A 46-year-old woman with a past medical history significant for hypertension presented to the emergency department (ED) with three days of fatigue, chills, decreased appetite, dry cough and progressive dyspnea. Review of systems was notable for intermittent fatigue and dyspnea on exertion for the preceding three months. Upon initial evaluation, she was in multi-system organ failure characterized by hypoxemic respiratory failure, shock and severe lactic acidosis. Following endotracheal intubation and volume resuscitation, her cardiopulmonary status initially stabilized; low-dose vasopressors and broad spectrum antimicrobials were initiated. Shortly thereafter, she suffered cardiopulmonary arrest with pulseless electrical activity. Following return of spontaneous circulation, gas exchange and hemodynamics deteriorated rapidly, and required high-levels of mechanical ventilatory support, vasopressors and inotropic agents, renal replacement therapy and emergent cannulation onto venoarterial extracorporeal membrane oxygenation. Despite these measures, she progressed to catastrophic multi-organ failure (severe acute respiratory distress syndrome, refractory shock and cardiac failure, and disseminated intravascular coagulation) and passed approximately 12 hours following presentation to the ED.
Approximately 5 years prior, she had presented to the ED with menorrhagia without anemia. Her gynecological/obstetrical history (G4P4; mode of delivery:vaginal vs C-section:unavailable) and exam were significant for a palpable anterior "fibroid" and 10 week size uterus. A transvaginal ultrasound was notable for 3 intramuscular "fibroids" and 1 cm thick endometrial stripe. Both ovaries were within normal limits and no free fluid was seen.
Imaging reports as part of 2nd/3rd trimester fetal evaluations 9 years earlier describe 2 suspected leiomyomata measuring up 3.5 cm in greatest dimension with a follow-up scan one year later showing increase to 3.7 cm.
A chest x-ray taken two days prior to presentation to the ED showed a diffuse granular pattern without consolidation bilaterally suggestive of infection (e.g., viral pneumonia, pneumocystis). At presentation to the ED, the chest computed tomography (CT) scan demonstrated diffuse, randomly-distributed, innumerable punctate nodules mostly <= 5 mm. Additionally, mild interstitial septal thickening, mildly heterogeneous diffuse ground-glass opacification of the lungs with lower lobe predominance and mediastinal lymphadenopathy were noted (Fig. 1). No honeycombing, significant confluent consolidation or mass(es) were seen. The differential included infectious/inflammatory conditions such as atypical pneumonia (e.g., military tuberculosis, fungus) and acute pneumonitis (e.g., hypersensitivity pneumonitis). Interstitial lung disease and sarcoidosis were less likely considerations in light of the acute presentation.
Consent for an autopsy, excluding the head, was granted. Serosanguineous fluid was noted in the pericardial sac (75 cc), pleural cavities (50 cc and 75 cc in the right and left lungs, respectively) and peritoneal cavity (300 cc). Vague nodularity was noted on the surface of the lungs; the left and right lungs weighed 1000 g and 1190 g, respectively. The heart weighted 390 g; the right ventricle was grossly dilated with the wall measuring 0.3 cm.
Gross sectioning of the lungs demonstrated diffuse consolidation and numerous whitish nodules bilaterally spanning between 0.5 cm and 0.8 cm without predilection for any specific region (Fig. 2). Mediastinal lymphadenopathy, with lymph nodes ranging from 1.0 to 1.5 cm, was also noted. There was dilatation of the right heart and left ventricular hypertrophy (1.3 cm thickness).
The uterus weighed 860 g and measured 13.5 x 9.0 x 6.0 cm. Myometrial sectioning revealed multiple 0.5 cm-4 cm white, well-circumscribed and firm nodules with a whorled appearance resembling leiomyomata in the subserosal, intramuscular and intramucosal locations. The endometrial lining, ovaries and fallopian tubes were unremarkable.
Other than hepatomegaly (2040 g), gross examination of the other sites, including breast, gastrointestinal organs, pancreas, spleen, adrenal, kidneys, bladder and bone was unremarkable and without any additional masses.