November 2018 Imaging Case of the Month: Respiratory Failure in a 36-Year-Old Woman

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Clinical History: A 36–year old woman presented with complaints of shortness of breath and worsening dyspnea on exertion. She had a reported history of central nervous system vasculitis of uncertain etiology, treated with azathioprine and prednisone currently, and cyclophosphamide in the past. Her symptoms reportedly responded well to this regimen. Her diagnosis of central nervous system vasculitis was established 6 months earlier when the patient presented with upper extremity paresthesia, headache, left arm weakness, diplopia, and a right eye visual field deficit, evidently with brain imaging showing some pathologic changes, although those records were not available at her presentation. Reportedly she responded well to her immunosuppressive therapy and her steroid and azathioprine doses had been tapered accordingly. Her past medical history was otherwise remarkable for a history of migraine headaches, depression, childhood asthma, hemorrhagic cystitis due to cyclophosphamide (which prompted discounting this drug in favor of azathioprine for the purported central nervous system vasculitis) in the past, and endometriosis.

The patient is a former smoker for a total of 5 pack-years, quitting years previously. She is the mother of a 3-year-old child. The patient denied alcohol and drug use. A history of penicillin allergy was elicited. In addition to azathioprine and prednisone, her medications included inhaled budesonide, Bactrim, escitalopram, topiramate, and sumatriptan/naproxen sodium as well as a multivitamin. There was some history of fenfluramine/phentermine (“Fen-Fen”) use years earlier.

Her physical examination was largely unremarkable. The patient complained of head pain and was visibly mildly dyspneic, but her lungs were clear and no abnormal heart sounds were detected. Her extremities appeared normal- no ecchymosis, cyanosis, or clubbing was detected. She did have some prior history suggesting the presence of erythema nodosum, now presenting as an erythematous region on the right lower extremity, which underwent biopsy, although changes characteristic of erythema nodosum were not present at her current examination. Reportedly this region had been injured when she bumped the right lower extremity on a chair, and this injury evidently became infected, requiring drainage, yielding cultures positive for Staphylococcus aureus and, about 1 month later, Actinomyces israelii. Her vital signs should normal pulse rate and blood pressure, breathing at 26 breaths / minute. Her room air oxygen saturation was 93%.

Frontal and lateral chest radiography (Figure 1) was performed.

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

Which of the following represents the most accurate assessment of the chest radiographic findings? (Click on the correct answer to be directed to the second of twelve pages)

1. Chest radiography shows basilar fibrotic opacities
2. Chest radiography shows bilateral pleural effusions
3. Chest radiography shows cavitary pulmonary lesions
4. Chest radiography shows marked cardiomegaly
5. Chest radiography shows numerous small nodular opacities

Cite as: Gotway MB. November 2018 imaging case of the month: Respiratory failure in a 36-year-old woman. Southwest J Pulm Crit Care. 2018;17(5):119-33. doi: https://doi.org/10.13175/swjpcc114-18 PDF

Medical Image of the Month: Superior Vena Cava Syndrome

Figure 1. Coronal CT thorax with contrast showing a large apical mass with near complete atelectasis of the right upper lobe, mediastinal extension and effacement of the superior vena cava (arrow).

Figure 2. Caval-superficial-umbilical-portal pathway.  EMV = external mammary vein, EV = epigastric vein, IEV = inferior epigastric vein, IMV = internal mammary vein, SEV= superior epigastric vein (2).

Figure 3. Axial CT thorax with contrast showing avid arterial enhancement of hepatic segment IV (arrow, hot quadrate sign), consistent with superior vena cava syndrome.

Although superior vena cava syndrome (SVCS) may result from internal or external occlusion of the superior vena cava, 60-90% of cases are caused by external compression from malignant tumors, predominately lung cancer and lymphoma (1). Additional causes of SVCS via external occlusion include fibrosing mediastinitis, while internal occlusion may result from pacemaker lead or indwelling central venous catheter thrombosis (1). Symptoms of SVCS, such as facial and neck swelling, dyspnea and cough, typically develop over 2-4 weeks prior to diagnosis, during which collateral vessels develop (2). More severe symptoms of disease include laryngeal edema, cerebral edema, orthostatic syncope secondary to decreased venous return and altered mental status (3). In the presence of SVCS, cavoportal collaterals that may develop include caval-superficial-umbilical-portal pathways and caval-mammary-phrenic-hepatic capsule-portal pathways (3). Figure 2 demonstrates the anastomosis of inferior and superficial epigastric veins with internal and external mammary veins, allowing for recanalization of the paraumbilical vein and drainage into left portal vein. The presence of a caval-superficial-umbilical-portal pathway may be detected as a wedge-shaped area of increased enhancement in segment IV of the liver on CT or MRI, a radiographic finding known as the hot quadrate sign (Figure 3). Following diagnosis of SVCS in the setting of malignancy, goals of management may be palliative or curative and should take into account life expectancy. Endovascular stenting can provide near immediate symptomatic relief of SVCS, but requires the addition of chemotherapy, radiotherapy or combined-therapy if the goals of treatment are curative (1). Although the median life expectancy of a patient with SVCS due to underlying malignancy is often reported as 6 months, the prognosis is dependent on tumor type and the presence or absence of poor prognostic factors, including age >50 years old, history of tobacco use and treatment with corticosteroids (3).

Elliot Breshears MS IV, Lev Korovin MD, and Veronica Arteaga MD.

Department of Medical Imaging

The University of Arizona

Tucson, AZ, USA

References

1. Wan JF, Bezjak A. Superior vena cava syndrome. Hematol Oncol Clin North Am. 2010;24(3):501-13. [CrossRef] [PubMed]
2. Kapur S, Paik E, Rezaei A, Vu DN. Where there is blood, there is a way: unusual collateral vessels in superior and inferior vena cava obstruction. RadioGraphics. 2010;30(1):67-78. [CrossRef] [PubMed]
3. Manthey DE, Ellis LR. Superior vena cava syndrome (SVCS). In: Todd KH, Thomas CR Jr. Oncologic Emergency Medicine: Principles and Practice. Switzerland: Springer; 2016:211-222. Available at: https://books.google.com/books?id=_qQqDAAAQBAJ&pg=PA211&lpg=PA211&dq=Manthey+DE,+Ellis+LR.&source=bl&ots=MWH6bcbHSf&sig=L7Ul5sfS1sSGBTF5cnK7MvKF9eA&hl=en&sa=X&ved=2ahUKEwjGkoTC9LrdAhUEEHwKHbV2CF4Q6AEwAHoECAEQAQ#v=onepage&q=Manthey%20DE%2C%20Ellis%20LR.&f=false (accessed 9/14/18).

Cite as: Breshears E, Korovin L, Arteaga V. Medical image of the month: superior vena cava syndrome. Southwest J Pulm Crit Care. 2018;17(4):114-5. doi: https://doi.org/10.13175/swjpcc103-18 PDF