Kawasaki disease (KD) is an acute systemic vasculitis of childhood. *e diagnosis can be made in a patient who presents witha prolonged high fever and meeting at least four of five criteria including polymorphous rash, mucosal changes, extremity changes(including swelling and/or palmar and plantar erythema), bilateral nonsuppurative conjunctivitis, and unilateral cervicallymphadenopathy. Atypical KD refers to patients who have not met the full criteria and in whom atypical features may be present.We discuss a case of a 6-year-old male who presented to the Emergency Department with torticollis. A series of investigations forelevated inflammatory markers revealed dilated coronary artery aneurysms on echocardiogram, and thus he was diagnosed withatypical KD. His only other criteria were bilateral nonsuppurative conjunctivitis and a prior brief febrile illness. He was treatedwith high-dose intravenous immune globulin (IVIG) and low-dose aspirin. Low-molecular-weight heparin and atenolol wereadded due to the presence of giant aneurysms.
1. Introduction
Kawasaki disease (KD) is an acute systemic vasculitis ofchildhood diagnosed by high fever for 5 days accompaniedby at least four of the following symptoms: polymorphousrash, mucosal changes (including dry, cracked lips andstrawberry tongue), extremity changes (including palmarand/or plantar erythema, swelling, and desquamation), bi-lateral nonsuppurative conjunctivitis, and cervical lymph-adenopathy [1]. It is the second most common vasculitis ofchildhood with a peak age of 2-3 years and rarely seen abovethe age of 7 years. KD is now the most common cause ofacquired heart disease in children in developed countries [2].Atypical KD refers to patients who have not met the fullcriteria and in whom atypical features may be present [3].Risk factors for development of coronary artery aneurysmswith KD include prolonged fever, prolonged elevation ofinflammatory markers, age younger than 1 year or olderthan 6 years at onset, and male gender [1]. KD is treated withaspirin and intravenous immune globulin (IVIG) whichreduces the incidence of coronary artery involvement fromapproximately 25% to less than 4% [4].
2. Case Presentation
A previously healthy 6-year-old boy presented to a pediatrichospital with a 3-week history of torticollis. He hadsymptoms of an upper respiratory tract infection four weeksprior and had 2 days of documented fever at home duringthat time. He had been treated with a 7-day course ofamoxicillin by the primary care physician for suspectedstreptococcal pharyngitis. Four days into the course ofantibiotics, he woke up from sleep with pain on the left sideof his neck. Despite taking ibuprofen and acetaminophen, hepresented to the Emergency Department 3 weeks later due topersisting torticollis. Pain was worse with movement. *erewas no history of head/neck trauma. At the time of pre-sentation, the infectious symptoms had resolved. Some fa-tigue was noted but he remained generally active, continuingto play hockey. *ere was no history of rash, peripheral jointpain, or weight loss. Past medical history and family historywere unremarkable.
On examination, the patient was afebrile with normalblood pressure for age and a maximum heart rate of 110beats per minute. *e patient’s head was tilted to the right
HindawiCase Reports in PediatricsVolume 2018, Article ID 4236264, 3 pageshttps://doi.org/10.1155/2018/4236264
with chin rotation to the left. No lymphadenopathy ormasses were noted on palpation of the neck. *ere was notenderness to palpation of bilateral sternocleidomastoidmuscles. *ere was a limited range of motion in all planesof rotation of the neck secondary to pain, particularly inlateral flexion. Bilateral injected conjunctivas were present.*e oropharynx was normal with no erythema or mucusmembrane changes. Cardiovascular exam revealed normalperipheral pulses, a quiet precordium with normal heartsounds, and no murmur. Respiratory exam was normal.*e abdomen was soft with no distension, tenderness, orhepatosplenomegaly. *ere were no bruits heard on aus-cultation of major vessel regions. *ere were no rashes ordesquamation of the skin. Neurological exam was normal.
At the time of presentation, laboratory investigationsrevealed an elevated white blood cell count of 17.4×109/Lwith a neutrophil count of 14.1× 109/L. Hemoglobin wasnormal for age at 110 g/L. Inflammatory markers were el-evated including platelet count of 860×109/L and CRP of38.5mg/L. Renal function (BUN and creatinine) and liverfunction (ALP and ALT) were normal for age. Because of theunexplained elevated white blood cell count and evidence ofinflammation, a chest X-ray was performed which revealednormal lung fields but an enlarged cardiac silhouette. X-rayof the cervical spine was normal with no atlantoaxial rotarysubluxation demonstrated. Ultrasound of the neck revealedmild thickening of the left sternocleidomastoid muscle andno lymphadenopathy. Abdominal ultrasound with Dopplerwas normal.
Additional investigations included a normal throat swabfor group A streptococci and a negative anti-streptolysin Oantibody titer. High-sensitivity troponin was elevated to176 ng/L. Creatinine kinase was normal. ANCA was normal.Electrocardiogram showed normal sinus rhythms withoutevidence of chamber hypertrophy.*e patient underwent anechocardiogram to further characterize the enlarged cardiacsilhouette identified on the chest X-ray. *is revealedmassive ectasia and aneurysmal dilatation of the rightcoronary artery, left main artery, left anterior descendingartery, and circumflex arteries, as seen in Figure 1. Leftventricular function was normal.*e aortic arch was normalas were the proximal neck vessels.
Because of the dilated coronary aneurysms, the pa-tient was diagnosed with KD. Despite lack of fever, giventhe evidence of ongoing inflammation and initial pres-ence of bilateral nonsuppurative conjunctivitis, in ad-dition to the coronary artery changes, the patient wastreated with high-dose IVIG (2 g/kg) and started on dailylow-dose aspirin. Low-molecular-weight heparin wasstarted as antithrombotic therapy and once stabilized,daily atenolol was initiated. Activity was restricted asmuch as possible.
