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Keratoglobus
Isolated Congenital Megalocornea

Presentation

Presenting Author: Dr. Saudamini Jadhav 2nd year resident, MVPS Dr.Vasantrao Pawar Medical College, Nashik Guide: Dr. Mrunal Patil Professor & Dean, MVPS Dr.Vasantrao Pawar Medical College, Nashik Co-Author: Dr. [slideshare.net]

This patient presented with corneal steepening on topography consistent with keratoglobus, as well as large areas of irregular polymorphous changes of the corneal endothelium on specular microscopy consistent with PPMD. [ncbi.nlm.nih.gov]

This case is presented to highlight the need for close follow-up and early intervention in keratoglobus to prevent sight-threatening complications. [ijoreports.in]

Vernal keratoconjunctivitis was present in one pediatric patient. Choroidal osteoma, retinitis pigmentosa, and retinal detachment were present in adults. [doaj.org]

Entire Body System

  • Surgical Procedure

    To describe the outcomes of femtosecond laser-assisted tuck-in penetrating keratoplasty as a single-step surgical procedure for visual and anatomical rehabilitation of patients with severe keratoglobus (KTG) and endothelial damage. [ncbi.nlm.nih.gov]

    Surgical procedures to treat keratoglobus included large-diameter lamellar and penetrating keratoplasty, and limbus-to-limbus epikeratoplasty. © Lippincott-Raven Publishers. Related Articles [journals.lww.com]

    […] reconstructive surgical procedures. [books.google.com]

  • Weakness

    The patient is at risk for globe perforation because the thinned out cornea is extremely weak. Surgical treatment Further progression of the disease usually leads to a need for surgery because of extreme thinning of the cornea. [wikidoc.org]

    The patient is at risk for globe perforation because the thinned out cornea is extremely weak. [ipfs.io]

    The mechanism of production of this globular corneal contour is not totally clear, but wound weakness in the area of the grafthost junction could be a possible cause. [ncbi.nlm.nih.gov]

  • Falling

    Klin Monbl Augenheilkd 2011; 228(4): 345-346 DOI: 10.1055/s-0031-1273225 Der interessante Fall © Georg Thieme Verlag KG Stuttgart · New York Keratoglobus und tief-stromale Hornhauttrübung in einem Fall von Arterial Tortuosity Syndrome Further Information [thieme-connect.com]

    Falling vision may not be corrected by glasses due to irregular astigmatism. Contact lenses (rigid gas permiable) usually improve the vision in early cases. In later stages penetrating keratoplasty may be required. [ophthalmologylife.blogspot.com]

    This talk will go over the rationale for and advances in visual skills training for learning, sports performance, TBI, and risk management in the areas of falls prevention, driving safety and law enforcement. [eye.conferenceseries.com]

    Bahn D, Falls H, Yarley G et al: Classification of corneal endothelial disorders based on neural crest origin. Ophthalmology 91:558, 1984. 14. Pe'er J, BenEzra D, Sela M et al: Cryptophthalmos syndrome. Clinical and histopathologic findings. [oculist.net]

Jaw & Teeth

  • Xerostomia

    In Sjögren’s syndrome, the immune system, not recognizing its own cells, tissues and organs, mainly attacks the exocrine glands (salivary, lacrimal) destroying them and creating considerable disorders of dry mouth (xerostomia) and dry eye (keratum dry [occhialhouse.it]

Skin

  • Blister

    Swelling also causes painful blisters on the epithelial layer. Corneal transplant may be needed to restore vision. Lattice dystrophy - involves an accumulation of amyloid deposits in the stroma. [patient.info]

Eyes

  • Blue Sclera

    Marshall-Stickler syndrome may exist in the differential diagnosis of keratoglobus with blue sclera. [ncbi.nlm.nih.gov]

    Visual acuity ranging from counting of fingers to no light perception was noted in 20/53 (37.74%) adults and 21/42 (50%) pediatric patients; 13/20 (65%) with blue sclera and 8/22 eyes (36.37%) without blue sclera. [doaj.org]

  • Microphakia

    […] congenital heart defects, joint hypermobility, obesity Lacrimal duct problems, high myopia, nystagmus, pupillary abnormalities, microcornea/microphthalmia, corneal abnormalities, congenital glaucoma, iris atrophy, congenital cataract, ectopia lentis, microphakia [ncbi.nlm.nih.gov]

  • Iridodonesis

    Iridodonesis was noted in left eye. Retinal evaluation of LE revealed retinal detachment (RD) with posterior staphyloma due to high myopia, hereas RE was within normal limits. Intraocular pressure was normal in both eyes. [mendeley.com]

    Associated findings include anterior megalophthalmos, enlarged ciliary ring with zonular stretching (leading to phacodonesis), ectopia lentis, iridodonesis, iris stromal hypoplasia, posterior embryotoxon, transillumination defects, Krukenberg spindles [medical-dictionary.thefreedictionary.com]

    Other ocular abnormalities associated with megalophthalmos anterior include iridodonesis, iris stromal hypoplasia, phacodonesis, ectopia lentis, cataracts, and pigmentary glaucoma with Krukenberg spindles (Vail, 1931; Neumann, 1984; Javadi, 2000; Chien-Kuang [webeye.ophth.uiowa.edu]

  • Night Blindness

    These small, white-yellow chalky concretions of the conjunctiva around the limbus are known today as the Horner-Trantas spots or Trantas dots.21 He also re-established the Hippocratic treatment of night blindness and in 1895 described an operation for [docksci.com]

Neurologic

  • Irritability

    Inflammation is the first response of the immune system to infection or irritation and may be referred to as the Innate immune system and as healthy nor unhealthy on its own: Inflammation helps fight disease, but it comes at the cost of suspending the [indiahospitaltour.com]

