Sharah Rahman

Abstract

The Hutchinson-Gilford Progeria (HGP) syndrome is an exceptionally rare genetic condition characterized by premature and accelerated aging in children. It is demonstrated by developmental delay along with progressive degenerative changes of the integumentary, musculoskeletal, cardiac, and vascular systems. In this case report, we describe the ocular manifestations of Hutchinson-Gilford Progeria (HGP) syndrome of a 20-year-old Bangladeshi boy. The patient had the classic triad of prominent eyes, loss of eyebrows or madarosis and lagophthalmos, which are the most common ocular manifestations.He also developed dry eye, keratinized ocular surface, Meibomian gland dysfunction, vascularized cornea, symblepharon, corneal opacification, and cataract. He had several systemic manifeastations that included senile facies, prominent scalp veins, generalized alopecia with plucked bird appearance, and sclerodermatous changes.   Keywords: Hutchinson­gilford, Progeria, Premature, Corneal keratinization, Loss of eyebrow

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Abstract

Purpose: To assess the impact on intraocular pressure (IOP) after Phacoemulsification (PE) with Posterior Chamber Intraocular Lens (PCIOL) implantation and its relation with ocular biometric parameters. Materials and Methods: The study was a prospective observational study included 524 eyes with normal IOP (11-21 mmHg) who underwent uneventful Phacoemulsification with PCIOL Implantation surgery for age-related cataract. IOP, Central Corneal Thickness (CCT), Anterior Chamber Depth (ACD), Axial Length (AL) were measured pre-operatively and one month post-operatively by Goldman Applanation Tonometer, Corneal Pachymeter and Optical Coherence Biometer, respectively. The post-operative IOP change and its relation with preoperative biometric parameters were determined. Results: The mean pre-operative and post-operative IOP was 13.44±1.98 & 11.54±1.66mmHg, respectively, with an average IOP reduction after one month of 1.90±0.61mmHg. The mean pre-operative and post-operative ACD was 3.27±0.24 and 3.66±0.27 mm, respectively, with an average increase of 0.39 ±0.3 mm. Paired t-test showed post-operative IOP reduction was significantly associated with preoperative IOP (p2 0.921. So 92.1% of the preoperative IOP is proportional to post-operative IOP reduction. The graphical representation shows more the pre-operative IOP, more the post-operative IOP reduction. Conclusion: IOP is significantly reduced, and ACD increased after PE with PCIOL implantation surgery and is associated with post-operative IOP reduction. The post-operative IOP reduction is proportional to pre-operative IOP, and more the preoperative IOP, more the post-operative IOP reduction.   Keywords: Intraocular pressure, Phacoemulsification, IOL implantation, Ocular biometric parameters.

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