Yu Xia, Liu Xiaoxia, Wang Yongchun, Lu Na, Wang Mengjiao, Sun Li
The First Hospital of Jilin University, Changchun.
NingBo N0.2 Hospital, NingBo.
Medicine (Baltimore). 2019 Feb;98(7):e14395. doi: 10.1097/MD.0000000000014395.
Kawasaki disease (KD), which is also known as mucocutaneous lymphnode syndrome, is a vasculitic disease and involves multi-system disorder with various clinical manifestations. KD is specific predilection for the coronary arteries and is the most common cause of childhood-acquired heart disease in developed countries. KD is rarely complicated by cranial nerve VII palsy.
This report described a 7-month-old infant who suffered from bilateral infranuclear facial nerve palsy (FNP) and multiple coronary artery aneurysms (CAAs) in parallel with KD. The patient had an intermittent fever for 18 days and had a medical history of bilateral conjunctival injection, strawberry tongue, reddened lips, and perianal excoriation. Physical examinations revealed fever (38.5°C), fingertips desquamation of the skin, and left cervical lymphadenopathies.
The diagnosis of KD is based on the presence of clinical features of persistent fever (≥5 days) together with polymorphous exanthema, cervical lymphadenopathy, non-purulent conjunctival injection, changes of the lips, oral cavity, and extremities. An echocardiogram has showed a beaded sample dilatation of all coronary arteries, in addition to aneurysms of the middle of the right coronary artery (6.2 mm in diameter; 14.5 Z score), and the left coronary artery (5.4 mm in diameter; 9.4 Z score). The physical examinations revealed incomplete closure of both eyes and bilateral drooping of the mouth, suggesting a bilateral infranuclear FNP.
The patient received intravenous immunoglobulin (IVIG) (2 g/kg) with high-dose aspirin according to the clinical guidelines.
Her fever finally resolved after 2 days' IVIG. All inflammatory indexes returned to normal or near-normal levels prior to discharge. However, the echocardiogram remained unchanged and the patient's facial nerve palsies had not recovered.
FNP in KD is uncommon. Yet, it may be a marker of disease progression. One should be aware of the diagnosis of KD when children suffer from high fever, FNP, and even with incomplete clinical features.
川崎病（KD），又称皮肤黏膜淋巴结综合征，是一种血管炎性疾病，涉及多系统紊乱，临床表现多样。KD对冠状动脉有特殊偏好，是发达国家儿童后天性心脏病最常见的病因。KD很少并发颅神经VII麻痹。
本报告描述了一名7个月大的婴儿，患有双侧核下性面神经麻痹（FNP）和多个冠状动脉瘤（CAA），同时患有KD。患者间歇性发热18天，有双侧结膜充血、草莓舌、嘴唇发红和肛周皮肤破损的病史。体格检查发现发热（38.5°C）、指尖皮肤脱屑和左颈部淋巴结肿大。
KD的诊断基于持续发热（≥5天）的临床特征，以及多形性皮疹、颈部淋巴结肿大、非脓性结膜充血、嘴唇、口腔和四肢的变化。超声心动图显示所有冠状动脉呈串珠样扩张，此外右冠状动脉中段有动脉瘤（直径6.2毫米；Z值14.5），左冠状动脉有动脉瘤（直径5.4毫米；Z值9.4）。体格检查发现双眼不完全闭合和双侧口角下垂，提示双侧核下性FNP。
患者根据临床指南接受了静脉注射免疫球蛋白（IVIG）（2克/千克）和高剂量阿司匹林治疗。
静脉注射免疫球蛋白2天后，她的发热最终消退。出院前所有炎症指标恢复到正常或接近正常水平。然而，超声心动图结果未改变，患者的面神经麻痹未恢复。
KD中的FNP并不常见。然而，它可能是疾病进展的一个标志。当儿童出现高热、FNP，甚至临床特征不完整时，应注意KD的诊断。
