Interactive Quiz: Constant Right-Sided Abdominal Pain

                        Welcome to Rheumatology Consultant's latest interactive diagnostic quiz. Over the next few pages, we'll present a case and ask you to make the diagnosis and treat the patient. Along the way, we'll provide details about the case, and at the end, we'll share the patient's outcome.   Ready to get started? >> , First, let’s meet the patient…   A 35-year-old man presented with moderate to severe, constant right-sided abdominal pain. The pain had been progressively worsening over the past 2 days and was associated with 1 watery bowel movement and nausea without vomiting. The pain was relieved with marijuana use and was not associated with food intake. Findings of a review of systems were otherwise negative.   His vital signs were normal. Cardiac and pulmonary examination findings also were normal. Abdominal examination revealed tenderness over the right upper and lower abdomen, with normal bowel sounds and no masses. Examination of the extremities revealed hypermobility of the fingers, talipes equinovarus, and knee hyperextensibility greater than 10°.   Based on the presentation, which study would you perform?Computed tomographyPositron emission tomography scanIntegrated PET-CT scan Are you correct? >>   , Answer: Computed tomography   Computed tomography scans of the abdomen and pelvis were suggestive of a renal infarct as opposed to pyelonephritis (Figure 1). The patient also underwent a transthoracic and transesophageal echocardiography, which revealed no apparent thrombi. Magnetic resonance angiography of the abdomen showed evidence of renal upper pole avascularization without abnormalities of the renal arteries (Figure 2).         Based on imaging findings and the presence of renal infarction, what is your diagnosis?Vascular EDSClassical EDSHypermobile EDS Are you correct? >> , Answer: Vascular EDS   Ehlers-Danlos syndrome (EDS) describes a spectrum of rare genetic disorders characterized by skin hyperextensibility, joint hypermobility, and tissue fragility. The patient did have findings suggestive of EDS that could be diagnosed clinically as the classic type. However, the presentation of renal infarction raised concern for vascular type EDS, which is associated with significantly higher morbidity and mortality rates. Vascular type EDS is notably associated with arterial, intestinal, and uterine fragility; vascular dissection or rupture; and organ rupture.   How would you treat this condition?Prescribe a medicationPerform surgeryNo treatment Are you correct? >> , Answer: Prescribe a medication Genetic medicine and vascular surgery teams were consulted, and it was decided that, irrespective of emboli or dissection, the patient should be placed on a medication. What medication would you prescribe this patient?AnticoagulantImmunosuppressantOpioid   Are you correct? >> , Answer: Anticoagulant   The patient was discharged home on long-term anticoagulation with warfarin and a goal international normalized ratio of 2 to 3. Decisions on anticoagulation are made on the basis of clinical discretion and the patient’s presentation. At the same time, no ideal studies are available for determining the value of long-term anticoagulation for such patients.     To read the full case report, see: Maanit K, Balaji Y. Renal infarction in Ehlers-Danlos syndrome. Consultant. 2017;57(12):718-719. https://www.consultant360.com/articles/renal-infarction-ehlers-danlos-syndrome.