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Keratoglobus

Isolated Congenital Megalocornea


Presentation

  • 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]
Easy Bruising
  • He had hypermobility of large joints, easy bruising, thin and hyperextensible skin with visible veins, which were also described in his elder brother.[ncbi.nlm.nih.gov]
Marfanoid Habitus
  • Other systemic features in this type are the presence of hypermobile joints, skeletal abnormalities like scoliosis, pectus excavatum, a marfanoid habitus, and hearing loss.[aimu.us]
Pseudotumor
  • Associated ocular conditions included orbital pseudotumor, vernal keratoconjunctivitis in three patients, chronic marginal blepharitis with chronic eye rubbing, and glaucoma after penetrating keratoplasty surgery.[ncbi.nlm.nih.gov]
Fishing
  • The long-curved needle of one end of a 10-0 double-armed Prolene suture was then passed through the paracentesis incision into the anterior chamber to reach the edges of the fish-mouthed unapposed posterior flap of the host pocket, before being passed[karger.com]
Aspiration
  • A paracentesis was made, and aqueous was aspirated until the anterior chamber had collapsed enough to take up a more physiologic shape. The donor corneoscleral graft was sutured into the prefashioned scleral bed with long, interrupted sutures.[ncbi.nlm.nih.gov]
High Arched Palate
  • Systemic examination may show features of blue sclera, skeletal abnormalities, hyper-mobility of joints, hearing loss, abnormal dentition or high-arched palate. There may be features specific to each syndrome.[aimu.us]
  • These include features mentioned earlier of blue sclera, joint hypermobility, skeletal abnormalities, hearing loss, abnormal dentition, high-arched palate, as well as other features specific to each syndrome.[ncbi.nlm.nih.gov]
Back Pain
  • My PT thinks my back pain is a fibromyalgia related issue, but my PCP thinks it could be my gallbladder....don’t you love when doctors have conflicting opinions I’ll be going in for an ultrasound on Wednesday to check my gallbladder so hopefully we’ll[thepicta.com]
Blue Sclera
  • Marshall-Stickler syndrome may exist in the differential diagnosis of keratoglobus with blue sclera.[ncbi.nlm.nih.gov]
  • Two siblings had in common the features of keratoglobus, blue sclera, atypical face, hearing loss, and hypermobile joints.[ingentaconnect.com]
Iridodonesis
  • 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]
  • 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]
  • 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]

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]
  • Treatment [ edit ] Treatment includes the use of protective eye glasses. [2] A number of surgical options are also available. [2] Further progression of the disease usually leads to a need for corneal transplantation because of extreme thinning of the[en.wikipedia.org]
  • Treatment of acute hydrops can be both conservative and surgical.[doi.org]
  • Helps you make optimal use of the newest drug therapies, including Anti-VEGF treatment for wet ARMD and bevacizumab treatment for complications of diabetes.[books.google.com]

Prognosis

  • Prognosis [ edit ] Keratoglobus continues to be a somewhat mysterious disease, but it can be successfully managed with a variety of clinical and surgical techniques.[en.wikipedia.org]
  • 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]
  • The prognosis for keratoglobus is poor.[eyewiki.aao.org]

Etiology

  • 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

  • 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]
  • 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: Incidence: Unknown Gender: 90% male predominance Genetics: X-linked recessive. Band Xq12-q26 (most likely q21-q23) Small number via autosomal dominant or recessive.[webeye.ophth.uiowa.edu]
  • Genetic epidemiology study of keratoconus: evidence for major gene determination. Am J Med Genet. 2000;93:403-09. 26. Gondhowiardjo TD, van Haerington NJ.[reviewofcontactlenses.com]
Sex distribution
Age distribution

Pathophysiology

  • 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 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]
  • Pathophysiology The pathophysiologic mechanism is unknown. Primary prevention An effective means of prevention has not been discovered.[eyewiki.aao.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]
  • Complete removal of the epithelium is important to prevent cystic formations or even melting of the donor lamella, Prof. Busin said.[healio.com]
  • Primary prevention An effective means of prevention has not been discovered. 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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