Medical Image of the Month: Late-Onset Pompe Disease

Figure 1. Non-contrasted CT scans. A: Chest CT demonstrates a large mucous plug in the left mainstem bronchus (blue arrow) resulting in complete collapse of the left lung. There is near complete fatty replacement of the musculature of the shoulder girdles except for a small residual portion of the left infraspinatus muscle (red arrow). B: Abdominal CT demonstrates fatty replacement of the rectus abdominis musculature (red arrows) and lumbar musculature (blue arrows) consistent with the patient’s history of Pompe disease. C: Pelvic CT demonstrates near complete fatty replacement of the muscular compartments of the thigh except for residual portions of the bilateral sartorius muscles (blue arrows).

Clinical Presentation: A 63-year-old lady with a past medical history significant for late-onset Pompe disease complicated by chronic hypoxemic and hypercarbic respiratory requiring continuous mechanical ventilation via a tracheostomy tube presented to the emergency room from her care facility with worsening hypoxemia. She had been feeling poorly for three days prior to her presentation with fevers, chills, and thicker secretions from her tracheostomy tube with routine suctioning.

On arrival, she was febrile with a temperature of 39 °C and had diminished breath sounds on the left. Her lab work demonstrated a leukocytosis along with an increase in her creatinine consistent with acute kidney injury. CT scans of the chest, abdomen, and pelvis (Figure 1) demonstrated collapse of the left lung secondary to a large mucous plug in the left mainstem bronchus and fatty replacement of most of her visualized skeletal musculature consistent with her diagnosis of Pompe disease. Sputum cultures grew Pseudomonas aeruginosa. Through a combination of fluid resuscitation, antibiotics, and aggressive chest physiotherapy her clinical condition improved to the point that she was able to return to her care facility.

Discussion: Pompe disease results from a deficiency of acid alpha-glucosidase (GAA) which leads to the accumulation of glycogen resulting in tissue destruction (1,2). Adult patients present with progressive, proximal weakness in a limb-girdle distribution (particularly the hip flexors) along with respiratory insufficiency secondary to diaphragmatic involvement (3,4). Some patients may require noninvasive respiratory support and may progress to requiring mechanical ventilation (5). Diagnosis is made by clinical history and electromyogram. The rate of progression and sequence of respiratory and skeletal involvement vary substantially. Intravenous enzyme replacement therapy with alglucosidase alfa has shown efficacy for late-onset Pompe disease. Gene therapy is under investigation. In untreated patients with late-onset disease, the estimated 5-year survival is 95% and 40% at 30 years (6).

Zachary Hernandez MD, Kelly Wickstrom DO, and Tammer El-Aini MD.

Department of Pulmonary Medicine and Critical Care

University of Arizona College of Medicine

Tucson, AZ USA

References

1. Hirschhorn R, Reuser A. Glycogen storage disease type II: Acid alpha-glucosidase (acid maltase) deficiency. In: The metabolic and molecular bases of inherited disease, Scriver C, Beaudet A, Sly W, Valle D (Eds), McGraw-Hill, New York 2001. p.3389.
2. Raben N, Plotz P, Byrne BJ. Acid alpha-glucosidase deficiency (glycogenosis type II, Pompe disease). Curr Mol Med. 2002 Mar;2(2):145-66. [CrossRef] [PubMed]
3. Engel AG. Acid maltase deficiency in adults: studies in four cases of a syndrome which may mimic muscular dystrophy or other myopathies. Brain. 1970;93(3):599-616. [CrossRef] [PubMed]
4. Winkel LP, Hagemans ML, van Doorn PA, Loonen MC, Hop WJ, Reuser AJ, van der Ploeg AT. The natural course of non-classic Pompe's disease; a review of 225 published cases. Neurol. 2005 Aug;252(8):875-84. [CrossRef] [PubMed]
5. Mellies U, Stehling F, Dohna-Schwake C, Ragette R, Teschler H, Voit T. Respiratory failure in Pompe disease: treatment with noninvasive ventilation. Neurology. 2005 Apr 26;64(8):1465-7. [CrossRef] [PubMed]
6. van der Meijden JC, Güngör D, Kruijshaar ME, Muir AD, Broekgaarden HA, van der Ploeg AT. Ten years of the international Pompe survey: patient reported outcomes as a reliable tool for studying treated and untreated children and adults with non-classic Pompe disease. J Inherit Metab Dis. 2015 May;38(3):495-503. [CrossRef] [PubMed]

Cite as: Hernandez Z, Wickstrom K, El-Aini T. Medical image of the month: late-onset Pompe disease. Southwest J Pulm Crit Care. 2020;20(4):124-5. doi: https://doi.org/10.13175/swjpcc022-20 PDF 

Medical Image of the Week: Diffuse Alveolar Hemorrhage in a Patient with ANCA Vasculitis and IgG4-Related Disease

Figure 1. Well-circumscribed, high-density, airspace opacities with a “crazy-paving” pattern in the upper and lower lobes with peripheral sparing (blue arrows) consistent with alveolar hemorrhage.

Figure 2. Well-circumscribed, high-density, airspace opacities with a “crazy-paving” pattern in the upper and lower lobes with peripheral sparing (blue arrows) consistent with alveolar hemorrhage.

The patient is a 47-year-old man with a history of bilateral orbital pseudotumor associated with immunoglobulin G4-related disease (IgG4-RD). He presented with progressively worsening exertional dyspnea evolving into multisystemic failure.  During the hospitalization, the patient was found to have pauci-immune ANCA-positive vasculitis and glomerulonephritis.

CT images (Figures 1 and 2) show relatively well-circumscribed and extensive upper lung predominant airspace opacities with high attenuation, in some cases with a patchy configuration. A background of interstitial prominence was also noted resulting in a "crazy paving" pattern”, consistent with diffuse alveolar hemorrhage.  This was confirmed with bronchoalveolar lavage.

Discussion

IgG4-RD (IgG4 related disease), is an autoimmune condition capable of causing inflammation and fibrosis of multiple organs, most classically the pancreas (1). IgG4 is the least abundant IgG in the serum and the least likely to stimulate immune activation due to its inability to activate complement (2).

The thoracic manifestations that have been described in cases of pure IgG4-RD include solid nodules, which can appear similar to malignant lesions. Interstitial changes have also been described in the form of non-specific interstitial pneumonia pattern, organizing pneumonia, bronchiolitis obliterans, acute interstitial pneumonitis and a sarcoid-like reaction. There may also be pleural involvement and thickening/irregularity of the central airways. The multiple varying presentations and their potential concomitance can lead to misinterpretation of findings (1-2).

This patient presented with the known history of IgG4-RD. The acute symptoms included hemoptysis/diffuse alveolar hemorrhage and renal failure. To the best of our knowledge, pulmonary hemorrhage has not been described as a potential manifestation of this IgG4-RD.   Therefore, the later diagnosed concomitant ANCA paucimmune vasculitis, likely explained the observed pulmonary findings. The coexistence of two different autoimmune vasculitides has been described before, both contributing to multiorgan-involvement (3).

Mariam Mostamandy BS and Diana Palacio MD

Department of Medical Imaging

The University of Arizona – Banner Medical Center

Tucson, AZ

References

1. Kurowecki D, Patlas MN, Haider EA, Alabousi A. Cross-sectional pictorial review of IgG4-related disease. Br J Radiol. July 2019:20190448. [CrossRef] [PubMed]
2. Campbell SN, Rubio E, Loschner AL. Clinical review of pulmonary manifestations of IgG4-related disease. Ann Am Thorac Soc. 2014;11(9):1466-75. [CrossRef] [PubMed]
3. Carruthers I, Shingare S, Khosroshahi A, et al. IgG4 plasma cell infiltration in granulomatosis with polyangiitis (formerly Wegener’s) lung biopsies. 2012 ACR/ARHP Annual Meeting. [Abstract 1534].

Cite as: Mostamandy M, Palacio D. Medical image of the week: diffuse alveolar hemorrhage in a patient with ANCA vasculitis and IgG4-related disease. Southwest J Pulm Crit Care. 2020;20(3):98-9. doi: https://doi.org/10.13175/swjpcc009-20 PDF 

Medical Image of the Month: Coccidioidal Pneumatocele Complicated by Pulmonary Hemorrhage

Figure 1. Axial CT of the chest without contrast 12 years prior to this hospitalization demonstrates an irregularly-marginated right upper lobe cyst measuring 1.5 x 1.6 cm (red arrow).

