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BRIEF COMMUNICATION
Year : 2010  |  Volume : 58  |  Issue : 4  |  Page : 328-329

Alcohol epitheliectomy with mechanical debridement in a case of granular corneal dystrophy with r555w homozygous mutation of TGF B1 gene


Cornea and Anterior Segment Service, L V Prasad Eye Institute, L V Prasad Marg, Hyderabad, India

Date of Submission20-Jul-2008
Date of Acceptance28-Mar-2009
Date of Web Publication7-Jun-2010

Correspondence Address:
Prashant Garg
L V Prasad Eye Institute, L V Prasad Marg, Banjara Hills, Hyderabad - 500 034
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0301-4738.64126

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  Abstract 

An eight-year-old girl, an offspring of a consanguineous marriage presented with multiple anterior stromal geographic corneal opacities in both eyes. She was diagnosed to have superficial variant of granular dystrophy based on the family history, clinical features and mutation of TGF B1 gene. She was treated by alcohol-assisted removal of epithelium followed by mechanical debridement of abnormal deposits. Postoperatively, the cornea in both eyes was clear with no trace of opacity and the patient had an unaided visual acuity of 20/20 partial.

Keywords: Alcohol epitheliectomy, granular corneal dystrophy, homozygous mutation of TGF B1 gene


How to cite this article:
Garg P, Jabbar A. Alcohol epitheliectomy with mechanical debridement in a case of granular corneal dystrophy with r555w homozygous mutation of TGF B1 gene. Indian J Ophthalmol 2010;58:328-9

How to cite this URL:
Garg P, Jabbar A. Alcohol epitheliectomy with mechanical debridement in a case of granular corneal dystrophy with r555w homozygous mutation of TGF B1 gene. Indian J Ophthalmol [serial online] 2010 [cited 2020 May 27];58:328-9. Available from: http://www.ijo.in/text.asp?2010/58/4/328/64126

The classical granular corneal dystrophy, an autosomal dominant disorder of TGF B1 gene is characterized by sharply demarcated breadcrumb-like deposits. [1],[2] The patients usually present in the fourth decade of life and the disease progresses very slowly. However, patients who are homozygous for the gene present in the first decade of life and the disease is characterized by deposits predominantly located in the anterior stroma / Bowman's plane. These deposits have a high tendency to recur necessitating repeated surgery. [3-6] These facts are important considerations in the management of this disorder. We describe a new surgical option for this disorder.


  Case Report Top


An eight-year-old girl presented to us with complaints of blurring of vision and episodes of ocular irritation in both eyes since the past four years. Her nine-year-old brother also had similar complaints. Both children were offsprings of a consanguineous marriage and the family pedigree is shown in [Figure 1].

On examination her visual acuity was 20/50 with -0.75 diopter cylinder (D Cyl) Χ 90 0 in the right eye and 20/50 with -1.00 D Cyl Χ 90 0 in the left eye. Slit-lamp examination showed multiple anterior stromal geographic corneal opacities in both eyes [Figure 2]a. The rest of the anterior and posterior segment examination was essentially within normal limits. Her brother had identical corneal findings [Figure 3]a. Both parents had classical granular dystrophy [Figure 3]b. Genetic analysis of family members showed the presence of Arg555Trp mutation of TGF B1 gene. The mutation was homozygous in the children.

The patient underwent alcohol epitheliectomy with mechanical debridment under general anesthesia. An 8-mm trephine was placed on the cornea and 2 ml of 20% ethyl alcohol poured in the well of the trephine was allowed to stay for 60 sec. The alcohol was thoroughly washed with saline. The epithelium was removed with a dry Q-tip applicator. Underlying deposits were then scraped off with a No. 15 surgical blade. The deposits came off easily leaving behind clear corneal stroma. At the conclusion a bandage contact lens was placed.

Postoperatively she was given prednisolone acetate eye drops four times per day and ofloxacin eye drops four times per day. After one week the bandage contact lens was removed; ofloxacin was discontinued and the prednisolone acetate was tapered. The procedure was repeated for her left eye after four weeks. At the end of six weeks follow-up after the second eye surgery the patient's unaided visual acuity improved to 20/20 partial in both eyes. Cornea was clear except for mild anterior stromal haze on slit-lamp examination [Figure 2]b.


  Discussion Top


Homozygous granular corneal dystrophy poses a therapeutic challenge. Various treatment options for this condition are superficial keratectomy, photo therapeutic keratectomy (PTK), lamellar keratoplasty, and penetrating keratoplasty. [3],[4],[5] PTK has the following drawbacks: need for an access to excimer laser, expense, difficulty of general anesthesia in the laser room especially for treating young children, thinning and consequent hyperopic shift. Therefore, PTK might not be the best option in managing this condition. Penetrating keratoplasty and lamellar keratoplasty are challenging in children due to the need for repeated examinations under anesthesia, an unpredictable refractive outcome with amblyogenic potential, and increased risk of rejection. These disadvantages have a major bearing on the treatment of the disorder due to a high potential for recurrence.

In contrast the treatment described in this paper is safe for children, does not alter corneal thickness and refraction and can be easily repeated. We got the clue for this new treatment from the observation that the accumulation of the abnormal material is restricted to the Bowman's layer and removal of the corneal epithelium would expose the deposits for mechanical debridement.

 
  References Top

1.
Mannis MJ, DeSousa LB, Gross RH. The stromal dystrophies. In: Krachmer JH, Mannis MJ, Holland EJ, editors. Cornea. Vol. 2. St Louis: Mo: Mosby Publications; 1997. p. 1043-62.  Back to cited text no. 1
    
2.
Kannabiran C, Sridhar MS, Chakravarthi K, Vemuganti GK, Lakshmipathi M. Genotype -Phenotype correlation in 2 Indian families with severe granular corneal dystrophy. Arch Ophthalomol 2005;123:1127-33.  Back to cited text no. 2
    
3.
Fujiki K, Hotta Y, Nakayasu K, Kanai A. Homozygotic patient with: βig h3 mutaton in Granular dystrophy. Cornea 1998;17:288-92.  Back to cited text no. 3
    
4.
Rapuano CJ. Excimer laser phototherapeutic keratectomy in eyes with anterior corneal dystrophies. Cornea 1997;16:151-7.  Back to cited text no. 4
    
5.
Lyons CJ, McCarteny AC, Kirkness CM, Ficker LA, Steele AD, Rice NS. Granular corneal dystrophy. Visual effects and pattern of recurrence after lamellar or penetrating keratoplasty. Ophthalmology 1994;101:1812-7.  Back to cited text no. 5
    
6.
Haddad R, Font RL, Fine BS. Unusual superficial variant of granular dystrophy of cornea. Am J Ophthalmol 1977;83:213-8.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


This article has been cited by
1 Phototherapeutic keratectomy versus alcohol epitheliectomy with mechanical debridement for superficial variant of granular dystrophy: A paired eye comparison
Jatin N. Ashar,Madhavi Latha,Pravin K. Vaddavalli
Contact Lens and Anterior Eye. 2012; 35(5): 236
[Pubmed] | [DOI]



 

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