- Ectatic diseases. [Journal Article]Exp Eye Res. 2020 Dec 01 [Online ahead of print]EE
- 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…
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- Lamellar Keratoplasty for Advanced Keratoconus. [Journal Article]Asia Pac J Ophthalmol (Phila). 2020 Dec; 9(6):580-588.AP
- 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…
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- Hypermature Intumescent Cataract in Advanced Keratoglobus. [Case Reports]Int Med Case Rep J. 2020; 13:507-511.IM
- 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…
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- Keratoglobus: Ancillary testing for differential diagnosis. [Journal Article]J Fr Ophtalmol. 2020 Oct 24 [Online ahead of print]JF
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- Total or subtotal full thickness recipient bed cut to repair donor-recipient curvature disparity in cases of DM rupture with manual DALK. [Case Reports]Eur J Ophthalmol. 2020 Sep; 30(5):1172-1178.EJ
- CONCLUSIONS: The existence of a donor-recipient curvature disparity should be investigated as a possible underlying mechanism of refractory double AC. Total or subtotal full thickness recipient bed cut may be considered to repair donor-recipient curvature disparity in cases of DM rupture occurring during manual DALK. Repairing the DM rupture and avoiding a conversion to PK in high-risk transplant cases are crucial.
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- Descemet stripping automated endothelial keratoplasty: An alternate surgical modality for Descemet's membrane detachment following hydrops in keratoglobus. [Journal Article]Indian J Ophthalmol. 2020 03; 68(3):513-514.IJ
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- [Surgical treatment of keratoglobus: key techniques of bridge-shaped flap penetrating keratoplasty and whole lamellar keratoplasty with corneoscleral limbal]. [Journal Article]Zhonghua Yan Ke Za Zhi. 2019 Dec 11; 55(12):916-922.ZY
- CONCLUSIONS: The whole lamellar keratoplasty with corneoscleral limbal could effectively reinforce corneal, avoid corneal penetrating injury caused by minor trauma, and improve visual acuity in keratoglobus whose corneas were gradually thinning and corrected vision cannot be improved. The patients with corneal perforating injury or descemet's membrane detachment could obtain good visual prognosis receiving bridge-shaped flap penetrating keratoplasty. (Chin J Ophthalmol, 2019, 55:916-922).
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- Treatment of Acute Corneal Hydrops With Combined Intracameral Gas and Approximation Sutures in Patients With Corneal Ectasia. [Journal Article]Cornea. 2020 Feb; 39(2):258-262.C
- CONCLUSIONS: Combined intracameral gas injection and approximation sutures are probably effective and safe for the treatment of acute CH. This treatment results in rapid recovery with very rare complications.
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- Keratoglobus with ARCL1B (EFEMP2 gene) cutis laxa. [Case Reports]Am J Ophthalmol Case Rep. 2019 Sep; 15:100477.AJ
- CONCLUSIONS: We believe that this is the first reported case of keratoglobus associated with cutis laxa.
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- Prosthetic replacement of the ocular surface ecosystem for corneal irregularity: Visual improvement and optical device characteristics. [Journal Article]Cont Lens Anterior Eye. 2019 10; 42(5):526-532.CL
- CONCLUSIONS: The PROSE device resulted in significant improvement in vision in this population. A trend of prescribing higher lens vault with increased keratometry value was evident especially in keratoconus. ACD appeared to be more important than Steep K in vault selection. An FSE of 0.6 and vertical peripheral lens toricity were used most frequently in this subset of patients.
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- [Ocular manifestations in Ehlers-Danlos Syndromes: Clinical study of 21 patients]. [Journal Article]J Fr Ophtalmol. 2019 Sep; 42(7):722-729.JF
- 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.
