A rare cervical complication of Kawasaki disease: a retrospective study of clinical and radiological features
Original Article

A rare cervical complication of Kawasaki disease: a retrospective study of clinical and radiological features

Chunyi Yan1,2#, Jinquan Liu1,2#, Xiaoliang Liu1,2, Fuqiang Liu1,2, Zexi Li3, Yarui Cui1,2, Nanjun Zhang1,2, Bowen Li1,2, Yimin Hua1,2, Kaiyu Zhou1,2, Shuran Shao1,2, Chuan Wang1,2

1Department of Pediatric Cardiology, West China Second University Hospital, Sichuan University, Chengdu, China; 2Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, China; 3Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China

Contributions: (I) Conception and design: C Yan, J Liu, S Shao, C Wang; (II) Administrative support: S Shao, C Wang; (III) Provision of study materials or patients: X Liu, F Liu, Y Cui, N Zhang, B Li, Y Hua, K Zhou; (IV) Collection and assembly of data: X Liu, F Liu, Z Li, Y Cui, N Zhang, B Li, Y Hua, K Zhou; (V) Data analysis and interpretation: C Yan, J Liu, X Liu; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.

#These authors contributed equally to this work.

Correspondence to: Shuran Shao, MD; Chuan Wang, MD. Department of Pediatric Cardiology, West China Second University Hospital, Sichuan University, No. 20, Section 3, South Renmin Road, Chengdu 610041, China; Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, China. Email: 36882191@qq.com; wangchuan1308@163.com.

Background: Grisel’s syndrome (GS) is a rare cervical complication of Kawasaki disease (KD). Evidence from larger cohorts remains limited. We aimed to characterize the clinical and imaging features of KD with GS, identify factors associated with GS, and examine relationships between atlantoaxial instability metrics and local pharyngeal findings.

Methods: We conducted a single-center retrospective cohort study involving children with KD treated between 2015 and 2023. KD was diagnosed according to the American Heart Association guidelines. GS was confirmed by clinical and imaging criteria. We collected demographics, clinical features, laboratory indices, and cervical imaging metrics, including the anterior atlanto-dental interval (AADI), lateral atlanto-dental interval (LADI), and retropharyngeal space (RS). GS versus non-GS groups were compared using appropriate parametric/nonparametric and categorical tests. Variables with P<0.10 in univariable analyses were entered into multivariable logistic regression to identify factors associated with GS. Within the GS subset, we stratified by AADI and evaluated correlations among AADI, RS, and cervical lymph-node size.

Results: Among 2,137 KD patients, 46 (2.15%) developed GS. GS patients were older (6.97±1.96 vs. 2.29±2.00 years, P<0.001) and had more frequent cervical lymphadenopathy (80.4% vs. 47.0%, P<0.001). They also showed higher neutrophil percentage, ESR, creatinine, and urea nitrogen (all P<0.05). Multivariate analysis identified age, ESR, and creatinine as independent predictors of GS. Excluding age, female sex, lymphadenopathy, and neutrophil percentage remained significant. Patients with higher AADI (>2.57 mm) had larger lymph nodes and thicker RS. AADI correlated positively with lymph node size and RS, suggesting local inflammation contributes to atlantoaxial instability in KD-associated GS.

Conclusions: GS is an uncommon complication of KD that occurs more often in older children and female patients. Imaging markers of atlantoaxial instability correlate with retropharyngeal crowding and nodal enlargement, while systemic inflammatory indices show limited associations. Early recognition and targeted cervical imaging may aid timely diagnosis and management.

Keywords: Kawasaki disease (KD); Grisel’s syndrome (GS); atlantoaxial rotatory fixation (AARF); cervical lymphadenopathy (CL)


Submitted Aug 14, 2025. Accepted for publication Nov 03, 2025. Published online Dec 31, 2025.

doi: 10.21037/qims-2025-1762


Introduction

Kawasaki disease (KD) is a common acute vasculitis syndrome primarily affecting children, though its etiology remains largely unknown. It presents as a multisystemic disorder, with symptoms including conjunctival congestion, rash, and lymphadenopathy, and it can also involve the respiratory, gastrointestinal, nervous, and urinary systems (1). Research indicates that cervical lymphadenopathy (CL) occurs in approximately 50–75% of the KD patients (2). Approximately 12% of patients present with fever and CL as the initial symptom. These patients often exhibit a cervical mass, tenderness, and restricted neck movement (3).