Figure 2. Axial CT of the chest without contrast obtained 4 months prior to this admission demonstrated a cavitary lesion now measuring 6.3 x 8.2 cm, thin-walled, with small internal air-fluid level and adjacent small pleural effusion without any internal debris (red arrow).

Figure 3. An axial CT angiogram of the chest in lung windows demonstrated a right upper lobe pulmonary cavitary lesion increased in size to 10.5 cm in largest dimension with almost complete opacification (red star) concerning for a superimposed infection.

A 77-year-old man with emphysema, hypertension, hypothyroidism, and diabetes mellitus presented with two days of worsening cough that progressed to massive hemoptysis. His hemoptysis included clots the size of golf balls and multiple episodes of frank blood, measuring half a cup each. His symptoms included dyspnea at rest, fatigue, and a 15-20-pound weight loss in three weeks. He denied fevers, night sweats, chest pain, hematemesis, and prior hemoptysis. Additionally, he had a history of coccidioidomycosis complicated by a cavitary lung lesion. Per chart review, 12 years prior to this hospitalization the patient had an irregularly-marginated right upper lobe cyst measuring 1.5 x 1.6 cm (Figure 1). A CT scan obtained 4 months prior to admission showed the cavity to be 6.3 x 8.2 cm thin-walled and clear of debris (Figure 2) – consistent with a pneumatocele. The patient was referred to thoracic surgery for possible resection at that time but was lost to follow up.

Admission labs showed a decrease in hemoglobin to 13.4 from a baseline of 15.1 g/dL and white blood cells of 10,300 cells/µL. Blood cultures were negative. CT angiography now demonstrated an increase in the right upper lobe pulmonary cavitary lesion to 10.5 cm in largest dimension with almost complete opacification of the lesion - concerning for a superimposed infection. Imaging also showed tree-in-bud nodules in right middle and lower lobes without evidence of a pulmonary embolism (Figure 3). Coccidioidomycosis serologies by EIA showed a non-reactive IgM with reactive IgG. Acid fast bacilli staining of the sputum was negative. Bronchoscopy performed in the hospital showed fresh blood present in the trachea and in the visualized tracheobronchial tree. Active bleeding was noted only from the posterior segment of the right upper lobe. A bronchoalveolar lavage was performed confirming alveolar hemorrhage centered in the right upper lobe. Lidocaine with epinephrine was instilled to stop bleeding. No endobronchial lesion was seen.

The case was evaluated by an interventional radiologist and cardiothoracic surgeon at our institution. They both felt the patient would benefit from transfer to a larger medical center for definitive management of his hemorrhage. He was transferred to a tertiary academic center for a right upper lobectomy, which he tolerated well.  Surgical pathology and bronchoscopy cultures ultimately grew coccidioides immitis and the patient was discharge on a treatment course of oral fluconazole.

Pulmonary pneumatoceles are thin-walled, air-filled cystic structures. Most pneumatoceles are encountered in infancy; however, they can appear at any age (1). Pneumatoceles are known sequelae of pneumonia but can also occur due to blunt thoracic injury or as a rare side effect of chemotherapy (2,3). While the mechanism of pneumatocele formation is unclear, several theories have been postulated including check-valve bronchial obstruction and narrowing or from parenchymal necrosis with accompanying focal collections of air within the interstitial tissue (5). Such cases are typically asymptomatic and do not require intervention as they resolve within weeks to months (6). While many pneumatocele resolve on their own without additional intervention, complex pneumatoceles may result in uncontrolled hemorrhage, as portrayed in this case, or infected lesions unresponsive to antibiotics - necessitating surgical intervention (7). Other complications of pneumatoceles are rare and may include a tension pneumatocele with cardiorespiratory compromise or pneumothorax (8). 

Staphylococcal pneumonia is frequently complicated by pneumatocele development, with pneumatoceles thought to occur in 61% of cases of staphylococcal pneumonia (9). However, the literature of pneumatocele development following cocci infection is scant. In immunocompetent hosts, infections from coccidiosis are transient, with pulmonary complications (often nodules and self-limited thin-walled cavities) occurring in less than 10% of patients (10).  Complications from coccidiosis infection are usually brief fatigue, dyspnea, cough, and arthritis, with chronic infection or severe complication being rare. Here, we report a case of a gradually enlarging pneumatocele in the setting of cocci infection that eventually eroded into the pulmonary vasculature. The resulting massive hemoptysis was refractory to epinephrine injection and not amenable to catheter embolization. Upper lobectomy was required for definite treatment of the pulmonary hemorrhage.

Sylvester Moses MD, Gregory Gardner MD, Ella Starobinska MD, and Arthur Wolff MD

Department of Internal Medicine

University of Arizona

Tucson, AZ USA

References

1. Flaherty RA, Keegan JM, Sturtevant HN. Post-pneumonic pulmonary pneumatoceles. Radiology. 1960;74:50-3. [CrossRef] [PubMed]
2. Aissaoui O, Alharrar R. Traumatic pulmonary pseudocyst: a rare complication of blunt thoracic injury. Pan Afr Med J. 2019 Apr 11;32:180. [CrossRef] [PubMed]
3. Sangro P, Bilbao I, Fernández-Ros N, Iñarrairaegui M, Zulueta J, Bilbao JI, Sangro B. Pneumatocele during sorafenib therapy: first report of an unusual complication. Oncotarget. 2017 Dec 22;9(5):6652-6. [CrossRef] [PubMed]
4. Quigley MJ, Fraser RS. Pulmonary pneumatocele: pathology and pathogenesis. AJR Am J Roentgenol. 1988 Jun;150(6):1275-7. [CrossRef] [PubMed]
5. Zuhdi MK, Spear RM, Worthen HM, Peterson BM. Percutaneous catheter drainage of tension pneumatocele, secondarily infected pneumatocele, and lung abscess in children. Crit Care Med. 1996 Feb;24(2):330-3. [CrossRef] [PubMed]
6. Kaira K, Ishizuka T, Yanagitani N, Sunaga N, Hisada T, Mori M. Pulmonary traumatic pneumatocele and hematoma. Jpn J Radiol. 2009 Feb;27(2):100-2. [CrossRef] [PubMed]
7. Kesieme EB, Kesieme CN, Akpede GO, Okonta KE, Dongo AE, Gbolagade AM, Eluehike SU. Tension pneumatocele due to Enterobacter gergoviae pneumonia: a case report. Case Rep Med. 2012;2012:808630. [CrossRef] [PubMed]
8. Dines DE. Diagnostic significance of pneumatocele of the lung. JAMA. 1968 Jun 24;204(13):1169-72. [CrossRef] [PubMed]
9. Nayeemuddin M, Jankowich MD, Noska A, Gartman EJ. A strange case of coccidioidomycosis: utilization of bronchoscopy to diagnose a chronic cavitary lesion. Am J Resp Crit Care Med. 2018;197:A5427 [Abstract].

Cite as: Moses S, Gardner G, Starobinska E, Wolff A. Medical image of the month: coccidioidal pneumatocele complicated by pulmonary hemorrhage. Southwest J Pulm Crit Care. 2020;20(3):84-6. doi: https://doi.org/10.13175/swjpcc008-20 PDF 

Medical Image of the Month: Metastatic Pulmonary Calcifications in a Kidney Transplant Recipient

Figure 1. Axial and coronal views of thoracic CT scan showing upper lobe predominant centrilobular ground glass nodules.

Figure 2. Transbronchial biopsy yielded nine tissue fragments, each of which demonstrated moderate to marked interstitial calcification (amorphous purple material) along the alveolar septae, perivascular spaces and within the bronchioles consistent metastatic calcification.  There were secondary changes of mild alveolar fibrosis and interstitial hemosiderin laden macrophages (golden brown pigment).  There was no evidence of an inflammatory response or malignancy to otherwise explain the CT findings in this patient.

A 40-year-old man presented with shortness of breath, cough and abnormal imaging. He had a past medical history of end stage renal disease (ESRD) secondary to Alport syndrome and underwent three kidney transplants in 2004, 2010 and 2016. He was intermittently on dialysis between the transplants. He also had a history of coronary artery disease, congestive heart failure and parathyroidectomy. His CT scan (Figure 1) from 2019 showed diffuse centrilobular ground glass opacities sparing the peripheral lung and lung bases. Pulmonary function testing showed obstruction, with reduced diffusion capacity. Bronchoscopy with bronchoalveloar lavage and transbronchial biopsy of the right upper and middle lobes was consistent with metastatic pulmonary calcification (MPC) (Figure 2).

