Corneal Crystalline Deposits Research Articles

Paraproteinaemic keratopathy simulating a crystalline keratopathy

BackgroundParaproteinemic keratopathy is a rare disorder characterized by the bilateral accumulation of polychromatic deposits diffusely in all corneal layers together or not with diffuse or patchy pseudo lipid deposits. We present an atypical case of paraproteinemic keratopathy which lead to an initial misdiagnosis of infectious crystalline keratopathy.Case presentationa 69-year-old woman with an asymptomatic keratopathy detected during a cataract intervention. Slit-lamp examination revealed several hyper refringent subepithelial foci with fern-shaped branches, resembling crystalline keratopathy, in her left eye. Anterior segment optical coherence tomography revealed exclusively subepithelial hyperreflective lesions limited to the anterior stroma. The progressive bilateralization and progression of the condition prompted us to include other entities with crystalline corneal deposits in our differential diagnosis. Hematological analysis showed a high number of free Kappa light chains. Despite the typical clinical appearance of crystalline keratopathy, the atypical evolution and test results led us to consider that monoclonal gammopathy could be the cause of this entity.ConclusionsParaproteinemic keratopathy may present in its early stages as a unilateral subepithelial crystalline keratopathy. Thus, it must always be taken into account in the differential diagnosis of any crystalline keratopathy, particularly when there are no predisposing factors for an infectious crystalline keratopathy. Early recognition of this rare entity is important to address the associated potentially serious systemic disease.

Crystal-storing histiocytes and plasma cells, crystalline corneal deposits and atypical MRI lesions in multiple myeloma.

Crystal-storing histiocytes and plasma cells, crystalline corneal deposits and atypical MRI lesions in multiple myeloma.

Genotype and Ocular Phenotype in Sixteen Chinese Patients with Bietti Corneoretinal Crystalline Dystrophy

ABSTRACTObjectiveTo investigate CYP4V2 gene variants and ocular clinical characteristics of Bietti corneoretinal crystalline dystrophy in China so as to provide more references for genotype and phenotype of BCD.MethodsSixteen Chinese probands were recruited in Beijing Tongren Hospital in a retrospective study. All patients underwent CYP4V2 gene detection and ophthalmic clinical examinations.ResultsCYP4V2 gene variants were detected in all patients. Eight variants were identified, and the most common one was c.802–8_810del17bpinsGC. Onset age of BCD was from 12 to 44 years, and the first symptoms mostly were decreased visual acuity or night blindness. Corneal crystalline depositions were observed in all patients and were found not only in epithelium and superficial stroma near the limbus but also in corneal endothelium. OCT showed atrophy of RPE in all patients, outer retinal tubulation in ten patients, macular edema in four patients, macular hole in three patients with one accompanied with retinal detachment, and choroidal neovascularization in one patient.ConclusionCYP4V2 gene variants were detected in all patients consistent with the genetic locus homogeneity of BCD, and c.802–8_810del17bpinsGC was the most common mutation. Corneal crystalline depositions were observed in all patients, which may be features of BCD and helpful for the diagnosis of BCD patients, especially those in the advanced stage without typical fundus crystalline depositions or without gene detection. However, considerable phenotypic variability was detected. Corneal crystalline deposits were observed not only in epithelium and superficial stroma but also in endothelium, which has not been reported before. This may provide further evidence for the variable phenotypic expression between affected individuals.

Crystalline corneal deposits after one month of ciprofloxacin treatment for bacterial keratitis

Crystalline corneal deposits after one month of ciprofloxacin treatment for bacterial keratitis

Noninvasive assessment of corneal alterations associated with monoclonal gammopathy.

Aggregation of monoclonal immunoglobulin can lead to organ damage. However, the necessity of invasive examination such as biopsy has hampered better understanding of the pathophysiology. Corneal crystalline deposition is a rarely reported but known ocular manifestation of multiple myeloma. It is unclear whether the cornea is a common target of monoclonal immunoglobulin deposition. We conducted a prospective clinical case-control study to objectively quantify monoclonal gammopathy-associated corneal changes as well as any therapeutic response. Using an ophthalmic Scheimpflug camera imaging for noninvasive corneal assessments, we quantified densitometry values in 30 patients. Although none had crystalline keratopathy, corneal transparency in monoclonal gammopathy patients was significantly impaired compared to that in age-matched controls, based on noninvasive Scheimpflug camera imaging. Furthermore, treatment for multiple myeloma seemed to eradicate the diffuse aggregation of monoclonal proteins. Our results indicate that exposure to monoclonal immunoglobulin may induce the accumulation of monoclonal immunoglobulin in the cornea, and ophthalmic examinations such as corneal densitometry measurements with a Scheimpflug camera may be useful for noninvasive evaluation of monoclonal immunoglobulin deposition diseases.