Inflammatory markers were followed. Platelets revealeda peak of 952×109/L and CRP a peak of 54.6mg/L. Aftertreatment, both platelet and CRP levels normalized.
*e patient’s neck pain and the limited range ofmovement resolved immediately after treatment, as did the
bilateral conjunctivitis. *e patient was stable and appearedwell at time of discharge. His aspirin, low-molecular-weightheparin, and atenolol were continued. *e CT angiogramperformed after discharge revealed massively dilated andaneurysmal coronary arteries, as shown in Figure 2.
In follow-up cardiology and rheumatology clinics, hehas been doing well with no further neck pain or stiffness.He did not develop desquamation during follow-up, andthe repeat echocardiogram one month after discharge wasunchanged. He will continue long-term anticoagulationtherapy with low-dose heparin with a target level greaterthan 0.5 IU/ml. He will also continue low dose aspirin andatenolol. His family was advised to have the annual in-fluenza vaccine.
3. Discussion
Our patient was diagnosed with KD after dilated coronaryartery aneurysms were found on the echocardiogram. Hehad a history of fever for two days that occurred three weeksprior to presentation, and no further fevers were docu-mented or recognized by his parents. *e only criteria of KDmet on history and examination at presentation was bilateralnonsuppurative conjunctivitis. Blood work did reveal evi-dence of ongoing inflammation. Risk factors for KD withcoronary involvement were male gender and a delayedpresentation prior to diagnosis. It is possible that he hadfever longer than the reported two days as the parents hadnot measured it regularly at home. Presumably, the in-flammatory markers had been elevated for up to three weeksprior to diagnosis; however, no blood work had been per-formed during the initial febrile period.
Although unusual, there have been several reports in theliterature of KD presenting as torticollis or neck tilt. Dif-ferent pathophysiologies have been described including KDassociated with Grisel’s syndrome, a rare, nontraumaticatlantoaxial subluxation [5]; KD with retropharyngealedema and arthritis of the small joints in the head and neckregion [6]; and KD with severe cervical spine and bilateraltemporomandibular joint arthritis [7]. Our patient did nothave any other signs of arthritis, and there was no cervicallymphadenopathy to explain the torticollis. During theadmission, an X-ray of the cervical spine and ultrasound ofthe neck did not reveal any underlying pathology. *etorticollis resolved after treatment with IVIG and aspirinand did not recur.
Despite giant coronary aneurysms, our patient hasremained well since discharge from hospital and is closelyfollowed by Cardiology and Rheumatology. He has had nofurther neck pain or stiffness and has not developed anyfurther symptoms including desquamation or arthritis.Repeat echocardiograms have remained stable. He will re-quire long-term anticoagulation therapy.
Our patient was brought to medical attention due to historticollis. While the exact reason for the torticollis is un-clear, we feel it is important to raise the awareness of this rare
2 Case Reports in Pediatrics
manifestation of Kawasaki disease.
Consent
Written consent was obtained.
Conflicts of Interest
*e authors declare that there are no conflicts of interestregarding the publication of this article.
References
[1] W. E. Nelson, K. J. Marcdante, and R. M. Kliegman, NelsonEssentials of Pediatrics, Elsevier Saunders, Philadelphia, PA,USA, 2015.
[2] B. W. Mccrindle, A. H. Rowley, J. W. Newburger et al., “Di-agnosis, treatment, and long-term management of Kawasakidisease: a scientific statement for health professionals from theamerican heart association,” Circulation, vol. 135, no. 17,pp. e927–e999, 2017.
[3] R. E. Petty, R. M. Laxer, C. B. Lindsley, and L. Wedderburn,Textbook of Pediatric Rheumatology, Elsevier, New York, NY,USA, 2016.
[4] J. W. Newburger, M. Takahashi, A. S. Beiser et al., “A singleintravenous infusion of gamma globulin as compared with fourinfusions in the treatment of acute Kawasaki syndrome,” NewEngland Journal of Medicine, vol. 324, no. 23, pp. 1633–1639,1991.
[5] F. Nozaki, T. Kusunoki, Y. Tomoda et al., “Grisel syndrome asa complication of Kawasaki disease: a case report and review ofthe literature,” European Journal of Pediatrics, vol. 172, no. 1,pp. 119–121, 2012.
[6] L. Puhakka, R. Saat, T. Klockars, L. Kajosaari, E. Salo, andT. Nieminen, “Retropharyngeal involvement in Kawasakidisease—a report of four patients with retropharyngeal edemaverified by magnetic resonance imaging,” International Journalof Pediatric Otorhinolaryngology, vol. 78, no. 10, pp. 1774–1778,2014.
[7] M. Jen, L. A. Brucia, A. N. Pollock, and J. M. Burnham,“Cervical spine and temporomandibular joint arthritis ina child with Kawasaki disease,” Pediatrics, vol. 118, no. 5,pp. e1569–e1571, 2006.
A B
C
(a)
D
(b)
Figure 1: Echocardiogram: (a) Massive ectasia and aneurysmal dilatation of the right coronary artery (16mm× 17mm) (A), aorta (B), andleft coronary artery (C). (b) Massive ectasia and aneurysmal dilatation of left main artery (13mm× 13mm) (D).
CB
A
Figure 2: CT angiogram: there is a long fusiform aneurysm in-volving the proximal LAD measuring approximately12mm× 12mm and extending over a length of 2.3 cm (A). *e leftcircumflex is aneurysmal proximally measuring approximately5mm× 5.5mm (B). *ere is a long fusiform aneurysm of rightcoronary artery measuring 16mm× 16mm and extending overa length of at least 3.2 cm (C).
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