Laura Sartori, MD
Fellow, Pediatric Emergency Medicine
Vanderbilt University
Other Contributors
Leah Holmes, FNP
Jaime Kaye Otillio, MD
Joshua Chew, MD
Vanderbilt University Divisions of Cardiology and Pediatric Emergency Medicine
The Case
History of Present Illness
A six year old male with ADHD & resolved refractory Kawasaki presents with two weeks of intermittent fever, maximum temperature 100.5 F, and fatigue. He had been seen by his PCP early in the course and was treated with amoxicillin following a positive rapid strep. His symptoms continued and he was later found to be positive for adenovirus.
Past Medical History
Developed Kawasaki 10 months ago. He had manifested with fever, rash, lip swelling, anemia, leukocytosis, and elevated inflammatory markers. He was treated with IVIG x1 and discharged home. He presented again one week later for refractory symptoms and received a second dose of IVIG. Aspirin therapy was discontinued seven months ago following a normal echocardiogram.
Physical Exam
HR 128 BP 108/77 RR 34 T 38.6 C Sat 99% on RA
General Alert, active, talkative
HEENT No conjunctival erythema, no mucous membrane changes or lymphadenopathy
Cardiac tachycardia, regular rhythm, no murmurs, faint friction rub at the apex
Respiratory CTAB, normal work of breathing
Abdomen Soft, contender, non distended, liver edge palpable at the right costal margin
Extremities no swelling
Skin no rashes
Routine labs – you know, CBC, ESR, CRP – the “I had Kawasaki before and you get labs because that’s what you do” were all normal. The team obtained a sequence of images which helped make the diagnosis.
A cardiac MRI was then obtained
What is the diagnosis?
A. Cardiac Rhabdomyoma
B. Giant Coronary Artery Aneurysm
C. Infectious Pericarditis
D. Histocytoid Cardiomyopathy
E. Hemorrhagic Pericardial Cyst
E. Hemorrhagic Pericardial Cyst
The ultrasound showed the following:
There is a large, well-circumscribed mass measuring ~ 4.5 x 5.1 cm in the pericardial space adjacent to the LV wall. There is rightward shift of the heart within the pericardium due to mass effect. The mass is heterogenous in appearance without evidence of vascularity by color Doppler.
He was ultimately taken to the OR where the following mass was (carefully) removed form the pericardial sac.
Gross pathology specimen – Courtesy Laura Sartori
The biopsy results were consistent with a hemorrhagic pericardial cyst. Work-up for infectious etiologies, including TB, histoplasma, mycoplasma and toxoplasma were all negative. He was discharged following an unremarkable post-op course and had a normal echo at 1 month follow-up. It was unclear why he had such a rapidly growing cyst following a normal echo a few months prior to the ED visit and admission.
Pericardial cysts are rare, occurring in 1/1000,000 humans. They are usually discover on routine imaging in patients in their 30s and 40s. Most are completely asymptomatic, though some (25-30%) complain of chest pain, cough and tachycardia. Reported complications include tamponade, cystic rupture, erosion into nearby tissue, right mainstem bronchus obstruction, and sudden death. Management is displayed in the following flowchart. Percutaneous aspiration of pericardial cysts (smaller than our patient’s) which is guided by ultrasonography have been reported with excellent results.
References
Noyes B E, Weber T, Vogler C. Pericardial cysts in children: surgical or conservative approach? Journal of Pediatric Surgery, Volume 38, Issue 8, August 2003, Pages 1263-1265.
Klein AL, Abbara S, Agler DA, et al. American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with pericardial disease: endorsed by the Society for Cardiovascular Magnetic Resonance and Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr 2013; 26:965.
