Affiliation:
1. Department of Pediatrics Hazrat‐e‐Rasool General Hospital Iran University of Medical Science Tehran Iran
2. Department of Pediatrics Hazrate Ali Asghar Children Hospital Iran University of Medical Science Tehran Iran
3. Department of Pediatric Firouzabadi Clinical Research Development Unit Iran University of Medical Sciences Tehran Iran
Abstract
Key Clinical MessageAutoimmune pancreatitis (AIP) is a form of chronic pancreatitis scarcely found in children. Raghib syndrome is a rare congenital heart defect known as persistent left superior vena cava (LSVC) draining into the left atrium. Total signs of Raghib syndrome in AIP case accompanied by an IgG4‐related disease were described.AbstractAutoimmune pancreatitis (AIP) is a form of chronic pancreatitis scarcely found in children. Raghib syndrome is a rare congenital heart defect known as persistent left superior vena cava (LSVC) draining into the left atrium. Here, we describe Raghib syndrome in AIP case accompanied by an IgG4‐related disease (AIP/IgG4RD). A 13‐year‐old boy presented with a 3‐month history of fever and abdominal pain. The laboratory findings showed SGOT and SGPT, ALP was increased, while amylase and γ‐GT were normal. Immunoglobulins were normal, except for IgG. Endosonography, spiral CT of the abdomen, and cholangiopancreatography showed an enlargement of the pancreas. Contrast echocardiography discovered opacification of the coronary sinus and left atrium. Transesophageal echocardiography for LSVC revealed a dilatation in the coronary sinus, indicating persistent LSVC. Following the injection of agitated saline into the left antecubital vein, bubbles entered both left and right atria in LSVC. It is reasonable to exclude some of these rare disorders as Raghib syndrome, in cases that will be started on medications like corticosteroids, which increases the susceptibility to thromboembolic events.