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- Pellucid marginal degeneration: Detection, discrimination from other corneal ectatic disorders and progression. [Review]Cont Lens Anterior Eye. 2019 08; 42(4):341-349.CL
- 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…
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- [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.O
- 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…
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- Trans-scleral explantation of posteriorly dislocated IOL-CTR complex in a case of anterior megalophthalmos with an unusually thin cornea. [Case Reports]BMJ Case Rep. 2018 Jul 19; 2018BC
- Anterior megalophthalmos and keratoglobus are characterised by corneal thinning and deep anterior chamber. They are clinically distinguished on the basis of normal to slightly decreased corneal thickness with a large corneal diameter (>13 mm) in case of anterior megalophthalmos, and marked limbus-to-limbus corneal thinning with globular protrusion in keratoglobus. To achieve and maintain a centre…
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- Posterior Amorphous Corneal Dystrophy Associated With Keratoglobus: A Case Report. [Case Reports]Cornea. 2017 Dec; 36(12):1562-1566.C
- CONCLUSIONS: In this case report, we illustrate progressive corneal ectasia in a patient with PACD. Although both conditions may represent changes in the structure and integrity of corneal collagen, whether an association exists between the 2 conditions is unknown.
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- Ophthalmic findings in patients with arterial tortuosity syndrome and carriers: A case series. [Case Reports]Ophthalmic Genet. 2018 Jan-Feb; 39(1):29-34.OG
- 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.
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- Femtosecond Laser-Assisted Tuck-In Penetrating Keratoplasty for Advanced Keratoglobus With Endothelial Damage. [Case Reports]Cornea. 2017 Sep; 36(9):1145-1149.C
- CONCLUSIONS: Femtosecond laser-assisted tuck-in penetrating keratoplasty can provide excellent anatomical and functional rehabilitation of patients with severe KTG and endothelial damage, through a single-step surgical procedure and a single donor cornea per eye. The femtosecond laser permits accurate dissection of these already thin corneas without inadvertent perforation risk.
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- Limbal Stem Cell-Sparing Corneoscleroplasty with Peripheral Intralamellar Tuck: A New Surgical Technique for Keratoglobus. [Case Reports]
- CONCLUSIONS: Limbal stem cell-sparing corneoscleroplasty is a single-step technique for restoring the structural integrity of the cornea in advanced keratoglobus while preserving the host limbal stem cells.
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- Congenital keratoglobus with blue sclera in two siblings with overlapping Marshall/Stickler phenotype. [Case Reports]
- 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…
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- Unusual case of globe perforation: the brittle cornea without systemic manifestations. [Case Reports]
- 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…
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- Presumptive keratoglobus in a great horned owl (Bubo virginianus). [Case Reports]Vet Ophthalmol. 2017 Nov; 20(6):560-567.VO
- 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…
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- Keratoglobus: a close entity to megalophthalmos. [Journal Article]
- 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.
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- Identification of Mutations in the PRDM5 Gene in Brittle Cornea Syndrome. [Case Reports]
- CONCLUSIONS: The current study revealed a novel mutation in the PRDM5 gene in a BCS family and recurrent mutation in a sporadic BCS patient. A variant in the SEC24D gene also segregated in the BCS family, although its role in the disease remains unclear.
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- New Technique of Exposed Glaucoma Drainage Tube Repair: Report of a Case. [Case Reports]
- 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 …
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- Management of advanced corneal ectasias. [Review]
- 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…
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- Keratoglobus: An experience at a tertiary eye care center in India. [Journal Article]
- 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.
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- Use of a novel lamellar keratoplasty with pleat technique to address the abnormal white-to-white diameter in keratoglobus. [Case Reports]
- 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.
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- Pediatric genetic disease of the cornea. [Journal Article]J Pediatr Genet. 2014 Dec; 3(4):195-207.JP
- 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…
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- An ocular surface prosthesis for keratoglobus and Terrien's marginal degeneration. [Case Reports]
- CONCLUSIONS: Modification of not only the vault but also the haptic and total diameter of the device is required to achieve an optimal fit. Though challenging, successful fitting of the scleral lens in ectatic corneas is attainable, with the aid of anterior imaging and spline technologies.
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- Congenital keratoglobus with multiple cardiac anomalies: a case presentation and literature review. [Case Reports]Semin Ophthalmol. 2015 Jul; 30(4):305-12.SO
- 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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