Corneal Crystalline Deposits Associated With Topically Applied Gatifloxacin

To report a case of corneal crystalline deposits from the use of gatifloxacin 0.5% topical antibiotic after combined cataract extraction and trabeculotomy ab interno surgery. A 59-year-old woman presented after combined cataract extraction and trabeculotomy ab interno with crystalline deposits in the anterior corneal stroma. Clinical examination and slit-lamp photography were performed. The slit-lamp examination showed inferior white crystal deposition in the anterior stroma with overlying punctate epithelial erosions 4 weeks postoperatively. The eye was asymptomatic, but the deposition was cosmetically noticeable to the patient. Serial slit-lamp photography demonstrated resolution of the crystalline deposits 30 days after the discontinuation of eye drops. The authors present a rare case of stromal crystallization from topical gatifloxacin treatment. Complete resolution of corneal deposits was seen 30 days after the discontinuation of the drops without sequelae.

Phenotype–Genotype Correlation in Patients With Schnyder Corneal Dystrophy

The aim of this study was to analyze the corneal morphology features and define mutations in the UbiA prenyltransferase domain-containing 1 (UBIAD1) gene in patients with Schnyder corneal dystrophy from a Polish population. Five affected and 15 unaffected members originating from 3 families with Schnyder corneal dystrophy were included in the study. Phenotype analysis consisted of visual acuity, slit-lamp biomicroscopy with photography, time domain optical coherence tomography, spectral domain optical coherence tomography, and confocal microscopy. Three patients underwent a penetrating keratoplasty. Corneal buttons obtained from the penetrating keratoplasty were processed for light microscopy. A novel mutation I245N of the UBIAD1 gene was revealed in 1 proband and associated with the phenotype without central corneal opacities. The analysis of the other patients showed the N102S mutation. In vivo corneal morphology analysis using optical coherence tomography and confocal microscopy confirmed the presence of multiple crystalline corneal deposits in all affected corneas. The histological examination revealed multiple empty widenings of the corneal lamellae that could represent lipids removed from the specimen. N102S may also be a mutation hotspot in the Polish population, as in other previously reported populations. Corneal crystals formed a characteristic pattern on optical coherence tomography scans.

Confocal microscopy in a case of crystalline keratopathy in a patient with smouldering multiple myeloma

We report the clinical and confocal microscopic findings of the cornea in a patient with smouldering multiple myeloma (SMM) using in vivo scanning laser confocal microscopy. A 72-year-old female underwent a complete ophthalmological examination including slit-lamp biomicroscopy with digital photography, HRT II laser scanning in vivo confocal microscopy and haematological laboratory assessment. Corneal biomicroscopy revealed the presence of bilateral diffuse microgranular tiny grey opacities. In vivo confocal microscopy showed randomly oriented hyper-reflective needle-shaped crystals throughout all levels of the stroma, sparing epithelium and endothelium. In vivo confocal microscopy was very helpful in the differential diagnosis by allowing the nature of the corneal deposits to be established, revealing the typical aspect of the crystals, and excluding granular dystrophy, leading to a suspected diagnosis of SMM. Crystalline corneal deposits may easily be confused as crumb-like opacities typical of granular dystrophy on slit-lamp examination even by experienced ophthalmologists.