MPC is a rare metabolic pulmonary disease which is usually found incidentally or on autopsy. It occurs with chronically elevated calcium and phosphorus levels (1). It is very commonly associated with ESRD and rarely in primary hyperparathyroidism, osteoporosis, sarcoidosis, renal and liver transplant and hematological malignancies (2-5). CT shows diffuse, nodular areas of ground-glass opacity or consolidation seen in the upper lung zones with pleural sparing. Diagnosis is made on histopathology. There is no definitive treatment for MPC. MPC should be considered with radiological nodular ground glass opacities, particularly in the context of chronic kidney disease or kidney transplant.

Nikhil Madan¹ MD FCCP, Vipul Patel¹ MD, Christine Minerowicz² MD, Harpreet Greewal¹ MD, and Thomas Kaleekal MD FCCP

¹Division of Pulmonary and Critical Care and Transplant

Newark Beth Israel Medical Center

Newark, NJ USA

²Department of Pathology and Laboratory Medicine

Rutgers Robert Wood Johnson Medical School

New Brunswick, NJ USA 

References

1. Chan ED, Morales DV, Welsh CH, McDermott MT, Schwarz MI. Calcium deposition with or without bone formation in the lung. Am J Respir Crit Care Med. 2002 Jun 15;165(12):1654-69. [CrossRef] [PubMed]
2. Kuhlman JE, Ren H, Hutchins GM, Fishman EK. Fulminant pulmonary calcification complicating renal transplantation: CT demonstration. Radiology. 1989; 173:459e60. [CrossRef] [PubMed]
3. Bendayan D, Barziv Y, Kramer MR. Pulmonary calcifications: a review. Respir Med. 2000; 94:190e3. [CrossRef] [PubMed]
4. Izadyar M, Mahjoub F, Ardakani SN, Ahmadi J. Pulmonary metastatic calcification in a leukemic patient: a case report. J Pediatr Hematol Oncol. 2010;32:e108e10. [CrossRef] [PubMed]
5. Surani SR, Surani S, Khimani A, Varon J. Metastatic pulmonary calcification in multiple myeloma in a 45-year-old man. Case Reports Pulmonol. 2013; 2013:341872. [CrossRef] [PubMed]

Cite as: Madan N, Patel V, Minerowicz C, Greewal H, Kaleekal T. Medical image of the month: metastatic pulmonary calcifications in a kidney transplant recipient. Southwest J Pulm Crit Care. 2020;20(2):71-2. doi: https://doi.org/10.13175/swjpcc001-20 PDF

Medical Image of the Month: Complexity of Healthcare Payment

Figure 1. An illustration taken from an advertisement from a company that claims to simplify healthcare payment.

It is generally agreed that healthcare costs are too high in the US. In ground-breaking work published in 1991, Woolhandler and Himmelstein (1) found that US administrative health care costs increased 37% between 1983 and 1987. They estimated these costs accounted for nearly a quarter of all health care expenditures. Himmelstein now estimates that administrative costs may now account for up to 40% of healthcare costs (2). The cartoon shows one provider and one patient but 8 healthcare administrators. This payment system is overly complex involving multiple steps and personnel, with each administrator “dipping their beaks” adding to healthcare costs.

Alp Umar, MD and Richard A. Robbins, MD

Arizona Chest and Sleep Medicine

Phoenix, AZ USA

References

1. Woolhandler S, Himmelstein DU. The deteriorating administrative efficiency of the US health care system. N Engl J Med. 1991;324(18):1253-8. [CrossRef] [PubMed]
2. Robbins RA, Natt B. Medical image of the week: Medical administrative growth. Southwest J Pulm Crit Care. 2018;17(1):35. [CrossRef]

Cite as: Umar A, Robbins RA. Medical image of the month: complexity of healthcare payment. Southwest J Pulm Crit Care. 2020;20(2):59. doi: https://doi.org/10.13175/swjpcc073-19 PDF 

February 2020 Imaging Case of the Month: An Emerging Cause for Infiltrative Lung Abnormalities

Prasad M. Panse MD*, Fiona F. Feller MD^†, Yasmeen M. Butt MD^‡, Michael B. Gotway MD*

Departments of *Radiology, ^†Medicine, and ^‡Laboratory Medicine

Mayo Clinic, Arizona

Phoenix, Arizona

Clinical History: A 25-year-old man with no previous medical history presented to the Emergency Room with complaints of worsening non-productive cough and fever to 102°F over the previous 7 days. The patient also complained of some nausea, vomiting, and generalized muscle aches. The patient denies rhinorrhea, sore throat, congestion, and diarrhea. The patient also illicit drug use, and drinks alcohol only occasionally. He said he previously smoked 1-2 packs-per day, having quit 6 months earlier.

The patient’s physical examination showed normal vital signs, although his respiration rate was approximately 18/minute. The physical examination showed some mild basilar crackles bilaterally, but was otherwise entirely within normal limits.

Basic laboratory data showed a white blood cell count near the upper of normal= 10.3 x 10⁹ / L (normal, 4–10.8 x 10⁹/L) with a normal platelet count and no evidence of anemia, normal serum chemistries and renal function parameters, and normal liver function tests. The patient was referred for chest radiography (Figure 1).

Figure 1. Frontal (A) and lateral (B) chest radiography at presentation.

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

1. The chest radiograph shows bilateral consolidation
2. The chest radiograph shows findings suggesting increased pressure pulmonary edema
3. The chest radiograph shows mediastinal and peribronchial lymph node enlargement
4. The chest radiograph shows mild perihilar infiltration
5. The chest radiograph shows normal findings

Cite as: Panse PM, Feller FF, Butt YM, Gotway MB. February 2020 imaging case of the month: an emerging cause for infiltrative lung abnormalities. Southwest J Pulm Crit Care. 2020;20(2):43-58. doi: https://doi.org/10.13175/swjpcc004-20 PDF 

Medical Image of the Month: Air Embolism in Transit

 

Figure 1. A: Axial CT of the chest without contrast in a lung window demonstrates air in the peripheral vein of the right upper extremity (arrow A). B: Coronal CT demonstrates air in the peripheral vein of right upper extremity (arrow A). C: Air in the right axillary vein (arrow A) and air in the right subclavian vein (arrow B). D: Air in the right atrium (arrow A). E: air in the right ventricle (arrow A).

Case Presentation

Venous air embolism after fluid resuscitation with pressure infuser bag is rare but can occur without appropriate precautionary measures and can be fatal. We report a case of a 51-year-old male patient who presented to the emergency room (ER) with alcohol withdrawal, atrial fibrillation with rapid ventricular response, seizures, and massive aspiration leading to severe hypoxia and cardiac arrest. He was intubated, CPR was performed, and he received fluid resuscitation using the pressure infuser bag over the collapsible polypropylene (PP) based fluid bag through peripheral intravenous access in the dorsum of right hand. He was admitted to intensive care unit for further management after stabilization in the ER. Computed tomography (CT) of the chest without contrast was performed which showed air tracking along the peripheral vein in right upper extremity (Figure 1A,B) as well as in the right axillary and subclavian veins (Figure 1C). Air was also seen within the right atrium (Figure 1D) and right ventricle (Figure 1E) along with bilateral consolidative changes in the lower lobes. Retrospectively, we discovered that the IV fluid bag was lying down on the bed during CT imaging, and the fluid bag had not been primed before placing it in the pressure infuser bag – both of which were the likely reasons for air entrapment. The patient was placed in left lateral decubitus (Durant's maneuver) and Trendelenburg position, given 100% supplemental oxygen, and provided with supportive care. A central line was placed in right internal jugular vein and blood was aspirated without any air. A transthoracic echocardiogram performed three days later showed no air in the right atrium or ventricle. A repeat CT of the chest without contrast performed seven days later demonstrated resolution of the air in the veins and right heart chambers. The patient improved from this episode, but unfortunately passed way from complications related to a large subdural hematoma.

Discussion

The risk of air embolism is less with collapsible fluid bags compared to glass or plastic bottles. However, there is still a risk of air entrapment with collapsible fluid bags without appropriate priming to remove small amounts of air present in the bag (1). Also, air can enter into the vascular system when the bag position is changed to a horizontal position from an upright position - which occurred in our case. Generally, a small amount of air in the venous system is absorbed without any major side effects but fatal consequences can occur with large amount of air (>3-5 ml/kg) and rapid air entry (2).

