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Ophthalmologic Manifestations of Kawasaki Disease

  • Author: Shauna Berry, DO; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Jul 07, 2016
 

Overview

Kawasaki disease is a systemic vasculitis of unknown etiology that affects the small- and medium-sized blood vessels of the body, particularly the coronary arteries.

Since its original description, in Japan in 1967, Kawasaki disease has been reported worldwide in children of all ethnic origins. The yearly incidence of endemic Kawasaki disease in the United States is 67 cases per 100,000 children who are younger than age 5 years. Approximately 3,000 hospitalizations occur annually in the United States as a result of Kawasaki disease.

The incidence of Kawasaki disease in Europe is comparable to that in the United States. In Japan, where Kawasaki disease has the highest incidence, an estimated 5000-6000 cases occur annually. Individuals of Asian descent are 20 times more likely to develop Kawasaki disease than whites.[1] The exact etiology is unknown; however, recent evidence suggests a combination of genetic factors and a possible infectious source.[2]

Go to Kawasaki Disease and Dermatologic Manifestations of Kawasaki Disease for complete information on these topics.

See Kawasaki Disease: Do You Know the Signs?, a Critical Images slideshow, to help identify the specific criteria for diagnosis. Also note the general images below.

Patchy generalized macular erythema, which is also Patchy generalized macular erythema, which is also typical of some viral exanthems.
Peeling and erythema of the fingertips. Peeling and erythema of the fingertips.
Strawberry tongue. Strawberry tongue.
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Ophthalmologic Presentation

The most common ophthalmologic finding in Kawasaki disease is bilateral conjunctival injection. Iridocyclitis, superficial punctate keratitis, vitreous opacities, papilledema, and subconjunctival hemorrhage also may occur.

In a study by Ohno et al, the incidence of superficial punctate keratitis, vitreous opacities, papilledema, and subconjunctival hemorrhage was, respectively, 22%, 11%, 11%, and 6%.[3]

Bilateral conjunctival erythema is one of the classic diagnostic criteria for Kawasaki disease, being present in more than 90% of children with the disorder. These findings occur in the acute phase of the disease and appear shortly after the fever.[3] It is mostly confined to the bulbar region and is nonexudative. Chemosis, follicles, and papillae are characteristically absent.

Conjunctival pathology obtained during the acute phase showed an increased number of neutrophils surrounding the conjunctival epithelial cells. This pattern, termed neutrophilic rosetting, is present in 36% of cases.[4] A 2016 study in Korea found that individuals with Kawasaki disease who did not initially present with anterior uveitis had a higher risk of coronary events and longer hospitalization. Therefore, one can hypothesize that the detection of anterior uveitis leads to an earlier diagnosis of Kawasaki disease, as opposed to a potentially delayed diagnosis in individuals who present without anterior uveitis.[5]

Burns et al found evidence of anterior uveitis in 83% of children who were diagnosed with Kawasaki disease in the first week of their illness.[6] The anterior uveitis, which is usually mild and bilateral, is sometimes associated with keratic precipitates. Anterior uveitis peaks about a week after fever onset. It generally resolves within 2-8 weeks after disease onset without any of the common sequelae that often are seen with many other infectious causes of uveitis.

Ryan and Walton described a case of a 10-month-old male infant in whom bilateral scarring of the superior and inferior fornices developed in association with Kawasaki disease.[7]

Less common posterior segment manifestations usually are limited to case reports. One such report, by Font et al, described bilateral inner retinal ischemia due to thrombotic occlusion as a result of systemic vasculitis.[8]

One case documented periorbital vasculitis in an 8-month-old infant with Kawasaki disease, a previously unreported manifestation of the disorder.