Optical coherence tomographic findings of crystal deposits in the lens and cornea in Bietti crystalline corneoretinopathy associated with mutation in the CYP4V2 gene

We report the optical coherence tomography (OCT) findings of crystalline deposits in the cornea and lens of a patient with Bietti crystalline dystrophy (BCD), thus providing evidence for a better understanding of the pathophysiology of BCD. A 49-year-old man showing typical chorioretinal degeneration with a CYP4V2 mutation was diagnosed with BCD. The anterior segment OCT images clearly showed flat hyperreflective plaques just beneath the corneal epithelium and in the lens epithelium. The crystals were not located on the outer surface of the lens capsule as previously described but on the inner surface of the anterior capsule. This finding suggests that the crystals in the lens of patients with BCD may be produced in the same way as corneal or retinal crystalline deposits and therefore result from a systemic abnormality of lipid metabolism rather than by previously considered possibilities, such as release from the retina adhering to the lens capsule.

Localized conjunctival extra-nodal marginal zone B cell lymphoma with presumed paraproteinic crystalline keratopathy

Crystalline corneal deposits have been well reported in individual cases of lymphoproliferative disorders associated with hyper-gammaglobulinemia, hence called 'Crystalline Paraproteinemic Keratopathy'. This is the first report of corneal deposits in a case of localised conjunctival B-cell Lymphoma without paraproteinaemia/hyper-gammaglobulinemia, hence called 'Presumed Paraproteinic Crystalline Keratopathy'.

Decrease in Bilateral Corneal Deposits After Bortezomib/ Dexamethasone Chemotherapy in Monoclonal Gammopathy: Case Report

Purpose: To evaluate changes of bilateral crystalline corneal deposits via biomicroscopic photography and in vivo confocal microscopy (IVCM) after systemic chemotherapy in a patient with monoclonal gammopathy. Methods: Ophthalmological examination, slit-lamp biomicroscopic photography and IVCM of each corneal quadrant were performed before and after 4 cycles of combined bortezomib and dexamethasone chemotherapy. Results: IVCM revealed mainly subepithelial crystalline deposits not affecting keratocytes or antigen presenting cells. After therapy, corneal opacity in slit-lamp biomicroscopic photography and crystalline deposits decreased visibly. Visual acuity remained stable. Subjective symptoms (haziness, photophobia) improved. Conclusions: This patient suffered from initially unknown monoclonal gammopathy manifesting in bilateral corneal deposits. Crystalline corneal deposits may be associated with systemic disorders such as monoclonal gammopathy. Correct diagnosis is crucial as systemic treatment may improve not only ophthalmological symptoms. Penetrating keratoplasty alone may lead to relapse.

Corneal Crystals: A Precursor to Cancer

Corneal crystalline deposits are a sign of a limited number of conditions that can often be differentiated based on the appearance and pattern of the deposits. Although, there are limited treatment options available to reduce the appearance or effects of corneal crystals, it is important to identify the correct etiology of these deposits. There are several associated systemic conditions, some of which are life threatening, for which treatments are available. A 51-year-old black male presented to the clinic with the complaint of "glare at night due to crystals on [the] cornea." Slitlamp findings revealed diffuse subepithelial and anterior stromal crystals across both corneas. The patient had a medical history of renal insufficiency and arthralgia. Through systemic testing, the patient was found to have monoclonal gammopathy of undetermined significance (MGUS), and subsequent follow-up of this patient revealed a conversion of MGUS to multiple myeloma. Corneal crystals can appear diffusely through the cornea secondary to corneal dystrophies, gammopathies, cystinosis, or other rare systemic conditions. In this case report, these crystals are secondary to MGUS, which progressed to multiple myeloma. These crystals consist of immunoglobulin, primarily IgG, and excessive kappa light chains. It is important to obtain laboratory tests and make appropriate referrals to determine whether corneal crystal deposits are associated with a serious systemic disease or merely a corneal dystrophy.

A Case of Multiple Myeloma Presented With Bilateral Corneal Crystalline Deposition

Purpose: Multiple myeloma is characterized by the neoplastic proliferation of a single clone of plasma cells. Multiple myeloma rarely involves the eyeball or the orbital tissues. We report a case of multiple myeloma that presented with corneal crystalline depositions in a patient complaining of decreased vision and irritation of both eyes without any systemic symptoms. Case summary: A 63-year-old woman complained of decreased vision and irritation of both eyes that had started suddenly 20 days before. Uncorrected visual acuity was 0.2 in the right eye and 0.3 in the left eye. Best-corrected visual acuity (BCVA) was 0.9 in the right eye and 1.0 in the left eye. Slit lamp examination showed gray-white crystalline depositions on the epithelium, stroma and Descemet membrane of the cornea extensively, except for the limbus. There were no specific findings on intraocular pressure measurement and fundus examination. The patient did not complain of any systemic symptoms. Multiple myeloma was diagnosed by blood examination and bone marrow biopsy. Two months later, the BCVA decreased to 0.3 in both eyes and artificial tears were prescribed to relieve irritation. Conclusions: When irritation and decreased vision occur abruptly even without systemic symptoms, there is a possibility of multiple myeloma. Systemic evaluations are needed in these cases.