Naga S Sirikonda, MD, FCCP and Abdulmonam Ali, MD

Pulmonary and Critical Care

Good Samaritan Hospital, SSM Health

Mount Vernon, IL USA

References

1. Bakan M, Topuz U, Esen A, Basaranoglu G, Ozturk E. Inadvertent venous air embolism during cesarean section: Collapsible intravenous fluid bags without self-sealing outlet have risks. Case report. Braz J Anesthesiol. 2013 Jul-Aug;63(4):362-5. [CrossRef] [PubMed]
2. Shamim F, Abbasi S. Fatal vascular air embolism during fluid resuscitation as a complication of pressure infuser bag. J Emerg Trauma Shock. 2016 Jan-Mar;9(1):46-7. [CrossRef] [PubMed]

Cite as: Sirikonda NS, Ali A. Medical image of the month: air embolism in transit. Southwest J Pulm Crit Care. 2019;20(1):41-2. doi: https://doi.org/10.13175/swjpcc053-19 PDF 

Medical Image of the Month: Idiopathic Right Lower Lobe Pulmonary Vein Thrombus

Figure 1. CT angiogram chest sagittal view: showing low density filling defect consistent with pulmonary vein thrombus (yellow arrow).

Figure 2. A: CT angiogram chest axial view showing right lower lobe pulmonary vein thrombus. B: the vein (red arrow) is well differentiated by his lower contrast than the adjacent artery (blue arrows).

A 62-year-old man with a medical history notable only for a seasonal allergy, presented to the emergency department with complaints of shortness of breath with productive cough for 2 months which were worsening for the last 2 weeks. CTA chest revealed low density filling defect in the RLL vein consistent with RLL vein thrombus (Figures 1 and 2). After a comprehensive work up to rule out malignancy and hypercoagulable disorders, a diagnosis of idiopathic pulmonary vein thrombosis was made. The patient received heparin and was discharged with rivaroxaban.

Pulmonary vein thrombosis is a rare disease but can be fatal, usually patient presents with non-specific symptoms such as cough and shortness of breath (1). The etiology in most of cases is hypercoagulable disorders, malignancies, atrial fibrillation, post lung operations such as lobectomy and lung transplantation, or could be idiopathic as in our patient.

Timothy Jon Rolle MD¹ and Mohammad Abdelaziz Mahmoud MD, DO^2
1Department of Radiology and the ²Internal Medicine Residency

Midwestern University Arizona College of Osteopathic Medicine

Canyon Vista Medical Center
Tucson, AZ USA

Reference

1. Chaaya G, Vishnubhotla P. Pulmonary vein thrombosis: a recent systematic review. Cureus. 2017 Jan 23;9(1):e993. [CrossRef] [PubMed]

Cite as: Rolle TJ, Mahmoud MA. Medical image of the month: idiopathic right lower lobe pulmonary vein thrombus. Southwest J Pulm Crit Care. 2020;20(1):7-8. doi: https://doi.org/10.13175/swjpcc048-19 PDF

Medical Image of the Month: Radiation-induced Organizing Pneumonia

Figure 1. Axial contrast enhanced CT depicting marked skin thickening of the right breast with fibrotic changes in the adjacent costal lung parenchyma.

Figure 2. Axial/Coronal CT images in lung window showing central ground glass attenuation with surrounding consolidation areas in both lung fields involving regions beyond the radiation field.

Radiotherapy post breast conserving surgery has been in vogue for the treatment of early breast cancer. Organizing pneumonia is one of the responses the lung has to acute lung injury. However, an unusual organizing pneumonia is being recognized with peculiarity of involving the lung zones beyond the actual irradiated parenchyma. Clinically patients may be asymptomatic or present with fever, nonproductive cough, dyspnea, malaise, fatigue and weight loss. The “reverse halo” sign describes the central ground glass haze surrounded by consolidation. Subsequent imaging may reveal migratory infiltrates.

The recognition of this entity is important as a differential with a good prognosis. Though the response to steroids is marked, radiation-induced organizing pneumonia can quickly relapse once the steroid is withdrawn (1,2).

Saika Amreen MD, Nidha Nazir MBBS, Naseer A. Choh MD, and Tariq Gojwari MD.

Department of Radiodiagnosis

Sher-i-Kashmir Institute of Medical Sciences (SKIMS)

Soura, Srinagar, India

References

1. Takigawa N, Segawa Y, Saeki T, et al. Bronchiolitis obliterans organizing pneumonia syndrome in breast-conserving therapy for early breast cancer: radiation-induced lung toxicity. Int J Radiat Oncol Biol Phys. 2000 Oct 1;48(3):751-5. [CrossRef] [PubMed]
2. Otani K, Seo Y, Ogawa K. Radiation-induced organizing pneumonia: a characteristic disease that requires symptom-oriented management. Int J Mol Sci. 2017 Jan 27;18(2). pii: E281. [CrossRef] [PubMed]

Cite as: Amreen S, Nazir N, Choh NA, Gojwari T. Medical image of the month: radiation-induced organizing pneumonia. Southwest J Pulm Crit Care. 2019;19(6):167-8. doi: https://doi.org/10.13175/swjpcc014-19 PDF

Medical Image of the Month: Penicillium Pneumonia Presenting as a Lung Mass

Figure 1. Representative image from thoracic CT scan in lung windows.

Figure 2. Panel A: Culture plate showing growth on culture plate. Panel B: Photomicrograph showing the dimorphic fungus taken from the culture plate.

A 72-year-old woman who is a non-smoker was referred for evaluation of a suspected lung cancer. She had progressive shortness of breath at rest for 5 months associated with right-sided chest pain, cough and yellowish sputum. She failed multiple courses of antibiotics.

Her past medical history was significant for hypertension, dyslipidemia, hypothyroidism and poorly controlled diabetes mellitus type 2. She also had mild coronary artery disease for which she was on dual antiplatelet therapy. On physical examination, her oxygen saturation was 94% on room air her other vital signs also being unremarkable. Her physical exam revealed decreased breath sounds on the right associated with dullness to percussion.

Her chest radiograph demonstrated right middle lobe opacities. Her chest CT showed a right hilar mass surrounded by multiple nodules along with interlobular septal thickening, a right middle lobe consolidation with air bronchograms, and multiple mediastinal lymph nodes – all suggestive of malignancy (Figure1).

The patient underwent bronchoalveolar lavage and multiple transbronchial biopsies from the right upper and right middle lobes. The lung biopsy showed nonspecific lymphocytic inflammatory infiltrates. Her bronchoalveolar lavage was positive for fungus on PAS stain. The BAL culture showed germ tube negative yeast, which were identified to be Penicillium species (Figure 2).

Fungi are uncommon causes of pneumonia in the general population, but they are more prevalent in immunocompromised hosts with HIV infection, bone marrow transplant, patients on steroids, or patients with neutropenia (1). Penicillium are thermally dimorphic fungi, widely spread in the environment (2). They found especially in soil or where decaying organic material is present. They are saprophytic and capable of causing food spoilage. Patients usually inhale the spores of penicillium present in soil, and so lungs are the primary site of infection. However, disseminated Penicilliosis with lymphadenopathy and organomegaly (especially in immunocompromised patients) can be seen. There was no evidence of disseminated Penicilliosis in our patient. She was not immunocompromised, and her only risk factor was poorly-controlled diabetes mellitus. If not recognized early, Penicillium pneumonia can be fatal. The diagnosis depends on obtaining tissue, sputum and/or BAL samples for fungal cultures. Use of a serum galactomannan antigen assay may facilitate earlier diagnosis of Penicillium infections, however it is not specific for this pathogen as it is a polysaccharide cell wall component of most Aspergillus species as well (3).

There is no consensus about the treatment of Penicillium pneumonia, however standard therapy consists of intravenous amphotericin B, followed by oral itraconazole for several weeks. The optimal duration of treatment is unknown as several cases of relapse have been reported in the literature.

The patient received two weeks of intravenous amphotericin B deoxycholate followed by 12 months of oral itraconazole. The patient improved significantly with resolution of the consolidation seen on her previous chest radiography.