Although exceptionally rare, neuro-ophthalmic findings may be associated with Kawasaki disease. Forty-one reported cases of facial nerve palsies associated with Kawasaki disease have been reported in the current literature. Such patients are reported to have a higher risk of coronary aneurysms and therefore should be monitored with increased vigilance.[9] Other neuro-ophthalmic findings include cranial nerve palsies such as oculomotor palsy (reported by Thapa et al[10] ) and supranuclear vertical palsy (reported by Delafay et al[11] ). A few cases of papilledema have been reported.[12] These cranial nerve findings are presumed to result from inflammation of the nerves and increased intracranial pressure due to an aseptic meningitis component in some individuals.[9, 12, 10]

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Diagnostic Considerations

Kawasaki disease is uncommon, while bilateral conjunctivitis is extremely common. The astute primary care physician, emergency room specialist, urgent care physician, or ophthalmologist should scrutinize cases of bilateral ocular redness associated with persistent fever, rash, and lymphadenopathy, particularly in the pediatric, Asian demographic groups most at risk. The lack of definitive autoimmune markers and laboratory tests stresses the importance of a prompt clinical diagnosis based on the constellation of symptoms. However, ongoing research is investigating the association with specific human leukocyte antigen (HLA) alleles.[2] Referral to the appropriate pediatric subspecialist will ascertain community standard care in selected cases.[13, 14, 15]

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Treatment & Management

Consultation with a pediatrician, a pediatric intensivist, or a pediatric cardiologist is essential to the delivery of community standard care.

Treat Kawasaki disease uveitis with topical corticosteroids, such as prednisolone acetate, loteprednol etabonate, or dexamethasone, as well as with topical cycloplegics. Although it generally resolves within days of intravenous immunoglobulin therapy, conjunctival inflammation also may be treated with topical corticosteroids.[15, 16]

Keratitis is treated with supportive care using topical tear replacement, preservative-free tears, and ophthalmic ointments, and through the avoidance of irritants, tap water, rubbing behavior, and excessive use of topical preserved drops.

Cranial neuropathies are treated with supportive care and generally resolve after the initiation of intravenous immunoglobulin therapy. No long-term complications have been reported in these cases.[11, 9]

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Contributor Information and Disclosures
Author

Shauna Berry, DO Resident Physician, Department of Ophthalmology, Larkin Community Hospital, Nova Southeastern University College of Osteopathic Medicine

Shauna Berry, DO is a member of the following medical societies: American Academy of Ophthalmology, American Osteopathic Association, American Osteopathic Colleges of Ophthalmology and Otolaryngology-Head and Neck Surgery, Florida Osteopathic Medical Association, Women in Ophthalmology, Inc

Disclosure: Nothing to disclose.

Coauthor(s)

Andrew G Lee, MD Chair, Department of Ophthalmology, Houston Methodist Hospital; Clinical Professor, Associate Program Director, Department of Ophthalmology and Visual Sciences, The University of Texas Medical Branch; Clinical Professor, Department of Surgery, Division of Head and Neck Surgery, University of Texas MD Anderson Cancer Center; Professor of Ophthalmology, Neurology, and Neurological Surgery, Weill Medical College of Cornell University; Clinical Associate Professor, University of Buffalo, State University of New York School of Medicine

Andrew G Lee, MD is a member of the following medical societies: American Academy of Ophthalmology, Association of University Professors of Ophthalmology, American Geriatrics Society, Houston Neurological Society, Houston Ophthalmological Society, International Council of Ophthalmology, North American Neuro-Ophthalmology Society, Pan-American Association of Ophthalmology, Texas Ophthalmological Association

Disclosure: Received ownership interest from Credential Protection for other.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

R Christopher Walton, MD Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, University of Tennessee College of Medicine

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Retina Society, American College of Healthcare Executives, American Uveitis Society

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

John D Sheppard, Jr, MD, MMSc Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard, Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, American Uveitis Society

Disclosure: Nothing to disclose.

Robert William Enzenauer, MD, MPH, MBA, MSS Professor, Department of Ophthalmology, University of Colorado School of Medicine; Chairman, Department of Ophthalmology, Children's Hospital

Robert William Enzenauer, MD, MPH, MBA, MSS is a member of the following medical societies: American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, American Association for Physician Leadership, American Academy of Ophthalmology

Disclosure: CLEAR DONOR: Received consulting fee from Clear Donor for consulting; Partner received salary from Clear Donor for employment.

Kalpana K Jatla, MD Private Practice, Clarity Eye Center

Kalpana K Jatla, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Spencer B Witcher, MD,to the development and writing of the source article.