Novel CYP4V2 Gene Mutation in a Mexican Patient with Bietti's Crystalline Corneoretinal Dystrophy

Purpose: To report the clinical and genetic analysis of a Mexican female patient with a sporadic Bietti's crystalline corneoretinal dystrophy. Methods: Ophthalmological examination included best-corrected visual acuity, slit lamp examination, applanation tonometry, fundus photography, fluorescein retinal angiography, Goldmann kinetic perimetry, corneal rotating Scheimpflug imaging, and anterior segment optical coherence tomography (Visante OCT). Genetic analysis included PCR amplification and direct nucleotide sequencing of the entire CYP4V2 gene in DNA from the propositus and her relatives. Results: A late-stage retinal dystrophy was established in the patient. No retinal or corneal crystalline deposits were evident during clinical evaluation. Retrospective analysis of fundus imaging disclosed the presence of retinal crystalline deposits, suggesting the diagnosis of Bietti's crystalline corneoretinal dystrophy. Molecular analysis of the CYP4V2 gene revealed the presence of a novel C to T mutation at nucleotide position 974 (exon 7), predicting a threonine to isoleucine replacement at amino acid position 325. Corneal deposits were not seen by biomicroscopy, corneal OCT, or specular microscopy but were evidenced by means of the corneal rotating Scheimpflug imaging. Conclusion: Our results expand the allelic heterogeneity of Bietti's crystalline corneoretinal dystrophy. This is the first patient of Latin-American origin in which a molecular analysis of the disease has been performed. Our results suggest that the use of corneal rotating Scheimpflug imaging can evidence corneal deposits that are not apparent by other methods.

Central Corneal Mosaic Opacities in Schnyder's Crystalline Dystrophy

To report an unusual presentation of Schnyder's corneal crystalline dystrophy (SCCD), sharing the feature of central corneal mosaic opacities. Observational case report. A 51-year-old man and his family members were examined. Investigations included slit-lamp biomicroscopy, radiography of knee joint, plasma lipid level, and genotyping of the SCCD candidate region in chromosome 1p34.1-1p36. A symmetric, central, disciform, full-thickness opacity was seen in both corneas of the patient. The opacities appeared in a mosaic pattern, instead of collections of crystals or a diffuse haze as typically detected in SCCD. Small clumps of crystalline deposits and arcus lipoides were also observed. Systemically, hyperlipidemia and bilateral genu valgus were identified. He had 2 daughters, and both of them had bilateral corneal crystalline deposits and genu valgus. No other family members had findings suggesting SCCD. The genetic study demonstrated that all of the affected individuals shared a common haplotype within the region of previously reported SCCD locus. However, 1 unaffected sibling of the proband also had the same haplotype. Central corneal mosaic opacities may be another variant of SCCD.

Fine mapping of the Schnyder's crystalline corneal dystrophy locus.

Schnyder's crystalline corneal dystrophy (SCCD) is a rare autosomal dominant eye disease with a spectrum of clinical manifestations that may include bilateral corneal clouding, arcus lipoides, and anterior corneal crystalline cholesterol deposition. We have previously performed a genome-wide linkage analysis on two large Swede-Finn families and mapped the SCCD locus to a 16-cM interval between markers D1S2633 and D1S228 on chromosome 1p36. We have collected 11 additional families from Finland, Germany, Turkey, and USA to narrow the critical region for SCCD. Here, we have used haplotype analysis with densely spaced microsatellite markers in a total of 13 families to refine the candidate interval. A common disease haplotype was observed among the four Swede-Finn families indicating the presence of a founder effect. Recombination results from all 13 families refined the SCCD locus to 2.32 Mbp between markers D1S1160 and D1S1635. Within this interval, identity-by-state was present in all 13 families for two markers D1S244 and D1S3153, further refining the candidate region to 1.58 Mbp.

