CASE REPORT

Appendicitis as the presenting manifestation of Kawasaki disease

Gwendolyn M. Garnett • Sarah Kimball • Marian E. Melish •

Karen S. Thompson • Devin P. Puapong • Sidney M. Johnson •

Russell K. Woo

Accepted: 27 November 2013 / Published online: 7 December 2013

� Springer-Verlag Berlin Heidelberg 2013

Abstract In cases of Kawasaki’s disease (KD) presenting

as acute surgical abdomen, rarely has the presence of acute

appendicitis been found. We report two cases of histolog-

ically confirmed acute appendicitis in the presence of KD

and a review of the literature as it pertains to acute abdo-

men and atypical presentations of KD.

Keywords Kawasaki disease � Acute appendicitis �Acute abdomen � Vasculitis � Coronary artery aneurysm

Introduction

While the pathogenesis of Kawasaki’s disease (KD)

remains unclear, the prevalence of serious and potentially

life-threatening sequelae has been reduced with early rec-

ognition of classic clinical features of the disease and

prompt initiation of therapy. A diagnostic challenge is

posed when the disease presents atypically, as is frequently

seen in patients with severe gastrointestinal complications,

and can result in detrimental therapeutic delays. While it is

known that KD can present as an acute abdomen, rarely has

acute appendicitis been found as a source of peritonitis at

the time of diagnosis. We report two such cases of histo-

logically confirmed acute appendicitis in the presence of

KD. In both cases, Kawasaki’s disease was diagnosed

postoperatively. Evaluation for Kawasaki’s disease was

initiated because of unusual clinical features during the

postoperative period. Describing the critical manifestations

seen in this unique presentation has important diagnostic

and treatment implications, as special consideration must

be given to the timing and appropriateness of certain

therapies. While KD is not considered a surgical disease by

convention, surgeons may be the first to recognize this

atypical presentation. Favorable outcomes for these atypi-

cal KD patients may depend upon the keen judgment of

surgeons familiar with the disease’s clinical presentation

and course.

Case histories

Case 1

A 3-year-old previously healthy male presented with a 72-h

history of periumbilical abdominal pain, nausea, vomiting,

diarrhea, and fever. Laboratory data were significant for

leukocytosis with bandemia, acidosis, and elevated

C-reactive protein (CRP). Abdominal ultrasound was

inconclusive. Computed tomography (CT) abdomen/pelvis

showed free fluid in the peritoneal cavity, and while por-

tions of the appendix appeared normal, the entire appendix

was not visualized. Tachycardia combined with the

development of peritoneal signs prompted laparoscopic

exploration. Intraoperative findings included free pelvic

fluid, and an injected appendix with serosal changes but

minimal thickening. No other sources of pathology were

identified and appendectomy was performed without

complication. Pathology showed an intact appendix with

focal intramuscular inflammation with neutrophil invasion.

G. M. Garnett (&) � S. Kimball

Department of Surgery, University of Hawai’i,

John A. Burns School of Medicine, Honolulu,

HI 96813, USA

e-mail: ggarnett@hawaii.edu

M. E. Melish � K. S. Thompson � D. P. Puapong �S. M. Johnson � R. K. Woo

Kapi’olani Medical Center for Women and Children,

Honolulu, HI 96826, USA

123

Pediatr Surg Int (2014) 30:549–552

DOI 10.1007/s00383-013-3439-9

On final report this was felt to be consistent with acute

appendicitis. The patient’s postoperative course was char-

acterized by anorexia, persistent abdominal pain, diarrhea,

transaminitis, and high fevers. Physical exam was notable

for peripheral edema involving the hands, fingers, and feet,

conjunctival injection, and dry, cracked lips. Suspecting

KD, an echocardiogram was obtained which revealed

pericardial effusion, and dilatation of the coronary arteries.

Intravenous gammaglobulin (IVIG) and aspirin were star-

ted on hospital day 7. One additional IVIG dose was given

for recurrent fever. The patient was discharged 3 days later

upon complete resolution of symptoms. At 2-year follow-

up, echocardiogram revealed normal caliber coronary

arteries.

