588979
research-article2015
CPJXXX10.1177/0009922815588979Clinical PediatricsBinnetoglu et al
Brief Report
Association Between Atypical/ Incomplete Kawasaki Disease and Sensorineural Hearing Loss: A Case Report
Clinical Pediatrics 1–4 © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815588979 cpj.sagepub.com
Adem Binnetoglu, MD1, Tekin Baglam, MD1, Berat Demir, MD1, Kiymet Kecelioglu Binnetoglu, MD1, and Murat Sari, MD1
Case Report A 6-month-old male was admitted to our Pediatrics Department because of fever that had persisted for 13 days. He had no history of drug allergy and infectious disease, or no recent vaccination, and no abnormal finding on physical examination. The laboratory and radiological findings were as follows: white blood cell count, 9.2 × 103/μL; platelet count, 975 × 103/μL; biochemical parameters of liver, thyroid, kidney and urine, and all serological assays of infectious and rheumatological diseases, and chest radiograph results were normal. The patient’s C-reactive protein level and erythrocyte sedimentation rate (ESR) were 9 mg/dL (normal range 0.00.8 mg/dL) and 107 mm/h (normal range 3-13 mm/h), respectively. Lumbar puncture revealed lymphocytic pleocytosis in the cerebrospinal fluid, but cultures were negative, indicating that aseptic meningitis was in play. Echocardiographic findings were considered to be suggestive of coronary artery involvement with aneurysmal changes. The patient was diagnosed with atypical/ incomplete Kawasaki disease. As therapy, acetylsalicylic acid (ASA) and intravenous immunoglobulin were given for 6 months. At this time, the patient’s family noted that he had hearing difficulties. On suspicion of sensorineural hearing loss (SNHL), a click-evoked auditory brainstem response test was performed and revealed no wave in either ear at maximum volume levels at 1000, 2000, or 4000 Hz, confirming a diagnosis of bilateral profound SNHL. Computed tomography and magnetic resonance imaging (Figure 1) of the temporal bone revealed no abnormality. As the patient did not benefit (in terms of speech perception) from 6 months of bilateral hearing aid use, he underwent right cochlear implantation after obtaining of informed consent from the parents.
Final Diagnosis Based on clinical picture and echocardiographic findings, the patient was diagnosed with sensorineural
hearing loss as a part of atypical/incomplete Kawasaki disease (KD).
Hospital Course No postoperative complication was noted. Stenvers and transverse scans were obtained to confirm accurate device placement in the cochlea (Figure 2). A follow-up evaluation showed that the implant facilitated speech perception. The patient could hear a whispering voice and enunciated two-word sentences, but had some difficulty with the letters “R” and “K.” The patient also began to establish connections between 2 separate events and was able to count from 1 to 10. The patient became accustomed to the implant very quickly, and even warned his mother when the battery went dead.
Discussion Kawasaki disease is an acute vasculitic syndrome that may lead to coronary artery disease and cardiac failure if untreated.1 KD is usually seen in infants and in children younger than 5 years. Recent studies strongly propose that an infection may be in play, but the exact etiology of KD still remains unknown.2 There is no specific test for the diagnosis of KD. Thus, diagnosis is made using the following clinical criteria: fever accompanied by bilateral nonexudative conjunctival injection, oral mucosal changes, rash, extremity changes, and cervical lymphadenopathy. These criteria were defined by the Japanese Ministry of Health and adopted by the American Heart Association3,4 (Table 1). Some patients suspected of KD 1
Marmara University Pendik Training and Research Hospital, Istanbul, Turkey Corresponding Author: Adem Binnetoglu, Department of Otorhinolaryngology Head & Neck Surgery, Marmara University Faculty of Medicine, Fevzi Cakmak Mahallesi, Mimar Sinan Caddesi No. 41, Endik, Istanbul, Turkey. Email: [email protected]
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Clinical Pediatrics
Figure 1. Imaging of the inner ear: Normal cochleovestibular structures were apparent on axial temporal computed tomography (A). Coronal temporal bone computed tomography (B). Imaging of the internal acoustic canal, normal cochleovestibular nerves were evident on axial magnetic resonance imaging (C).