Grisel’s syndrome (GS) primarily occurs in children and is a rare non-traumatic atlantoaxial subluxation, also known as atlantoaxial rotatory fixation (AARF) (4). It can result from any upper cervical inflammation, such as CL, or following otolaryngologic surgery (5). Given that CL is a common symptom of KD (6), it is reasonable to expect that GS might also occur in KD patients. However, current reports of GS predominantly come from the fields of otolaryngology, neurosurgery, and orthopedics, with limited documentation in the context of KD in children (7,8). Although there are sporadic case reports of GS in KD, systematic studies remain lacking. When diagnosed early, GS can typically be managed conservatively; however, delayed diagnosis may lead to severe atlantoaxial deformities necessitating complex surgical intervention (9,10).

Therefore, this study aims to: (I) compare the clinical and laboratory features of KD patients with and without GS; (II) identify risk factors for GS development in KD; and (III) assess factors associated with the severity of AARF in GS patients. The goal is to improve understanding of this rare KD complication, to facilitate early recognition and to improve prognostic assessment in practice. We present this article in accordance with the STROBE reporting checklist (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1762/rc).


Methods

Study population

This retrospective study was conducted at West China Second University Hospital, Sichuan University, where we reviewed the medical records of 2,451 patients diagnosed with KD and treated between January 2015 and December 2023. The diagnosis of KD was established according to the 2004 American Heart Association criteria (11). Patients aged ≤18 years with complete clinical and laboratory data prior to initial intravenous immunoglobulin (IVIG) treatment were included. Those with incomplete records, uncertain diagnosis, prior treatment at other institutions, or concomitant conditions such as trauma, surgery, or congenital cervical disorders were excluded.

The diagnosis of GS was based on both clinical and radiological criteria. For a patient without recent trauma who presents with neck pain, fever, restricted range of motion, and pain on attempted repositioning, the combination of these clinical features with radiological evidence of AARF establishes the diagnosis of GS (12-14). All patients received standardized treatment for KD according to established clinical guidelines (11). The treatment strategy for GS was based on the Fielding-Hawkins classification, with different subtypes corresponding to specific management approaches, including non-steroidal anti-inflammatory drugs (NSAIDs), physical therapy, analgesics, or surgical intervention (12). However, all patients in this study were treated with non-surgical approaches.

The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Ethics Committee of Sichuan University (No. 201712160121). Written informed consent was obtained from the guardians of all pediatric patients.

Data collection

To compare KD patients with and without concomitant GS, we retrospectively collected baseline characteristics, clinical manifestations at admission, complications, prognosis, and laboratory data from medical records. Baseline data included age, sex, body mass index (BMI), and the number of days of fever before IVIG treatment. Clinical features at admission included rash, edema and erythema of the extremities, bilateral bulbar conjunctival injection, erythema of the oral and pharyngeal mucosa, and CL. Complications and clinical outcomes were evaluated based on the presence of coronary artery lesions (CALs), resistance to IVIG, and length of hospital stay. CAL was diagnosed via echocardiography and determined using the Z-score, calculated based on body surface area, and a Z-score greater than 2 was defined as the presence of CAL (15,16).

Laboratory examinations data included white blood cell (WBC) count, neutrophil percentage, hemoglobin (HGB), platelet (PLT) count, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TB), creatinine, urea nitrogen and serum sodium and potassium. All laboratory tests were performed upon admission and prior to IVIG administration.

Radiological data were obtained from cervical spine computed tomography (CT), including coronal, sagittal, and axial views (Figure 1). The lateral atlanto-dental interval (LADI) was measured on coronal images as the distance between the odontoid process and the lateral masses of the atlas on each side. Lateral atlanto-dental interval asymmetry (LADIA) was defined as the difference between the left and right LADI [LADIA = absolute value of (right LADI − left LADI)]. The anterior atlanto-dental interval (AADI), measured on sagittal images, was defined as the horizontal distance between the anterior arch of the atlas and the odontoid process. The thickness of the retropharyngeal space (RS) was measured on sagittal views as the distance from the odontoid process—at the level of the inferior margin of the atlas—to the posterior pharyngeal wall. The atlantoaxial rotational angle (ARA) was measured on axial views as the angle between the midline of the atlas and the midline of the odontoid process. The longest diameter of the largest lymph node reported in radiology was used to define the lymph node size.

