Kenneth Sakata, MD¹ and Clinton Jokerst MD²

Division of Pulmonology¹ and Department of Radiology²

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venereol. 2009 Sep;23(9):1008-17. [CrossRef] [PubMed]
2. Ashchyan HJ, Butler DC, Nelson CA, et al. The Association of Age With Clinical Presentation and Comorbidities of Pyoderma Gangrenosum. JAMA Dermatol. 2018 Apr 1;154(4):409-413. [CrossRef] [PubMed]
3. Binus AM, Qureshi AA, Li VW, Winterfield LS. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol. 2011 Dec;165(6):1244-50. [CrossRef] [PubMed]
4. Vadillo M, Jucgla A, Podzamczer D, Rufi G, Domingo A. Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia. Br J Dermatol. 1999 Sep;141(3):541-3. [CrossRef] [PubMed]
5. Scherlinger M, Guillet S, Doutre MS, Beylot-Barry M, Pham-Ledard A. Pyoderma gangrenosum with extensive pulmonary involvement. J Eur Acad Dermatol Venereol. 2017 Apr;31(4):e214-e216. [CrossRef] [PubMed]
6. Abdelrazeq AS, Lund JN, Leveson SH. Pouchitis-associated pyoderma gangrenosum following restorative proctocolectomy for ulcerative colitis. Eur J Gastroenterol Hepatol. 2004 Oct;16(10):1057-8. [CrossRef] [PubMed]
7. Gade M, Studstrup F, Andersen AK, Hilberg O, Fogh C, Bendstrup E. Pulmonary manifestations of pyoderma gangrenosum: 2 cases and a review of the literature. Respir Med. 2015 Apr;109(4):443-50. [CrossRef] [PubMed]
8. Sakata KK, Penupolu S, Colby TV, Gotway MB, Wesselius LJ. Pulmonary pyoderma gangrenosum without cutaneous manifestations. Clin Respir J. 2016 Jul;10(4):508-11. [CrossRef]  [PubMed]
9. Maverakis E, Ma C, Shinkai K, et al. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts. JAMA Dermatol. 2018 Apr 1;154(4):461-466. [CrossRef] [PubMed]
10. Teasley LA, Foster CS, Baltatzis S. Sclerokeratitis and facial skin lesions: a case report of pyoderma gangrenosum and its response to dapsone therapy. Cornea. 2007 Feb;26(2):215-9. [CrossRef] [PubMed]

Cite as: Goswami U, Gotway M, Rojas C, Panse P, Cummings K, Jensen E, Sakata K, Jokerst C. July 2022 Medical Image of the Month: Pulmonary Nodule in the Setting of Pyoderma Gangrenosum (PG). Southwest J Pulm Crit Care Sleep. 2022:25(1):4-6. doi: https://doi.org/10.13175/swjpccs029-22 PDF 

June 2022 Medical Image of the Month: A Hard Image to Swallow

Alessandra Carrillo, DO

Robert Ondracek, DO

Shil Punatar, DO

Andrew Ondracek, DO

Ravi Sundaram, DO

Department of Critical Care Medicine

Franciscan Health

Olympia Fields, Illinois USA

Figure 1. Portable chest x-ray demonstrating marked dilatation of the esophagus with food impaction and bilateral aspiration of food particles. There is also a small left pleural effusion.

Figure 2. Coronal view CT-chest/abdomen/pelvis demonstrating marked dilatation of the esophagus with food impaction seen and food particles seen in his lungs bilaterally.

Introduction

Esophageal food impactions are common occurrences in gastroenterology, however, under 20% of cases require intervention (1)  .The clinical condition of the esophagus and the consistency of food being swallowed contribute to the development of food bolus impactions, with patients having underlying esophageal pathology in most cases (2). Unfortunately, radiographic evidence is often difficult to obtain as food is radiolucent and poorly visualized on radiograph. Here, we demonstrate the risk associated with severe food impaction.

