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Shilpa Ajit Joshi, Shalomith Uppapalli, Pranav More, Madan Deshpande
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, hyperelasticity of skin, kyphoscoliosis and dental abnormalities alongwith ophthalmic features. We report a case of globe perforation following trivial trauma, in an individual with brittle cornea without any extraocular manifestations, posing a challenge in the diagnosis and dilemma in surgical repair of cornea, restoration of globe integrity and visual rehabilitation...
October 7, 2016: BMJ Case Reports
Rachael K Lau, Anneke Moresco, Sarah J Woods, Christopher M Reilly, Michelle G Hawkins, Christopher J Murphy, Steven R Hollingsworth, Dennis Hacker, Kate S Freeman
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 ophthalmic feature. The fundus was normal in both eyes (OU). Over the course of several days, both corneas developed edema combined with further dessication at the ocular surface associated with diffuse dorsal fluorescein stain uptake...
July 31, 2016: Veterinary Ophthalmology
Noopur Gupta, Anita Ganger
BACKGROUND: Keratoglobus closely resembles buphthalmos and anterior megalophthalmos. FINDINGS: A 45-year-old man presented with gradually progressive, painless, diminution of vision in both eyes since childhood. On examination, visual acuity of right (RE) and left eye (LE) was 20/60 and 2/20 respectively. Clinical pictures of the patient are shown in panel A, B, C, D. Keratometry values were 46.47/47.94 D at 42/132° in RE and 46.90/47.23 D at 174/84° in LE, signifying steep, ectatic cornea...
2016: SpringerPlus
Shazia Micheal, Muhammad Imran Khan, Farrah Islam, Farah Akhtar, Raheel Qamar, Marie-José Tassignon, Bart Loeys, Anneke I den Hollander
BACKGROUND: Brittle cornea syndrome (BCS) is a rare autosomal recessive connective tissue disease characterized by variable combinations of corneal thinning and fragility, corneal ruptures either spontaneously or after minor trauma, blue sclerae, keratoconus, keratoglobus, and high myopia. So far, mutations in 2 genes, PRDM5 and ZNF469, have been associated with BCS. The purpose of this study is to describe novel mutations in the PRDM5 gene in patients with BCS. METHODS AND RESULTS: Using homozygosity mapping with single-nucleotide polymorphism markers followed by whole-exome sequencing, we identified a novel homozygous splice site variant (c...
June 2016: Cornea
Tamara L Berezina, Robert D Fechtner, Amir Cohen, Eliott E Kim, David S Chu
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 eye. Ahmed drainage device with sclera patch graft was implanted to control the intraocular pressure. Two years later the tube eroded through sclera graft and conjunctiva...
May 2015: Journal of Current Glaucoma Practice
Prafulla K Maharana, Aditi Dubey, Vishal Jhanji, Namrata Sharma, Sujata Das, Rasik B Vajpayee
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 astigmatism. Management of these disorders is difficult due to the peripheral location of ectasia and associated severe corneal thinning...
January 2016: British Journal of Ophthalmology
Varsha M Rathi, Somasheila I Murthy, Bhupesh Bagga, Mukesh Taneja, Sunita Chaurasia, Virender S Sangwan
CONTEXT: This study was carried out as a part of an internal audit and is the largest series of patients having keratoglobus, published in the literature. Poor visual acuity of the patients indicates the blinding nature of the disease. AIMS: We report our experience with patients having keratoglobus at a tertiary eye care center in India. SETTINGS AND DESIGN: Retrospective study. MATERIALS AND METHODS: We analyzed adults and pediatric patients (<16 years) with keratoglobus, seen during 2008-2012...
March 2015: Indian Journal of Ophthalmology
David Lockington, Kanna Ramaesh
PURPOSE: We describe a novel surgical technique that seeks to address the fundamental mechanical problem of the superiorly and vertically displaced limbus and the corresponding abnormal corneal white-to-white diameter in keratoglobus. METHOD: A 42-year-old woman had progressive reduction of vision due to hydrops of her right cornea with secondary peripheral corneal perforation due to progressive bilateral keratoglobus. Her visual acuity was hand movements in the right eye, with an uncorrectable refractive error...
February 2015: Cornea
Rajeswari Mahadevan, Asra Fathima, Rajni Rajan, Amudha Oli Arumugam
PURPOSE: This case report describes the challenges in fitting corneas using the prosthetic replacement of ocular surface ecosystem (PROSE) device in a unique case of bilateral keratoglobus (KG) with Terrien's marginal degeneration (TMD). CASE REPORT: KG and TMD are uncommon corneal ectatic conditions, characterized by protrusion and thinning of the cornea. Optical correction with spectacles is limited, as this may not provide the best-corrected visual acuity because of irregular corneal astigmatism...
April 2014: Optometry and Vision Science: Official Publication of the American Academy of Optometry
Pinar A Ozer, Zuleyha Yalniz-Akkaya
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 syndrome, Marfan syndrome, and osteogenesis imperfecta. Some concomitant abnormalities reported with kertoglobus include joint hypermobility, dental and skeletal abnormalities, osteal fragility, and deafness...
July 2015: Seminars in Ophthalmology
Farid Karimian, Alireza Baradaran-Rafii, Amir Faramarzi, Mitra Akbari
PURPOSE: To describe the technique and outcomes of limbal stem cell-sparing lamellar keratoplasty (LSCS-LKP) for the management of advanced keratoglobus (KGB). METHODS: In a sequential interventional case series from March 2010 to December 2012, 8 eyes of 6 patients with advanced KGB underwent an LSCS-LKP. Three patients had isolated KGB, 2 were affected with the Ehlers-Danlos syndrome, and the other subject had osteogenesis imperfecta. Epithelial healing, anatomical results (corneal thickness and keratometry), and visual outcomes were evaluated after this intervention...