References
  1. Greco A, De Virgilio A, Rizzo MI, Tombolini M, Gallo A, Fusconi M, et al. Kawasaki disease: an evolving paradigm. Autoimmun Rev. 2015 Aug. 14 (8):703-9. [Medline].

  2. Parthasarathy P, Agarwal A, Chawla K, Tofighi T, Mondal TK. Upcoming biomarkers for the diagnosis of Kawasaki disease: A review. Clin Biochem. 2015 Nov. 48 (16-17):1188-94. [Medline].

  3. Ohno S, Miyajima T, Higuchi M, et al. Ocular manifestations of Kawasaki's disease (mucocutaneous lymph node syndrome). Am J Ophthalmol. 1982 Jun. 93(6):713-7. [Medline].

  4. Al-Abbadi MA, Abuhammour W, Harahsheh A, Abdel-Haq NM, Hasan RA, Saleh HA. Conjunctival changes in children with Kawasaki disease: cytopathologic characterization. Acta Cytol. 2007 May-Jun. 51 (3):370-4. [Medline].

  5. Lee KJ, Kim HJ, Kim MJ, Yoon JH, Lee EJ, Lee JY, et al. Usefulness of anterior uveitis as an additional tool for diagnosing incomplete Kawasaki disease. Korean J Pediatr. 2016 Apr. 59 (4):174-7. [Medline].

  6. Burns JC, Joffe L, Sargent RA, et al. Anterior uveitis associated with Kawasaki syndrome. Pediatr Infect Dis. 1985 May-Jun. 4(3):258-61. [Medline].

  7. Ryan EH, Walton DS. Conjunctival scarring in Kawasaki disease: a new finding?. J Pediatr Ophthalmol Strabismus. 1983 May-Jun. 20(3):106-8. [Medline].

  8. Font RL, Mehta RS, Streusand SB, et al. Bilateral retinal ischemia in Kawasaki disease. Postmortem findings and electron microscopic observations. Ophthalmology. 1983 May. 90(5):569-77. [Medline].

  9. Stowe RC. Facial nerve palsy, Kawasaki disease, and coronary artery aneurysm. Eur J Paediatr Neurol. 2015 Sep. 19 (5):607-9. [Medline].

  10. Thapa R, Mallick D, Biswas B, Chakrabartty S. Transient unilateral oculomotor palsy and severe headache in childhood Kawasaki disease. Rheumatol Int. 2011 Jan. 31 (1):97-9. [Medline].

  11. Delafay MC, Matoussi Z, Remy-Piccolo V, Gay C, Veyrier M, Stéphan JL. [Kawasaki disease and cranial nerve involvement: two cases]. Arch Pediatr. 2015 Aug. 22 (8):853-6. [Medline].

  12. Anand S, Yang YC. Optic disc changes in Kawasaki disease. J Pediatr Ophthalmol Strabismus. 2004 May-Jun. 41 (3):177-9. [Medline].

  13. Blatt AN, Vogler L, Tychsen L. Incomplete presentations in a series of 37 children with Kawasaki disease: the role of the pediatric ophthalmologist. J Pediatr Ophthalmol Strabismus. 1996 Mar-Apr. 33(2):114-9. [Medline].

  14. Cerman E, Eraslan M, Turhan SA, Usta SA, Akalin F. Orbital cellulitis presenting as a first sign of incomplete kawasaki disease. Case Rep Ophthalmol. 2013 Sep. 4(3):294-8. [Medline]. [Full Text].

  15. Erdem E, Kocabas E, Taylan Sekeroglu H, Ozgür O, Yagmur M, Ersoz TR. Crystalline-like keratopathy after intravenous immunoglobulin therapy with incomplete kawasaki disease: case report and literature review. Case Rep Ophthalmol Med. 2013. 2013:621952. [Medline]. [Full Text].

  16. Saadoun D, Bodaghi B, Bienvenu B, Wechsler B, Sene D, Trad S, et al. Biotherapies in inflammatory ocular disorders: Interferons, immunoglobulins, monoclonal antibodies. Autoimmun Rev. 2013 May. 12(7):774-83. [Medline].

 
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Patchy generalized macular erythema, which is also typical of some viral exanthems.
Peeling and erythema of the fingertips.
Strawberry tongue.
 
 
 
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