ABSTRACT
Lymphangioleiomyomatosis (LAM) is a disorder that causes cystic disease in the lungs. This condition is associated with renal angiomyolipomas and commonly occurs in individuals with tuberous sclerosis. Despite its frequent association with tuberous sclerosis, LAM is a rare condition and is often underdiagnosed. An identification of the array of signs for LAM is necessary to start the patient on appropriate long-term guideline-directed medical therapy. A 24-year-old female patient with a past medical history of tuberous sclerosis, gestational hypertension, stable renal angiomyolipoma, and recent pneumothorax presented to our emergency department complaining of four weeks of productive cough. On presentation, she was found to have stable vital signs, and on examination, she had absent breath sounds in the right basilar and anterior mid-lung field compared to the left. Subsequent imaging confirmed a recurrence of pneumothorax from her visit three months prior to her current presentation as well as progressive cystic lung disease and an unchanged angiomyolipoma, suggestive of LAM. We present this article with the hope of raising the index of suspicion for LAM in the setting of the particular signs and symptoms and to encourage prompt stabilization of the patient and initiation of guideline-directed medical therapy and strict follow-up to provide the greatest possible improvement in the patient's quality of life.
ABSTRACT
Necrotizing soft tissue infections typically begin with direct inoculation of bacteria into the subcutaneous tissues. Here, we present a case with no such exposure, but with severe necrotizing fasciitis. We present a middle-aged man presented to the emergency department for a presumed allergic reaction after having initially sought care twice at an urgent care facility. The patient had swelling, but no tenderness of his right lateral chest and flank. Subsequent imaging showed extensive fluid in the fascial planes of the right chest wall requiring surgical debridement. Necrotizing fasciitis that is not treated with surgical debridement carries a mortality rate approaching 100%. This case highlights a potential atypical presentation as well as highlights the fact that the Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score lacks sensitivity to rule out a necrotizing soft tissue infection, requiring surgical debridement for diagnosis.
ABSTRACT
BACKGROUND: Apical hypertrophic cardiomyopathy is a rare, complex phenotypic variant of the classically taught hypertrophic cardiomyopathy. It is highly variable in its pathophysiology as well as its clinical course, spanning the spectrum from a healthy asymptomatic lifestyle to sudden cardiac death and severe diastolic dysfunction. The hallmark electrocardiographic findings of unusually large T-wave inversions, in conjunction with the most common presenting symptom being chest pain, makes this disease entity concerning in the emergency department (ED) setting. CASE REPORT: A 61-year-old man with a history of hypertension presented to the ED with chest pain. His electrocardiogram exhibited a biphasic T wave in lead V2 with ST depressions in leads V3-V6 with deep symmetrical T-wave inversions in these leads as well. His troponin was negative and the patient was taken for cardiac catheterization. Catheterization revealed no coronary artery disease; however, it revealed a "spade like" filling pattern of the left ventricle, suggestive of an apical variant of hypertrophic cardiomyopathy. Subsequent cardiac magnetic resonance imaging confirmed the diagnosis of apical hypertrophic cardiomyopathy and the patient was started on a beta-blocker and discharged with cardiology follow-up. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Electrocardiographic interpretation is a critical skill of the emergency physician. Awareness of the syndrome and its specific electrocardiogram findings may help facilitate further testing that will aid in timely diagnosis and interventions.