Figure 2. Electrodes can be seen in the postoperative radiographs of the cochlea: Stenvers x-ray (a) and transorbital x-ray (b).
Downloaded from cpj.sagepub.com by guest on July 26, 2015
3
Binnetoglu et al Table 1. American Heart Association Diagnostic Clinical Criteria for Kawasaki Disease (KD). KD: Fever persisting for >5 days, plus at least 4 of the following 5 features Atypical KD: Fever persisting for >5 days without explanation, with fulfillment of fewer than 4 of the 5 clinical criteria for KD, but with coronary artery involvement 1 2 3 4 5
Changes in the extremities, including indurative angioedema and desquamation Polymorphous exanthema Bilateral bulbar conjunctival injection without an exudate Changes to the lips and oral cavity, including pharyngeal injection, dry fissured lips, and/or strawberry tongue Acute nonpurulent cervical lymphadenopathy (>1.5 cm in diameter)
do not meet all diagnostic clinical criteria and may have presented with atypical symptoms that are not frequent in classical KD patients, including renal failure, an acute surgical abdomen, pleuritis, and aseptic meningitis.4 Such cases are defined as “atypical/incomplete KD” and diagnosis is achieved by findings of coronary artery involvement. Involvement of the cardiovascular system, especially of coronary arteries, is the most commonly encountered complication of KD. Also, some neurological complications of ataxia, cranial nerve palsies, emotional disorders, and ocular involvement can develop in concert with cardiac abnormalities.5-7 However, SNHL is not a frequent complication of KD and usually leads to irreversible the inner ear damage.8 The main strategy for management of KD is to prevent coronary artery disease and to relieve the symptoms of complications with the use of intravenous immunoglobulin and ASA.9 In our case, the child had a fever, thrombocytosis without leucocytosis, an increased C-reactive protein level and ESR, lymphocytic pleocytosis in the cerebrospinal fluid compatible with aseptic meningitis, and coronary artery aneurysms. Considering all the clinical, biochemical, and radiological aspects, our patient did not fulfill at least 4 of the 5 classical criteria for KD, and was diagnosed with atypical/incomplete KD. Kawasaki disease and SNHL may be associated. The etiology of any such association remains unclear, but few theories have been suggested. SNHL develops principally in the acute phase of KD and lead to SNHL both ears irreversibly, suggesting that a systemic inflammatory vasculitis may affect the inner ear cells.10 Some authors have proposed that ASA can lead to SNHL associated with KD. The condition (KD) is because of hypoalbuminemia, which can rise the levels of unbound, free-circulating ASA. However, SNHL
associated with ASA ototoxicity prone to be bilateral, symmetric, mild, and transient. In contrast, Knott et al8 found no significantly increased risk of SNHL associated with elevated levels of ASA in patients with KD. Our patient suffered bilateral profound hearing loss 6 months after an attack of atypical/incomplete KD; this is not compatible with ASA ototoxicity because it was not mild nor transient SNHL. Our case supports the notion that the inner ear suffered an inflammatory insult because it was bilateral and irreversible SNHL. However, SNHL usually develops in the acute phase of KD according to inflammatory insult notion; this is a contradiction for our case who had SNHL after 6 months of the episode of atypical/incomplete KD. This contradiction could be explained that the patient may have had SNHL during the initial active phase of the disease, but diagnosis might have been delayed. Although no association between atypical/incomplete KD and SNHL has been described. SNHL may be a complication of KD.
