Chief of Pediatric Critical Care Maria Fareri Children's Hospital At Westchester Medical Center Valhalla, New York, United States
Introduction: Hypotension is associated with decreased systemic perfusion, resulting in inadequate tissue oxygenation and anaerobic lactate production. Although often a sign of shock, the following case highlights a less common cause of persistent hypotension.
Description: A 17 year old female presented with two days of abdominal pain, fever, diarrhea, and nausea. The patient was non-toxic appearing with right lower quadrant abdominal tenderness on exam. Vital signs were significant for hypotension with normal heart rate. Laboratory evaluation revealed elevated inflammatory markers and normal lactate. Abdominal ultrasound showed possible appendicitis. With hypotension in the setting of an infectious source, there was concern for septic shock. IV fluids and antibiotics were administered with minimal response. Due to fluid refractory hypotension, a central venous catheter was placed for vasopressor support. Blood pressures were monitored via a radial arterial line and vasopressors were titrated accordingly. A CT abdomen was equivocal for appendicitis and revealed mural thickening of the aorta. Further vascular imaging showed stenosis of the right subclavian, right vertebral, left subclavian, left axillary, and left brachial arteries. Findings were consistent with large vessel vasculitis. Four limb BPs were obtained with notable discrepancy between the upper and lower extremities. Rheumatology was consulted for further workup of vasculitis and patient was diagnosed with Takayasu Arteritis (TA).
Discussion: TA is a systemic inflammatory vasculitis, characterized by autoimmune-mediated inflammation and transmural thickening of the arterial walls. It can initially present with nonspecific symptoms. Once ischemia develops, symptoms become more acute depending on the location of the occluded vessel, and there may be discrepant blood pressures due to redirection of flow. This patient was initially presumed to be in septic shock and norepinephrine was administered in an effort to maintain adequate perfusion. The normal lactate value was interpreted as a reassuring sign that perfusion and oxygen delivery were maintained. When a discrepancy between upper and lower extremities blood pressures was identified and CT findings were considered, the diagnosis became evident and the patient was treated accordingly.
