PEDIATRICS (doi:10.1542/peds.2007-3238)
EXPERIENCE & REASON |
Facial Nerve Palsy Complicating Kawasaki Disease
a School of Paediatrics and Child Health, University of Western Australia, Perth, Australia
b Infectious Diseases and General Paediatrics, Princess Margaret Hospital for Children, Perth, Australia
c Division of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
ABSTRACT
The diagnosis of Kawasaki disease, the most common cause of pediatric acquired heart disease, is difficult and often delayed for children whose age falls outside the typical range of 6 months to 5 years, especially in those with incomplete Kawasaki disease and atypical features. Delayed diagnosis is associated with an increased incidence of coronary artery pathology. Here we describe 2 cases of lower motor neuron facial nerve palsy complicating Kawasaki disease. In both cases the diagnosis of Kawasaki disease was not made acutely, and both patients developed extensive coronary artery lesions. These cases highlight the importance of considering Kawasaki disease in children with unexplained prolonged fever at any age, particularly those without full diagnostic criteria and with unusual features.
Key Words: Kawasaki disease • facial nerve palsy • vasculitis
Abbreviations: KD, Kawasaki disease • CA, coronary artery • HSV, herpes simplex virus • IVIg, intravenous immunoglobulin • RCA, right coronary artery • LAD, left anterior descending coronary artery
Kawasaki disease (KD) is an acute panvasculitis with a striking affinity for the coronary arteries (CAs). The diagnosis is made on the basis of prolonged fever (
5 days) and at least 4 of 5 principal clinical criteria, but it can also be made with fewer criteria when CA abnormalities are present. "Incomplete KD," in which 
3 principal clinical criteria are fulfilled, has been increasingly recognized, especially in younger children.1
Facial nerve palsy is a recognized but uncommon manifestation of KD.1 It has been associated with a higher incidence of CA lesions and may be an indicator of more severe disease.2 Here we describe 2 cases of facial nerve palsy in KD in which the initial diagnosis was not made during the acute illness; both patients developed extensive CA involvement.
CASE REPORTS
Case 1
A formula-fed, fully immunized 3-month-old boy was admitted to a general hospital with a 5-day febrile illness associated with a maculopapular rash and mild, nonexudative pharyngitis (Table 1). No causative organism was found on blood cultures and he received empiric antibiotics for 6 days, during which time he became afebrile. Two weeks after discharge he was admitted to a tertiary pediatric facility with a short history of pallor, poor feeding, and left-sided facial weakness. He was afebrile, tachycardic, pale, and irritable, with a normotensive anterior fontanelle and a lower motor neuron left facial nerve palsy.
|
Laboratory investigations during the second admission included a mild normocytic normochromic anemia (hemoglobin: 90 g/L [reference range: 95–140g/L]), leukocytosis (19.9 x 109/L with 52% neutrophils [reference range: 6.0–17.5 x 109/L]), thrombocytosis (974 x 109/L [reference range: 150–400 x 109/L]), and a raised C-reactive protein level (117 mg/L [reference range: 0–7 mg/L]). Blood cultures were sterile. Serologic testing was negative for herpes simplex viruses type 1 (HSV1) and HSV2, Mycoplasma pneumoniae, Epstein-Barr virus, cytomegalovirus, and influenza A and B. Blood polymerase chain reaction results were negative for enterovirus and meningococcus. A cerebrospinal fluid sample was heavily blood stained, grew no organisms, and was negative for viral nucleic acid, including enterovirus, HSV1, HSV2, varicella zoster virus, Epstein-Barr virus, and cytomegalovirus. Throat and fecal cultures were negative for bacterial and viral pathogens. Results of a cerebral ultrasound were normal.
The child was commenced on empiric antibiotics while the laboratory results were pending. Over the first 24 hours of admission, the infant became febrile, remained pale and irritable, and was observed to have cracked, reddened lips, a bilateral nonexudative conjunctivitis, and perineal desquamation. His left facial nerve palsy persisted.
