引起周围溃疡性角膜炎的系统性疾病及眼局部治疗

周围溃疡性角膜炎（PUK）是一种罕见的周围角膜疾病,严重时可致失明,合并系统性疾病者甚至危及生命。因此,及时寻找病因进行早期诊断及干预性治疗有助于控制疾病进展,提高患者生活质量及延长寿命。近年来对于系统性疾病致PUK的研究取得了很多重要进展。本研究旨在阐述PUK相关的系统性疾病及眼局部治疗的研究进展。     

Mycobacterium Chelonei Keratitis：Report of a Case and Review of the Literature

Background: Mycobacterium (M) chelonei keratitis is a rare opportunistic eye infection that can cause significant morbidity when not being treated properly. The first case was documented by Gangadharam et al in 1978 and since then, a total of 49 cases were reported in the literature. One alarming fact is that more than 50% of cases were found in the Chinese population and mostly reported in recent years. The key to successful management of M. chelonei keratitis is early diagnosis by high index of suspicion. In order to alert ophthalmologists of this condition, we report a typical case of M. chelonei keratitis and review the literature of all the reported cases with special reference to its risk factors, treatments and outcome.Methods: The cases reported in the literature and a case of our own were reviewed and analyzed.Results: Our case was a 42-year-old gentleman who developed M. chlonei keratitis following pterygium surgery. He had typical clinical features of irregular infiltrates with radiating proj     

Subacute Acrylamide Intoxication with Severe Visual Disturbance: A Case Report

A 35-year-old man was admitted due to somnolence and progressive sensory-motor polyneuropathy, followed by severe visual impairment in both eyes after direct skin exposure to an acrylamide monomer solution. The results of an ophthalmological examination including central critical flicker fusion frequency and the decreased amplitude of action potentials observed in the visual evoked potential studies suggested that acrylamide intoxication resulted in neuronal degeneration in the optic pathways. Additional attention should be directed to the potential effect of acrylamide on the human visual system.     

Bilateral Orbital IgG4-Related Disease with Systemic and Corneal Involvement Showing an Excellent Response to Steroid and Rituximab Therapy: Report of a Case with 11 Years Follow-Up

IgG4-related disease is a newly recognized fibro-inflammatory condition. The purpose of this report is to present a patient with 11 years of follow-up, who revealed characteristic features of IgG4-related disease with systemic, orbital and corneal involvement and showed a favorable response to steroids and rituximab treatment.     

Dysthyroid Orbitopathy Presenting with Gaze-Evoked Amaurosis: Case Report and Review of the Literature

Background: Gaze-evoked amaurosis (GEA) describes visual loss associated with eccentric gaze that recovers when the eye is returned to primary position. Here we describe an unusual case of bilateral GEA as the presenting feature of dysthyroid orbitopathy. This is only the third such case to be reported in the literature and the first to feature bilateral GEA in all positions of gaze without accompanying proptosis or ophthalmoplegia.

Case Presentation: A 50-year-old man who had recently commenced treatment for thyrotoxicosis presented with a 3-week history of typical GEA in both eyes in all positions of gaze. He subsequently developed a bilateral compressive optic neuropathy which was only partially responsive to high dose steroid therapy.

Conclusion: Although an uncommon presenting feature of dysthyroid orbitopathy, GEA is an ominous symptom that may precede sight-threatening optic nerve compromise. When present, early immunosuppressive and/or decompressive treatment should be considered.     

Stromal Keratitis with Endophthalmitis Caused by Vittaforma Corneae in an Immunocompetent Patient: A Case Report

Purpose: To describe a case of microsporidial stromal keratitis with endophthalmitis in an immunocompetent patient.Methods: Case reportResults: A 58-year-old HIV-negative man presented with stromal keratitis in his right eye. The patient demonstrated subsequent vitritis, multifocal retinitis and arteritis, and macular edema with recurrent vitreous hemorrhage after therapeutic keratoplasty. Numerous microsporidial spores were detected in corneal tissues by modified trichrome stain. Both corneal tissues and vitreous sample of the affected eye showed positive results by polymerase chain reaction targeting the microsporidial small subunit rRNA gene whose sequences belonged to Vittaforma corneae. Post-keratoplasty and vitrectomy, his best-corrected visual acuity was hand motion due to pale optic disc.Conclusion: Endophthalmitis can be a consequence of microsporidial stromal keratitis in an immunocompetent host. Early recognition and prompt treatment should be considered in patients diagnosed with microsporidial keratitis presenting with mild vitritis, retinitis, and recurrent vitreous hemorrhage.     