Figure 1 Radiological measurements of cervical spine parameters. (A) The right and left LADIs (a, b) were measured on the coronal CT image of the cervical spine. (B) The AADI (c) and the RS (d) were measured on the sagittal CT image. (C) The ARA (e) was measured on the axial CT image of the cervical spine. AADI, anterior atlanto-dental interval; ARA, atlantoaxial rotation angle; CT, computed tomography; LADI, lateral atlanto-dental interval; RS, retropharyngeal space.

Statistical analyses

Statistical analyses were performed using SPSS (version 21.0). Continuous variables were compared using appropriate parametric or non-parametric tests, and categorical variables with the Chi-squared or Fisher’s exact test. Variables with significance in univariate analyses were entered into a multivariate logistic regression model. Correlations between radiological parameters and lymph node size were assessed using Pearson correlation. A two-tailed P<0.05 was considered statistically significant.


Results

A total of 2,137 KD patients were included, comprising 1,214 males and 923 females. Of the 2,137 patients, 46 (2.15%) developed GS, while the remaining were classified as the non-GS group.

Univariate analysis (Table 1) showed that the GS group was significantly older than the non-GS group (6.97±1.96 vs. 2.29±2.00 years, P<0.001). CL was present in 80.4% of GS patients versus 47.0% of non-GS patients (37/46 vs. 983/2,091, P<0.001).

Table 1

Comparison of clinical features between GS group and non-GS group

Variables GS group (n=46) Non-GS group (n=2,091) P
Baseline
   Age (years) 6.96±1.81 2.29±2.00 <0.001*
   Gender <0.001*
    Male 13 1,201
    Female 33 890
   BMI (kg/m2) 15.35±1.55 16.52±1.52 0.364
   Length of fever before IVIG (days) 7.52±2.36 5.76±2.13 0.142
Clinical symptoms
   Rash 36 1,416 0.189
   Edema & erythema of the extremities 21 1,094 0.292
   Bilateral bulbar conjunctival injection 40 1,871 0.278
   Erythema of oral & pharyngeal mucosa 39 1,852 0.187
   CL 37 983 <0.001*
Complication and outcome
   CALs 2 335 0.710
   IVIG resistance 2 213 0.802
   Length of hospital stay (days) 7.12±3.53 6.64±5.29 0.610

Data are presented as mean ± standard deviation or n. *, P<0.05. BMI, body mass index; CALs, coronary artery lesions; CL, cervical lymphadenopathy; GS, Grisel’s syndrome; IVIG, intravenous immunoglobulin.

As summarized in Table 2, patients with GS had higher neutrophil percentage (75.36%±5.31% vs. 66.23%±15.63%, P<0.001), ESR (88.40±17.95 vs. 63.03±26.96 mm/h, P=0.047), creatinine (32.61±6.43 vs. 27.21±9.29 µmol/L, P=0.003), and urea nitrogen (3.65±1.63 vs. 3.11±1.59 µmol/L, P=0.022).

Table 2

Comparison of laboratory features between GS group and non-GS group

Variables GS group (n=46) Non-GS group (n=2,091) P
WBC count (×109/L) 14.48±5.30 13.78±5.11 0.378
Neutrophil percentage (%) 75.36±5.31 66.23±15.63 <0.001*
HGB (g/L) 112.81±8.66 110.25±11.89 0.165
PLT count (×109/L) 330.60±85.37 348.25±116.11 0.327
CRP (mg/L) 82.92±53.25 79.34±49.65 0.640
ESR (mm/h) 88.40±17.95 63.03±26.96 0.047*
ALT (U/L) 39.93±44.92 35.46±22.41 0.195
AST (U/L) 37.25±19.61 37.82±23.52 0.870
TB (μmol/L) 12.26±13.78 10.02±12.92 0.324
Creatinine (μmol/L) 32.61±6.43 27.21±9.29 0.003*
Urea nitrogen (mmol/L) 3.65±1.63 3.11±1.56 0.022*
Serum sodium (mmol/L) 136.18±2.29 135.95±3.27 0.361
Potassium (mmol/L) 3.98±0.52 3.72±0.36 0.230

Data are presented as mean ± standard deviation. *, P<0.05. ALT, alanine aminotransferase; AST, aspartate aminotransferase; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; GS, Grisel’s syndrome; HGB, hemoglobin; PLT, platelet; TB, total bilirubin; WBC, white blood cell.

