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120 results
  • Ectatic diseases. [Journal Article]
    Exp Eye Res. 2020 Dec 01 [Online ahead of print]Salomão MQ, Hofling-Lima AL, … Ambrósio R
  • Ectatic corneal disease (ECD) comprises a group of disorders characterized by progressive thinning and subsequent bulging of the corneal structure. Different phenotypes have been recognized, including keratoglobus, pellucid marginal degeneration (PMD), and keratoconus (KC). Keratoconus has been widely investigated throughout the years, but the advent of laser refractive surgery boosted an immedia…
  • Lamellar Keratoplasty for Advanced Keratoconus. [Journal Article]
    Asia Pac J Ophthalmol (Phila). 2020 Dec; 9(6):580-588.Patil M, Mehta JS
  • Ectatic corneal diseases are a group of disorders resulting in progressive corneal steepening and thinning. Keratoconus, keratoglobus, pellucid marginal degeneration, post-keratorefractive ectasia, and corneal graft ectasia are the types of corneal ectasia. Early diagnosis with appropriate diagnostic tools can help prevent progression, eliminating the need for corneal transplantation. Lamellar or…
  • Hypermature Intumescent Cataract in Advanced Keratoglobus. [Case Reports]
    Int Med Case Rep J. 2020; 13:507-511.Singh B, Sharma S, … Bharti S
  • We report a rare case of bilateral keratoglobus with hypermature intumescent cataract in a 55-year-old woman. Clinical examination and corneal topography confirmed generalized corneal bulging and global corneal thinning. A Pentacam® (Oculus Optikgerate, Wetzlar, Germany) demonstrated bilateral diffuse corneal thinning (368 μm in the right eye and 371 μm in the left eye). Phacoemulsification was p…
  • [Ocular manifestations in Ehlers-Danlos Syndromes: Clinical study of 21 patients]. [Journal Article]
    J Fr Ophtalmol. 2019 Sep; 42(7):722-729.Perez-Roustit S, Nguyen DT, … Bremond-Gignac D
  • CONCLUSIONS: In this study, the main ophthalmological sign was convergence insufficiency present in more than 60% of the patients. This highlights the importance of an orthoptic examination in patients with Ehlers-Danlos syndrome. Dry eye syndrome with tear film instability was frequent, even though the patients were young. Blue sclera was seen in 38% of the patients. We reported two patients with high myopia and one patient with keratoglobus in our cohort. No patients presented with angioid streaks, and mean pachymetry was normal in our series.An ophthalmological and orthoptic evaluation should be performed in all patients with Ehlers-Danlos syndrome to detect and treat ocular manifestations. If Ehlers-Danlos syndrome is suspected, ophthalmological examination can also provide support for the diagnosis.
  • Pellucid marginal degeneration: Detection, discrimination from other corneal ectatic disorders and progression. [Review]
    Cont Lens Anterior Eye. 2019 08; 42(4):341-349.Martínez-Abad A, Piñero DP
  • Pellucid marginal degeneration (PMD) is a non-inflammatory ectatic corneal disease characterized by a narrow band of corneal thinning separated from the limbus by a relatively uninvolved area 1-2 mm in width. It is a rare corneal disorder that shares many clinical characteristics with other corneal ectasias, such as keratoconus, keratoglobus or Terrien marginal degeneration. PMD usually starts la…
  • [Brittle cornea syndrome type 1 caused by compound heterozygosity of two mutations in the ZNF469 gene]. [Case Reports]
    Ophthalmologe. 2019 Aug; 116(8):780-784.Menzel-Severing J, Meiller R, … Atalay D
  • We report the case of a 3-year-old boy presenting with bilateral keratoglobus and blue sclera in addition to hallux valgus, arachnodactyly, small joint hypermobility, mitral valve dysfunction and a history of generalized muscular hypotonia in early infancy. Molecular genetics provided evidence of two pathogenic mutations in the ZNF469 gene (compound heterozygosity) leading to the diagnosis of bri…
  • Ophthalmic findings in patients with arterial tortuosity syndrome and carriers: A case series. [Case Reports]
    Ophthalmic Genet. 2018 Jan-Feb; 39(1):29-34.Hardin JS, Zarate YA, … Warner DB
  • CONCLUSIONS: We document a spectrum of ophthalmic manifestations of ATS with universal findings of myopia, corneal thinning, and a propensity for corneal ectasia leading to keratoconus or keratoglobus. Heterozygous carriers may develop keratectasia after corneal refractive surgery. Our data support regular eye examinations for all patients carrying SLC2A10 mutations with follow-up tailored to clinical findings.