Case Presentation

An 86-year-old man with a past medical history of achalasia with laparoscopic Heller myotomy complicated by distal esophageal perforation, was admitted after presenting with complaints of chest pain and inability to tolerate a solid diet. Additionally, he suffered a 90-pound weight loss over 1 year. He was seen by speech therapy and provided with a dysphagia appropriate diet. Eight days into the patient stay, the family presented to the patient's bedside to assist in 1-to-1 feeding of the patient per their request. One hour following the completion of the patient’s feeding, a CODE BLUE was called.  The patient was unresponsive and without a pulse. PEA protocol was initiated and return of spontaneous circulation was achieved. Post intubation chest x-ray demonstrated a markedly dilated esophagus (Figures 1). Thereafter, CT chest was ordered demonstrating markedly dilated appearance of the patient’s esophagus with internal food material without as a large obstructing lesion (Figure 2). This was deemed to be the cause of the patient's cardiac arrest with concomitant aspiration. Overall, the dilatation significantly progressed from previous imaging. The patient was made NPO, transitioned to total parenteral nutrition and plans were made for a follow-up disimpaction via esophagogastroduodenoscopy (EGD). Ultimately, the patient was too unstable to pursue EGD and expired 9 days after his initial arrest.

Discussion

Through literature review, a majority of cases of food bolus impaction are self-limited. In most cases described, boluses pass on their own or with the assistance of an EGD. In most cases, underlying esophageal or motility dysfunction is known. With few case reports, food disimpaction has been assisted with cola products or nifedipine (3,4). Though trivially regarded, our case demonstrates that food bolus revel against more gruesome esophageal pathology in both presentation, prompt intervention, and adverse on outcomes.

Conclusions

We illustrate a common presentation to gastroenterologists and physicians of a food bolus impaction. Though, due to the profound radiographic presentation and severe morbidity of our clinical scenario, we hope to bring attention to the need for rapid evaluation, treatment, and consideration of adverse outcomes in patients presenting with food boluses as well as the severity and life-threatening outcomes that may preside with the previously trivially described pathology.

References

1. Yao CC, Wu IT, Lu LS, Lin SC, Liang CM, Kuo YH, Yang SC, Wu CK, Wang HM, Kuo CH, Chiou SS, Wu KL, Chiu YC, Chuah SK, Tai WC. Endoscopic Management of Foreign Bodies in the Upper Gastrointestinal Tract of Adults. Biomed Res Int. 2015;2015:658602. [CrossRef] [PubMed]
2. Sperry SL, Crockett SD, Miller CB, Shaheen NJ, Dellon ES. Esophageal foreign-body impactions: epidemiology, time trends, and the impact of the increasing prevalence of eosinophilic esophagitis. Gastrointest Endosc. 2011 Nov;74(5):985-91. [CrossRef] [PubMed]
3. Gelfond M, Rozen P, Gilat T. Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology. 1982 Nov;83(5):963-9. [PubMed]
4. Karanjia ND, Rees M. The use of Coca-Cola in the management of bolus obstruction in benign oesophageal stricture. Ann R Coll Surg Engl. 1993 Mar;75(2):94-5. [PubMed]

Cite as: Carrillo A, Ondracek R, Punatar S, Ondracek A, Sundaram R. June 2022 Medical Image of the Month: A Hard Image to Swallow. Southwest J Pulm Crit Care Sleep. 2022;24(6):93-95. doi: https://doi.org/10.13175/swjpccs022-22 PDF 

April 2022 Medical Image of the Month: COVID Pericarditis

Figure 1. A: Pericardial enhancement on thoracic CT (red arrows). B: Thoracic CT in lung windows showing mosaic attenuation (black arrows) and bilateral pleural effusions (red arrows).

Figure 2. A: Static image of parasternal short axis on transthoracic echocardiogram showing moderate, generalized pericardial effusion with right ventricular diastolic collapse (red arrow). B. Static image of parasternal long axis on transthoracic echocardiogram again showing a moderate, generalized pericardial effusion (red arrow). Lower panel: video of echocardiogram in parasternal long axis view.

