stromal dystrophy

  • 文章类型: Case Reports
    Deep anterior lamellar keratoplasty or penetrating keratoplasty are currently considered the optimal methods of surgical treatment of stromal dystrophies and corneal degeneration. Despite certain advantages and benefits of these methods, they also have significant limitations: involvement of superficial corneal layers in the surgery, need for suturing, development of post-keratoplasty astigmatism etc.
    OBJECTIVE: This study aimed to test and describe the new method of closed sutureless keratoplasty (intracorneal selective stromal transplantation), which was indicated in isolated dystrophic and degenerative pathology of the stroma.
    METHODS: Intracorneal selective stromal transplantation was performed in a 62-year-old patient with stromal degeneration and intact corneal layers between the altered stroma and the Descemet\'s membrane posteriorly, and the Bowman\'s layer anteriorly. The patient also had immature senile cataract. Corneal stroma was removed and replaced with a graft in the optical center of the lens, while the endothelium, the Descemet\'s membrane and the Bowman\'s layer remained intact.
    RESULTS: The proposed technique of intracorneal selective stromal transplantation makes it possible to replace only the pathologically altered stroma through closed surgical approach, without affecting the anterior and posterior surfaces of the cornea. Best-corrected visual acuity has increased in the patient from 0.01 to 0.6, while mean endothelial cell density has not changed in the course of 24-months follow-up.
    CONCLUSIONS: The proposed keratoplasty method can be used in patients with dystrophy or degeneration of the corneal stroma and preserved endothelial cells, intact Descemet\'s membrane and Bowman layer. Since the superficial corneal layers are not involved during the surgery, intracorneal selective stromal transplantation combined the advantages of both deep anterior lamellar keratoplasty and endothelial keratoplasty. The biologically favorable result in this first clinical case allows a preliminary conclusion on the technical possibility and functional effectiveness of the proposed method, but further long-term observation and more clinical cases are required.
    В настоящее время оптимальным способом хирургического лечения стромальных дистрофий и дегенераций роговицы являются глубокая передняя послойная кератопластика или сквозная кератопластика. Несмотря на определенные преимущества и достоинства этих методов, они обладают существенными недостатками: вовлечение в хирургию поверхностей роговицы, наложение швов, развитие посткератопластического астигматизма и др.
    UNASSIGNED: Апробировать и описать новый метод бесшовной закрытой кератопластики (внутрироговичная селективная трансплантация стромы), показанный при изолированной патологии стромы дистрофического и дегенеративного генеза.
    UNASSIGNED: Внутрироговичная селективная трансплантация стромы выполнена пациенту 62 лет со стромальной дегенерацией и интактными слоями роговицы между измененной стромой и десцеметовой мембраной (ДМ) сзади и боуменовым слоем (БС) спереди. Имела место сопутствующая незрелая сенильная катаракта. В оптическом центре была удалена и далее трансплантирована только строма роговицы, в то время как эндотелий, ДМ и БС были сохранены интактно.
    UNASSIGNED: Предложенная техника внутрироговичной селективной трансплантации стромы позволила заменить только патологически измененную строму закрытым хирургическим подходом, не затрагивая переднюю и заднюю поверхности роговицы. Максимально корригированная острота зрения увеличилась с 0,01 до 0,6, а средняя плотность эндотелиальных клеток не изменилась в течение 24 мес периода наблюдения.
    UNASSIGNED: Предложенный метод кератопластики может быть использован при дистрофиях и дегенерациях стромы роговицы с сохранными эндотелиальными клетками, интактной ДМ и БС. Поскольку поверхности роговицы не вовлекаются в хирургию, внутрироговичная селективная трансплантация стромы обладает одновременно преимуществами глубокой передней послойной и эндотелиальной кератопластики. Благоприятный биологический результат в нашем первом клиническом случае предварительно позволяет сделать заключение о технической возможности и функциональной эффективности предложенного метода. Необходимы длительные наблюдения большего количества клинических случаев.
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  • 文章类型: Journal Article
    角膜营养不良是一组非炎性遗传性角膜疾病。这篇综述考虑了上皮基质和基质角膜营养不良的治疗选择:即Reis-Bücklers,Thiel-Behnke,晶格,Avellino,颗粒状,黄斑和Schnyder角膜营养不良。哪里有视觉减少,治疗方案可包括光疗性角膜切削术(PTK)或角膜移植.由于Reis-Bücklers和Thiel-Behnke营养不良中沉积物的前部位置,PTK被认为是选择的治疗。对于晶格,Avellino,颗粒状和黄斑角膜营养不良,PTK提供了暂时的视觉改善;然而,随着复发,需要重复PTK或角膜移植。对于施耐德营养不良,如果需要治疗,由于PTK在角膜移植中可能复发,因此PTK可能是首选。这篇综述从视觉结果和复发率方面讨论了治疗角膜营养不良的文献和证据基础。
    Corneal dystrophies are a group of non-inflammatory inherited disorders of the cornea. This review considers treatment options for epithelial-stromal and stromal corneal dystrophies: namely Reis-Bücklers, Thiel-Behnke, lattice, Avellino, granular, macular and Schnyder corneal dystrophies. Where there is visual reduction, treatment options may include either phototherapeutic keratectomy (PTK) or corneal transplantation. Due to the anterior location of the deposits in Reis-Bücklers and Thiel-Behnke dystrophies, PTK is considered the treatment of choice. For lattice, Avellino, granular and macular corneal dystrophies, PTK provides temporary visual improvement; however, with recurrences, repeat PTK or a corneal transplant would be needed. For Schnyder dystrophy, should treatment be required, PTK may be the preferred option due to the potential for recurrence of the disease in corneal transplantation. This review discusses the literature and evidence base for the treatment of corneal dystrophies in terms of visual outcomes and recurrence rate.