  • Congenital keratoglobus with blue sclera in two siblings with overlapping Marshall/Stickler phenotype. [Case Reports]
    Indian J Ophthalmol. 2016 Nov; 64(11):856-859.Imamoglu S, Kaya V, … Gok K
  • We aimed to describe congenital keratoglobus with blue sclera in two siblings with overlapping Marshall/Stickler phenotype. Two sisters (ages four and six) with bilateral high astigmatism were evaluated by slit-lamp microscopy. Corneal topography and pachymetry maps were also obtained. Slit-lamp examination revealed that both corneas were globular in shape with peripheral corneal thinning. Pachym…
  • Unusual case of globe perforation: the brittle cornea without systemic manifestations. [Case Reports]
    BMJ Case Rep. 2016 Oct 07; 2016Joshi SA, Uppapalli S, … Deshpande M
  • Brittle cornea syndrome is a rare generalised connective tissue disorder with ocular features like keratoglobus or keratoconus, severe corneal thinning and a high risk of perforation. Various authors in different case reports and case series have brought out the fact that brittle cornea is a disorder with characteristic systemic manifestations such as deafness, joint hypermobility, hyperelasticit…
  • Presumptive keratoglobus in a great horned owl (Bubo virginianus). [Case Reports]
    Vet Ophthalmol. 2017 Nov; 20(6):560-567.Lau RK, Moresco A, … Freeman KS
  • A juvenile to young adult, male, great horned owl (Bubo virginianus,GHOW) was presented to the wildlife rehabilitation hospital at Lindsay Wildlife Museum (WRHLWM) due to trauma to the right patagium from barbed wire entanglement. On presentation, both corneas were irregular, dry, and no movement of the third eyelid was noted. A severe corneal enlargement/globoid appearance was the predominant op…
  • Keratoglobus: a close entity to megalophthalmos. [Journal Article]
    Springerplus. 2016; 5:634.Gupta N, Ganger A
  • CONCLUSIONS: Keratoglobus, Megalophthalmos and Buphthalmos are exceedingly close entities and it is very essential to make correct diagnosis, as management options differ significantly for all three diseases.
  • New Technique of Exposed Glaucoma Drainage Tube Repair: Report of a Case. [Case Reports]
    J Curr Glaucoma Pract. 2015 May-Aug; 9(2):62-4.Berezina TL, Fechtner RD, … Chu DS
  • We present the case of successful repair of an exposed glaucoma drainage tube by cornea graft fixation with tissue adhesive, and without subsequent coverage by adjacent conjunctiva or donor tissues. Patient with history of keratoglobus with thin cornea and sclera, and phthisical contralateral eye, underwent three unsuccessful corneal grafts followed by Boston type 1 keratoprosthesis in the right …
  • Management of advanced corneal ectasias. [Review]
    Br J Ophthalmol. 2016 Jan; 100(1):34-40.Maharana PK, Dubey A, … Vajpayee RB
  • Corneal ectasias include a group of disorders characterised by progressive thinning, bulging and distortion of the cornea. Keratoconus is the most common disease in this group. Other manifestations include pellucid marginal degeneration, Terrien's marginal degeneration, keratoglobus and ectasias following surgery. Advanced ectasias usually present with loss of vision due to high irregular astigma…
  • Keratoglobus: An experience at a tertiary eye care center in India. [Journal Article]
    Indian J Ophthalmol. 2015 Mar; 63(3):233-8.Rathi VM, Murthy SI, … Sangwan VS
  • CONCLUSIONS: About 50% of pediatric eyes (65% having blue sclera) had no functional vision. Trivial trauma was responsible for corneal rupture indicating need for protective glasses. About 50% patients had post-PK glaucoma though grafts were clear.
  • Use of a novel lamellar keratoplasty with pleat technique to address the abnormal white-to-white diameter in keratoglobus. [Case Reports]
    Cornea. 2015 Feb; 34(2):239-42.Lockington D, Ramaesh K
  • CONCLUSIONS: We have demonstrated a modified anterior lamellar keratoplasty with pleat technique to achieve satisfactory visual rehabilitation in extreme keratoglobus. This pleat technique addresses the fundamental structural problem of a vertically displaced limbus in keratoglobus. We highlight the optical illusion of the white-to-white diameter in keratoglobus. We also recommend the baking analogy of muffin to cupcake as a communication aid when explaining the mechanics of surgery to the patient.
  • Pediatric genetic disease of the cornea. [Journal Article]
    J Pediatr Genet. 2014 Dec; 3(4):195-207.Fecarotta CM, Huang WW
  • Our objective is to evaluate the literature regarding selected genetic diseases of the cornea, including megalocornea, keratoglobus, keratoconus, cystinosis, the mucopolysaccharidoses, sclerocornea, Peters' anomaly, familial dysautonomia, and various corneal dystrophies. The transparency of the cornea is a consequence of uniformity in both size and spacing of the collagen lamellae. The cornea's c…
  • Congenital keratoglobus with multiple cardiac anomalies: a case presentation and literature review. [Case Reports]
    Semin Ophthalmol. 2015 Jul; 30(4):305-12.Ozer PA, Yalniz-Akkaya Z
  • Keratoglobus is a rare condition of bilateral corneal ectasia, which results in high myopia, irregular astigmatism, scarring, and rarely spontaneous globe rupture. Globoid protrusion of a clear, diffusely thin cornea is the pathology. The congenital form has been associated with blue sclera in which there is a systemic connective tissue disorder with abnormal collagen synthesis like Ehlers-Danlos…
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