【摘要】 结节病是一种罕见的和潜在的致残儿科疾病。此病临床治疗困难，需要长期监测。我们报告了1例9岁的白人女孩的情况，就诊最初表现为双侧慢性前葡萄膜炎、白内障和继发于结节病的青光眼。在过去的2a，患者接受左布诺洛尔滴剂，局部和全身类固醇治疗，随后要求折叠式人工晶状体植入。在白内障手术后的2mo，患者葡萄膜炎以局部类固醇和低剂量甲氨蝶呤一直控制良好。她全血计数和肾功能定期监测，没有甲氨蝶呤不良影响的报告。结节病是一多系统疾病，需要眼科学家，神经学家和儿科医师多学科的投入。内科和外科治疗本病富于挑战。本例强调了低剂量甲氨蝶呤在儿童慢性葡萄膜炎治疗中的安全性和对此病及时治疗以防止显著发病的需要。
【关键词】 结节病;白内障;葡萄膜炎
Childhood ocular sarcoidosis: a case report
Terrence Soong, TKJ Chan, Hardeep Mudhar, IG Rennie, Victor Soong, Angela Loo Voon Pei
Department of Ophthalmology, University of Malaya, 50603 Lembah Pantai, Kuala Lumpur, Malaysia
Department of Ophthalmology, Royal Hallamshire Hospital, Glossop Road, Sheffield, S10 2JF, United Kingdom
Department of Histopathology, Royal Hallamshire Hospital, Glossop Road, Sheffield, S10 2JF, United Kingdom
Academic Unit of Ophthalmology and Orthoptics, the University of Sheffield, Glossop Road, Sheffield, S10 2JF, United Kingdom
Department of Ear, Nose and Throat, Salford Royal Teaching Hospitals NHS Trust, United Kingdom
AbstractSarcoidosis is a rare and potentially disabling disease in the paediatric age group. Clinical management of such cases is difficult and requires longterm monitoring. We report a case of a 9 years old Caucasian girl who presented initially with bilateral chronic anterior uveitis, cataracts and glaucoma secondary to sarcoidosis. She was treated with Guttae Levobunolol, topical and systemic steroids over the last 2 years but subsequently required cataract extractions with foldable intraocular lens implants (Alcon Laboratories, Inc. Acrysof IOL Model: MA60AC). Her uveitis was well controlled with both topical steroids and low dose methotrexate over the last 2 months following cataract surgery. Her full blood counts and renal functions are monitored regularly. There was no adverse effect from the methotrexate reported so far. Sarcoidosis is a multisystem disease and requires multidisciplinary input from ophthalmologists, neurologists and paediatricians. Medical and surgical treatment of such ocular manifestations is challenging. This case highlights both the safety of low dose methotrexate in the management of childhood chronic uveitis and the need for prompt treatment in such cases to avert significant morbidity from this disease.
KEYWORDS: sarcoidosis; cataract; uveitis
CASE REPORT
A 9 years old Caucasian girl presented initially with an acute onset of bilateral red and painful eyes associated with reduced vision. She had no significant respiratory or musculoskeletal symptoms but admitted getting tired on exertion. Her medical and family histories were unremarkable. There was no evidence of any exposure to TB. On examination, her visual acuity was 6/60 on the right and 6/36 on the left which improved with pinhole to 6/12 and 6/9 respectively. She had bilateral incomplete band keratopathy. Slitlamp biomicroscopy revealed mild flare and odd cell in her anterior chambers with absence of keratic precipitates. She had few posterior synaechiae, but the pupils dilated reasonably well. The intraocular pressures were 20mmHg on both eyes. There were signs of early lenticular haze (Figure 1). Her vitreous showed a few cells on both sides but otherwise the optic discs, retinal vessels and fundi were normal. She was commenced on topical steroid therapy and was investigated further with preliminary blood tests and a chest Xray. Blood results revealed a raised angiotensin converting enzyme level of 100U/L, Creactive protein of 12mg/L and corrected calcium of 2.58mmol/L. Chest Xray revealed focal scoliosis at the level of T4/5 with concavity to the left side. In light of her investigative blood results and radiological findings, both the ophthalmologist and rheumatologist diagnosed bilateral chronic anterior uveitis secondary to childhood ocular sarcoidosis. Figure 1 Early band keratopathy, posterior synaechia and cortical cataract.(略)
She was then started with low dose oral methotrexate (MTX) 10mg and folic acid 5mg weekly. There was mild abdominal upset at the commencement of MTX therapy but it resolved quickly when the dose was reduced to 5mg per week. Her uveitis was well controlled with both topical corticosteroid and MTX. Subsequently, she developed raised intraocular pressure which was treated with guttae levobunolol. Her cataracts had progressed within the last 2 years and required cataract extractions. She was given prophylactic intravenous methylprednisolone and remained on MTX therapy before cataract surgery of her right eye. She underwent routine phacoemulsification cataract surgery and a foldable intraocular lens (Alcon laboratories, Inc. Acrysof IOL Model: MA60AC) was implanted in the capsular bag (Figure 2). Both the lens aspirate and anterior capsule were sent for histopathology analysis.Figure 2 Three months post cataract showing quiet anterior chambers with clear intraocular lens implant.(略)
Her postoperative visual recovery was uneventful and her bestcorrected visual acuity had improved to 6/9. Seven months later, the visual acuity of her right eye remained stable. Microscopy of the anterior capsule revealed abnormal lens epithelial cells exhibiting pleomorphism with microvacuolation of the cytoplasm (Figure 3). Lens aspirate revealed typical cataract degenerative changes characterized by cell membrane lysis with exudation of granular eosinophilic proteinaceous bodies (Figure 4). Unfortunately, no granuloma formation was identified. She remains treated with both topical steroids and low dose 7.5mg MTX. Her full blood counts, renal and liver functions are monitored regularly. There was no adverse effect from the MTX reported so far. She is awaiting cataract surgery and lens implant on her fellow eye.Figure 3 Anterior lens capsule, lens basement membrane (thin arrow). Attenuated, vacuolated degenerate lens epithelial cells (thick arrow) (toluidine blue).(略) Figure 4 Lens aspirate showing granular eosinophilic degenerate lens epithelial cells (arrows) amongst viable epithelial fibres (hematoxylin and eosin).(略)
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