Background

Kawasaki disease is an acute, self-limiting febrile vasculitis, most common in infants aged 6 months–2 years. It is challenging to make a diagnosis of incomplete Kawasaki disease, particularly in infants younger than 6 months with fewer clinical manifestations. ^{Reference Uysal, Bostan, Celebi, Uysal, Hamitoglu and Cil1} Various neurologic complications have been described to occur in 1–30% of patients with Kawasaki disease, ^{Reference Gallagher2} but facial nerve palsy secondary to Kawasaki disease is an extremely rare (0.9%) and atypical presentation. ^{Reference Alves, Magalhaes, Almeida Rde, Santos, Gandolfi and Pratesi3} Patients with Kawasaki disease and facial palsy tend to be under 6 months old, and there is a higher association with coronary artery aneurysm in such patients compared to those without facial palsy who never received treatment. ^{Reference Yeo, Kim and Ha4} Kawasaki disease associated with facial nerve palsy may indicate increased inflammatory burden, and patients may require additional anti-inflammatory agents and more vigilant echocardiography. Herein, we describe a 4-month-old female who presented with unilateral facial nerve palsy in the acute phase of incomplete Kawasaki disease, in which the diagnosis was delayed until day 10 of the illness.

Case Presentation

A 4-month-old female presented with fever, irritability, and reduced mobility of the right side of the face, in addition to an inability to close the right eye and epiphora for 3 days before admission. The onset of the disease, with fever and a slight cough, was 10 days before admission. Therefore, she was thought to have a respiratory tract infection and was admitted to a regional hospital where she was treated with antibiotics and antipyretics, but there was no improvement. On day 7 of illness, the patient developed a right facial droop, false right ptosis secondary to the inability to raise her right eyebrow, and decreased movement of the right aspect of her mouth. Her personal history revealed a 10-day episode of persistent fever and conjunctival injection that resolved spontaneously 5 days earlier. She also presented with generalised cracking and reddening of the lips and was transferred to our clinic with suspected Kawasaki disease.

Physical examination revealed fever (39°C), cracking and reddened lips, and extreme irritability. On examination, she was noted to have right painless cervical lymphadenopathy, left-sided deviation of the labial commissure, obliteration of the right nasolabial fold, and incomplete closure of the right eye, suggesting a right-sided infranuclear facial nerve palsy (Fig 1). A detailed neurologic assessment did not reveal any more defects. The remainder of the physical examination was unremarkable.

Figure 1. Photograph of the patient showing right-sided facial nerve palsy at presentation.

The laboratory tests on the day of admission revealed leukocytosis (36,590/µl) with neutrophilia (29,720/µl), anemia (Hb: 7.8 g/dl), thrombocytosis (1155,000/µl), hypo-albuminemia (2.59 g/dl), elevated N-terminal of the prohormone brain natriuretic peptide level (1130 pg/mL), elevated CRP (139 mg/l), and elevated erythrocyte sedimentation rate (65 mm/h). An echocardiogram was performed showing normal left ventricular function, mild pericardial effusion, and mitral regurgitation, in addition to aneurysms of the right main coronary artery (maximum diameter of 8.5 mm; Z-score 11.62), left main coronary artery (maximum diameter of 5.1 mm; Z-score 6.97), left anterior descending coronary artery (maximum diameter of 3.3 mm; Z-score 4.26), and left circumflex coronary artery (maximum diameter of 2.2 mm; Z-score 3.01), thus confirming the diagnosis of incomplete Kawasaki disease (Fig 2). The electrocardiogram demonstrated sinus tachycardia, and the chest X-ray and cerebral magnetic resonance imaging were normal. Blood, urine, cerebrospinal fluid, and stool cultures were sterile. Throat and fecal cultures were negative for bacterial and viral pathogens.

Figure 2. Two-dimensional Doppler echocardiogram. Parasternal short axis plane showing a large-size aneurysm of the left main coronary artery (2A: RMCA; maximum diameter of 8.5 mm; Z-score 11.62), and a small-size aneurysm of the right main coronary artery (2B: LMCA; maximum diameter of 5.1 mm; Z-score 6.97). Ao = aortic root.

Based on the patient’s medical history, the physical and laboratory findings were compatible with incomplete Kawasaki disease. She received intravenous immunoglobulin (2 g/kg/16 hours), and acetyl salicylic acid (50 mg/kg/d) was started on the 10th day of illness. The patient’s fever and facial nerve palsy resolved within 48 hours after intravenous immunoglobulin treatment. An echocardiogram performed on day 5 of admission revealed similar coronary findings. Enoxaparin was stopped, and anticoagulation was continued with a combination of warfarin and low-dose acetyl salicylic acid after the 2nd week of the treatment.

The patient’s inflammatory markers all improved to normal or near-normal levels prior to discharge; all infectious studies returned to negative. Her right facial weakness was unappreciable on the day of discharge (20 days after onset). At the 3-month follow-up after discharge, the right-sided facial nerve palsy had resolved completely, and echocardiography showed improvement in the right coronary artery aneurysms, left coronary artery and left anterior descending coronary artery, and normal left circumflex coronary arteries. The pericardial effusion and mitral regurgitation disappeared completely.