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  • 文章类型: Case Reports
    193nm准分子激光的光疗角膜切除术(PTK)是治疗浅层角膜病理的安全有效方法。我们旨在回顾PTK在治疗角膜黄斑营养不良(MCD)中的应用。
    病例报告及文献复习。
    一个16岁的男孩出现在眼科医生面前,他有4年的视力下降史,他的左眼眩光和畏光。他被诊断为角膜黄斑营养不良,并接受了CHST6基因测序。用丝裂霉素C进行左准分子PTK。直到术后18个月,他的视力下降,基质看起来更“乳白色”,他一直没有复发。在最初的PTK后24个月,他在左眼接受了穿透性角膜移植术。
    光疗性角膜切除术是黄斑角膜营养不良患者视觉恢复的有效手段,可能会延迟穿透性角膜移植术。应就复发的高风险对患者进行咨询。这是第一例报道的患有MCD的CHST6基因阳性患者接受光疗角膜移植术治疗。
    Phototherapeutic keratectomy (PTK) with 193-nm excimer laser is a safe and effective procedure for the treatment of superficial corneal pathology. We aimed to review the use of PTK for the treatment of corneal macular dystrophy (MCD).
    Case report and literature review.
    A 16-year-old boy presented to an ophthalmologist with a 4-year history of reduced vision, glare and photophobia in his left eye. He was diagnosed with corneal macular dystrophy and underwent sequencing of the CHST6 gene. Left excimer PTK with mitomycin C was performed. He remained relapse free until 18 months post procedure when his visual acuity declined and the stroma appeared more \"milky\". He underwent a penetrating keratoplasty in his left eye 24 month following the initial PTK.
    Phototherapeutic keratectomy is an effective means of visual restoration in patients with macular corneal dystrophy and may delay penetrating keratoplasty. Patients should be counselled regarding the high risk of recurrence. This is the first reported case of a CHST6 gene positive patient with MCD that was treated with phototherapeutic keratoplasty.
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  • 文章类型: Journal Article
    OBJECTIVE: To describe the phenotype of canine macular corneal dystrophy (MCD) including the clinical presentation, multimodal ocular imaging, histopathology, and ultrastructural analysis in ten Labrador Retrievers.
    METHODS: Multicentered data collection.
    RESULTS: Labrador Retrievers affected by MCD were presented between the age of 4.5 and 6 years of age with a history of cloudy eyes and/or visual impairment. Findings on ophthalmic examination included a diffuse haze of the corneal stroma and multiple, well-demarcated, off-white to yellow-brown, punctate corneal opacities heterogeneous in size. Corneal vascularization developed in most dogs as the disease progressed. Disease progression was associated with increased density of the corneal haze as well as increased number and size of the focal opacities and dogs developed significant visual impairment. Spectral domain-optical coherence tomography revealed multifocal hyper-reflective regions within the stroma. In vivo confocal microscopy revealed marked alterations in reflectivity throughout the entire stroma. Normal keratocytes could not be identified in affected areas. Histopathology showed stromal collagen fibers separated by acidophilic granular material on hematoxylin and eosin stain. The material stained with periodic acid-Schiff and colloidal iron stain but not with Masson trichrome stain, confirming the accumulation of glycosaminoglycans. On electron microscopic ultrastructural examination, keratocytes presented with vacuolated rough endoplasmic reticulum and multiple electron dense cytoplasmic inclusions. In areas keratocytes appeared ruptured, with cell organelles and proteinaceous material grouped together between collagen fibers.
    CONCLUSIONS: MCD in Labrador Retrievers has similarities with the human counterpart of the condition and is an important differential diagnosis in dogs with corneal disease.
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  • 文章类型: Journal Article
    退行性或遗传性角膜疾病有时难以区分。角膜营养不良影响约0.09%的人口。它们根据其表型通过IC3D分类进行识别,基因型和为他们的诊断收集的证据。实际上,眼科医生管理功能性症状,比如经常性的糜烂,视力丧失和弱视,畏光,异物感,有时疼痛和美学问题。药物治疗包括促进愈合的滴剂,软膏,高渗剂和绷带隐形眼镜。微创手术治疗被用作二线治疗(光疗角膜切除术,板层角膜切除术)。最终可以使用更多的侵入性程序(层状或穿透性角膜移植术)。前板层或内皮角膜移植术现在优于穿透性角膜移植术,尽管在某些情况下,后者仍然是唯一可能的选择。一些罕见的营养不良需要协调和全面的医疗护理。
    Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09 % of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. Practically, the ophthalmologist manages functional symptoms, such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses. Less invasive surgical treatments are used as second line therapy (phototherapeutic keratectomy, lamellar keratectomy). More invasive procedures may eventually be utilized (lamellar or penetrating keratoplasty). Anterior lamellar or endothelial keratoplasty are now preferred to penetrating keratoplasty, although the latter still remains the only possible option in some cases. Some rare dystrophies require coordinated and comprehensive medical care.
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  • 文章类型: Journal Article
    Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09% of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. In practice, the ophthalmologist manages functional symptoms such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses. Less invasive surgical treatments are used as second line therapy (phototherapeutic keratectomy, lamellar keratectomy). More invasive procedures may eventually be utilized (lamellar or penetrating keratoplasty). Anterior lamellar or endothelial keratoplasty are now preferred to penetrating keratoplasty, although the latter still remains the only possible option in some cases. Some rare dystrophies require coordinated and comprehensive medical care.
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