Hasan S. Yamin MD¹, Amro Alastal MD², Abbas Iter MD¹, Murad Azamttah¹

¹Pulmonary and Critical Care, An-Najah University Hospital, Nablus, Palestine

²Pulmonary and Critical Care, Marshall University, WV, USA

References

1. Kang Y, Feitelson M, de Hoog S, Liao W. Penicillium marneffei and its pulmonary Involvements. Current Respiratory Medicine Reviews. 2012;8(5):356-64. [CrossRef]
2. Visagie CM, Houbraken J, Frisvad JC, Hong SB, Klaassen CH, Perrone G, Seifert KA, Varga J, Yaguchi T, Samson RA. Identification and nomenclature of the genus Penicillium. Stud Mycol. 2014 Jun;78:343-71. [CrossRef] [PubMed]
3. Hung CC, Chang SY, Sun HY, Hsueh PR. Cavitary pneumonia due to Penicillium marneffei in an HIV-infected patient. Am J Respir Crit Care Med. 2013 Jan 15;187(2):e3-4. [CrossRef][PubMed]

Cite as: Yamin HS, Alastal A, Iter A, Azamttah M. Medical image of the month: Penicillium pneumonia presenting as a lung mass. Southwest J Pulm Crit Care. 2019;19:164-6. doi: https://doi.org/10.13175/swjpcc033-19 PDF 

Medical Image of the Month: Pneumoperitoneum with Rigler’s Sign

Figure 1. CT of the abdomen with contrast showing a large quantity of free air within the peritoneal cavity. The etiology of her free intraperitoneal air was not evident on this imaging study.

Figure 2. An upright chest radiograph performed six months later again demonstrates a large amount of free air under the hemidiaphragms, outlining both the spleen and the superior surface of the liver. Rigler’s sign (air on both the peritoneal and luminal side of bowel wall (arrows) - which clearly delineates the bowel wall) is in noted and supports the diagnosis of free intraperitoneal air.

Clinical Presentation: A 70-year-old Asian-American woman presented to the hospital with a distended and tympanic abdomen. She was otherwise asymptomatic. Her past medical history was significant only for an uncomplicated colonoscopy the previous summer. A CT scan showed free air within the peritoneal cavity (Figure 1). She was managed conservatively without a surgical intervention. After six months without a chest x-ray continued to show free air (Figure 2). She underwent an elective exploratory laparotomy without identification of a cause for her free intraperitoneal air. Her pneumoperitoneum completely resolved on follow up imaging.

Discussion: Pneumoperitoneum is a condition which commonly presents as an acute abdomen (1). Causes are numerous and include penetrating and blunt abdominal trauma, perforation of viscus, diaphragmatic rupture, fistula formation – among other etiologies. Work-up of pneumoperitoneum varies depending on the suspected etiology. In the presence of hemodynamic instability or peritoneal signs, the patient should proceed to an exploratory laparotomy immediately following airway maintenance and resuscitation. In the setting of a perforation or sepsis, broad-spectrum intravenous antibiotics are indicated. Stable patients are managed expectantly with NPO status, intravenous fluids resuscitation, serial vitals/abdominal imaging/labs, and nasogastric tube decompression if indicated for obstructive etiologies.

Rigler’s sign is well-demonstrated in the abdominal radiograph (figure 2). Rigler’s sign is the presence of air on both the luminal and peritoneal side of the bowel wall – which clearly delineates the bowel wall (1). This sign is highly suggestive of free intraperitoneal air. Rigler’s sign can be seen on a supine abdominal radiograph and can be helpful in the identification of free intraperitoneal air in a patient who may be too ill for upright radiographs or CT imaging.

Mohammad A. Mahmoud MD DO, Jonathon P. Mahn DO, and Alexander E. Brahmsteadt, MSIV.

Midwestern University | Arizona College of Osteopathic Medicine

Canyon Vista Medical Center

Sierra Vista, AZ USA

Reference

1. Levine MS, Scheiner JD, Rubesin SE, Laufer I, Herlinger H. Diagnosis of pneumoperitoneum on supine abdominal radiographs. AJR Am J Roentgenol. 1991 Apr;156(4):731-5.

Cite as: Mahmoud MA, Mahn JP, Brahmsteadt AE. Medical image of the month: pneumoperitoneum with Rigler's sign. Southwest J Pulm Crit Care. 2019;19(6):156-7. doi: https://doi.org/10.13175/swjpcc047-19 PDF 

Medical Image of the Month: Emphysematous Cystitis

Figure 1. Abdominal CT with contrast showing a thickened, trabeculated bladder wall containing pockets of gas consistent with emphysematous cystitis due to E. coli infection.

Emphysematous cystitis is a rare infection of the urinary bladder caused by gas producing organisms which can be bacterial or fungal characterized by gas collections inside the bladder wall (1). Most common organisms are E. coli, Klebsiella and Proteus are also commonly isolated. Fungi, such as Candida, have also been reported as causative organisms. Presentation range from asymptomatic up to septic shock.

Jonathon P. Mahn DO¹ and Mohammad A. Mahmoud MD, DO²

¹Canyon Vista Medical Center and ²Internal Medicine Residency, Midwestern University, Arizona College of Osteopathic Medicine, Canyon Vista Medical Center

Tucson, AZ USA

Reference

1. Amano M, Shimizu T. Emphysematous cystitis: a review of the literature. Intern Med. 2014;53(2):79-82. [CrossRef] [PubMed]

Cite as: Mahn JP,  Mahmoud MA. Medical Image of the month: emphysematous cystitis. Southwest J Pulm Crit Care. 2019;19(5):148. doi: https://doi.org/10.13175/swjpcc045-19 PDF 

Medical Image of the Month: Hampton Hump and Palla Sign

Figure 1. A chest radiograph demonstrates a wedge-shaped opacity in the right lung base (red circle) and enlargement of the right descending pulmonary artery branch (blue arrow) consistent with a Hampton hump and Palla sign, respectively.

Figure 2. A computed tomography angiogram (CTA) of the chest in a lung window demonstrates a wedge-shaped opacity in the right middle lobe consistent with a Hampton hump (red circle).

Figure 3. A CTA of the chest demonstrates an embolus in the right main pulmonary artery which appears slightly dilated (red circle).

Figure 4. A CTA of the chest demonstrates extension of the pulmonary embolus into the right lower lobe pulmonary arterial branch (blue circle) along with a right middle lobe pulmonary infarction (red circle) which is better demonstrated in Figure 2.

A 51-year-old lady presented to emergency room with acute, severe, right-sided pleuritic chest pain, mild cough and dyspnea at rest. She underwent a lumbar spine laminotomy and foraminotomy twelve days prior to her presentation with limited mobility after her operation. On examination, she was tachypneic and tachycardic. Her blood pressure and oxygen saturations on room air were normal. Chest auscultation revealed a few crackles in the right lung base. There was no pedal edema or calf tenderness.

A chest radiograph demonstrated a right lower lobe wedge-shaped opacity along with right hilar prominence (Figure 1). She was initially diagnosed with a right lower lobe pneumonia and was admitted to step-down unit for further management. However, her history, clinical examination, and chest radiograph findings suggested the high likelihood a pulmonary embolism. A computed tomography angiogram (CTA) of the chest confirmed the diagnosis of a pulmonary embolism (Figures 2-4).

Based her clinical presentation and radiology results, the patient was diagnosed with a sub-massive pulmonary embolism (PE). She was treated with an intravenous heparin drip. She was hemodynamically stable throughout the hospital admission. Her echocardiogram showed no evidence of right ventricular strain. Eventually, she was transitioned to oral anticoagulation and was discharged home in good condition.

Discussion

The wedge-shaped right lower lobe opacity and right hilar prominence correspond to a Hampton hump and Palla sign, respectively. A Hampton hump represents a pulmonary infarction secondary to PE, and it was named by the radiologist Aubrey Hampton in 1940 (1). The Palla sign is an enlarged right descending pulmonary artery, an observation made in 1983 by a radiologist, Antonio Palla (2). Both signs can be seen on chest radiography and may aid in the diagnosis of a PE.

Although these radiologic findings of PE are rare, practicing physicians should be aware of these findings as they can be extremely helpful and expediate the diagnosis of a PE. On the other hand, misinterpretation of these findings can lead to a delay in the diagnosis of other significant chest pathologies.