    Health News and Articles Comprehensive information about Diabetes, depression, weight management, osteoarthritis and more from MerckSource.com Health Resource Library - Information about Irritable Bowel Syndrome (IBS), high blood pressure, osteoporosis [web.archive.org]

    Lubricants reduce the redness and provide relief from the chronic irritation. [patient.info]

    Corneal irritation is not accompanied by activation of the corneal reflex. In the later stages, the friction of eyelashes on the cornea leads to increased lacrimation. [medic-journal.com]

    Ten days later, the patient complained of pain, irritation, and tearing. Vision decreased to 20/200 with no pinhole improvement. Conjunctival injection with corneal bullae was noted. [hindawi.com]

Workup

Bilateral cases are often associated with systemic disorders and warrant a complete genetic workup. [entokey.com]

NOTE: Children should be referred for an extensive developmental workup Differential Diagnosis Simple megalocornea Megalophthalmos anterior Congenital glaucoma Keratoglobus High myopia References Basti S and Koch DD. [webeye.ophth.uiowa.edu]

Treatment

Intracameral injection of C(3)F(8) may possibly be a viable treatment option for acute hydrops in a case of corneal ectasia. [ncbi.nlm.nih.gov]

Our current treatment method for patients who present with acute corneal hydrops can be seen in the sidebar, Acute Corneal Hydrops Treatment. [crstoday.com]

Nonetheless, there is no clear consensus on the surgical treatment for a PAA. [tgkdc.dergisi.org]

Treatment Treatment Options: Treatment beyond corneal repair is limited. References [disorders.eyes.arizona.edu]

Prognosis

Prognosis: The prognosis for penetrating keratoplasty in treating kerato-conus is good because the cornea is avascular in keratoconus. [brainkart.com]

Prognosis and prevention Specific measures for the prevention of keratoglobus have not been developed, since in most cases this pathology is congenital. [medic-journal.com]

Prognosis often depends on the extent of opacity and disorganization of the anterior segment. Corneal transplantation has been performed for these disorders with variable success. [content.iospress.com]

Prognosis Keratoglobus continues to be a somewhat mysterious disease, but it can be successfully managed with a variety of clinical and surgical techniques. [wikidoc.org]

Etiology

The etiology and pathogenesis of these disorders are unknown but may be associated with a variety of factors, including contact lens wear, eye rubbing, Down's syndrome, atopic disease, connective tissue disease, tapetoretinal degeneration and inheritance [scholars.northwestern.edu]

We aimed to discuss the etiology and the association of keratoglobus with some systemic abnormalities caused by collogen tissue disturbance, and make a brief review about the recent literature concerning the management of keratoglobus patients. [ncbi.nlm.nih.gov]

The etiology is unknown. [eyewiki.aao.org]

Epidemiology

Beginning with basic science, examination techniques and epidemiology, the following chapters discuss the diagnosis and the medical and surgical treatment of numerous different conditions and diseases that may affect the cornea. [books.google.ro]

Epidemiology: Keratoconus is the mostfrequently encountereddeformationof the cornea. Occurrence is familial, although women are more likely to be affected than men. Etiology: Keratoconus is probably a genetic disorder. [brainkart.com]

Epidemiology It is a much rarer condition than keratoconus, which is the most common dystrophy of the cornea. [2] Similar to keratoconus it is typically diagnosed in the patient's adolescent years and attains its most severe state in the twenties and [wikidoc.org]

Epidemiology It is a much rarer condition than keratoconus, which is the most common dystrophy of the cornea. [4] Similar to keratoconus it is typically diagnosed in the patient's adolescent years and attains its most severe state in the twenties and [ipfs.io]

Epidemiology[edit] It is a much rarer condition than keratoconus, which is the most common dystrophy of the cornea.[4] Similar to keratoconus it is typically diagnosed in the patient's adolescent years and attains its most severe state in the twenties [en.wikipedia.org]

Pathophysiology

Pathophysiology Keratoglobus is a little-understood disease with an uncertain cause, and its progression following diagnosis is unpredictable. [wikidoc.org]

It is sometimes equated with "megalocornea". [1] Pathophysiology Keratoglobus is a little-understood disease with an uncertain cause, and its progression following diagnosis is unpredictable. [ipfs.io]

It is sometimes equated with "megalocornea".[1] Pathophysiology[edit] Keratoglobus is a little-understood disease with an uncertain cause, and its progression following diagnosis is unpredictable. [en.wikipedia.org]

Pathophysiology The pathophysiologic mechanism is unknown. Primary prevention An effective means of prevention has not been discovered. [eyewiki.aao.org]

Discussion A structural collagen tissue disorder or infection, thinning/weakening of the pulmonary artery wall due to chronic inflammation, wall tension created by degeneration, and chronic pressure/volume overload have been suggested to create the basic pathophysiological [tgkdc.dergisi.org]

Prevention

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. [ncbi.nlm.nih.gov]

Preventing progression of a central Descemet tear is essential to bypass the need for a large-diameter penetrating keratoplasty graft and its complications in a young patient with a history of bilateral corneal hydrops. [eye.hms.harvard.edu]

Complete removal of the epithelium is important to prevent cystic formations or even melting of the donor lamella, Prof. Busin said. [healio.com]

Prognosis and prevention Specific measures for the prevention of keratoglobus have not been developed, since in most cases this pathology is congenital. [medic-journal.com]

Primary prevention An effective means of prevention has not been discovered. Diagnosis History Patients usually present with a stable or worsening bilateral visual impairment that cannot be properly corrected with spectacles or contact lenses. [eyewiki.aao.org]

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