A 76-year-old patient presented with fatigue and shortness of breath after missing one session of dialysis. Past medical history included end stage renal disease on hemodialysis and atrial fibrillation on anticoagulation. Initial labs showed that she was COVID positive with mild elevation in troponin and a BNP 1200. While an inpatient, she had received a few sessions of dialysis and treatment for COVID (including dexamethasone and remdesivir). Initial echo showed an ejection fraction of 60-65% with a small generalized pericardial effusion, a thickened pericardium with calcification. A few days after admission patient was suddenly noted to be hypotensive with systolic blood pressure in the 70s and altered mental status. Repeated labs showed a D-Dimer of 17,232, leukocytosis, lactic acidosis, troponin 0.556 ng/ml and arterial blood gas with metabolic acidosis. With a worsening clinical picture, repeat imaging was obtained. CT angiography of the chest was negative for pulmonary embolism; however, it showed a large pericardial effusion with reduced size of the right ventricle more so than left, concerning for cardiac tamponade (Figure 1A). CT chest also showed moderate-to-large pleural effusions with scattered mosaic attenuation of the lung parenchyma (Figure 1B). Repeat transthoracic echocardiogram had a moderate generalized pericardial effusion with right ventricular diastolic collapse concerning for pericardial tamponade (Figure 2). Her airway was secured with endotracheal intubation and vasopressors added for hemodynamic support. Pericardiocentesis was indicated however, patient’s INR was severely elevated in the setting of anticoagulation use. Efforts were made to lower INR with FFP; however, patient had a PEA arrest the following day and expired.

COVID-19 has been classically known for its detrimental lung damage; however, it has shown to cause extrapulmonary effects as well. Cardiac injury is one phenomenon that has been seen with the fulminant inflammatory state that COVID is known to cause. With a few cases reported for COVID pericarditis, it is a possible culprit when all other causes have been ruled out. Pericardial involvement can be seen in about 20% of COVID 19 cases, with effusion found in about 5% of patients (1). Concomitant myocarditis can also be found in up to 17% of patients. Having isolated cardiac involvement with COVID is rare, with most cases presenting mainly as lung involvement in addition to other organs affected as well. Clinically, patients with pericarditis typically experience chest pain and in the setting of COVID infection, an increase in inflammatory markers. Characteristic findings of pericarditis include friction rub on auscultation, diffuse ST elevations on EKG and a potential progression to pericardial effusion on echo. When a pericardial effusion becomes large enough, it can progress to cardiac tamponade (2). Having a high clinical suspicion for tamponade is crucial in a patient who has developed respiratory distress and hypotension in the setting of recent viral pericarditis. It is a clinical diagnosis and requires rapid treatment with pericardiocentesis to prevent cardiac arrest.

Sarah Youkhana, MD¹ and Maged Tanios, MD²

St. Mary Medical Center, Long Beach, CA USA

¹Internal Medicine Resident, PGY-3

²Medical Director, Critical Care Services

References

1. Diaz-Arocutipa C, Saucedo-Chinchay J, Imazio M. Pericarditis in patients with COVID-19: a systematic review. J Cardiovasc Med (Hagerstown). 2021 Sep 1;22(9):693-700. [CrossRef] [PubMed]
2. Imazio M, Gaita F, LeWinter M. Evaluation and Treatment of Pericarditis: A Systematic Review. JAMA. 2015 Oct 13;314(14):1498-506. [CrossRef] [PubMed]

Cite as: Youkhana S, Tanios M. April 2022 Medical Image of the Month: COVID Pericarditis. Southwest J Pulm Crit Care Sleep 2022;24(4):62-3. doi: https://doi.org/10.13175/swjpccs006-22 PDF

February 2022 Imaging Case of the Month: Between A Rock and a Hard Place

Michael B. Gotway MD

Department of Radiology, Mayo Clinic, Arizona

5777 East Mayo Boulevard

Phoenix, Arizona USA

Clinical History: A 46-year-old woman presented to her primary care physician with longstanding complaints of difficulty with aerobic exercise, near syncope, headache, poor sleep, and pain in both legs and arms, exacerbated when flying in commercial aircraft. The patient had also complained of several gastrointestinal disturbances recently that prompted evaluation, revealing a normal colonoscopy. The patient was diagnosed with probable food intolerance by breath testing showing fructose intolerance, managed with a low fermentable oligosaccharides, disaccharides, monosaccharides and polyols (FODMAP) diet with positive results.

