Granular dystrophy

Granular dystrophy: Description, Causes and Risk Factors:Granular dystrophyGranular dystrophy is an autosomal dominant, bilateral, non-inflammatory condition that results in deposition of opacities in the cornea by adulthood. It specifically affects the middle portion of the cornea (stroma) and eventually can cause decreased vision and eye discomfort.As with most dominantly-inherited corneal dystrophies, the pathology is central rather than peripheral and does not cause inflammation or vascularization of the cornea. Characteristics of this dystrophy are clear areas between the hyaline deposits in the stroma, which allows for good vision late into the course of disease. In some families, however, the clear stroma in the middle of the pathologic areas are compromised, causing decreased vision. Recurrent corneal erosions can occur later in the course of the disease, but much of the visual loss is caused by the stromal hyaline deposits.A new variation of granular dystrophy called Avellino corneal dystrophy has been recognized. It has amyloid deposits in addition to the hyaline stromal deposits. Several families with this unusual combination have been traced back to Avellino, Italy for which the granular dystrophy variation was named.The genetics of granular, lattice, and macular dystrophy have revealed that they all involve a mutation in the BIGH3 gene. The disease is very uncommon Worldwide.Symptoms:Patients with granular dystrophy may have decreased vision, photosensitivity, and/or eye pain (from recurrent corneal erosions).Because granular dystrophy is autosomal dominant, one of the parents likely has granular corneal dystrophy as well.Diagnosis:Diagnosis is based on,Masson trichrome stains deposits bright red.
  • Optical coherence tomography (OCT) has been used in assessing granular corneal dystrophyand has been used to guide phototherapeutic keratectomy (PTK).
  • Corneal biopsy (which is not clinically indicated in patients with granular dystrophy) reveals eosinophilic hyaline deposits in corneal stroma. When a corneal transplant is performed, the specimen is submitted for histopathologic evaluation.
Treatment:In the first few decades of life, no treatment is needed because vision is good and erosions usually do not occur. In some families confluent pathology in the central cornea is found during the fourth decade and beyond. Depending on the extent of the central opacity and the response to visual correction with contact lenses and other means penetrating corneal transplantation may be treatment of choice. Excimer laser phototherapeutic keratectomy and lamellar keratoplasty usually are not options because of the depth of the defect in the middle and deeper stroma. Recurrence of hyaline and amyloid degeneration in the new transplant tissue is common. The prognosis for corneal transplantation in granular dystrophy is generally excellent with well over 90% of the corneas remaining clear for as least several years. Eventually, granular deposits may form in the superficial stroma and cause recurrent opacification and possible recurrent erosion.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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