Multivariate logistic regression (Table 3) identified age, ESR, and creatinine as significant predictors of GS, with age showing the highest odds ratio (OR; OR =4.806). To account for the potential confounding effect of age, an additional regression model excluding this variable was constructed (Table 4). In this model, female sex, CL, and neutrophil percentage remained significant predictors.

Table 3

Multivariate logistic regression analysis of relevant parameters for GS

Variables β SE Wald test df P Exp(β)
Age 1.570 0.189 69.100 1 <0.001* 4.806
Gender −1.406 0.437 10.364 1 0.001* 0.245
CL 0.779 0.499 2.436 1 0.119 2.180
Neutrophil percentage −0.021 0.013 2.834 1 0.092 0.979
ESR 0.000 0.007 0.005 1 0.944 1.000
Creatinine −0.045 0.029 2.339 1 0.126 0.956
Urea nitrogen 0.152 0.155 0.958 1 0.328 1.164

*, P<0.05. CL, cervical lymphadenopathy; ESR, erythrocyte sedimentation rate; GS, Grisel’s syndrome; SE, standard error.

Table 4

Multivariate logistic regression analysis of relevant parameters for GS

Variables β SE Wald test df P Exp(β)
Gender (male) −1.321 0.358 13.651 1 <0.001* 0.267
CL 1.148 0.384 8.951 1 0.003* 3.150
Neutrophil percentage 0.034 0.013 7.386 1 0.007* 1.035
ESR 0.007 0.006 1.663 1 0.197 1.007
Creatinine 0.026 0.014 3.392 1 0.066 1.026
Urea nitrogen −0.009 0.112 0.006 1 0.938 0.991

*, P<0.05. CL, cervical lymphadenopathy; ESR, erythrocyte sedimentation rate; GS, Grisel’s syndrome; SE, standard error.

The methods for diagnose AADI was 2.57±0.85 mm. Based on this mean value, patients were stratified into high- and low-AADI subgroups (22 vs. 24 cases, Table 5). The high-AADI subgroup had significantly larger lymph nodes (3.04±1.01 vs. 2.24±0.75 cm, P=0.004) and more frequent cervical traction use (8/22 vs. 2/24, P=0.022).

Table 5

Comparison of clinical data between high-AADI group and low-AADI group

Variables High-AADI group (n=22) Low-AADI group (n=24) P
Baseline
   Age (years) 6.91±1.54 7.00±2.06 0.867
   Gender 0.430
    Male 5 8
    Female 17 16
   BMI (kg/m2) 15.75±1.42 15.20±1.60 0.225
   Length of fever before IVIG (days) 7.91±2.03 7.21±1.93 0.269
Clinical symptoms
   Rash 12 14 0.961
   Edema & erythema of the extremities 10 11 0.980
   Bilateral bulbar conjunctival injection 18 22 0.327
   Erythema of oral & pharyngeal mucosa 19 20 0.777
   Cervical rotation (right side) 10 11 0.979
   CL 19 18 0.866
   Lymph node (right side) 9 12 0.541
   Lymph node size (cm) 3.04±1.01 2.24±0.75 0.004*
Complication and treatment
   Cervical traction 8 2 0.022*
   Cervical orthosis 12 11 0.559
   CALs 0 2 0.188
   IVIG resistance 1 1 0.950

Data are presented as mean ± standard deviation or n. *, P<0.05. AADI, anterior atlanto-dental interval; BMI, body mass index; CALs, coronary artery lesions; CL, cervical lymphadenopathy; IVIG, intravenous immunoglobulin.

Although laboratory parameters did not differ between subgroups (Table 6), radiological analysis showed greater LADIA (2.96±1.46 vs. 1.97±1.01 mm, P=0.010) and RS thickness (10.99±3.02 vs. 8.50±2.10 mm, P=0.002) in the high-AADI group.