Conclusion We report the first case of atypical/incomplete KD associated with bilateral profound SNHL. Clinicians should have a high index of suspicion of SNHL in patients with atypical/incomplete KD to prevent delays in diagnosis and treatment. Author Contributions Conceptualised by AB, TB, MS. Designed by AB. Supervisied by AB, TB, MS. Materials provided by KKB, BD, AB. Data Collection and/or processing done by KKB, BD, analysis and/ or interpretation done by BD, AB. Literature Review by KKB, BD writing by KKB, BD, AB. Critical review by TB, MS.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Jamieson N, Singh-Grewal D. Kawasaki disease: a clinician’s update. Int J Pediatr. 2013;2013:645391. 2. Burns JC, Glodé MP. Kawasaki syndrome. Lancet. 2004;364:533-544. 3. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome (MCLS) prevailing in Japan. Pediatrics. 1974;54:271-276.
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Clinical Pediatrics
4. Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 2004;110:2747-2771. 5. Poon LK, Lun KS, Ng YM. Facial nerve palsy and Kawasaki disease. Hong Kong Med J. 2000;6:224-226. 6. King WJ, Schlieper A, Birdi N, Cappelli M, Korneluk Y, Rowe PC The effect of Kawasaki disease in cognition and behavior. Arch Pediatr Adolesc Med. 2000;154:463-468.
7. Ohno S, Miyajima T, Higuchi M, et al. Ocular manifestations of Kawasaki disease (mucocutaneous lymph node syndrome). Am J Ophthalmol. 1982;93:713-717. 8. Knott PD, Orloff LA, Harris JP, Novak RE, Burns JC. Sensorineural hearing loss associated and Kawasaki disease: a prospective study. Am J Otolaryngol. 2001;22:343-348. 9. Smith KA, Yunker WK. Kawasaki disease is associated with sensorineural hearing loss: a systematic review. Int J Pediatr Otorhinolaryngol. 2014;78:1216-1220. 10. Thapa R, Chakrabartty S. Atypical Kawasaki disease with remarkable paucity of signs and symptoms. Rheumatol Int. 2009;29:1095-1096.
Downloaded from cpj.sagepub.com by guest on July 26, 2015
research-article2015
CPJXXX10.1177/0009922815588979Clinical PediatricsBinnetoglu et al
Brief Report
Association Between Atypical/ Incomplete Kawasaki Disease and Sensorineural Hearing Loss: A Case Report
Clinical Pediatrics 1–4 © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815588979 cpj.sagepub.com
Adem Binnetoglu, MD1, Tekin Baglam, MD1, Berat Demir, MD1, Kiymet Kecelioglu Binnetoglu, MD1, and Murat Sari, MD1
Case Report A 6-month-old male was admitted to our Pediatrics Department because of fever that had persisted for 13 days. He had no history of drug allergy and infectious disease, or no recent vaccination, and no abnormal finding on physical examination. The laboratory and radiological findings were as follows: white blood cell count, 9.2 × 103/μL; platelet count, 975 × 103/μL; biochemical parameters of liver, thyroid, kidney and urine, and all serological assays of infectious and rheumatological diseases, and chest radiograph results were normal. The patient’s C-reactive protein level and erythrocyte sedimentation rate (ESR) were 9 mg/dL (normal range 0.00.8 mg/dL) and 107 mm/h (normal range 3-13 mm/h), respectively. Lumbar puncture revealed lymphocytic pleocytosis in the cerebrospinal fluid, but cultures were negative, indicating that aseptic meningitis was in play. Echocardiographic findings were considered to be suggestive of coronary artery involvement with aneurysmal changes. The patient was diagnosed with atypical/ incomplete Kawasaki disease. As therapy, acetylsalicylic acid (ASA) and intravenous immunoglobulin were given for 6 months. At this time, the patient’s family noted that he had hearing difficulties. On suspicion of sensorineural hearing loss (SNHL), a click-evoked auditory brainstem response test was performed and revealed no wave in either ear at maximum volume levels at 1000, 2000, or 4000 Hz, confirming a diagnosis of bilateral profound SNHL. Computed tomography and magnetic resonance imaging (Figure 1) of the temporal bone revealed no abnormality. As the patient did not benefit (in terms of speech perception) from 6 months of bilateral hearing aid use, he underwent right cochlear implantation after obtaining of informed consent from the parents.
