A 27-year-old man presented for left-sided neck and chest pain that had started 24 hours earlier. He said he went to a driving range for approximately 90 minutes, woke up the next day with a crick in his neck, and then began to have substernal chest pain later that night. His pain was not localized to his chest, and it worsened with deep inspiration and movement. He had no prior medical problems, smoked marijuana occasionally, and had not been sick recently. He denied any family history of early cardiac death or disease.
He was well-appearing but looked uncomfortable. His cardiac exam showed tachycardia and a pericardial rub best heard over the left sternal border. An ECG was obtained shortly after arrival, and it showed subtle ST changes in the inferior precordial leads. Labs were obtained, and were unremarkable except for a mild leukocytosis (11.8). He told his nurse shortly after his labs returned that his pain had worsened. A chest CT was ordered because of his out-of-proportion pain and pericardial rub.
Find the diagnosis and case discussion on p. 10.
Diagnosis: Epipericardial Fat Necrosis
Pericardial or epipericardial fat necrosis is a rare condition presenting as acute chest pain that mimics the pain of myocardial infarction or pulmonary embolism. (J Thorac Surg 1957;33:723.) Only 25 cases had been reported in the literature up until 2011. (AJR Am J Roentgenol 2016:207:773; Arch Med Sci 2011;7:337.) The pain may begin in the neck as it did in our patient, and is typically located anteriorly near the diaphragm. The pain is usually described as severe and pleuritic.
No unifying risk factors or identifiable cases have been discovered for this disease. Obesity has been discussed, but less than half of the patients in the case reports were obese. (Arch Med Sci 2011;7:337.) Structural abnormalities (other masses) or pedicles could put a patient at risk if he lifts heavy objects and elicits a Valsalva response or twists himself like our patient did. (J Thorac Imaging 2011;26:W140.) These movements could cause hemorrhage into the tissue and begin necrosis. Unfortunately, many questions remain about the etiology because the literature is limited.
Patients usually present with tachycardia, dyspnea, distress, and diaphoresis. A friction rub can be heard or chest wall tenderness elicited with palpation in rare cases like our patient's.
Labs typically yield normal results. Electrocardiograms may show nonspecific ST or T wave changes and tachycardia, or be normal. Chest x-rays are typically normal. The diagnosis is almost purely based on the physician's suspicion and awareness of this disease and the persistence to get further imaging. CT will show a well-circumscribed mass in the anterior chest with a density similar to fat. Biopsy of the tissue will show necrotic fatty tissue similar to infarcted breast or omental tissue. (N Engl J Med 1968;279:473.)
Treatment in 22 of the 25 cases reported prior to 2011 had thoracotomy and resection. (Arch Med Sci 2011;7:337.) Pineda, et al., found that symptomatic treatment alone rather than invasive thoracotomy is appropriate for these patients. (AJR Am J Roentgenol 2005;185:1234.) Since then, most cases have been treated with NSAIDs only, with pain typically resolving in a few weeks. That can rarely last for almost a year, however. (J Thorac Surg 1957;33:723.) Surgical management or thoracotomy is reserved for cases of intractable pain or significant concern for another etiology such as malignancy.
The number of cases is likely underreported because CT imaging is needed to make the diagnosis. Any patient with pleuritic chest pain should undergo a CT scan, according to a 2016 retrospective review of patients with acute chest pain and CT findings or epipericardial fat pad necrosis. (AJR Am J Roentgenol 2016:207:773.) This recommendation, along with the patient's presentation and history, should prompt a physician to remember this diagnosis and the next steps.
Our patient's severe pain, pericardial rub, and ECG that showed subtle signs of ischemia led the physician to CT for further information. The one and a half hours at the driving range and repetitive swinging action and chest rotation before the onset of pain twisted a pedicle and possibly initiated his symptoms. The physician was able to discharge the patient home with scheduled NSAIDs, strict return precautions, and follow-up in the surgery clinic in two to three weeks. Emergency physicians should be aware of this benign, typically self-limiting disease with a well-described treatment plan that avoids further unnecessary testing and invasive surgical procedures.
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