Diagnostic DilemmaFollow the Beads: Fibromuscular Dysplasia
Section snippets
Presentation
Imaging provided a definitive diagnosis for a 58-year-old woman who presented to the emergency department with abdominal pain. The previous day, she had nonbloody diarrhea followed by sharp epigastric pain that migrated to her right lower quadrant and flank. The pain improved while she was lying flat and worsened with upright posture. Additionally, she had distinct, persistent pressure over the epigastrium and multiple episodes of nonbloody emesis that developed after the diarrhea.
The patient's
Assessment
On examination, the patient appeared comfortable. Her temperature was 36.6°C (97.9°F), her blood pressure was 143/88 mm Hg, her heart rate was 115 beats per minute, her respiratory rate was 17 breaths per minute, and her oxygen saturation was 100% on room air. A cardiopulmonary examination was normal, with no carotid or femoral bruits. She had 2+ radial and dorsalis pedis pulses. Mild tenderness without rebound or guarding was noted in the right lower quadrant and right costovertebral angle.
Diagnosis
The presence of multiple arterial stenoses and aneurysms strongly suggested a primary vascular disorder, either inflammatory (vasculitis) or noninflammatory (vasculopathy). The lack of involvement of the aorta and pulmonary vessels made Takayasu arteritis or giant cell arteritis unlikely. “Sausage-like” dilatation is a described feature of polyarteritis nodosa, a medium-vessel vasculitis with a propensity for the mesenteric vessels, but the absence of systemic inflammatory symptoms such as
Management
Management consists of surveillance imaging as well as medical, endovascular, and surgical therapies. In general, surveillance with noninvasive imaging at 6- or 12-month intervals is recommended for patients with vertebral and carotid disease.5 Angiotensin-converting enzyme inhibitors are preferable for patients with hypertension induced by fibromuscular dysplasia, and aspirin is recommended for preventing thromboembolic events.5 Revascularization, via either endovascular or surgical
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Cited by (1)
Uncommon Cause of Internal Mammary Artery Pseudoaneurysm
2022, Ochsner Journal
Funding: RM is supported by the Jeremiah A. Barondess Fellowship in the Clinical Transaction of the New York Academy of Medicine, in collaboration with the Accreditation Council for Graduate Medical Education (ACGME).
Conflict of Interest: The authors have no conflict of interest to report.
Authorship: All authors listed have contributed sufficiently to the project to be included as authors, and all those who are qualified to be authors are listed in the author byline. To the best of our knowledge, no direct conflict of interest, financial or other, exists.