Tuberculous Uveitis Presenting with a Bullous Exudative Retinal Detachment: A Case Report and Systematic Literature Review

Purpose: To describe tuberculous uveitis (TU) presenting as a bullous retinal detachment (RD) and to perform a comprehensive literature review on TU with similar features.

Methods: Observational case report and systematic literature review.

Results: An 84-year-old woman presented with bilateral granulomatous uveitis and bullous RD in the left eye. The interferon gamma release assay was strongly positive, but all other tests were unremarkable. The patient was diagnosed with TU and started on anti-tubercular therapy (ATT) and systemic steroids with excellent treatment response. Twenty-six articles (32 cases) reported TU with exudative RD. Choroidal tuberculoma was the most common clinical manifestation, followed by optic disc edema and retinal exudate. Systemic steroids with ATT improved vision in more patients (78.6%) than ATT alone (50.0%) or oral steroids followed by ATT (50.0%).

Conclusion: Atypical presentations of TU make diagnosis and treatment difficult. A high level of suspicion for TU is needed to minimize ocular morbidity.     

Infectious Keratitis Following Corneal Crosslinking: A Systematic Review of Reported Cases: Management,Visual Outcome,and Treatment Proposed

Aim: To describe the infectious complications and the group of pathogens involved in the infection following corneal crosslinking, the visual outcome, and the treatment proposed. Methods: A Medline (National Library of Medicine, Bethesda, MD, USA) search from October 2000 to October 2013 was performed to identify all articles describing infectious keratitis following corneal crosslinking treatment. Nineteen articles were selected. Ten articles reported infectious complications of corneal crosslinking treatment were included. Nine articles were excluded, because seven described sterile keratitis, one article was in German, and one reported general complication without describing the infection complication. Results: A total number of infections reported included 10 eyes. The infectious keratitis was associated with bacteria in five eyes (50%): gram-positive bacteria in three eyes (30%) (staphylococcus epidermidis, S. aureus and streptococcus salivarius plus S. oralis, respectively) and gram-negative bacteria in two eyes (20%) (E. coli; P. aeruginosa); there was herpes virus in two eyes, fungus in two eyes (Fusarium and Microsporidia) (20%), and Acanthamoeba in one eye (10%). Conclusions: Only 10 cases of infectious keratitis following corneal crosslinking are published. The most virulent pathogens were Pseudomonas aeruginosa and Acanthamoeba. Less virulent organisms were Escherichia coli and S. epidermidis. Two cases of herpes keratitis were described, suggesting the possibility of systemic antiviral prophylaxis before corneal crosslinking treatment. The most common risk factor of infections identified was postoperative incorrect patient behavior.     

Epithelioid Angiosarcoma of the Orbit Presenting as Tolosa-Hunt Syndrome: A Clinicopathologic Case Report with Review of the Literature

The clinical diagnosis of Tolosa-Hunt syndrome was first considered in a 66-year-old man with facial pain and diplopia. A complete neuroradiologic evaluation as well as an oncologic work-up yielded normal results. Several courses of oral prednisone provided no significant benefit. Within a year the patient became clinically worse and a CT scan disclosed an abnormal area of enhancement at the left orbital apex. An orbital exploration was performed elsewhere and a histologic diagnosis of myositis was obtained. Because of further worsening the patient was re-evaluated 3 months later and a CT scan showed a mass in the left orbital apex and superior orbital fissure. A second orbital exploration was performed and a sausage-shaped mass encompassing the optic nerve was excised. By light microscopy a poorly differentiated malignant tumor was infiltrating the orbital tissues with areas of intra- and perineural invasion. The tumor cells were arranged in strands and tubules with a definite tendency to form lumens that often contained red blood cells. Electron microscopic studies disclosed features consistent with a neoplasm of endothelial cell origin displaying a polarized basal lamina and rare micropinocytotic vesicles on the luminal side. The presence of multiple, slender microvilli and sometimes tonofilaments as well as desmosomes were interpreted as epithelioid metaplasia of an angiosarcoma.     

Malignant Peripheral Nerve Sheath Tumor of Lacrimal Nerve: A Case Report

Background: Orbital and periorbital presentation is rare for malignant peripheral nerve sheath tumors. These tumors are poorly defined spindle cell neoplasms of peripheral nerves and have not been reported to develop in the lacrimal gland to date.