Case 2

A previously healthy 7-year-old female presented with a

4-day history of abdominal pain, emesis, diarrhea, and

fever. Laboratory data showed a markedly elevated CRP

level and white blood cell count. CT abdomen/pelvis with

rectal contrast demonstrated dilated loops of small bowel,

free fluid in the pelvis, but poor visualization of the

appendix. Perforated appendicitis was suspected and the

patient was taken for laparoscopic exploration. Intraoper-

ative findings included suppurative appendicitis, purulent

pelvic fluid, and uniform dilatation of the entire small

bowel. Pathology was consistent with mild neutrophilic

and eosinophilic appendicitis, with acute appendicitis being

given as the final diagnosis. The postoperative course was

complicated by persistent abdominal pain, fever, the

development of a raised rash on the arms, trunk and but-

tocks, and dry, cracked lips. Bilateral non-exudative con-

junctivitis was present since admission. A repeat CT

abdomen/pelvis showed colitis, though no evidence of

abscess formation. Again suspecting KD, an echocardio-

gram was obtained and revealed a medium-sized aneurysm

of the left anterior descending coronary artery. IVIG and

antiplatelet therapy was initiated. All symptoms of KD

resolved and the patient was discharged on hospital day 11.

The patient required one additional hospitalization after

follow-up echocardiogram showed enlargement of the

coronary artery aneurysm. Recent echocardiograms have

shown aneurysm regression.

Discussion

KD is an acute, self-limited vasculitis predominately

occurring in early childhood [1]. While there are clinical

and epidemiological features suggestive of an infectious

cause and a possible role for genetic predisposition, the

etiology of KD remains unknown [1, 2]. The major

sequelae of KD are cardiovascular in nature with coronary

artery involvement found in all fatal cases. While coronary

artery aneurysm formation is the most dreaded complica-

tion, it is important to recognize KD is a systemic disease

involving blood vessels throughout the entire body, and can

manifest in several organ systems. KD thus has the

capacity to present in a number of ways often times mas-

querading as other illnesses particularly in the early stages

of disease onset [1].

While there are many clinical and laboratory findings

associated with KD, diagnosis is based on the presence of

persistent fever for five or more days duration, accompa-

nied by at least four of five principle clinical findings.

These findings include bilateral non-exudative conjuncti-

vitis, changes of the extremities, rash, cervical lymphade-

nopathy, and erythema of the lips and oral mucosa [1–3].

Atypical or incomplete Kawasaki’s disease may be diag-

nosed in patients who have some clinical features of the

disease but fail to meet the classic criteria listed above.

These patients carry a higher risk of coronary artery

involvement [4–6]. Because delay in treatment raises

morbidity and mortality of KD particularly in infants and

older children, KD should be considered in any child with

persistent fever, laboratory evidence of systemic inflam-

mation, and no other explanation of febrile illness. Echo-

cardiographic abnormalities, chiefly coronary artery

involvement, often provide the basis for diagnosis in

patients with persistent fever and two or three of the

principle KD clinical features [1].

Owing to its propensity to involve vessels throughout

the entire body, KD often mimics other disease processes

including those that are surgical in nature [1, 7]. Gastro-

intestinal symptoms including abdominal pain with dis-

tention, vomiting, and diarrhea are common and occur in

approximately two-thirds of patients [3]. On rare occasion,

KD has presented as acute surgical abdomen thus landing

these unusual patients on the surgical service. Two tertiary

care centers with a large referral base reported acute sur-

gical abdomen as the presenting manifestation in 4.6 % of

their Kawasaki patients. Notable clinical features of these

patients distinguishing them from patient’s with classic KD

include older age ([5 years old), elevated transaminases,

drastically elevated white blood cell count ([20,000 units)

and incomplete presentation. These patients also had a high

incidence of coronary artery aneurysm development at

50 %. Operative findings in these patients most commonly

revealed gallbladder involvement such as gallbladder

hydrops and cholestasis, though paralytic ileus, and hem-

orrhagic duodenitis were other pertinent findings. Just one

case of acute appendicitis was identified in this series [7].