Further history revealed that between admissions he had been afebrile, with bilateral skin peeling of his hands and feet in the third week of the illness. His medical notes from the first hospital admission documented conjunctival injection and cervical lymphadenopathy as part of the acute presentation.
In view of the history suggestive of recent KD, recrudescence of fever, ongoing inflammation, and further mucocutaneous signs, the patient was treated with intravenous immunoglobulin (IVIg) (2 g/kg) and aspirin (75 mg/kg per day) on day 2 of the second admission. Within 24 hours there was sustained resolution of fever and dramatic systemic improvement. The facial nerve palsy resolved within 2 days. An echocardiogram on day 3 of the second admission revealed normal left ventricular function, with diffusely dilated and mildly ectatic CAs. The right CAs (RCAs) had a "beaded" appearance, and there was ectasia of the proximal RCA and left anterior descending CA (LAD). The CA diameter measurements were: left main coronary artery, 2.4 mm (z score = 1.64); proximal LAD, 2.3 mm (z score = 4.20); and proximal RCA, 3.7 mm (z score = 7.93). There was mild mitral valve incompetence. A repeat echocardiogram 6 weeks after admission showed marked improvement with minimal residual dilatation of the RCA (2 mm) and slight ectasia of the other CAs.
Case 2
A 7-month-old previously well boy presented with prolonged fever, cracked red lips, a polymorphous rash, and bilateral nonpurulent conjunctivitis. His erythrocyte sedimentation rate was raised, and no causative infectious organism was identified. During the course of his illness he developed a left-sided lower motor neuron facial nerve palsy, which was attributed to possible concurrent otitis media. He was treated with prednisolone, and the facial nerve palsy resolved over a few days. A diagnosis of KD was not made at the time.
At the age of 2 years 3 months, the patient's mother noted bilateral axillary masses, which were pulsatile and consistent with axillary aneurysms, a recognized complication of KD.1 Subsequent echocardiography and cardiac and axillary catheterization demonstrated widespread and severe coronary and axillary artery involvement. His CA measurements were: left main coronary artery, 5 mm (z score = 6.03); proximal LAD, 9 mm (z score = 25.84); and proximal RCA, 10.5 mm (z score = 22.64). The giant RCA aneurysm was occluded at its distal end, with multiple small tortuous collateral vessels circumventing the aneurysm. There were bilateral huge axillary artery aneurysms. Left ventricular function and valve function were normal. A stress 2-methoxy isobutyl isonitrile scan, to exclude the possibility of a past myocardial infarction, showed no evidence of reversible or fixed ischemia. The patient's condition was managed with warfarin and aspirin, and the family was advised that the patient should avoid high-impact and contact sports. He remained well at 10 years of age.
DISCUSSION
The diagnosis of KD relies on the recognition of often sequential clinical features that together constitute the cardinal diagnostic criteria. Incomplete KD, in which 3 principal clinical criteria are fulfilled, is increasingly recognized, especially in younger children, and often delays diagnosis. Such cases sometimes exhibit atypical clinical features, including neurologic signs.1
Initiation of treatment with IVIg before day 10 of the illness results in a markedly reduced frequency of CA lesions; prompt diagnosis and treatment, therefore, is essential.3 The majority of KD cases occur in children between the ages of 6 months and 5 years. However, up to one quarter of children develop KD outside this age range and have worse coronary outcomes, possibly because of delayed diagnosis and treatment.3 The diagnosis at the extremes of age is often delayed, because physicians are reluctant to consider a diagnosis of KD.4 Infants <6 months of age may exhibit fewer cardinal signs,5 and older children may present with clinical features that divert clinicians from a diagnosis of KD.6 KD may present with a number of noncardiac clinical and laboratory features.1 Recognition of these additional clinical features of KD and consideration of a diagnosis of KD in children outside the usual age range may, therefore, aid timely diagnosis and improve outcome.
