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Effect of ocular rigidity and biomechanics on intraocular pressure in mucopolysaccharidosis I-S (Scheie’s syndrome)

Poster Details

First Author: M.Rana UK

Co Author(s):    A. Gupta   S. Shah   M. Quinlan   I. Masood     

Abstract Details



Purpose:

To study the effect of biomechanical properties of the cornea on the intraocular pressure in eyes affected with Mucopolysaccharidosis type I-S (Scheie’s syndrome).

Setting:

Birmingham Midlands Eye Centre, City Hospital, Birmingham , United Kingdom.

Methods:

Four eyes of two patients in their mid-fifties diagnosed with Scheie’s syndrome were investigated for corneal biomechanical properties. There was corneal clouding and stromal oedema causing poor vision and also had other characteristic features of Scheie’s syndrome, well published in the literature. The corneal biomechanical profile was assessed by the use of Ocular response analyzer (ORA, Reichert Inc., of Buffalo, NY). Intraocular pressures were also checked using various tonometry devices to check for variability in the readings due to the corneal changes.

Results:

Two eyes of one patient had IOP readings of 30mmHg and 26mmHg, whereas the other patient had significantly high readings of 54mmHg and 56mmHg in their right and left eye respectively. Both the patients underwent successful bilateral deep anterior lamellar keratoplasty (DALK) procedures. The post procedure IOP measurement had a dramatic improvements. The first patient has IOP readings of 14mmHg and 15mmHg and the second patient had readings of 9mmHg and 10mmHg in the right and left respectively. Corneal hysteresis and corneal resistance factor were measured using the ORA post graft in the first patient and pre and post graft in the second patient. For the first patient, the measurements for corneal hysteresis were 9.5 and 11 and corneal resistance factor were 7.3 and 9.2 in the right and left eye respectively. Corneal hysteresis was noted to be 19.2 and 16.8 pre-graft in the second patient and this improved to 7.9 and 10.5 after having bilateral DALK procedures. Corneal resistance factor was also noted to reduce from 19.2 and 18.5 to 11.3 and 9.6 in right and left eye respectively. The intraocular pressure readings measured using different tonometry devices were also noted to be lower after graft surgeries.

Conclusions:

Corneal rigidity and hysteresis is high in patients with MPS type I-S. This is reflected in the raised intraocular pressures checked by use of applanation or indentation tonometry. Use of the ocular response analyzer has helped study the biomechanical properties of such corneas and helped in assessment of true values of intraocular pressures thus preventing inappropriate intervention options especially surgical options.

Financial Disclosure:

NONE

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