Overall, there are five reported cases of Kawasaki dis-

ease with appendicular involvement [6–9]. Most of these

cases were limited to appendicular vasculitis (arteritis) with

550 Pediatr Surg Int (2014) 30:549–552

123

only two cases providing histological confirmation of acute

appendicitis [6, 7]. Both our patients were taken for sur-

gical exploration for peritonitis and the presumed diagnosis

of appendicitis. During surgical exploration, inflammatory

changes of the appendix were visualized in both cases

though were more prominent in case two. No other

abnormalities were seen. The pathology reports varied and

noted unusual features such as focal infiltration and the

presence of eosinophils, though both showed neutrophilic

invasion of the muscularis propria consistent with acute

appendicitis (Fig. 1). Though both cases met pathologic

criteria to receive the diagnosis of acute appendicitis, the

additional atypical findings may be considered clues to the

diagnosis of KD. In addition, in both cases, the patients’

advanced initial clinical presentation and prolonged post-

operative course without improvement did not correlate

with the gross surgical and pathologic findings of non-

complicated acute appendicitis.

Our two cases shared a number of clinical features with

other KD patients presenting with appendicitis and acute

abdomen. As in the vast majority of reported KD patients

presenting with acute abdomen, both of our cases had

incomplete presentations of KD, possessing persistent

fever and just three of the five principle features. Most of

the features developed after surgery were undertaken. As

imaging was inconclusive, the presumed diagnosis of

appendicitis in our two cases was based on clinical criteria,

mainly the presence of peritonitis. In both patients, there

was laboratory evidence of systemic inflammation, how-

ever, non-specific (drastically elevated CRP, WBC). A

mild transaminitis was confirmed in one patient, a feature

not classically seen in acute appendicitis [10] but not

uncommonly seen with KD [1]. Postoperatively, both

patients failed to improve and had persistent abdominal

pain and fever, later developing some of the principle

clinical features of KD. Diagnosis was confirmed with

echocardiographic findings of coronary artery dilatation.

Coronary artery aneurysm (CAA) development is the

most dreaded complication of KD as it places the patient at

risk for ischemic heart disease, myocardial infarction and

sudden death [1]. Coronary artery aneurysms are present in

15–25 % of untreated patients [11]. Treatment within the

first 10 days of disease onset with IVIG has been shown to

reduce the incidence of CAA to\5 % [7]. The 10–45 % of

children presenting with atypical KD have a higher risk of

developing CAAs [3]. In reported KD patients presenting

with acute surgical abdomen, the vast majority of which

presented with atypical KD, the incidence of CAA for-

mation was 50 % [7]. As gastrointestinal symptoms often

precede the classic features of KD, there may be a delay in

diagnosis and treatment as the initial focus is diverted

towards treating the abdominal pain [5]. However, despite

prompt diagnosis and early IVIG therapy in the select

group of KD patients presenting with acute abdomen, the

development of coronary artery aneurysms as well as

abdominal complications prompting surgical intervention

could not be arrested [7]. In our two patients, IVIG treat-

ment was initiated after surgery but within 10 days of the

onset of fever. Coronary artery aneurysms were already

present prior to treatment. These findings seem to support

Zulian’s suggestion that KD patients presenting with acute

abdomen may possess a more severe vasculitis involving

multiple systems including the intestinal tract and carry a

more aggressive form of the disease [7].

Conclusion

In cases of acute abdomen, surgery should not be delayed.

However, familiarity with the clinical features of KD is

essential as the persistence of fever, particularly in the

postoperative period, and the presence of two or three of

these features should prompt the physician to consider the

diagnosis of KD. While uncommon, Kawasaki patients

Fig. 1 Histopathology of cases 1 and 2 showing the presence of

neutrophils in the muscularis propria of the appendiceal wall

Pediatr Surg Int (2014) 30:549–552 551

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initially presenting with acute abdomen will likely be

admitted to the surgical service. As there is evidence to

suggest that these patients carry a more aggressive form of

the disease, positive outcomes in this unique group will

rely on a surgeon’s ability to promptly diagnosis and ini-

tiate treatment of this unusual disease.

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