Aim: To report a rare presentation of malignant peripheral nerve sheath tumor in the orbital cavity.

Case report: A 65-year-old man was admitted with the chief complaint of prominent right eye. His symptoms began 16 months prior to his admission. He had obvious limited ocular motion in upgaze and lateral gaze directions, as well as diplopia in all directions. He underwent imaging studies, and an iso-dense mass lesion in the lacrimal gland was revealed in orbital CT scan. In the MRI, there was a well-defined iso-intense mass lesion in T1-weighted images that was hyperintense in T2-weighted images and was enhanced with Gadolinium. Excisional biopsy revealed epithelioid and spindle cells with hyperchromatic rather than pleomorphic nuclei. Immunohistochemistry confirmed the presence of positive markers consistent with malignant peripheral nerve sheath tumor.

Conclusion: Malignant peripheral nerve sheath tumor should be considered in the differential diagnosis of lacrimal gland tumors. Imaging studies may be helpful but tissue biopsy should be performed for accurate diagnosis. Complete excision of the mass lesion and adjunctive chemotherapy and radiotherapy should be considered in these cases.     

Ocular Neuromyotonia: Case Reports and Literature Review

Ocular neuromyotonia (ONM) is a rare eye movement disorder, presenting as a paroxysmal involuntary spasm of one or more extra-ocular muscles, that can persist for a few seconds up to several minutes. The phenomenon is caused by the contraction of an extra-ocular muscle, excited by a damaged nerve, which leads to delayed muscle relaxation.

We present eight patients with this rare condition together with an overview of the literature on all published ONM cases. One of the presented cases is possibly secondary to hypovitaminosis D. This association has not been reported previously in the literature. A possible underlying mechanism is given.     

Orbital Paraganglioma Presenting as Lateral Rectus Enlargement and its Novel Management: A Case Report and Review of Literature

Extra-ocular and upper eyelid (levator) muscle damage in thyroid orbitopathy may be due to autoimmunity against eye muscle auto antigens. The main antigen appears to be the calcium binding protein calsequestrin. In this study we have tested for T lymphocyte sensitization to calsequestrin in patients with Graves’ disease, with and without orbitopathy, in standard proliferation assay. We have also tested total RNA prepared from thyroid tissue of patients with Graves’ disease with and without orbitopathy for expression across 20,589 genes using micro array analysis technology. We were looking for differences in gene expression between the two groups which might provide information about the early thyroid events that lead to the development of eye muscle autoimmunity. Positive lymphocyte reactivity to calsequestrin was demonstrated in 59% of Graves’ patients with orbitopathy, 33% without evident ophthalmopathy and in 43% of patients with Hashimoto’s thyroiditis and upper eyelid retraction (UER). Two hundred and ninety six genes were identified to be differentially expressed between in patients with Graves’ disease with and without orbitopathy. Of these, the cardiac calsequestrin gene CASQ2 was the most highly up regulated, 2.2-fold. The closely related skeletal muscle calsequestrin gene CASQ1 was also up-regulated, 4.1 fold, but this was not significant, while genes encoding the thyroid antigens thyroglobulin, thyroid peroxidase and the TSH-receptor were not differentially expressed. These findings provide further evidence for a prominent role of autoimmunity against calsequestrin in the pathogenesis of the eye muscle components of thyroid orbitopathy.     

Cutaneous Embryonal Rhabdomyosarcoma Presenting as a Nodule on Cheek; A Case Report and Review of Literature

We report a case of primary cutaneous rhabdomyosarcoma, solid embryonal type, presenting as a rapidly enlarging nodule on the right cheek of a 7-year-old boy. This lesion had begun as a pea-sized nodule 8 months previously, and, with suspected abscess, had been incised. It recurred 2 months later; at that time, incisional biopsy was consistent with malignant round cell tumor. Wide local excision of the tumor was then completed. Subsequent immunohistochemical staining with desmin and myoglobin confirmed embryonal rhabdomyosarcoma. The patient underwent radiation therapy followed by chemotherapy and continues to be disease free at 14 months after his wide local excision. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.     

Horizontal Gaze Palsy with Progressive Scoliosis: A Case Report and Literature Review

Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare autosomal recessive disorder. The ROBO 3 gene mutation is responsible for the disease. We present a boy aged 12 years who was admitted for scoliosis surgery who had also had horizontal gaze palsy since birth. His brainstem abnormalities were compatible with the syndrome of HGPPS. HGPPS is one of the rare congenital diseases of childhood. Horizontal gaze palsy, ametropia, and progressive scoliosis are the main findings of the disease. This syndrome should be kept in mind for both ophthalmologists and orthopaedic surgeons in patients who present with gaze palsy and scoliosis. Early diagnosis of scoliosis makes it possible to treat the disease at an early stage, and early diagnosis of ametropia is important in the prevention of amblyopia.     

