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Challenges in Diagnosing Kawasaki Disease With Atypical Presentation Rare Case Report

Authors

Abdelmoneim Elhadidy*, Emad Balah, Hani Ghoneim, Ahmed Eltenahy
Gastroenterology and Hepatology department, Damietta Fever and Gastroenterology hospital, Ministry of Health and Population, Egypt.

Article Information

*Corresponding author: Abdelmoneim Elhadidy, Gastroenterology and Hepatology department, Damietta Fever and Gastroenterology hospital, Ministry of Health and Population, Egypt.

Received: November 20, 2025            |         Accepted: November 28, 2025         |    Published: December 02, 2025

Citation: Elhadidy A, Balah E, Ghoneim H, Eltenahy A. (2025) “Challenges in Diagnosing Kawasaki Disease With Atypical Presentation Rare Case Report” Clinical Case Reports and Clinical Study, 12(5); DOI: 10.61148/2766-8614/JCCRCS/222.

Copyright: © 2025 Abdelmoneim Elhadidy. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Kawasaki disease(KD) is an acute febrile illness primarily affecting children under five, characterized by inflammation of medium-sized blood vessels, or vasculitis, with a particular risk to the coronary arteries. Acute acalculous cholecystitis (AAC) is an inflammatory disease of the gallbladder in the absence of gallstones. AAC has been linked to various systemic illnesses including Kawasaki disease (KD).  We report a case of a five-year-old male brought to the emergency department (ED) with a history of fever and pharyngitis for four days. He was admitted developed tinge of jaundice, abdominal pain, cough and expectoration. Then, we discovered that he had Kawasaki disease.

Reporting this case is crucial due to unusual presentation as Acute acalculous cholecystitis and abdominal pain contributing to the limited literature on Kawasaki disease.

Keywords:

Atypical Kawasaki Disease, Abdominal pain, Jaundice and coronary artery aneurysms

Introduction:

Kawasaki disease (KD) is defined as an acute systemic vascular disorder primarily impacting medium and small blood vessels. It is typically a self-limiting illness, with the highest occurrence in children being below five years old (1). The diagnosis of KD relies mostly on clinical signs and symptoms. The diagnostic criteria require the presence of fever for at least 5 days along with four of five other clinical features (rash, mucositis, conjunctival injection, cervical lymphadenopathy or extremity changes). The American Heart Association suggests the use of supportive laboratory testing and echocardiography to help detect patients who are at risk (2). Atypical Kawasaki disease includes patients who meet only 2 or 3 of the 5 criteria for diagnosis (3).

 Most children with KD are brought to medical attention because of prolonged fever. There are two forms of KD: complete and incomplete. Diagnosis of complete KD requires fever of at least 5 days' duration along with 4 or 5 of the principal clinical features. The most recent version of the AHA guidelines suggest that if a patient presents with 4 or more of the principal criteria, KD can be diagnosed on day 4 of fever. (4) Experienced clinicians who have treated many KD patients may establish diagnosis before day 4, and patients who present with coronary artery disease can be diagnosed if they have at least 3 of the 5 major diagnostic criteria. (5)  

Up to 48.4% of patients with Kawasaki disease (KD) have an incomplete manifestation, and an atypical presentation may delay an accurate diagnosis (6-7). In the unusual manifestations, hepatobiliary issues during the progression of KD are infrequent. Burns et al. indicated that gallbladder hydrops and hepatobiliary dysfunction are closely associated with the acute phase of KD(8), and Falcini et al. and Grewal et al. both reported acute febrile jaundice as an initial presentation of KD (9-10). An atypical presentation of jaundice often occurs in the early stage of KD; however, the typical presentation can manifest 14 days after the first manifestations of jaundice (10). Certain research has indicated a link between jaundice-dominant symptoms of KD in the acute phase and a higher risk of coronary artery abnormalities. An early identification of acute febrile jaundice in patients with KD could enable swift treatment to lower the likelihood of deadly heart complications.

 Also, AAC is considered a sign of a severe disease. Therefore, rapid recognition of AAC is crucial as it can indicate the presence of an underlying systemic illness, particularly in children. This is essential to prevent any delays in diagnosing and treating the condition and to effectively mitigate the risk of developing coronary lesions (11).

In this article, we report a case involving a pediatric patient who was diagnosed with Kawasaki disease (KD) complicated by AAC and coronary artery aneurysms were observed. The patient's condition was effectively managed by intravenous immunoglobulin (IVIG) treatment.