January 2014: Cornea
Ilya Ortenberg, Shmuel Behrman, Wasim Geraisy, Irina S Barequet
OBJECTIVES: To evaluate the visual correction and clinical performance with scleral contact lenses (CL) for the visual rehabilitation of irregular astigmatism and to report the effect of brief wearing breaks on the wearing time and success rate. METHODS: A retrospective review was performed on consecutive patients who were fitted with scleral CL because of irregular astigmatism following failure of other optical corrections. Visual acuity (VA) and wearing times were abstracted...
November 2013: Eye & Contact Lens
B S Wallang, S Das
Keratoglobus is a rare noninflammatory corneal thinning disorder characterised by generalised thinning and globular protrusion of the cornea. It was first described as a separate clinical entity by Verrey in 1947. Both congenital and acquired forms have been shown to occur, and may be associated with various other ocular and systemic syndromes including the connective tissue disorders. Similarities have been found with other noninflammatory thinning disorders like keratoconus that has given rise to hypotheses about the aetiopathogenesis...
September 2013: Eye
Anton M Kolomeyer, David S Chu
Purpose. To report the use of Descemet stripping endothelial keratoplasty (DSEK) in a patient with keratoglobus and chronic hydrops. Case Report. We describe a case of a 28-year-old man with bilateral keratoglobus and chronic hydrops in the right eye secondary to spontaneous Descemet membrane tear. The patient presented with finger counting (CF) vision, itching, foreign body sensation, and severe photophobia in the right eye. Peripheral corneal thinning with central corneal protrusion and Descemet membrane tear spanning from 4 to 7 o'clock was noted on slit lamp examination...
2013: Case Reports in Ophthalmological Medicine
Marianne Rohrbach, Helen L Spencer, Louise F Porter, Emma M M Burkitt-Wright, Céline Bürer, Andreas Janecke, Madhura Bakshi, David Sillence, Hailah Al-Hussain, Matthias Baumgartner, Beat Steinmann, Graeme C M Black, Forbes D C Manson, Cecilia Giunta
Brittle cornea syndrome (BCS; MIM 229200) is an autosomal recessive generalized connective tissue disorder caused by mutations in ZNF469 and PRDM5. It is characterized by extreme thinning and fragility of the cornea that may rupture in the absence of significant trauma leading to blindness. Keratoconus or keratoglobus, high myopia, blue sclerae, hyperelasticity of the skin without excessive fragility, and hypermobility of the small joints are additional features of BCS. Transcriptional regulation of extracellular matrix components, particularly of fibrillar collagens, by PRDM5 and ZNF469 suggests that they might be part of the same pathway, the disruption of which is likely to cause the features of BCS...
July 2013: Molecular Genetics and Metabolism
Sotiria Palioura, James Chodosh, Roberto Pineda
PURPOSE: To report a case of corneal hydrops in a patient with keratoglobus that was managed with endothelial keratoplasty to achieve corneal stability and prevent a limbus-to-limbus tear in Descemet membrane. METHODS: A 30-year-old man with keratoglobus presented with corneal hydrops in his left eye resulting from a central vertical tear in Descemet membrane. His other eye had been previously treated with penetrating keratoplasty using a large graft (an 11-mm donor graft to a 10-mm recipient bed) because of a limbus-to-limbus tear in Descemet membrane without resolution of his edema...
March 2013: Cornea
Stephan Riss, Ludwig M Heindl, Bjoern O Bachmann, Friedrich E Kruse, Claus Cursiefen
PURPOSE: To describe the clinical results of Pentacam-based big bubble deep anterior lamellar keratoplasty (DALK) to achieve an intended 90% depth of initial lamellar trephination. METHODS: Fifty consecutive eyes of 50 patients with keratoconus, keratoglobus, and anterior stromal scars were included. DALK was performed with the big bubble technique using a 90% intended depth for initial lamellar trephination based on preoperative pachymetry by Pentacam. Main outcome measures were success of surgery, best spectacle-corrected visual acuity, endothelial cell count, refractive astigmatism at 12-month follow-up, and rate of intra- and postoperative complications...
June 2012: Cornea
S Hasler, J Stürmer, C Kaufmann
No abstract text is available yet for this article.
April 2011: Klinische Monatsblätter Für Augenheilkunde
Sayan Basu, Pravin K Vaddavalli, Muralidhar Ramappa, Sushmita Shah, Somasheila I Murthy, Virender S Sangwan
PURPOSE: This study is aimed to evaluate the role of 14% nonexpansile concentration of perfluoropropane (C(3)F(8)) gas in the management of acute hydrops in corneal ectasias. DESIGN: Retrospective, nonrandomized, comparative, interventional case series. PARTICIPANTS AND CONTROLS: The study group consisted of 62 eyes of 57 patients and the control group included 90 eyes of 82 patients with acute corneal hydrops who presented within 30 days of onset of symptoms...
May 2011: Ophthalmology
Sangita P Patel, Manoj M Sajnani, Roberto Pineda
A 13-year-old boy presented with gradually progressive deterioration of vision in both eyes, bilateral photophobia, and regular headaches. Clinical examination, anterior segment findings, and specular microscopy findings were consistent with the diagnosis of posterior polymorphous dystrophy and keratoglobus. To the authors' knowledge, this is the first pediatric case and the second case overall of the simultaneous occurrence of posterior polymorphous dystrophy and keratoglobus.
2011: Journal of Pediatric Ophthalmology and Strabismus
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