PMH, SH, FH: The patient’s past medical history was remarkable for a history of Raynaud’s phenomenon and head trauma at age 16. She noted that her presenting complaints have been present since childhood to some extent. Her poor sleep was characterized as frequent awakenings, daytime somnolence, mouth dryness, and waking up with severe headaches. The patient had been diagnosed with COVID-19 4 months earlier, with her presenting complaints all exacerbated and accompanied by shortness of breath, but she recovered uneventfully. The patient denied other significant past medical history and had no surgical history. Her family history was remarkable for a sister diagnosed with obstructive sleep apnea, diabetes, and thyroid carcinoma, and hypertension in a number of her 13 siblings. The patient’s mother had been diagnosed with colonic malignancy and her father died of melanoma. The patient’s social history was remarkable for abuse during childhood by a male sibling. The patient denied tobacco, alcohol, and illicit drug use.

Physical Examination: The patient’s physical examination showed her to be slender and in no distress although anxious, afebrile, pulse rate= 73, normal respiratory rate, with a blood pressure of 116/95 mmHg. Her cardiovascular, pulmonary, musculoskeletal, and neurologic examinations were within normal limits.

Results from prior outside examinations, including funduscopic, abdominal MRI, and brain MRI and MRA were within normal limits. An outside audiology consultation when the patient complained of hearing loss several months after her SARS-CoV-2 infection showed normal findings. Her complete blood count, coagulation parameters, electrolytes, and liver panel showed no abnormal values. A frontal chest radiograph from an outside institution (Figure 1) from 4 months prior to her primary care appointment, around the time when the patient was diagnosed with COVID-19.

P

Figure 1. Frontal (A) and lateral (B) chest radiography obtained around the time the patient was diagnosed with COVID-19.

Which of the following represents an appropriate interpretation of her frontal chest radiograph? (Click on the correct answer to be directed to the second of 11 pages)

1. Frontal chest radiography shows findings typical for coronavirus (SARS-CoV-2) pulmonary infection
2. Frontal chest radiograph shows bilateral peribronchial lymphadenopathy
3. Frontal chest radiography shows focal consolidation
4. Frontal chest radiography shows multiple lung nodules
5. Frontal chest radiography shows pleural effusion

Cite as: Gotway MB. February 2022 Imaging Case of the Month: Between A Rock in a Hard Place. Southwest J Pulm Crit Care Sleep. 2022;24(2): 12- . doi: https://doi.org/10.13175/swjpccs004-22  PDF

January 2022 Medical Image of the Month: Bronchial Obstruction Due to Pledget in Airway Following Foregut Cyst Resection

Figure 1. Enhanced chest CT in axial (A), sagittal (B), and coronal (C) planes shows a mediastinal foregut duplication cyst (*) compressing medial basal subsegmental airways (arrows). Axial enhanced chest CT displayed in lung (A and B) and soft tissue (C) windows 7 years after surgical resection of the foregut duplication cyst shows post-resection changes with a focus of hyperattenuation (curved arrow) related to the medial basal segmental bronchus; this bronchus is dilated and fluid-filled more distally (arrow). Click here to view Figure 1 enlarged in a new window.

Figure 2.  Bronchoscopic images (G-J) show an object obstructing the medial basal segmental right lower lobe bronchus (arrow); a blue suture is attached to the object. The object (arrow) was retrieved using forceps (arrowhead) and was found to reflect a surgical pledget. Follow up unenhanced axial (K), sagittal (L), and coronal (M) chest CT shows mildly stenosed medial basal subsegmental bronchi (curved arrow) with distal bronchiectasis (double arrowheads); these airway abnormalities are shown to advantage using minimum intensity projected images (N-P). Click here to view Figure 2 enlarged in a new window.

A 37-year-old woman complaining of chest pain and cough underwent resection of a mediastinal foregut duplication cyst complicated by a 10-day hospitalization with a prolonged air leak. Seven years later, she presented with worsening cough and shortness of breath, complaining of similar symptoms intermittently in the 7 years between her surgery and presentation. Chest CT showed a hyperattenuating lesion obstructing the medial basal segmental airways (Figure 1). Bronchoscopy revealed a suture and a pledget obstructing the medial basal segmental right lower lobe bronchus (Figure 2). The pledget and suture were successfully removed. Repeat bronchoscopy several months later showed no residual airway foreign body, although medial basal subsegmental bronchial stenosis prevented advancement of the bronchoscope distally; this finding correlated with the CT impression of airway stenosis or occlusion in this region on the follow up CT.