Table 6

Comparison of laboratory and radiology data between high-AADI group and low-AADI group

Variables High-AADI group (n=22) Low-AADI group (n=24) P
Laboratory
   WBC count (×109/L) 12.90±5.60 15.98±4.65 0.056
   Neutrophil percentage (%) 72.77±17.34 77.84±13.10 0.284
   HGB (g/L) 112.59±8.51 113.09±8.98 0.828
   PLT count (×109/L) 343.20±97.71 319.14±72.81 0.368
   CRP (mg/L) 67.67±42.15 97.48±59.36 0.066
   ESR (mm/L) 75.05±17.95 67.29±23.24 0.316
   ALT (U/L) 38.86±20.83 32.69±15.89 0.562
   AST (U/L) 37.25±19.61 32.72±11.68 0.085
   TB (μmol/L) 11.22±12.05 12.96±13.54 0.648
   Creatinine (μmol/L) 33.32±8.04 30.33±5.84 0.154
   Urea nitrogen (μmol/L) 3.48±0.71 3.49±1.76 0.974
   Serum sodium (mmol/L) 136.40±0.57 135.98±2.23 0.540
   Potassium (mmol/L) 3.98±0.57 4.02±0.52 0.779
Radiology
   Right LADI 4.13±1.85 4.19±1.50 0.912
   Left LADI 4.81±2.15 3.73±1.61 0.058
   LADIA 2.96±1.46 1.97±1.01 0.010*
   ARA 12.36±6.47 10.00±9.51 0.334
   RS 10.99±3.02 8.50±2.10 0.002*

Data are presented as mean ± standard deviation. *, P<0.05. LADIA = absolute value of (right LADI − left LADI). AADI, anterior atlanto-dental interval; ALT, alanine aminotransferase; ARA, atlantoaxial rotational angle; AST, aspartate aminotransferase; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; HGB, hemoglobin; LADI, lateral atlanto-dens interval; LADIA, lateral atlanto-dental interval asymmetry; PLT, platelet; RS, retropharyngeal space; TB, total bilirubin; WBC, white blood cell.

Pearson correlation analysis (Table 7) demonstrated significant positive correlations between AADI and lymph node size (r=0.492, P<0.05), AADI and RS (r=0.375, P<0.05), as well as between lymph node size and RS (r=0.504, P<0.05). This quantifies the relationship between local anatomical changes and atlantoaxial instability in GS.

Table 7

Pearson correlation analysis of radiological features and lymph node size in the GS group

Variables AADI LADIA ARA RS Lymph node size
AADI 0.204 0.265 0.375* 0.492*
LADIA 0.204 0.175 0.262 0.189
ARA 0.265 0.175 −0.010 0.184
RS 0.375* 0.262 −0.010 0.504*
Lymph node size 0.492* 0.189 0.184 0.504*

*, P<0.05. AADI, anterior atlanto-dental interval; ARA, atlantoaxial rotational angle; GS, Grisel’s syndrome; LADIA, lateral atlanto-dental interval asymmetry; RS, retropharyngeal space.


Discussion

GS was first described in 1830 by Charles Bell, who, during an autopsy of a patient who died from acute spinal cord compression due to syphilitic pharyngeal ulcers, discovered erosion and ulceration of the atlantoaxial transverse ligament (17). In 1930, French physician Pierre Grisel further characterized the clinical manifestations, pathophysiology, and radiographic features of the condition in two case reports, which subsequently led to its eponym (8,18). Current evidence suggests that inflammation secondary to head and neck infections or surgical procedures is the primary trigger for GS. In recent years, KD has also been recognized as a potential cause of AARF and should therefore be considered a risk factor for GS (13,19-21). However, KD does not fall into these categories, as it is a non-infectious systemic inflammatory condition. KD is a common systemic inflammatory condition in children that may involve the cervical region and is often associated with CL, making the development of GS in KD patients theoretically plausible (6). However, published reports remain limited, consisting primarily of isolated case studies, with few investigations based on larger clinical cohorts (22-25). To our knowledge, large-sample, systematic evaluations of the clinical and imaging features of KD with GS are still scarce, and our study adds further evidence.

In this study, 46 cases of GS were identified, corresponding to an incidence rate of 2.15%, which is higher than the 0.6% reported in our previous investigation (26) and also greater than the rates reported by Michihata et al. (24) (0.23%), Oshita et al. (27) (1.08%). We speculate the higher incidence is due to increased clinical awareness. After our initial report identifying GS as a KD complication, clinicians became more proactive in obtaining cervical imaging for KD patients with suspicious symptoms, thereby raising the detection rate. This also indicates that cervical imaging should be given greater consideration for KD patients with neck symptoms to avoid missed GS diagnoses.