Case presentation

A previously healthy five-year-old male, was brought to the Damietta Fever and Gastroenterology hospital, with a fever, pharyngitis, vomiting and CXR on admission revealed left mid-zonal patch of consolidation.

for four days. The fever was documented at 39°C, his vital signs were as follows: temperature of 38.9°C, pulse rate of 130 beats per minute, blood pressure of 84/41 mmHg, and oxygen saturation of 98% on room air. Next day after admission she developed abdominal pain in the right hypochondrium and the appearance of jaundice with hypocolic faeces and hyperchromic urine, cough and expectoration with bilateral non purulent conjunctivitis, red lips with congested throat.

Laboratory studies showed white blood cell of 6.9 × 103/UI, hemoglobin of 10.3 g/dL, platelet of 242 × 103/UI, erythrocyte sedimentation rate (ESR) of 65 mm/hour, positive C-reactive protein (CRP) 192, total bilirubin of 3.2 umol/L, direct bilirubin of 2.9 umol/L, aspartate aminotransferase (AST) of 168 IU, alanine transaminase (ALT) of 287 IU, and alkaline phosphatase (ALP) of 230 IU. HAV-IgM: negative twice with negative other virology panel.

Nasopharyngeal swab: negative. 

On imaging abdominal ultrasound revealed thick wall gall bladder with clear mild ascites, CT chest revealed suboptimal due to movement yet, no masses nor consolidation and Echocardiography show normal.

One week after admission Laboratory studies showed white blood cell of 16.6 × 103/UI, hemoglobin of 10.3 g/dL, platelet of 676 × 103/UI, erythrocyte sedimentation rate (ESR) of 65 mm/  hour,positive C-reactive protein (CRP) 6 , total bilirubin of 1.3 umol/L, direct bilirubin of 0.9 umol/L, aspartate aminotransferase (AST) of 31 IU, alanine transaminase (ALT) of 30  IU, and alkaline phosphatase (ALP) of 175 IU and we notice bilateral minimal desquamation of the skin at his fingertips.  However, five days later the fever persisted, and repeated blood tests showed leukocytosis showed white blood cell of 20,3 × 103/UI, along with elevated ESR  130 mm / hour and now we suspected atypical Kawasaki disease and repeat Echocardiography is requested (with provisional diagnosis of?? Kawasaki disease) at the same day which revealed Kawasaki disease with dilatation of left main coronary artery (LMCA), dilatation of left circumflex artery (LCX), dilatation of Left anterior descending artery (LAD) and minimal pericardial effusion

Table (I): Laboratory findings of the studied child with atypical Kawasaki.

 

31/7

2/8

5/8

6/8

7/8

8/8

9/8

 

ESR

 

 

 

 

 

65

 

 

INR

 

 

1.05

 

 

 

 

 

Albumin

 

 

3.4

 

 

 

 

 

WBCs

6.9

 

15.1

16.6

17.7

20.6

20.3

 

Platelet

242

 

322

399

643

642

917

 

Hg

10.3

 

10.8

9.9

10

9.5

10.3

 

CRP

192

 

 

 

 

95

 

 

Creatinine

0.3

 

0.2

 

 

 

 

 

RBS

93

 

 

 

 

 

 

 

ALT

188

121

102

 

 

 

45

 

AST

287

193

156

 

 

 

38

 

T bilirubin

3.2

3.1

3.8

2.2

 

 

1.3

 

D bilirubin

2.9

2.9

3.5

0.9

 

 

0.9

 

CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; Hb: hemoglobin; MCV: mean corpuscular volume; PLT: platelets; TLC: total leucocytic count; RBS: random blood sugar; ALT; alanine transaminase; AST: aspartate aminotransferase.

Following the diagnosis of atypical Kawasaki disease, the patient was transferred to the Pediatric Intensive Care Unit (PICU) for specialized management. The patient started receiving treatment with a single infusion of 2 g/kg of intravenous immunoglobulin (IVIG) , methylprednisolone as pulse therapy as 10mg/kg for 4 days then oral steroid 2mg /kg , and maintenance prednisolone and oral aspirin at a dose of 60 mg/kg/day until the fever resolved. Fever subsided and platelet count was the first to normalize.

The general condition improved with normalization of previously elevated inflammatory markers and thrombocytosis started to appear in blood picture.  

. Echocardiograms were performed daily for the first week and then on a weekly basis.

On the 21 th day after the hospitalization he performed a Coronary Computed Tomography Angiography which was normal. Two months later the patient was completely asymptomatic.

Discussion

The KD's aetiology is still unknown, and has been hypothesized that it develops in the genetically predisposed patients due to a possibly infectious trigger.