Morphological evaluation of Schnyder’s central crystalline dystrophy by confocal microscopy before and after phototherapeutic keratectomy

A 29-year-old man with Schnyder’s central crystalline dystrophy was treated with phototherapeutic keratectomy (PTK) in his right eye. Ocular examination revealed abnormal deposits of cholesterol and lipid within the corneal stroma (appearing as crystalline spindle-shaped deposits), high myopia, phakic anterior chamber intraocular lens implantation, and myopic macular degeneration in both eyes. Phototherapeutic keratectomy ablation to a central zone of 7.0 mm and a depth of 96 μm was performed with an Aesculap Meditec MEL-70 excimer laser. Confocal microscopy performed before PTK showed multiple deposits of large, brightly reflective crystalline material extending from the anterior to the mid stroma. The Z-scan curves revealed that the highest density of crystalline deposits was located within the first 140 μm of corneal depth. Six months after PTK, confocal microscopy showed a markedly decreased density of corneal crystalline deposits in the anterior stroma.

Noninfectious crystalline keratopathy after postoperative subconjunctival 5-fluorouracil

PURPOSE: To describe a case of transient, noninfectious crystalline keratopathy after postoperative subconjunctival 5-fluorouracil. METHOD: Case report. Slit-lamp biomicroscopic examinations were performed during serial office examinations. RESULTS: A 69-year-old woman underwent an uncomplicated trabeculectomy with adjunctive sponge 5-fluorouracil application for 5 minutes in her left eye. Postoperative treatment included topical prednisolone acetate, ciprofloxacin, and scopolamine. She was initially examined with intrastromal corneal crystalline deposits 5 days after her first postoperative subconjunctival injection of 5-fluorouracil. The deposits completely disappeared 4 days later without addition to or change in treatment. CONCLUSION: Transient, noninfectious, crystalline, intrastromal corneal deposits may develop after adjunctive subconjunctival 5-fluorouracil administration.

Schnyder Crystalline Dystrophy Sine Crystals: Recommendation for a Revision of Nomenclature

To determine the percentage of patients with Schnyder crystal line dystrophy who had corneal crystal deposition. Thirty-three patients with Schnyder crystalline dystrophy were identified by the author since 1987. Each patient had a complete ophthalmic evaluation, including slit-lamp examination by the author. Only 51% (17 of 33) of patients with Schnyder crystalline corneal dystrophy actually had clinical evidence of corneal crystalline deposits. Because of the confusing nomenclature, many ophthalmologists presume that the presence of corneal crystals is an integral part of the diagnosis of Schnyder crystalline dystrophy. The clinician should be aware that despite the fact that the noncrystalline form of the dystrophy has been poorly recognized, it is equally common.

Eye lesions in pet birds

Amongst eye lesions in birds that died in quarantine, cataracts were the most common disorders (37/241, 15.4%), being prevalent in the annular pads of cockatiels (Nymphicus hollandicus), Amazon parrots (Amazona aestiva aestiva) and budgerigars (Melopsittacus undulatus). The incidence in male birds was more than twice that in females. Deposition of crystals, mostly in the cornea, was the second most frequent lesion (21/293, 8.7%), mainly found in cockatiels, parakeets (Psittacula krameri manillensis), Amazon parrots (Amazona aestiva aestiva), budgerigars and finches (Poephila gouldiae gouldiae). These corneal crystals were negative to PAS and Kossa's stains. Six parakeets (Psittacula krameri manillensis) had calcium salts deposited in the inner plexiform layer of the retina and occasionally in the iris and ciliary body. Neither inflammation nor neo-vascularization was observed when cataracts, corneal crystalline deposition, and retinal and ciliary calcification were present. Intranuclear inclusion bodies typical for papovavirus infection were found in the eyelids of six budgerigars (2.5%). Similar inclusions were simultaneously found in the pars ciliaris retinae (4, 1.7%), inner plexiform of retina (1, 0.4%) and anterior epithelium of the cornea (1, 0.4%). Other lesions such as candidial endophthalmitis, conjunctival cryptosporidiosis, corneal dystrophy, keratitis, corneal perforation and iridocyclitis, were occasional findings.