Abdulmonam Ali MD and Naga S Sirikonda MD

SSM Health

Mount Vernon, IL USA

References

1. Hampton AO, Castleman B. Correlation of postmortem chest teleroentgenograms with autopsy findings with special reference to pulmonary embolism and infarction. Am J Roentgenol. 1940;43:305–26.
2. Palla A, Donnamaria V, Petruzzelli S, Rossi G, Riccetti G, Giuntini C. Enlargement of the right descending pulmonary artery in pulmonary embolism. AJR Am J Roentgenol. 1983;141:513-7. [CrossRef] [PubMed]

Cite as: Ali A, Sirikonda NS. Medical image of the month: Hampton hump and Palla sign. Southwest J Pulm Crit Care. 2019;19(5):144-5. doi: https://doi.org/10.13175/swjpcc041-19 PDF

November 2019 Imaging Case of the Month: A 56-Year-Old Woman with a Rash

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Clinical History: A 56-year-old post-menopausal woman with a remote history of asthma and asymptomatic uterine fibroids presented with a macular-papular rash over the upper chest, upper medial left forearm, and medial legs, without scaling that has intermittently recurred over the previous few years. The rash is unaccompanied by fever, chills, rigors, abdominal pain, cough, conjunctivitis, urethritis, or any other mucocutaneous lesions. The patient did not note any seasonal relationships or association with food, and the rash regresses promptly with H₁ or H₂-blocker therapy.

The patient’s past medical history was otherwise unremarkable. Her surgical history was positive only for a laparoscopic left inguinal hernia repair 7 years earlier. The patient indicated she was neither a smoker nor a drinker. Her medications included an as-needed albuterol inhaler, a steroid inhaler, a nasal steroid spray, a multivitamin, and a topical steroid.

The patient’s physical examination showed normal vital signs, although her pulse rate was 95 beats / minute. The physical examination was otherwise entirely within normal limits aside from her presenting complaint of rash; in particular, her lungs were clear to auscultation. 

About 2 weeks later, the patient began to complain of myalgias and some wheezing in addition to her rash, with some mild fatigue as well. She denied other complaints, such as coryza, cough, nasal drainage, ear pain, and neck pain or stiffness. At repeat physical examination, her lungs remained clear to auscultation; no wheezing was noted. A dermatology consult suggested that the rash was most consistent with atopic dermatitis, for which a topical steroid was prescribed.

Basic laboratory data showed a white blood cell count in the normal range, mild anemia (hemoglobin / hematocrit = 11.5 mg/dL / 34.7%), a normal platelet count, normal serum chemistries and renal function parameters, and normal liver function tests aside from a mildly elevated alkaline phosphatase level of 145  U/L147 (normal, 35 – 104 U/L). A C-reactive protein level was elevated at 38.5 mg/L (normal, ≤8 mg/L). The patient was referred for chest radiography (Figure 1).

Figure 1.  Frontal chest radiograph.

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

1. The chest radiograph shows mediastinal and peribronchial lymph node enlargement
2. The chest radiograph shows bilateral consolidation
3. The chest radiograph shows cavitary lung disease
4. The chest radiograph shows findings suggesting increased pressure pulmonary edema
5. The chest radiograph shows numerous small nodules

Cite as: Gotway MB. November 2019 imaging case of the month: a 56-year-old woman with a rash. Southwest J Pulm Crit Care. 2019;19(5):127-43. doi: https://doi.org/10.13175/swjpcc065-19 PDF 

Medical Image of the Month: Giant Bulla

Figure 1. A chest radiograph demonstrates marked oligemia of the left lung with displacement of the cardiomediastinal silhouette to the right. Subtle, linear lung parenchymal markings are noted in the base of the left lung hinting at extensive bullous disease and not a pneumothorax (red arrows).

Figure 2. A CT of the chest with contrast in lung windows demonstrates a giant bulla centered in the left upper lobe. Adjacent bullous disease is also present.

Clinical Background: A 49-year-old gentleman with an extensive smoking history who was transferred from an outside hospital for higher level of care for management of his acute hypoxemic respiratory failure. His outside chest radiograph (Figure 1) demonstrated marked oligemia of the left lung with displacement of the cardiomediastinal silhouette to the right. Subtle linear parenchymal markings are noted in the lower lobe suggesting bullous disease. There is extensive airspace disease of the right lung. A CT of the chest (Figure 2) demonstrated extensive bullous disease with a giant bulla noted in the left upper lobe. The patient was transferred to the MICU for further management of his hypoxemic respiratory failure. A CT surgery consult was obtained, and he was deemed not to be a surgical candidate given his tenuous clinical status.

Discussion: A bulla is defined as an air-containing space measuring greater than 1 cm in diameter and surrounded by a thin wall which is less than 1 mm thick. Bulla are usually located in a subpleural location and can be seen with emphysema - both paraseptal and centrilobular types. A giant bulla is defined as a bulla occupying at least 30% of a hemithorax. In this case, the patient had a giant bulla centered in the left upper lobe.

Giant bullae typically develop because of long-term cigarette smoking, which is the most likely cause in this case. Bullous lung disease has also been associated with HIV infection and intravenous use of methadone, methylphenidate, or talc-containing drugs.

In asymptomatic patients, smoking cessation is recommended to prevent further progression. In dyspneic patients with COPD, medical therapy with bronchodilators, inhaled corticosteroids, supplemental oxygen, and pulmonary rehab are recommended. In patients who have dyspnea despite medical optimization or who have issues with a spontaneous, secondary pneumothorax, a bullectomy may be beneficial. Contraindications to a bullectomy include ongoing cigarette smoking, significant comorbid disease, poorly-defined bullae on chest imaging, pulmonary hypertension, and other comorbid conditions that make surgery high risk.

Leslie Littlefield MD and Mohammed Fayed MD

UCSF Fresno

Fresno, CA USA

References

1. Rosado-de-Christenson M, Abbott GF. Diagnostic Chest Imaging. 2^nd edition. Canada: Amirsys; 2012; Section 1, p 15.
2. Diaz PT, Clanton TL, Pacht ER. Emphysema-like pulmonary disease associated with human immunodeficiency virus infection. Ann Intern Med. 1992 Jan 15;116(2):124-8. [CrossRef] [PubMed]
3. Palla A, Desideri M, Rossi G, Bardi G, Mazzantini D, Mussi A, Giuntini C. Elective surgery for giant bullous emphysema: a 5-year clinical and functional follow-up. Chest. 2005 Oct;128(4):2043-50. [CrossRef] [PubMed]

Cite as: Littlefield L, Fayed M. Medical image of the month: giant bulla. Southwest J Pulm Crit Care. 2019;19(4):125-6. doi: https://doi.org/10.13175/swjpcc040-19 PDF

Medical Image of the Month: Air Bronchogram Sign

Figure 1. Chest radiograph showing bilateral dense airspace disease with air bronchograms. Veno-venous ECMO catheter is visible tracking from the right internal jugular vein to the inferior vena cava.

Figure 2. Chest radiograph on day 5 of ECMO after 4 days of induction chemotherapy demonstrating marked improvement of his airspace disease.

An 18-year-old man without any known past medical history presented with a one-day history of progressive shortness of breath. He reported a sudden onset of symptoms the morning of presentation, and an accompanying sensation of confusion with difficulty concentrating. Initial laboratory evaluation was significant for leukocytosis over 60 K/mm³. Due to his increased work of breathing and worsening lethargy, the patient was intubated and sedated for airway protection and ventilatory support. The patient was admitted to the ICU, and his initial chest radiograph was concerning for acute respiratory distress syndrome. Subsequent hematologic analyses from his admission CBC were consistent with a new diagnosis of acute myelogenous leukemia.

Despite aggressive alveolar recruitment maneuvers and maximum ventilator support, the patient’s oxygen saturation remained poor and his respiratory reserve continued to decline. The decision was made to place the patient on veno-venous extracorporeal membrane oxygenation (ECMO) prior to initiating therapy with doxorubicin and cytarabine (7+3 induction protocol). A dual-lumen ECMO catheter was placed in the right internal jugular vein. His initial chest radiograph demonstrated complete bilateral air bronchograms (Figure 1). The patient was started on chemotherapy while on ECMO and was successfully decannulated after five days on the circuit. His chest radiograph on day 5 of ECMO was significant for marked improvement in bilateral airspace disease (Figure 2).

In patients with hematologic malignancy, an inflammatory response can be generated by either the malignant cells themselves, or more commonly as a reaction to subsequent infection. This inflammation often results in protein-rich fluid infiltrating the alveoli. When this process becomes severe enough to cause hypoxic respiratory failure, it can progress to acute respiratory distress syndrome (ARDS) (1). The chest radiograph demonstrates dense airspace disease which developed in this patient. The fluid-filled alveoli in this extreme example of ARDS created a volume of uniform opacities throughout his lung parenchyma which make the conducting airways stand out clearly (2). Segmental air bronchograms can be seen in localized airspace disease, such as atelectasis or pneumonia, but a full-pulmonary air bronchogram of this clarity can only be seen on a patient undergoing ECMO as there are effectively no functional alveoli to participate in gas exchange.