Final Diagnosis Based on clinical picture and echocardiographic findings, the patient was diagnosed with sensorineural
hearing loss as a part of atypical/incomplete Kawasaki disease (KD).
Hospital Course No postoperative complication was noted. Stenvers and transverse scans were obtained to confirm accurate device placement in the cochlea (Figure 2). A follow-up evaluation showed that the implant facilitated speech perception. The patient could hear a whispering voice and enunciated two-word sentences, but had some difficulty with the letters “R” and “K.” The patient also began to establish connections between 2 separate events and was able to count from 1 to 10. The patient became accustomed to the implant very quickly, and even warned his mother when the battery went dead.
Discussion Kawasaki disease is an acute vasculitic syndrome that may lead to coronary artery disease and cardiac failure if untreated.1 KD is usually seen in infants and in children younger than 5 years. Recent studies strongly propose that an infection may be in play, but the exact etiology of KD still remains unknown.2 There is no specific test for the diagnosis of KD. Thus, diagnosis is made using the following clinical criteria: fever accompanied by bilateral nonexudative conjunctival injection, oral mucosal changes, rash, extremity changes, and cervical lymphadenopathy. These criteria were defined by the Japanese Ministry of Health and adopted by the American Heart Association3,4 (Table 1). Some patients suspected of KD 1
Marmara University Pendik Training and Research Hospital, Istanbul, Turkey Corresponding Author: Adem Binnetoglu, Department of Otorhinolaryngology Head & Neck Surgery, Marmara University Faculty of Medicine, Fevzi Cakmak Mahallesi, Mimar Sinan Caddesi No. 41, Endik, Istanbul, Turkey. Email: [email protected]
Downloaded from cpj.sagepub.com by guest on July 26, 2015
2
Clinical Pediatrics
Figure 1. Imaging of the inner ear: Normal cochleovestibular structures were apparent on axial temporal computed tomography (A). Coronal temporal bone computed tomography (B). Imaging of the internal acoustic canal, normal cochleovestibular nerves were evident on axial magnetic resonance imaging (C).
Figure 2. Electrodes can be seen in the postoperative radiographs of the cochlea: Stenvers x-ray (a) and transorbital x-ray (b).
Downloaded from cpj.sagepub.com by guest on July 26, 2015
3
Binnetoglu et al Table 1. American Heart Association Diagnostic Clinical Criteria for Kawasaki Disease (KD). KD: Fever persisting for >5 days, plus at least 4 of the following 5 features Atypical KD: Fever persisting for >5 days without explanation, with fulfillment of fewer than 4 of the 5 clinical criteria for KD, but with coronary artery involvement 1 2 3 4 5
Changes in the extremities, including indurative angioedema and desquamation Polymorphous exanthema Bilateral bulbar conjunctival injection without an exudate Changes to the lips and oral cavity, including pharyngeal injection, dry fissured lips, and/or strawberry tongue Acute nonpurulent cervical lymphadenopathy (>1.5 cm in diameter)
do not meet all diagnostic clinical criteria and may have presented with atypical symptoms that are not frequent in classical KD patients, including renal failure, an acute surgical abdomen, pleuritis, and aseptic meningitis.4 Such cases are defined as “atypical/incomplete KD” and diagnosis is achieved by findings of coronary artery involvement. Involvement of the cardiovascular system, especially of coronary arteries, is the most commonly encountered complication of KD. Also, some neurological complications of ataxia, cranial nerve palsies, emotional disorders, and ocular involvement can develop in concert with cardiac abnormalities.5-7 However, SNHL is not a frequent complication of KD and usually leads to irreversible the inner ear damage.8 The main strategy for management of KD is to prevent coronary artery disease and to relieve the symptoms of complications with the use of intravenous immunoglobulin