Coronary Microaneurysms—An Early Manifestation of Kawasaki Disease: A Case Report
Corresponding Author: Shea-Lee Godin, South Texas Health System, Edinburg, Texas, United States, Phone: (708) 916-6600, e-mail: email@example.com
Received on: 27 May 2022; Accepted on: 16 October 2022; Published on: 30 June 2023
Introduction: Kawasaki disease (KD) is a medium-vessel vasculitis with a predilection for coronary arteries and has been recognized to be the most common cause of acquired heart disease in children (Hedrich et al., 2018; Newburger et al., 2016; Saguil et al., 2015; Singh et al., 2018; Sundel, 2015). Hedrich et al. (2018) outline how coronary artery aneurysms are a typical manifestation of KD that develops after several weeks of disease.
Case presentation: This case report describes the clinical course and outcomes of a patient, a 5-year-old Hispanic female with KD who displayed early development of coronary microaneurysms as opposed to the typical late sequelae development.
Discussion and conclusion: This unique presentation of KD highlights some crucial questions that should be addressed, including whether coronary artery aneurysms should be reconsidered as only a late sequela of untreated disease. Can coronary artery aneurysms be an early manifestation of KD?
How to cite this article: Godin SL. Coronary Microaneurysms—An Early Manifestation of Kawasaki Disease: A Case Report. Pediatr Inf Dis 2023;5(2):49-51.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient’s parents/legal guardians for publication of the case report details and related images.
Keywords: Coronary artery aneurysm, Kawasaki, Kawasaki disease, Pediatric.
Kawasaki disease is a medium-vessel vasculitis with a predilection for coronary arteries. It has been recognized as the most common cause of acquired heart disease in children.1-5 According to Saguil et al.,3 in the United States, 19 per 100,000 children younger than 5 years of age are hospitalized each year with KD. A literature search was conducted between December 2021 and March 2022. Databases searched include PubMed, EBSCO, Medline, and Cochrane Library electronic databases. The search was conducted utilizing the following search terms: “Kawasaki disease,” “Kawasaki,” “coronary artery aneurysm,” and “pediatric.”
On review of the literature, cardiac involvement is the most characteristic and specific manifestation of KD. This factor determines all diagnostic and therapeutic decisions.3,5 Further, the prognosis is determined by the extent and severity of coronary artery involvement at diagnosis and follow-up.6 Hedrich et al.1 outline how coronary artery aneurysms are a typical manifestation of KD that develops after several weeks of disease. This is further supported by the works of Newburger et al.,2 Sundel,5 and Rife and Gedalia,6 who emphasized that coronary artery aneurysms are late sequelae of KD.
Coronary microaneurysms are currently known as a late manifestation of KD. This case report describes a patient with KD’s clinical course and outcomes who displayed early development of coronary microaneurysms instead of the typical late sequelae development. This report will add to current existing literature and contribute an additional clinical presentation representing the need for clinicians to consider early screening for coronary microaneurysms in those with suspected and confirmed KD.
The patient was admitted to the hospital to treat clinical sepsis, moderate dehydration, and acute cervical adenitis. The patient was a 5-year-old Hispanic female, 104 cm tall who weighed 18.30 kg. She was referred to the children’s emergency department by her primary care provider (PCP) with a 3-day history of persistent fever, ranging from 103 to 104ºF. As well as a 24-hour history of a painful small left-sided neck mass, found to be lymphadenopathy by ultrasound on admission, and elevated white blood cell count discovered by the PCP. The patient had no known allergies, no significant past medical history, family and social history were noncontributory, and the patient was up to date on her immunizations.
On the second day of admission, the patient continued to have persistent fever, making 5 days of fever. The patient continued to have episodes of vomiting and developed a maculopapular rash on the neck, chest, and abdomen. The patient also had injected conjunctiva. Examination of the neck mass revealed a stable mass of approximately 1 inch in size, slight tenderness, and no fluctuation with overlying erythema. The left neck mass was stable and not increasing in size. Blood culture showed no growth at 18–24 hours at 29 hours; urine culture showed no growth at 18–24 hours. Due to the patient’s continued febrile state, clinical sepsis, and left neck lymphadenitis, a consult to infectious disease had been made. The infectious disease consult agreed with the plan set out by the pediatric team. During this point, the pediatric team began to consider KD and an echocardiogram was planned.
The echocardiogram revealed diffuse left coronary artery ectasia, with microaneurysm at the proximal segment of the left anterior descending coronary artery (Figs 1 and 2). These noted changes confirmed the diagnosis of KD. No additional evidence of cardiac defects or dysfunction was noted. The patient then began treatment for KD with high-dose aspirin and intravenous immunoglobulin (IVIG).
Coronary artery aneurysms are a typical manifestation of KD and typically develop after several weeks of disease.1 These coronary artery aneurysms are late sequelae of KD.2,5,6 Most coronary artery aneurysms develop in untreated children; approximately one-quarter of untreated children develop coronary artery aneurysms and account for a mortality rate of more than 1% without IVIG treatment.5 This is further supported by Newburger et al.,2 who outline that approximately one in five children who are not treated with IVIG in the acute phase of illness will develop coronary artery aneurysms. Coronary artery development can be reduced by more than 70% if therapy with IVIG is initiated within the first 10 days of illness.2,5 The use of IVIG decreases the risk of coronary artery development from 20–25% to 3–5% in those who are appropriately treated.1,2,6 The available literature surrounding coronary artery aneurysm development in KD patients focuses on the manifestations being a late sequela in largely untreated patients with KD after 10 days of development of the illness. On day 5 of fever, the presented patient had an echocardiogram that revealed microaneurysm development. The patient had developed a maculopapular rash and injected conjunctiva consistent with KD on the same day. The patient’s initial manifestations were only persistent fever and cervical adenitis, presumed to be lymphadenitis. Given the importance of prompt treatment with IVIG in preventing mortality in KD patients, it is important to be aware of the need for prompt diagnosis. The presented patient had already developed coronary microaneurysms on day 5 of persistent fever, which contradicts current literature which focuses on these coronary manifestations being a late sequela. Being aware that coronary artery aneurysms may develop early in KD can encourage practitioners to consider echocardiogram imaging in patients with suspected KD. This early intervention can facilitate expedited treatment and help mitigate any potential long-term complications, including mortality.
A significant limitation to consider in the presentation of this case report is the possibility of reporting bias by the patient’s mother. It is essential to consider that the patient’s mother may not have detected the patient’s fever promptly. This is an important consideration because if the patient had been febrile prior to the 3 days that the mother was aware of then, the coronary microaneurysms could genuinely be a late manifestation. However, the addition of an enlarged neck mass appearing simultaneously with the fever does support the mother’s reported timeline.
This unique presentation of KD highlights some crucial questions that should be addressed, including whether coronary artery aneurysms should be reconsidered as only a late sequela of untreated disease. Can coronary artery aneurysms be an early manifestation of KD? Is there an opportunity to reconsider echocardiograms in pediatric patients with persistent fever? This case raises the question of whether practitioners should adopt a practice of ordering echocardiograms on pediatric patients with persistent fever and leukocytosis even before meeting the criteria for KD.
DATA AVAILABILITY STATEMENT
The data supporting this study’s findings are available from the corresponding author, SG, upon reasonable request.
Written informed consent was obtained from the minor’s legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.
Shea-Lee Godin https://orcid.org/0000-0003-3227-368X
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