Bilateral Posterior Scleritis Presenting as the First Manifestation of Immunoglobulin A Nephropathy: Case Report and Review of the Literature

Purpose: We describe a case of posterior scleritis presenting as the first manifestation in a Chinese patient with immunoglobin A nephropathy (IgAN).

Methods: Retrospective review of our patient and review of the published case reports from PubMed search.

Results: A 43-year-old woman developed bilateral posterior scleritis. Microscopic hematuria and proteinuria was found by urinalysis, and a kidney biopsy revealed IgAN. The posterior scleritis was resolved with oral prednisolone. For the review, 12 cases of episcleritis (70.6%), 4 cases of scleritis (23.5%), and 1 case of posterior scleritis (5.9%) associated with IgAN were analyzed. Five patients (29.4%) had scleritis or episcleritis as the first presenting manifestation.

Conclusions: Posterior scleritis may be the first manifestation of IgAN in a patient. For any adult patient with scleral/episcleral inflammation, urinalysis should be done to rule out IgAN.     

Rituximab-Associated Retinal Occlusive Vasculopathy: A Case Report and Literature Review

ABSTRACT

Purpose

To describe a case of retinal occlusive vasculopathy associated with intravitreal administration of rituximab.     

Eyelid Lipomas: A Case Report and Review of the Literature

Simple lipomas of the eyelid are rare. We present a case of a 61-year-old man, who presented with 6 months of a slowly worsening blepharoptosis. On examination, that patient was noted to have a palpable, soft mass in the medial left upper eyelid. Histopathological examination of the mass revealed mature adipose tissue most consistent with lipoma. Simple lipomas of the eyelid are very unusual but should be considered in the differential diagnosis of patients presenting with mechanical ptosis.     

Certolizumab-Induced Uveitis: A Case Report and Review of the Literature

We report the case of a 64-year-old woman with rheumatoid arthritis and bilateral visual deterioration. The patient had been treated with certolizumab, a new tumor necrosis factor alpha (TNFα) antagonist, and her findings were consistent with bilateral uveitis, suggestive of sarcoidosis. Here, we review the literature on TNFα antagonist-induced sarcoidosis and report the first case of uveitis induced by certolizumab. Awareness of the possibility of this unique complication is important for both rheumatologists and ophthalmologists who treat patients with this new agent.Key words: Certolizumab, Ocular sarcoidosis, Sarcoidosis, Uveitis, Tumor necrosis factor alpha     

Type IV Hypersensitivity to Gold Weight Upper-Eyelid Implant: Case Report and Review of the Literature

Purpose: Complications associated with gold-weight insertion for lagophthalmos are uncommon, recent reports have provided evidence to suggest that type IV hypersensitivity to gold can cause a persistent inflammatory reaction.

Methods: We present a case of a 46-year-old man who experienced persistent post-operative inflammation, and summarize previously documented cases. This patient underwent uncomplicated insertion of an upper eyelid gold weight for right-sided facial nerve palsy. He had no allergies or implanted metalwork. Post-operatively erythema was noted at seven-weeks and did not resolve. The weight was removed after six-months.

Results: The histopathological findings were in keeping with type IV hypersensitivity and similar to previous cases.

Conclusions: Although infrequent, this complication has poor outcomes. The definitive management is removal of the weight. Information regarding implanted gold, and previous reactions should be elicited pre-operatively. Type IV hypersensitivity should be considered in patients with persistent inflammation that do not respond to antibiotic or steroid therapy.     

Acute Retinal Necrosis Presenting in Developmentally-delayed Patients with Neonatal Encephalitis: A Case Series and Literature Review

We report three cases of patients with developmental-delay from neonatal herpetic encephalitis and/or meningitis who presented years later with acute retinal necrosis due to herpes simplex virus. The diagnosis was delayed in all cases due to the patients’ inability to verbalize their ocular complaints and cooperate with eye examinations. This case series documents the clinical course, pathophysiologic mechanism, and treatment of acute retinal necrosis in this patient population. Clinicians should understand the importance of prudent consideration of acute retinal necrosis in patients with a history of neonatal herpetic encephalitis and/or meningitis presenting with a red eye.