Bronchogenic cysts result from abnormal lung budding and development of the ventral foregut during the first trimester (1). Many lesions are detected asymptomatically, but larger lesions, as in this patient, may induce symptoms prompting resection. The surgical note for this patient suggested the lesion resided in the right lower lobe, but most bronchogenic cysts arise in the mediastinum near the carina, and this patient’s large lesion extended from the subcarinal space into the azygoesophageal recess. It is possible the surgeon entered the right lower lobe to resect the lesion, resulting in the post-surgical air as well as the surgical pledgets in the medial basal right lower lobe airway that caused the patient’s recurrent chest complaints.

Prasad M. Panse MD¹ and Kenneth K. Sakata MD²

Departments of Radiology¹ and Pulmonary Medicine² 

Mayo Clinic Arizona, Scottsdale, AZ USA

Reference

1. Panchanatheeswaran K, Dutta R, Singh KI, Kumar A. Eleven-year experience in thoracoscopic excision of bronchogenic cyst. Asian Cardiovasc Thorac Ann. 2012; 20(5):570-574 perspective. Natl J Maxillofac Surg. 2015; 6(2):144-1451. [CrossRef][PubMed]

Cite as: Panse PM, Sakata KK. January 2022 medical image of the month: bronchial obstruction due to pledget in airway following foregut cyst resection. Southwest J Pulm Crit Care. 2022;24(1):6-7. doi: https://doi.org/10.13175/swjpcc065-21 PDF

November 2021 Imaging Case of the Month: Let’s Not Dance the Twist

Prasad M. Panse MD and Michael B. Gotway MD

Department of Radiology

Mayo Clinic, Arizona

5777 East Mayo Boulevard

Phoenix, Arizona 85054

Editor’s Note: Parts of this presentation were used in the June 2020 Pulmonary Case of the Month.

History of Present Illness: An 82-year-old man presented to his physician for general health maintenance as well as a complaint of persistently poor quality sleep and poor appetite with weight loss. The patient had undergone robotic-assisted radical left nephroureterectomy and cystectomy with pelvic lymph node dissection and urinary diversion for left clear cell renal cell carcinoma (staged T2a, grade 2) and transitional cell carcinoma of the bladder (carcinoma in situ at surgery), approximately 9 months earlier. The patient’s bladder malignancy was initially treated with transurethral resection, with histopathology at that procedure showing high-grade papillary urothelial malignancy with lamina propria invasion, but no muscular invasion; this procedure was followed by formal complete resection approximately 3 months later. The patient’s post-operative course was complicated by significant bleeding which required transfusion of 3 units of blood. He had undergone inferior vena caval filter placement prior to surgery when preoperative testing revealed lower extremity deep venous thrombus and pulmonary embolism.

Past Medical History: The patient’s past medical history was remarkable for atrial fibrillation treated with anticoagulation and hypertension. He also had a history of coronary artery disease and myocardial infarction with moderate systolic dysfunction His medical list included warfarin (for his atrial fibrillation), acetaminophen, vitamin supplementation, hydrochlorothiazide, atorvastatin, ramipril, metoprolol, and zolpidem. He denied allergies. The patient was a former smoker, previously smoking 2 packs-per day for 35 years, quitting over 30 years prior to presentation.

His past surgical history was remarkable for laminectomy in addition to the recent urinary surgery. He also had a history of rectal laceration complicating previous prostatectomy for prostate carcinoma (Gleason 3 + 4, T2).

Physical Examination: showed the patient to be afebrile with normal heart and respiratory rates and blood pressure. Her room air oxygen saturation was 99%. The physical examination did not disclose any salient abnormalities.

Initial Laboratory: The patient’s complete blood count and serum chemistries showed largely normal values, with the white blood cell count was normal at 6.7 x 10⁹ /L (normal, 4-10 x 10⁹ /L). His liver function testing and renal function testing parameters were also within normal limits. Echocardiography showed mildly decreased left ventricular systolic function, but this finding was stable. The patient underwent frontal chest radiography (Figure 1A).

Figure 1. A: Frontal chest radiography. B: Frontal chest radiography performed just over 1 year prior to A shows no specific abnormalities.

Which of the following represents an appropriate interpretation of his frontal chest radiograph? (Click on the correct answer to be directed to the second of fourteen pages).