Neurologic manifestations of KD may occur in up to 30% of patients, although definitive neurologic manifestations are relatively rare. Complications include irritability, lethargy, aseptic meningitis, ataxia, seizures, focal encephalopathy, cranial nerve palsies, cerebral infarction, and transient hemiplegia.7 Extreme irritability is more marked than that seen with other febrile illnesses in young children and may be a helpful, albeit subjective, diagnostic feature. Histopathologic features from autopsies of children who died as a result of KD include aseptic choriomeningitis, leptomeningitis, and ganglionitis and neuritis of both central and peripheral nerves, although there are no pathognomonic changes.8 It is likely that both ischemic vasculitis of the arteries supplying the facial nerve and immunologic mechanisms contribute to the facial nerve dysfunction.9
More than 30 cases of KD-associated facial palsy have now been reported in the literature, most of which predated IVIg therapy. Clinically, the facial palsy usually presents during the convalescent phase of KD, as in our case 1, but may develop any time from the second day of fever until >1 month after the initial KD presentation.2 The facial nerve palsy in case 2 evolved in a patient who had incomplete clinical features. CA abnormalities occur in more than half of the patients with facial nerve palsy,7 which may reflect late KD diagnosis, more severe inflammation and vasculitis, or a selection bias with a diagnosis of KD being made more often than in those who develop CA aneurysms. Facial nerve palsy in KD generally resolves spontaneously. Before the advent of IVIg, most cases of KD-associated facial palsy lasted from 1 week to 3 months.2
CONCLUSIONS
These cases highlight the importance of a low diagnostic threshold for KD in any child with prolonged fever, even with incomplete diagnostic features, as well as the need to be aware of unusual manifestations. KD should be considered in the differential diagnosis of a child who presents with facial nerve palsy, especially when the clinical presentation of the illness is still unfolding. Diagnostic algorithms for the management of children with possible incomplete KD1 may facilitate earlier treatment and reduced coronary morbidity, especially in younger children.
FOOTNOTES
Accepted Mar 17, 2008.
Address correspondence to David Burgner, MD, PhD, School of Paediatrics and Child Health, University of Western Australia, Princess Margaret Medical Centre, GPO Box D184, WA 6000, Perth, Australia. E-mail: dburgner{at}meddentuwa.edu.au
The authors have indicated they have no financial relationships relevant to this article to disclose.
REFERENCES
- 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
(17):2747
–2771
[Abstract/Free Full Text] - Poon LK, Lun KS, Ng YM. Facial nerve palsy and Kawasaki disease. Hong Kong Med J. 2000;6 (2):224 –226[Medline]
- Burns JC, Glodé MP. Kawasaki syndrome. Lancet. 2004;364 (9433):533 –544[CrossRef][Web of Science][Medline]
- Pannaraj PS, Turner CL, Bastian JF, Burns JC. Failure to diagnose Kawasaki disease at the extremes of the pediatric age range. Pediatr Infect Dis J. 2004;23 (8):789 –791[CrossRef][Web of Science][Medline]
- Rosenfeld EA, Corydon KE, Shulman ST. Kawasaki disease in infants less than one year of age. J Pediatr. 1995;126 (4):524 –529[CrossRef][Web of Science][Medline]
- Stockheim JA, Innocentini N, Shulman ST. Kawasaki disease in older children and adolescents. J Pediatr. 2000;137 (2):250 –252[CrossRef][Web of Science][Medline]
- Tizard E. Complications of Kawasaki disease. Curr Paediatr. 2005;15 :62 –68[CrossRef]
- Amano S, Hazama F. Neutral involvement in Kawasaki disease. Acta Pathol Jpn. 1980;30 (3):365 –373[Medline]
- Terasawa K, Ichinose E, Matsuishi T, Kato H. Neurological complications in Kawasaki disease. Brain Dev. 1983;5 (4):371 –374[Web of Science][Medline]
PEDIATRICS (ISSN 1098-4275). ©2008 by the American Academy of Pediatrics
CiteULike 
Connotea 
Del.icio.us 
Digg 
Facebook 
Reddit 
Technorati 
Twitter What's this?
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