The diagnosis of KD relies on clinical signs and symptoms since no specific diagnostic tests are available. In the classic form the patients are children, and they have fever for more than 5 days and at least 4 out of 5 diagnosis criteria (12-13-14-15):

  • oral lesions such as erythema of oral and pharyngeal mucosa, cracking or fissured lips, strawberry tongue;
  • bilateral conjunctivitis without exudate;
  • polymorphous rash;
  • oedema or erythema of the extremity with a progressing to desquamation of feet and hands;
  • cervical lymphadenopathy usually unilateral (≥ 1.5 cm)

The patient we studied presented 3 of these criteria however, some patients show only a few clinical features of the typical clinical picture of KD, defining as incomplete form of KD often underdiagnosed (16).

Reports of atypical findings of KD have increased in the past few decades, and gastrointestinal symptoms are not easily recognized in the early stages of the disease. Although gastrointestinal symptoms are not included in the classical diagnostic criteria, hydrops of the gallbladder and acute febrile jaundice have been reported as atypical findings in some studies (17-18).

 Our findings highlight the importance of the jaundice-predominant manifestation of KD in infants and children. An atypical presentation of jaundice often occurs in the early stage of KD, at an average of 3.3 days between the second and sixth day of the febrile course (19).

However, the typical presentation can manifest as late as 14 days after the onset of the jaundice manifestation (10). Thus, the early recognition of febrile jaundice as the initial presentation of KD would allow for a timelier diagnosis.

The pathophysiology of jaundice among patients with KD is still unclear (20). The proposed mechanisms include vasculitis of hepatobiliary vessels, edematous change of the cystic duct wall, inflammation of the serosa of the liver and gallbladder, and cholangitis, which has been suggested to be the cause of cholestasis and hepatitis (21). Many studies have proposed that liver function tests can detect inflammation and predict the possibility of developing IVIG-refractory disease or coronary artery aneurysms (22-23).

While steroids are the first line of management in most of the immune vasculitis; its use is controversial in Kawasaki disease (24-25).  Intravenous immunoglobulins is the first line of management to prevent the coronary artery disease in Kawasaki disease (26). Incomplete or atypical Kawasaki disease should be taken into consideration in case of all children with unexplained fever for more than 5 days, associated with 2 or 3 of the main clinical findings of Kawasaki disease (3). The diagnosis of incomplete Kawasaki disease is based on presence of any three of the supplemental laboratory criteria: anaemia, leucocytosis, thrombocytosis, elevated alanine aminotransferase, hypoalbuminemia and sterile pyuria. Cardiac complications, mostly coronary artery aneurysm, can occur in 20% to 25% of untreated patients and in 4% of treated patients (26). Other aneurysms in different medium sized arteries are documented (e.g. axillary and femoral arteries) (27).

 In our case, five years presented with fever abdominal pain, jaundice, bilateral non purulent conjunctivitis, red lips with congested throat, along with indications of dilatation of left main coronary artery (LMCA), dilatation of left circumflex artery (LCX), dilatation of Left anterior descending artery (LAD) and minimal pericardial effusion 

The patient started receiving treatment with a single infusion of 2 g/kg of intravenous immunoglobulin (IVIG) , methylprednisolone as pulse therapy as 10mg/kg for 4 days then oral steroid 2mg /kg , and maintenance prednisolone and oral aspirin at a dose of 60 mg/kg/day until the fever resolved. The general condition improved with normalization of previously elevated inflammatory markers and thrombocytosis started to appear in blood picture.  

. Echocardiograms were performed daily for the first week and then on a weekly basis.

On the 21 th day after the hospitalization he performed a Coronary Computed Tomography Angiography which was normal. Two months later the patient was completely asymptomatic.

Conclusion

This case highlights the challenges of diagnosing KD particularly when classic symptoms are absent. The patient initially presented with fever, abdominal pain and jaundice leading to a misdiagnosis. However, the detection of dilatation of left main coronary artery (LMCA), dilatation of left circumflex artery (LCX), dilatation of Left anterior descending artery (LAD) and minimal pericardial effusion confirmed KD.

Given the increased risk of severe cardiac complications in child, early recognition is crucial. This case emphasizes the need for heightened clinical suspicion and timely treatment to improve outcomes in atypical KD presentations.

Acknowledgements

Not applicable.

Authors and Affiliations

Gastroenterology and Hepatology department, Damietta Fever and Gastroenterology hospital, Ministry of Health and Population, Egypt.

Abdelmoneim Elhadidy& Emad Balah & Hani Ghoneim &

 Ahmed Eltenahy.

Authors’ contributions

All authors are responsible for the modification and giving final approval of the manuscript. Abdelmoneim Elhadidy was a contributor in writing the manuscript. All authors read and approved the final manuscript.

Funding

The authors received no funding for this study.

Availability of data and materials

Please contact the corresponding author for data requests.

Declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Not applicable.

Competing interests

The authors declare that they have no competing interests.

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