Case Session

SCMR 22nd Annual Scientific Sessions

6 - Value of magnetic resonance imaging techniques in diagnosing pericardial diseases: Teenager with constrictive pericarditis causing unusual presentation of protein losing enteropathy

Presenter(s)

• 

  Nazire Ozcelik, MD

  Fellow- Cardiac MRI
  Children's Hospital of Philadelphia

Author Only(s)

• YD

  Yoav Dori, MD

  Director of Pediatric Lymphatic Imaging and Interventions and Lymphatic Research
  Children's Hospital of Philadelphia
• EP

  Erin Pinto, RN

  Nurse Practitioner in the Center for Lymphatic Imaging and Interventions
  Children's Hospital of Philadelphia
• MF

  Mark Fogel, MD

  Director of Cardiac MRI
  Children's Hospital of Philadelphia
• KW

  Kevin Whitehead, MD, PhD

  Associate Professor of Pediatrics
  Children's Hospital of Philadelphia
• MH

  Matthew Harris, MD

  Associate Director of Cardiac MRI
  The Children's Hospital of Philadelphia
• SP

  Sara Partington, MD

  Assistant Professor of Clinical Medicine
  Hospital of the University of Pennsylvania
• DB

  David Biko, MD

  Assistant Professor of Radiology
  Hospital of the University of Pennsylvania

Description of Clinical Presentation:

Previously healthy 16 years old male presented with acute onset of lower extremity and facial edema, underwent extensive work-up which revealed hypoalbuminemia and elevated fecal alpha 1-antitrypsin leading to the diagnosis of protein losing enteropathy (PLE). Despite medical treatment, the edema and hypoalbuminemia progressed requiring further imaging work up with an intrahepatic dynamic contrast magnetic resonance lymphangiogram (IH-DCMRL) for evaluation of his PLE. IH-DCMRL is performed by ultrasound guided percutaneous needle placement within the intrahepatic lymphatics with dynamic magnetic resonance imaging (MRI) during the lymphatic injection of gadolinium.

Diagnostic Techniques and Their Most Important Findings:

IH-DCMRL demonstrated lymphatic perfusion of the duodenum with intraluminal leakage of lymphatic contrast into the duodenum with a dilated thoracic duct (Figure 1). Additionally, the central veins were dilated suggesting increased central venous pressure which prompted further hemodynamic evaluation with cardiac catheterization. Cardiac catheterization findings were consistent with constrictive pathology demonstrating elevated ventricular filing pressures with equalization of the right and left ventricular end-diastolic pressures, elevated wedge pressures and elevated pulmonary vascular resistance index, and low cardiac index. On further evaluation with cardiac MRI, small right and left ventricular volumes with dilation of the superior and inferior vena cava and pericardial thickening were noted, and viability imaging showed pericardial hyperenhancement (Figure 2). The patient underwent pericardiectomy with surgical and pathologic findings consistent with constrictive pericarditis.

Learning Points from this Case:

1. Constrictive pericarditis is a rare condition, especially in childhood, and PLE is a very rare and unusual presentation for this disorder.


2. Increased central venous pressure secondary to constrictive pericarditis may result in decreased lymphatic drainage to systemic veins and increased lymphatic production leading to PLE which can be diagnosed with IH-DCMRL.


3. Cardiac MRI can diagnose constrictive pericarditis by demonstrating thickening and hyperenhancement of the pericardium.