摘要
12岁男性患儿,因“双眼视力下降3年,加重半年”就诊。既往史:5年前双耳先后发生重度感音神经性耳聋,后行人工耳蜗植入术;右眼反复发作前葡萄膜炎3年。查体如下。最佳矫正视力:右眼CF/40 cm,左眼0.05。眼压:右眼19 mmHg(1 mmHg=0.133 kPa),左眼13 mmHg。裂隙灯检查:右眼角膜后弥漫分布中小型树突状角膜后沉着物(KP),相对性瞳孔传入障碍(RAPD)(+),瞳孔欠圆,瞳孔向鼻上移位,鼻上虹膜瞳孔领色素外翻,伴鼻上虹膜周边前粘连,虹膜表面散在小结节;左眼前节未见明显异常;双眼视盘色淡,右眼视盘边界清,左眼视盘鼻侧边界稍模糊。眼眶CT:右侧视神经明显增粗迂曲,累及眶内段及管内段,右侧视神经管增宽,左侧视神经轻度增粗。高频B超:双眼视神经鞘膜下间隙增宽,右眼增宽明显不对称,鼻侧和下方较宽,且呈较实性的中等回声,左眼增宽均匀。随诊期间左右两侧先后发生面瘫,颅脑CT:右侧面神经管迷路段和膝状神经节区增粗,双侧小脑幕增厚,涉及双侧海绵窦区。神经内科腰穿脑脊液检查发现大量形态单一的幼稚淋巴细胞。颈部磁共振成像(MRI):颈椎硬脊膜明显增厚,伴脊髓受压。行手术活检,术后病理未见典型肿瘤图像。初步诊断:多脑神经病变,脑脊膜病变,右眼葡萄膜炎。讨论体会:本例患者目前尚无最终诊断,根据患儿的临床表现及已有相关辅助检查,淋巴瘤或者炎性病变可能性较大,目前仍需进一步的检查予以明确诊断。
A 12-year-old boy presented with bilateral visual loss for 3 years and progressed in the past half a year.The patient was diagnosed with bilateral profound sensorineural hearing loss 5 years ago and then a cochlear was implanted.He was also diagnosed with recurrent uveitis in the right eye for 3 years.His best corrected visual acuity was CF/40cm in the right eye(OD)and 0.05 in the left(OS).The intraocular pressure was 19 mmHg(OD)and 13 mmHg(OS).Slit lamp examination of the right eye revealed small to medium-sized dendritic keratic precipitates(KPs).The right pupil was irregular and displaced superonasally.Ectropion uvea,superonasal anterior synechia,multiple iris nodules and RAPD were present in OD.The left anterior segment was unremarkable.The funduscopic examination showed bilateral pale optic discs with blurred disc margin nasally in OS.Orbital CT demonstrated significant enlargement of the intraorbital and intracranial segments of the right optic nerve and slight enlargement of the left optic nerve.High-resolution transbulbar ultrasonography revealed asymmetrically enlarged subarachnoid space(SAS)of the optic nerve in OD and evenly enlarged in OS.The subarachnoid echo of the right eye also revealed a moderately solid echogenic pattern.Bilateral facial nerve paralysis occurred successively during the period of follow-ups.Brain CT showed widened labyrinthine segment and the geniculate fossa of the right facial nerve canal,thickened tentorium cerebelli involving bilateral paracavernous sinus meninges.Cerebrospinal fluid examination revealed a large number of immature lymphocytes presenting uniform morphology.Cervical MRI showed obviously thickened dura mater with spinal compression,but tumor cells were not detected in biopsy tissue.Primary diagnosis:multiple cranial neuropathies,meningeal lesions and uveitis(OD).According to patient’s clinical manifestations and associated auxiliary tests,lymphoma or inflammatory pathogenesis was highly suspected.However,further examinations screening for systemic disorders were needed to obtain a finial diagnosis.
作者
孙平
陈倩
田国红
冯超逸
SUN Ping;CHEN Qian;TIAN Guohong;FENG Chaoyi(Department of Ophthalmology,Eye&ENT Hospital of Fudan University,Shanghai 200031,China)
出处
《中国眼耳鼻喉科杂志》
2024年第S01期62-66,72,共6页
Chinese Journal of Ophthalmology and Otorhinolaryngology
关键词
多脑神经
脑脊膜
淋巴瘤
炎症
Multiple cranial nerve
Meninges
Lymphoma
Inflammation