Clinically, this complication appears to affect older children with KD, with a mean age of 6.96±1.81 years. In addition, female patients appear to be at higher risk. Oshita et al. (27) conducted a retrospective analysis of insurance claims data to examine the incidence of AARF in children and adolescents. They reported a peak incidence around the age of 6 years, which is consistent with our findings. We further speculate that the current diagnostic approach in KD may influence the detection rate of GS. Older children are more likely to report neck pain or restricted mobility, and their caregivers may be more attentive to limitations in neck movement caused by the disease. Considering the potential risks associated with radiological imaging in children (28,29) and the difficulties in acquiring satisfactory images due to their limited cooperation (30), pediatricians typically do not include cervical imaging in the routine evaluation of KD unless neck-related symptoms are explicitly reported. This may partly explain the higher detection rate of GS in older children.

In contrast to our findings, Oshita et al. (27) reported in 2024 that observed a higher incidence of AARF in males. However, this cohort included patients without KD, which may account for the discrepancy from the present study. In a subsequent 2025 study, Oshita et al. (31) focusing specifically on KD-associated AARF found that female KD patients had a higher probability of developing AARF. Moreover, Michihata et al. (24), using a national database, observed a higher incidence of AARF in female KD patients. Furthermore, a case series by Nozaki et al. (23) involving nine patients reported that seven were female. Collectively, these findings are consistent with our results, suggesting that female KD patients may indeed be at greater risk of developing GS. Nevertheless, the existing evidence is largely derived from regionally confined cohorts. Future large-scale, multi-center studies with long-term follow-up will be necessary to validate these observations. Moreover, investigating the underlying molecular and biological mechanisms contributing to the higher prevalence of GS among female patients may provide deeper insights into the pathogenesis of this condition.

The GS and non-GS groups also demonstrated differences in laboratory findings. In this study, the neutrophil percentage was significantly higher in the GS group. Although WBC count was also higher in the GS group, the difference did not reach statistical significance. Jun et al. (6) reported that patients with cervical masses tend to have elevated WBC and neutrophil counts and are at higher risk of coronary artery dilation, suggesting that the presence of a CL may be indicative of more severe clinical presentation. However, our findings showed no significant increase in the incidence of CAL or IVIG resistance among patients with GS, suggesting that GS may be less strongly associated with the overall level of systemic inflammation and may have a limited relationship with the severity of vasculitis. Serum creatinine is a widely used marker for evaluating renal function. As creatinine is a product of muscle metabolism, its levels vary by age and sex. Previous studies have shown that serum creatinine levels increase with age (32). Similarly, urea nitrogen levels also change with age, and the ratio of urea nitrogen to creatinine significantly decreases as age increases (32,33), which is consistent with the findings of our study.

The methods for diagnosing AARF have been extensively described in previous studies. However, discussions regarding the gold standard for diagnosing AARF are still ongoing (34,35). Among the proposed measures, the AADI has received increasing attention in recent years and has been demonstrated to be effective in the diagnosis of AARF (36,37). In this study, AADI was used to stratify patients within the GS group. Bertozzi et al. (38) reported that the normal range of AADI in children should be less than 2.6 mm, which is very close to the cutoff value (2.57 mm) we used to distinguish between the high- and low-AADI groups in our study. Those in the high-AADI group had a higher incidence of CL, larger lymph nodes, and a greater need for cervical traction. These findings suggest a potential association between subluxation severity and the extent of cervical lymph node enlargement in KD patients with GS. Further analysis revealed no significant differences in laboratory parameters between the high- and low-AADI groups, suggesting that subluxation severity may not be closely related to systemic inflammatory activity. In contrast, radiological parameters such as LADIA and RS were significantly higher in the high-AADI group. Pearson correlation analysis demonstrated that AADI was positively correlated with both lymph node size and RS. These findings support the hypothesis that GS development in KD may be primarily driven by mechanical compression from enlarged cervical lymph nodes and thickened retropharyngeal tissues, rather than by systemic inflammation.