and ASA.9 In our case, the child had a fever, thrombocytosis without leucocytosis, an increased C-reactive protein level and ESR, lymphocytic pleocytosis in the cerebrospinal fluid compatible with aseptic meningitis, and coronary artery aneurysms. Considering all the clinical, biochemical, and radiological aspects, our patient did not fulfill at least 4 of the 5 classical criteria for KD, and was diagnosed with atypical/incomplete KD. Kawasaki disease and SNHL may be associated. The etiology of any such association remains unclear, but few theories have been suggested. SNHL develops principally in the acute phase of KD and lead to SNHL both ears irreversibly, suggesting that a systemic inflammatory vasculitis may affect the inner ear cells.10 Some authors have proposed that ASA can lead to SNHL associated with KD. The condition (KD) is because of hypoalbuminemia, which can rise the levels of unbound, free-circulating ASA. However, SNHL
associated with ASA ototoxicity prone to be bilateral, symmetric, mild, and transient. In contrast, Knott et al8 found no significantly increased risk of SNHL associated with elevated levels of ASA in patients with KD. Our patient suffered bilateral profound hearing loss 6 months after an attack of atypical/incomplete KD; this is not compatible with ASA ototoxicity because it was not mild nor transient SNHL. Our case supports the notion that the inner ear suffered an inflammatory insult because it was bilateral and irreversible SNHL. However, SNHL usually develops in the acute phase of KD according to inflammatory insult notion; this is a contradiction for our case who had SNHL after 6 months of the episode of atypical/incomplete KD. This contradiction could be explained that the patient may have had SNHL during the initial active phase of the disease, but diagnosis might have been delayed. Although no association between atypical/incomplete KD and SNHL has been described. SNHL may be a complication of KD.
Conclusion We report the first case of atypical/incomplete KD associated with bilateral profound SNHL. Clinicians should have a high index of suspicion of SNHL in patients with atypical/incomplete KD to prevent delays in diagnosis and treatment. Author Contributions Conceptualised by AB, TB, MS. Designed by AB. Supervisied by AB, TB, MS. Materials provided by KKB, BD, AB. Data Collection and/or processing done by KKB, BD, analysis and/ or interpretation done by BD, AB. Literature Review by KKB, BD writing by KKB, BD, AB. Critical review by TB, MS.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Jamieson N, Singh-Grewal D. Kawasaki disease: a clinician’s update. Int J Pediatr. 2013;2013:645391. 2. Burns JC, Glodé MP. Kawasaki syndrome. Lancet. 2004;364:533-544. 3. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome (MCLS) prevailing in Japan. Pediatrics. 1974;54:271-276.
Downloaded from cpj.sagepub.com by guest on July 26, 2015
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Clinical Pediatrics
4. Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 2004;110:2747-2771. 5. Poon LK, Lun KS, Ng YM. Facial nerve palsy and Kawasaki disease. Hong Kong Med J. 2000;6:224-226. 6. King WJ, Schlieper A, Birdi N, Cappelli M, Korneluk Y, Rowe PC The effect of Kawasaki disease in cognition and behavior. Arch Pediatr Adolesc Med. 2000;154:463-468.
7. Ohno S, Miyajima T, Higuchi M, et al. Ocular manifestations of Kawasaki disease (mucocutaneous lymph node syndrome). Am J Ophthalmol. 1982;93:713-717. 8. Knott PD, Orloff LA, Harris JP, Novak RE, Burns JC. Sensorineural hearing loss associated and Kawasaki disease: a prospective study. Am J Otolaryngol. 2001;22:343-348. 9. Smith KA, Yunker WK. Kawasaki disease is associated with sensorineural hearing loss: a systematic review. Int J Pediatr Otorhinolaryngol. 2014;78:1216-1220. 10. Thapa R, Chakrabartty S. Atypical Kawasaki disease with remarkable paucity of signs and symptoms. Rheumatol Int. 2009;29:1095-1096.
Downloaded from cpj.sagepub.com by guest on July 26, 2015