Eric Brucks, MD and Richard Young, MD

Department of Internal Medicine

Banner University Medical Center

University of Arizona

Tucson, AZ USA

References

1. Papazian L, Calfee CS, Chiumello D, Luyt CE, Meyer NJ, Sekiguchi H, Matthay MA, Meduri GU. Diagnostic workup for ARDS patients. Intensive Care Med. 2016 May;42(5):674-85. [CrossRef] [PubMed]
2. Natt B, Raz Y. Air Bronchogram. N Engl J Med. 2015 Dec 31;373(27):2663. [CrossRef] [PubMed]

Cite as: Brucks E, Young R. Medical image of the month: air bronchogram sign. Southwest J Pulm Crit Care. 2019;19(4):119-20. doi: https://doi.org/10.13175/swjpcc036-19 PDF 

Medical Image of the Month: Large Complex Cerebral Arteriovenous Malformation

Figure 1. Computed tomography angiography of the head showing the large complex arteriovenous malformation near the midline of the brain. A: sagittal plane the malformation is fed predominantly by the anterior circulation more on the right and the left. B: coronal plane.

A 70-year-old woman with a history of hypertension presented with left-sided weakness, headache, nausea, and vomiting. She denied loss of consciousness or seizure activity. On examination, she had receptive aphasia. Pupils were equal, round and reactive. She had neck pain on flexion. Her left upper extremity was plegic. Computed tomography of the brain showed acute hemorrhage involving the right thalamus, extending into the ventricular system, and a midline mass. She underwent a computed tomography angiogram, which showed a large, complex arteriovenous malformation (AVM) with a dilated branch of the right suprasellar internal carotid artery feeding the AVM, which then drained into the vein of Galen and straight sinus (Figure 1). She was monitored in the intensive care unit without worsening neurological deficit. She was discharged to a rehabilitation facility, having had no intravascular or surgical intervention.

AVMs are intracranial vascular anomalies which occur in 0.1% of the population (1). Clinical presentations include intracranial hemorrhage, seizures, headaches and neurological deficits, with hemorrhage being the most common and significant manifestation (2). The gold standard imaging modality is conventional cerebral angiography (1). Treating an AVM is a challenging clinical problem, as the risk of treatment has to be weighed against the natural history of the condition. Treatment modalities include observation with medical management, surgical resection, stereotactic radiosurgery, and endovascular embolization (1,2).

Vedhapriya Srinivasan MD, Piruthiviraj Natarajan MD, Reuben De Almeida, Safal Shetty MD, and Kulothungan Gunasekaran MD.

Bridgeport Hospital

Yale New Haven Health

New Haven, CT USA

References

1. Ajiboye N, Chalouhi N, Starke RM, Zanaty M, Bell R. Cerebral arteriovenous malformations: evaluation and management. ScientificWorldJournal 2014;2014:649036. [CrossRef] [PubMed]
2. Geibprasert S, Pongpech S, Jiarakongmun P, Shroff MM, Armstrong DC, Krings T. Radiologic assessment of brain arteriovenous malformations: what clinicians need to know. RadioGraphics. 2010;30:483-501. [CrossRef] [PubMed]

Cite as: Srinivasan V, Natarajan P, De Almeida R, Shetty S, Gunasekaran K. Medical image of the month: large complex cerebral arteriovenous malformation. Southwest J Pulm Crit Care. 2019;19(3):97-8. doi: https://doi.org/10.13175/swjpcc027-19 PDF 

Medical Image of the Month: Renal Cell Carcinoma with Extensive Tumor Thrombus

Figure 1. CT of the abdomen with contrast (axial image) shows a large right large heterogeneous mass (red arrow), consistent with renal cell carcinoma.

Figure 2. A: CT of the abdomen with contrast (coronal image) shows a large right renal mass (green arrow) and tumor thrombus in the IVC (orange arrow). B: Sagittal image showing extension of the tumor thrombus from the inferior vena cava into the right atrium (blue arrow). C: Axial image showing evidence of tumor thrombus in the right atrium (pink arrow).

A 53-year-old man with a right-sided renal cell carcinoma (RCC) presented with nausea, vomiting, intolerance of oral intake and melena. A contrast enhanced CT of the abdomen and pelvis showed near complete replacement of the right kidney by a large heterogeneous mass, measuring 10 x 16 cm (Figure 1). The mass invaded the renal vein and inferior vena cava (IVC) with extension to the level of the inferior cavo-atrial junction (Figure 2). The mass compressed the duodenum, causing a bowel obstruction. Liver and lung metastases were also found. A duodenal stent was placed with significant improvement in his nausea and vomiting. He was not able to receive anticoagulation due to severe gastrointestinal bleeding. The patient discontinued disease modifying therapy and died four weeks after discharge from the hospital.

Tumor thrombus occurs when a tumor invades a blood vessel. It occurs in approximately 10% of patients with renal cell carcinoma, which is a highly vascular malignancy with a propensity to invade the venous system (1). Extension of the tumor from the inferior vena cava into the right atrium is very uncommon, seen in only about 1% of RCCs (1). The American Joint Committee on Cancer staging system for RCC differentiates between tumor thrombus involving the renal vein (T3a), IVC below the diaphragm (T3b) and IVC above the diaphragm (T3c) (1). The presence of tumor thrombus changes staging, prognosis and surgical options. Surgical treatment may be the approach to tumor thrombus in RCC without metastatic disease. The surgical approach is often complex and requires extensive surgical planning and expertise (2). Perioperative morbidity and mortality appear to be proportional to the height of tumor growth, and tumor thrombus extending above the diaphragm carries increased perioperative risk. Wagner et al. (3) retrospectively studied 1,192 cases, and found reduced long-term survival in patients with any venous involvement. However, they found no significant difference in long-term survival between patients with IVC tumor thrombus below (T3b) or above (T3c) the diaphragm. In this study, the most important prognostic factors in RCC included renal tumor size, the presence of perinephric fat invasion, lymph node involvement and distant metastatic lesions.

David Horn MD, Sue Cassidy ANP-BC and Linda Snyder MD

Departments of Internal Medicine and Pulmonary, Critical Care, Allergy and Sleep Medicine

University of Arizona College of Medicine

Tucson, AZ USA

References

1. Wotkowicz C, Wszolek MF, Libertino JA. Resection of renal tumors invading the vena cava. Urol Clin N Am. 2008; 35: 657-71. [CrossRef] [PubMed]
2. Quencer KB, Friedman T, Sheth R, Rahmi O. Tumor thrombus: incidence, imaging, prognosis and treatment. Cardiovasc Diagn Ther. 2017;7(Suppl 3):S165-77. [CrossRef] [PubMed]
3. Wagner B, Patard JJ, Méjean A, et al. Prognostic value of renal vein and inferior vena cava involvement in renal cell carcinoma. Eur Urol. 2009;55:452-9. [CrossRef] [PubMed]

Cite as: Horn D, Cassidy S, Snyder L. Medical image of the month: renal cell carcinoma with extensive tumor thrombus. Southwest J Pulm Crit Care. 2019;19(3):95-6. doi: https://doi.org/10.13175/swjpcc031-19 PDF 

Medical Image of the Month: Mounier-Kuhn Syndrome

Figure 1. AP chest x-ray showing significant tracheomegaly (diameter 30.8 mm), bilateral interstitial infiltrates with dense consolidation more at the lower lobes (left>right).

Figure 2. Axial thoracic CT in lung windows (A-D) and soft tissue windows (E-F). Sagittal CT in soft tissue windows (G-H). A: tracheal diameters in 2 dimensions (coronal 30.4 mm, sagittal 37.6 mm), para-septal emphysema (yellow arrows). B: showing tracheomegaly (23.2 x 34.3 mm) and para-septal emphysema changes (yellow arrows. C: enlarged mainstem bronchi diameters (right mainstem 22.3 x 30.6 mm, left mainstem 24.4 x 16.0 mm). In addition to central bronchiectatic changes (red arrows), left lower lobe consolidative changes (blue arrow). D: dense left lower lobe consolidation and para-septal emphysema. E: Significant tracheomegaly (31.5 x 41.a mm) and dilated esophagus (orange arrow). F: Significant tracheomegaly and dilated esophagus.