In summary, GS is an uncommon complication of KD, and its incidence may be underestimated. Although all patients in this study responded effectively to conservative treatment without experiencing severe complications, it is important to consider that children may develop irreversible severe atlantoaxial joint deformities, requiring complex surgical intervention. Therefore, in any febrile child with CL (after excluding common infections), clinicians should promptly consider KD and administer IVIG without delay. This may help prevent the development of GS. At the same time, the current diagnostic process may have limitations. As mentioned earlier, the diagnosis of GS relies on radiological examination, and whether imaging is conducted often depends on the patient’s or caregiver’s reported symptoms of the children, potentially leading to missed diagnoses. Our study indicates that, in addition to age, female sex, CL, high neutrophil percentage, and elevated ESR are risk factors for the development of GS. We also propose a new hypothesis regarding the pathogenesis of GS: soft tissue swelling plays a central role in mechanically displacing the atlantoaxial joint. Enlarged lymph nodes, together with adjacent edematous soft tissues, may exert combined mechanical pressure leading to AARF. Current guidelines and related studies have suggested that CL may be associated with deep cervical inflammation, leading to manifestations such as parapharyngeal and retropharyngeal edema (1,39). However, its potential link to the subsequent development of GS has not been clearly established, and standardized diagnostic and therapeutic protocols remain lacking (40). Therefore, we recommend that in cases presenting with CL, early imaging evaluation should be considered. Once the risk of GS is identified, conservative management can be initiated as a first-line approach, with close monitoring of symptom progression during the disease course and timely escalation to further treatment if necessary.

In addition, several emerging considerations warrant attention. Pediatricians may consider exploring new methods for assessing neck symptoms in children. The Modified Japanese Orthopaedic Association (mJOA) score and the Neck Disability Index (NDI) are commonly used in the field of spinal surgery to evaluate cervical spine disorders and neck function (41,42). A study by Sergeenko et al. (43) demonstrated that the mJOA is a reliable and effective tool for assessing cervical spine diseases in pediatric patients. Therefore, pediatricians could explore and validate the applicability and effectiveness of these adult-focused assessment and diagnostic tools in the pediatric population in future studies.

This study has several constraints. First, as a single-center study with participants limited to a specific region, there may be potential bias. Therefore, larger multi-center studies are needed to reduce such bias and improve generalizability. Second, most cases of GS in KD are acute and transient, and cervical spine imaging is not routinely performed during KD evaluation. As a result, the true incidence of GS may be underestimated due to missed or delayed diagnoses. More importantly, given the rarity of GS in KD, long-term follow-up data—particularly regarding post-discharge functional outcomes—were not systematically collected. Therefore, the mid- and long-term prognosis of affected patients remains unclear. Despite these shortcomings, this study represents the first large-scale investigation of GS in KD and offers valuable insights that may inform future clinical practice and research.


Conclusions

GS is a relatively rare complication in patients with KD, with an incidence of 2.15% in this cohort. Our findings suggest that GS predominantly affects older children and is associated with female sex, CL, elevated neutrophil percentage, and increased ESR. The development of GS does not appear to increase the risk of CAL or IVIG resistance, indicating only a limited association with systemic inflammatory response. Instead, it may be more closely related to mechanical compression of the atlantoaxial joint caused by CL and thickening of the RS. Given the risk of missed diagnoses due to the lack of routine imaging, pediatricians should maintain a high index of suspicion for GS in KD patients presenting with neck symptoms. Early recognition and timely radiological evaluation are essential to ensure accurate diagnosis and prevent potential complications.


Acknowledgments

None.


Footnote

Reporting Checklist: The authors have completed the STROBE reporting checklist. Available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1762/rc

Data Sharing Statement: Available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1762/dss

Funding: This study was supported financially by grants from Natural Science Foundation of Sichuan Province (No. 2024YFFK0272) and Key Research and Development Project of Chengdu Science and Technology Bureau (No. 2024-YF05-00237-SN).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1762/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Ethics Committee of Sichuan University (No. 201712160121). Written informed consent was obtained from the guardians of all pediatric patients.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.


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Cite this article as: Yan C, Liu J, Liu X, Liu F, Li Z, Cui Y, Zhang N, Li B, Hua Y, Zhou K, Shao S, Wang C. A rare cervical complication of Kawasaki disease: a retrospective study of clinical and radiological features. Quant Imaging Med Surg 2026;16(1):45. doi: 10.21037/qims-2025-1762

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