1. Frontal chest radiography shows no specific abnormalities
2. Frontal chest radiograph shows a nodule
3. Frontal chest radiography shows bilateral interstitial thickening
4. Frontal chest radiography shows bilateral pleural effusions
5. Frontal chest radiography shows mediastinal and peribronchial lymph node enlargement

Cite as: Panse PM, Gotway MB. November 2021 Imaging Case of the Month: Let’s Not Dance the Twist. Southwest J Pulm Crit Care. 2021;23(5):115-25. doi: https://doi.org/10.13175/swjpcc053-21 PDF

Medical Image of the Month: COVID-19-Associated Pulmonary Aspergillosis in a Post-Liver Transplant Patient

Figure 1. Axial (A) and coronal (B) CT views before transplantation. The lung parenchyma appears normal.

Figure 2. Postoperative axial (A) and coronal (B) CT views 14 days later. Multiple consolidatory nodules with central break down seen involving both lungs, the largest 43mm x 47 mm in the lower lobe of right lung.

A previously healthy, 48-year-old woman, admitted with a working diagnosis of acute-on-chronic liver failure (Grade III) secondary to an autoimmune etiology, was found to be SARS COV-2 RTPCR positive on routine admission screening. She was initially managed with standard medical care for COVID, including steroids. She required invasive ventilation for worsening encephalopathy and when her antigen test was negative 10 days later, she underwent an urgent liver transplantation.

Her preoperative infection screen (culture of blood, bronchoalveolar lavage, urine) was negative and computerised tomography (CT) of the chest was normal (Figure 1). She was extubated on day 3 after liver transplantation. Her recovery was uneventful until the 10th postoperative day when she developed cough and oxygen desaturation. A repeat CT chest showed multiple multilobular consolidatory nodules with central breakdown involving both lung (Figure 2).  Her bronchoalveolar lavage culture grew Aspergillus fumigatus (azole sensitive) which fulfilled criteria for proven COVID-19 Associated pulmonary aspergillosis (pulmonary form) which the host criteria already met (1). Although she was aggressively managed with intravenous voriconazole and liposomal amphotericin, she subsequently succumbed to her illness.

COVID-19 infection shows a propensity to dysregulate the immune system and decreases T-cell lymphocytes. The dysfunctional immune system with a direct damage of respiratory epithelium by the viral infection facilitates superadded bacterial and fungal infections (2).  The use of corticosteroids and antiinterleukins in the therapy elevates the risk. Immunosuppression in an organ transplanted patient can complicate this further. Though patients with invasive fungal disease (IFD) are diagnosed and classified based on host factors, clinical factors and mycological evidences, CAPA essentially need not present with all the typical host and clinical elements (3). A high index of suspicion is essential for an early diagnosis of this condition.

Anandajith Kartha P¹, Zubair Umer Mohamed¹, Dinesh Balakrishnan², S Sudhindran²

Department of ¹Anaesthesiology and Critical Care, ²Gastrointestinal Surgery and Solid Organ Transplantation, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India

References

1. Koehler P, Bassetti M, Chakrabarti A, et al. Defining and managing COVID-19-associated pulmonary aspergillosis: the 2020 ECMM/ISHAM consensus criteria for research and clinical guidance. Lancet Infect Dis. 2021 Jun;21(6):e149-e162. [CrossRef] [PubMed]
2. Herold S, Becker C, Ridge KM, Budinger GR. Influenza virus-induced lung injury: pathogenesis and implications for treatment. Eur Respir J. 2015 May;45(5):1463-78. [CrossRef] [PubMed]
3. Donnelly JP, Chen SC, Kauffman CA, et al. Revision and Update of the Consensus Definitions of Invasive Fungal Disease From the European Organization for Research and Treatment of Cancer and the Mycoses Study Group Education and Research Consortium. Clin Infect Dis. 2020 Sep 12;71(6):1367-1376. [CrossRef] [PubMed]

Cite as: P AK, Mohamed ZU, Balakrishnan D, Sudhindran S. Medical Image of the Month: COVID-19-Associated Pulmonary Aspergillosis in a Post-Liver Transplant Patient. Southwest J Pulm Crit Care. 2021;23(4):98-99. doi: https://doi.org/10.13175/swjpcc029-21 PDF