Pearl-Like Chest Calcifications
Radiology Cases in Pediatric Emergency Medicine
Volume 4, Case 4
Loren G. Yamamoto, MD, MPH
Kapiolani Medical Center For Women And Children
University of Hawaii John A. Burns School of Medicine
     A 12-year old male Asian tourist visiting your town 
comes to the emergency department with a chief 
complaint of coughing and fever.  They do not speak 
English well.  From what you can tell, his sister has a 
cold and he has a past history of "Kawasaki".
     Exam  VS:  T38.2 (oral), P110, R32, BP 110/70, 
oxygen saturation in room air 98%.  He is alert and 
active.  He is not toxic.  He has an occasional moist 
cough.  Eyes clear.  Oral mucosa clear and moist.  
Nasal congestion with thick yellow-green mucus.  TM's 
normal.  Neck supple.  Heart regular, no murmurs.  
Lungs clear to auscultation.  Abdomen non-tender.  No 
CVA tenderness.  Color and perfusion are good.
     A chest radiograph is ordered to rule out pneumonia.  
The exam findings are not very suggestive of 
pneumonia, but the history is unclear because of the 
language problem.

View chest radiograph.

     PA and lateral views of the chest are shown here.  
You must enlarge the image to appreciate the findings 
here.  The heart size is normal.  There are no 
pulmonary infiltrates.  There are several spherical 
calcifications with central lucencies overlying the heart 
measuring up to 1.8 cm in size.  There are at least four 
of these clearly visible on the lateral view overlying the 
heart and possibly two more.  The PA view shows one 
of these clearly adjacent to the right inferior heart 
border.
     A translator is arranged on a three-way telephone 
translation access line so that more history can be 
obtained.  His parents indicate that he had a severe 
case of Kawasaki disease when he was two years old. 
(10 years ago).  During his hospitalization, he 
developed heart failure.  After his hospitalization, he 
had to take heart medicines and aspirin at home.  He 
sees a heart specialist at home who examines him 
twice a year.  He last had a chest radiograph one-year 
ago.  His parents give you the name and phone number 
of his cardiologist.
     With the translator still on the line, a phone call to 
his cardiologist across several time zones is successful.  
The cardiologist confirms his past history of Kawasaki 
disease.  The child developed severe coronary 
aneurysms and congestive heart failure at age 2 years.  
IV gamma globulin therapy that is used today to reduce 
the likelihood of developing coronary aneurysms, was 
not in use at the time of his initial illness 10 years ago.  
He is now followed periodically.  He no longer requires 
medications for congestive heart failure.  You describe 
the spherical pearl-like calcifications on his chest 
radiograph.  The cardiologist indicates that this is 
nothing new since these have been visible on his chest 
radiographs for many years now.  These represent 
calcifications of his coronary aneurysms.

     Some the clinical manifestations of Kawasaki 
disease are described in Case 1 of Volume 3, 
Myocardial Failure in a 2-Month Old.  Coronary 
aneurysms are a known complication of Kawasaki 
disease.  Acutely, coronary aneurysms may thrombose 
resulting in coronary insufficiency.  Myocarditis may 
also develop resulting in cardiogenic congestive heart 
failure and/or shock.  Cardiogenic shock in young 
children may present with vomiting.  While vomiting is 
often assumed to be due to viral gastrointestinal 
infections, a careful assessment of perfusion 
parameters and cardiac auscultation should prompt the 
physician to consider cardiac conditions.  Myocarditis 
may often present with muffled heart tones.  Thus, it is 
important to ascertain the integrity of the heart tones in 
children presenting with vomiting or other symptoms 
suggestive of congestive heart failure.
     During the years following the acute phase of 
Kawasaki disease, small aneurysms will usually resolve 
without complications.  Others may evolve resulting in 
coronary vessel stenosis subjecting such patients to an 
increased risk of myocardial ischemia and infarction in 
later life.  Large coronary calcifications such as the 
ones seen on this patient's chest radiograph are 
unusual.  This case is useful to appreciate the 
magnitude of coronary vessel damage in some children 
with Kawasaki disease.  Thus, children or teenagers 
with a past history of Kawasaki disease presenting with 
chest pain suggestive of ischemia should be treated as 
a rule out myocardial infarction since their degree of 
coronary vessel disease may be severe.

References
     Yamamoto LG, Martin JG.  Kawasaki syndrome in 
the ED.  Am J Emerg. Med 1994;12:178-182. 
     Melish ME. Hicks RV.  Kawasaki Syndrome: Clinical 
features, pathophysiology, etiology, and therapy.  J 
Rheumatoloty (suppl 24) `1990;17:2-10.
     Gersony WM.  Diagnosis and management of 
Kawasaki disease.  JAMA 1991;256(20):2699-2703.

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Web Page Author:
Loren Yamamoto, MD, MPH
Associate Professor of Pediatrics
University of Hawaii John A. Burns School of Medicine
loreny@hawaii.edu