Malignant Glaucoma Post Gonioscopy-Assisted Transluminal Trabeculectomy.

A 69-year-old male patient with Pseudoexfoliation and open angle glaucoma presented with malignant glaucoma 1 week after undergoing gonioscopy-assisted transluminal trabeculotomy (GATT). This is a rare sight-threatening complication following gonioscopy-assisted transluminal trabeculotomy . A high index of suspicion, early detection, and prompt institution of medical therapy and YAG hyaloidotomy, helped in the resolution of the condition with good intraocular pressure control and visual improvement.

Novel Proposed Algorithm in Congenital Hereditary Endothelial Dystrophy.

PURPOSE: Congenital hereditary endothelial dystrophy (CHED) is a rare, autosomal recessive, monogenic corneal condition with variable expressivity. Often presents in bilateral symmetrical progressive corneal cloudiness that starts in the early infancy. It is characterized by increased corneal thickness, profound corneal edema, and thickening of the Descemet membrane due to endothelial dysfunction. The published literature lacks uniform guidelines for grading corneal cloudiness and management algorithm for CHED cases. This article focuses on applying newer investigational modalities to fine-tune surgical outcomes and more recent CHED management strategies. METHODS: This comprehensive literature review was performed based on a search on the PubMed database of relevant CHED articles focusing on those published in the last 7 years. A total of around 70 articles were reviewed, and 17 of them were included in this review. These include systemic reviews, randomized controlled clinical trials, cohort studies, case-controlled studies, and case series. RESULTS: Corneal cloudiness grading in CHED using subjective and objective methods using Anterior Segment Optical Coherence Tomography (AS-OCT) and densitometry using Scheimpflug imaging can help select appropriate management plan for CHED cases. DSAEK outscores penetrating keratoplasty with much fewer complications and expedites visual recovery, which helps mitigate amblyopia. CONCLUSION: Managing cases of CHED has been a challenge due to the dilemma in timing and appropriate surgical method selection and lack of definitive medical or other conservative approaches. Currently, DSAEK has shown favorable results in cases of CHED. However, appropriate staging of CHED and selecting the appropriate management approach appears to play a critical role in managing such cases. Besides these, novel treatment modalities such as nonsteroidal anti-inflammatory agents (NSAIDS) that target restoring water-flux activity in subtype of CHED and gene editing using CRISPR-Cas9 are promising paradigm treatment modalities.

Updates on congenital hereditary endothelial dystrophy.

Congenital hereditary endothelial dystrophy (CHED) is a rare genetic corneal disorder causing progressive cornea clouding and significant visual impairment. CHED remains a leading indication for pediatric corneal transplantation despite its infrequency, particularly in regions with high consanguinity rates like Southeast Asia. Identifying the Solute Carrier Family 4 Member 11 (SLC4A11) gene as the genetic basis of CHED has led to the discovery of it's various genetic variations. However, a comprehensive understanding of its clinical-genetic correlation, pathophysiology, and optimal management is ongoing. This review aims to consolidate current knowledge about CHED, covering its genetic origins, pathophysiological mechanisms, clinical presentation, and management strategies. Surgical intervention, such as penetrating keratoplasty (PK), Descemet stripping automated endothelial keratoplasty (DSAEK), and Descemet membrane endothelial keratoplasty (DMEK), remains the primary treatment. DSAEK and DMEK offer advantages over PK, including quicker visual recovery, reduced complications, and longer graft survival, especially in the pediatric age group. The timing of surgical interventions depends on disease severity, age at presentation, comorbidities, and visual potential. Elevated oxidative stress in CHED corneal tissue suggests potential benefits from anti-inflammatory drugs to rescue mutated endothelial cells. Considering the limitations of corneal graft surgeries, exploring novel gene-based molecular therapies are essential for future management. Early diagnosis, appropriate surgical interventions, amblyopia control, and genetic counseling for predictive analysis are pivotal for optimizing CHED management. A multidisciplinary approach involving ophthalmologists, researchers, and genetic counselors is essential for precise diagnosis and optimal care for CHED patients.