背景:川崎病(KD)表现为急性,构成5岁以下儿童获得性心脏病的主要原因的自限性血管炎疾病。面神经麻痹(FNP)是KD患者与冠状动脉病变(CAL)相关的罕见并发症。KD和FNP患者通常不典型地出现,导致KD的诊断和治疗延迟。
方法:一个4个月大发烧男孩,左FNP和双侧结膜注射自发消退,被送进了医院,接受了短期静脉注射地塞米松,在第一次入院时经历了FNP的快速恢复。病人发烧了,双侧结膜注射,和正确的FNP,这导致了重新接纳。体格检查显示卡介苗接种部位发红,嘴唇发红,和远端脱皮。超声心动图显示右侧CAL。
方法:患者首次入院时最初错过KD,并在第二次入院时被诊断为完全KD伴FNP。
结果:经过短期静脉注射地塞米松,左FNP迅速解决。然而,右FNP在皮质类固醇停药后复发。同时,观察到更典型的症状,KD被诊断出来了.随后用静脉注射免疫球蛋白(IVIG)治疗,阿司匹林,还有地塞米松.病人迅速缓解,没有复发。在出院后的1年随访中,超声心动图继续显示正常结果。
结论:儿童FNP合并KD的临床症状不典型,易导致延误诊断和治疗。KD患者的FNP可能是CAL的危险因素,这比FNP本身更具挑战性。建议在IVIG中加入皮质类固醇以减少IVIG耐药性,降低开发CAL的风险,减轻CAL。
BACKGROUND: Kawasaki disease (KD) manifests as an acute, self-limited vasculitis disease that constitutes the primary cause of acquired heart disease in children under 5 years of age. Facial nerve palsy (FNP) is a rare complication associated with coronary artery lesions (CALs) in patients with KD. Patients with KD and FNP usually present atypically, leading to a delayed diagnosis and treatment of KD.
METHODS: A 4-month-old boy with fever, left FNP and bilateral conjunctival injection with spontaneous resolution, was admitted to the hospital, received a short course of intravenous dexamethasone, and experienced rapid FNP recovery on the first admission. The patient experienced a resurgence of fever, bilateral conjunctival injection, and right FNP, which led to readmission. Physical examination revealed redness at the site of Bacillus Calmette-Guérin inoculation, reddening of lips, and desquamation of the distal extremities. Echocardiography revealed right-sided CALs.
METHODS: The patient initially missed KD on the first admission, and was later diagnosed with complete KD with FNP on the second admission.
RESULTS: After a short course of intravenous dexamethasone, the left FNP resolved quickly. However, right FNP recurred after corticosteroids withdrawal. Meanwhile, more typical symptoms were observed, and KD was diagnosed. Treatment ensued with intravenous immunoglobulin (IVIG), aspirin, and dexamethasone. The patient achieved rapid remission, without recurrence. Echocardiography continued to show normal findings during 1-year follow-up after discharge.
CONCLUSIONS: The clinical symptoms of FNP complicating KD in children are atypical and can easily lead to delayed diagnosis and treatment. FNP in patients with KD may serve as a risk factor for CALs, which are more challenging to resolve than the FNP itself. Adding corticosteroids to IVIG may be recommended to reduce IVIG resistance, decrease the risk of developing CALs, and alleviate CALs.