Figure 3. A: Sagittal CT scan (soft tissue window) showing significant tracheomegaly (sagittal diameter 35.8 mm). B: Sagittal CT chest (lung window) showing significant tracheomegaly, multiple tracheal diverticuli (green arrows) on the upper posterior tracheal wall.

Figure 4. Pulmonary function testing.

A 52-year-old non-smoking, Caucasian male patient with a past medical history of reported chronic obstructive pulmonary disease (COPD), recurrent lower respiratory tract infections, prior history of pneumothorax, and dysphagia presented with fevers and shortness of breathing associated with a productive cough for one week. Clinically, he was mildly tachypneic and chest auscultation revealed crackles bilaterally - more prominent at the left base. A chest radiograph (Figure 1) showed bilateral lower lobe pulmonary opacities (left more than right). Computed tomography (CT) of the chest demonstrated airspace disease in the lower lobes in addition to significant tracheobronchomegaly along with paraseptal emphysema and central bronchiectatic changes (Figures 2 and 3). Upper posterior tracheal wall diverticulae were also noted (Figure 3). Serum α₁-antitrypsin level and serum immunoglobulins, including IgE levels, were normal. Our patient declined performing diagnostic bronchoscopy. He had a pulmonary function test performed few months prior to his hospital admission which showed combined mild obstructive/restrictive pattern (Figure 4). He responded well to empiric antibiotics and chest percussion therapy. He was discharged in stable condition.

Discussion

On the basis of above findings, a diagnosis of Mounier-Kuhn syndrome complicated by pneumonia was made. The syndrome was first described by P. Mounier-Kuhn in 1932 (1). The diagnosis is usually made when the tracheal diameter is greater than 3 cm on a CT chest (measured 2 cm above the aortic arch) (2). Other diagnostic criteria include a mainstem bronchial diameter of 20-24 mm (right) and 15-23 mm (left) (3). Our patient’s tracheal diameter was around 37 mm. Both mainstem bronchi were dilated.

The abnormal tracheobronchial dilatation in this syndrome is attributed to atrophy of the muscular and elastic tissues in the tracheal and the bronchial walls (3). Hence, in addition to tracheobronchomegaly, these patients can also develop tracheal diverticulosis along with varicose and cystic bronchiectasis (3). These patients usually present in the 3rd or 4th decade of life with nonspecific respiratory symptoms including recurrent bronchitis and subsequently end up being misdiagnosed with COPD (3).

Three subtypes of this syndrome had been described. Subtype 1 has symmetric dilation of the trachea and mainstem bronchi. Subtype 2 demonstrates tracheal dilation and tracheal diverticula. Subtype 3 has diverticular and saccular structures extending to the level of the distal bronchi (3). Our patient likely fits subtype 3 of this syndrome. Overall, treatment is supportive - usually with antibiotics, physiotherapy and postural drainage. In rare instances, tracheal stenting has been used (4). Special consideration should be taken post intubation as achieving good cuff seal can be potentially challenging.

Dysphagia has not been well documented in this syndrome and could be a coincidental finding in our case. However, theoretically, the etiology of this patient’s dysphagia could be secondary to extrinsic compression of the anterior esophageal wall by his markedly dilated trachea. Historically, he underwent multiple esophageal dilatations and at least one Botox injection over the last 5 years without any significant improvement.

Abdulmonam Ali MD and Naga S. Sirikonda MD

Pulmonary and Critical Care

Good Samaritan Hospital

Mount Vernon, Illinois

References

1. Mounier-Kuhn P. "Dilatation de la trachee: constatations, radiographiques et bronchoscopies." Lyon Med. 1932;150:106-9.
2. Menon B, Aggarwal B, Iqbal A. Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications. South Med J. 2008;101(1):83-7. [CrossRef] [PubMed]
3. Falconer M, Collins DR, Feeney J, Torreggiani WC. Mounier-Kuhn syndrome in an older patient. Age Ageing. 2008;37(1):115-6. [CrossRef] [PubMed]
4. Schwartz M, Rossoff L. Tracheobronchomegaly. Chest 1994;106(5):1589-90. [CrossRef] [PubMed]

Cite as: Ali A, Sirikonda NS. Medical image of the month: Mounier-Kuhn syndrome. Southwest J Pulm Crit Care. 2019;19(2):73-5. doi: https://doi.org/10.13175/swjpcc044-19 PDF 

Medical Image of the Week: Diffuse Pulmonary Ossification

 

Figure 1. Scout view from a high-resolution CT (HRCT) in this patient, demonstrating predominantly peripheral coarse interstitial thickening, with architectural distortion. Multiple calcific densities are associated with the interstitial abnormality.

Figure 2. A: High resolution CT axial image, 1 mm slice thickness, “lung windows”, bone algorithm. (Window width, 2500 HU; level, 500 H). Extensive peripheral/subpleural predominant reticulation and superimposed net-like, branching, and highly attenuating structures (dendriform configuration) are nicely depicted. Some coexisting less than 4 mm nodules are deposited predominantly in the areas of reticulation. B: Corresponding mediastinal window.

An 84-year-old man with a twelve-year history of interstitial lung disease with indolent course was referred for a new oxygen requirement. He had previously been diagnosed with usual interstitial pneumonia associated with occupational exposures. Over the previous six-months he became breathless with minimal activity. During this interval he had lost nearly 40 pounds. He had worked in uranium mining and had a mere four-pack-year smoking history. In his free time, he was an artisan and engaged in woodworking, metal craft and stonework. He was hypoxic with exertion and notably cachectic. His clinic exam was significant for grade 1 clubbing and soft inspiratory crackles that were audible at the bilateral bases. Pulmonary function testing demonstrated a restrictive ventilatory defect with severe reduction in diffusion capacity. A chest radiograph was followed by high resolution computed tomography (HRCT) with representative images shown in Figures 1 and 2. A diagnosis of diffuse pulmonary ossification (DPO) associated with UIP was made.

Pulmonary ossification indicates bone tissue formation; this in contrast to the deposition of calcium salts in pulmonary calcification. The pathogenesis is uncertain as most patients have no derangements in serum calcium and phosphorus levels. Transforming growth factor-β, implicated in idiopathic pulmonary fibrosis, is also thought to stimulate chondrocytes and osteoblasts in DPO. Other associated chemokines include bone morphogenic protein, and interleukins 1 and 4.

Patients with DPO may be minimally symptomatic or have significant disease to the level of respiratory failure. The diagnosis is most often made by a surgical biopsy or at the time of autopsy. Nodular and dendriform histologic types are described; the latter of which develops in areas of interstitial fibrosis. The nodular form often follows longstanding pulmonary venous congestion from cardiovascular disorders. Chest radiography is insensitive for diagnosis and may only demonstrate an interstitial pattern. Calcification is generally only seen once HRCT is obtained. ^99mTc-methylene diphosphonate (Tc-MDP) nuclear medicine scanning will also detect the presence of pulmonary ossification. Imaging-wise, the differential diagnosis for DPO, is restricted. Pulmonary alveolar microlithiasis could potentially be confused with DPO. The intra-alveolar accumulation of innumerable minute calculi called microliths are generally much smaller, usually less than 2 mm, with a uniform size and distribution throughout the lungs (‘sandpaper” appearance). At a later phase the number and volume of the calcific deposits increases and becomes more granular. The distribution follows the interlobular septa or bronchovascular bundles and can be confused with DPO. Previous granulomatous disease may have a somewhat similar appearance. However, the density per area unit of the calcific deposits tends to be much less, and the distribution is more random and not necessarily associated with underlying abnormal/ fibrosing tissue. There is a strong association between DPO and IPF, when compared with nonspecific interstitial pneumonia (NSIP) and chronic hypersensitivity pneumonitis. This may improve diagnostic specificity in patients with IPF.

Therapy with calcium binding agents, chelation, and corticosteroids has been disappointing, and there is currently no proven treatment.

Steven Sears DO¹, Bhupinder Natt MD¹, and Diana Palacio MD²

¹Division of Pulmonary, Critical Care, Allergy and Sleep and ² Department of Medical Imaging

University of Arizona College of Medicine. Tucson, AZ USA

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Cite as: Sears S, Natt B, Palacio D. Medical image of the week: diffuse pulmonary ossification. Southwest J Pulm Crit Care. 2019;19(2):65-7. doi: https://doi.org/10.13175/swjpcc028-19 PDF