GRANT'S SYNDROME

Grant's syndrome is a rare cause of secondary glaucoma.The syndrome was first described, and named, by Chandler and Grant in 1968. So far, only 16 primary cases have been reported. Two other cases have occurred after argon laser trabeculoplasty and intravitreal injection of bevacizumab and ranibizumab. 

The underlying mechanism for elevated intraocular pressure in these patients is apparently similar to that of other uveitic open-angle glaucomas – namely trabecular swelling, trabecular obstruction with cell/debris, and/or trabecular hyalinization.

Most of the cases have been Caucasians or blacks. Wei has reported one Asian patient with Grant's syndrome.

Patients, often >50 years old, present with high IOP (30-70 mmHg), frequently in both eyes (86% of cases). There is a female predominance in most cases (79%).

Characteristically there are yellowish trabecular meshwork (TM) precipitates with or without light anterior chamber flare and cells. Often, the TM precipitates are the only sign of inflammation. As other inflammatory signs usually were absent or minimal, it is easy to be misdiagnosed as primary open angle glaucoma (POAG).

Black arrow= PAS; White arrow= precipitates

The syndrome is diagnosed based on clinical manifestations. 

A few systemic conditions such as Sarcoidosis, rheumatoid arthritis (RA) and ankylosing spondylitis (AS) have been reported to be associated with Grant's syndrome. Scleritis, chronic uveitis and Posner-Schlossman syndrome (PSS) have also been reported to accompany Grant's syndrome. However, these diseases usually have other obvious inflammation signs, such as ciliary congestion, anterior chamber cells, and keratic precipitates.

Typical gonioscopic findings in Grant's syndrome are TM precipitates and scattered irregular peripheral anterior synechiae (PAS). Long-standing TM precipitates can prompt development of PAS. So, patients can be misdiagnosed with primary angle-closure glaucoma (PACG), especially those patients with a shallow anterior chamber. In PACG, PAS first appear superiorly and nasally, whereas inflammatory PAS are observed in areas where TM precipitates have been present and tend to distribute in all segments of the chamber angle.

Ultrasound biomicroscopy showing precipitates 

The patients typically do not respond well to anti-glaucoma treatment. However, the condition usually resolves with corticosteroid treatment.

The condition is prone to recurrence; up to 64% of cases may have recurrence of glaucoma in long-term follow-up.

Prognosis is usually good if treated promptly, the inflammation and pressure can be controlled, but regular follow-up is necessary due to the potential for recurrences.

AICARDI-GOUTIERES SYNDROME

Aicardi–Goutières syndrome (AGS) is a rare autoimmune neurological disorder belonging to type I interferonopathies. 

There are seven subtypes based on the different pathogenic genes: three prime repair exonuclease 1 (TREX1) (AGS1), RNASEH2B (AGS2), RNASEH2C (AGS3), RNASEH2A (AGS4), SAMHD1 (AGS5), ADAR1 (AGS6) and IFIH1 (AGS7).

Mutations affecting RNASEH2B and TREX1 are reported to be the most common, representing 35% and 17% of all cases, respectively.

AGS is usually inherited in an autosomal recessive manner. However, there may also be de novo or inherited autosomal dominant pathogenic variants in TREX1 or ADAR, as well as heterozygous autosomal dominant pathogenic variants in IFIH1. Mutations in the above genes affect the targeting and/or metabolism of nucleic acids, thereby promoting a type I interferon (IFN-I)-mediated innate immune response.

AGS was first described by Jean Aicardi and Francoise Goutières in 1984 with a case series of eight children from five families with severe early-onset encephalopathy. 

The major clinical features of AGS include encephalopathy, significant intellectual disability, acquired microcephaly during the first year of life, dystonia, spasticity, sterile pyrexias, intracranial calcifications, white matter lesions, brain atrophy, bilateral striatal necrosis, chilblain lesions on the feet, hands, ears or more diffuse throughout the skin.

The characteristic features include lymphocytosis, high levels of interferon α (IFN-α) in the cerebrospinal fluid (CSF) and serum with an increased expression of interferon-stimulated genes (ISGs) in the peripheral blood—the so-called “interferon signature”.

Patients may also have intracerebral vasculopathy, hepatosplenomegaly, elevated liver enzymes, thrombocytopenia, hemolytic anemia, elevated autoantibodies, hypothyroidism, insulin-dependent diabetes mellitus, transitory antidiuretic hormone deficiency, neonatal cardiomyopathy and demyelinating peripheral neuropathy.

In the most severe form, this condition features microcephaly, leukodystrophy, cerebral atrophy, intracranial calcifications, along with hepatosplenomegaly and thrombocytopenia. It is associated with elevated levels of central nervous system type I interferon signaling and often progresses with severe neurologic symptoms and death in early childhood. However, milder forms may show later onset with features of a lupus-like syndrome including painful skin lesions and congenital glaucoma.

AGS is often mistaken for TORCH (Toxoplasmosis, Others, Rubella, Cytomegalovirus, Herpes) infections.

Ocular manifestations include glaucoma (congenital or later onset), optic atrophy, and cortical blindness. 

Most cases of glaucoma are diagnosed within the first 6 months of life. Studies have shown that the risk of glaucoma development differs depending on the specific genetic mutation. In a study of AGS, glaucoma was diagnosed in 6.3%, of which 20.8% were patients with a pathological SAMHD1. The ADAR and IFIH1 mutations were found to be the least associated with glaucoma.

Congenital glaucoma can be considered as a part of the phenotypic spectrum of AGS; therefore, regular ophthalmological examinations are essential, especially in the first years of a child’s life, in order to diagnose the disease and promptly initiate treatment.

WORLD GLAUCOMA WEEK

The World Glaucoma Association celebrates the World Glaucoma Week every year with a novel theme. 

This year the week was marked from 8-14th March with the theme "Uniting for a glaucoma free world". 

This week is an important occasion to reinforce the importance of early detection of glaucoma, and using this period to perform extensive screenings for glaucoma. It is also necessary to disseminate information among the public about the disease, sharing awareness materials (like the green ribbon), and emphasise the necessity for regular follow ups with their eye health practitioners.

The at-risk categories such as people over 40, those with a family history of glaucoma, or diabetics should be reviewed more frequently.

In conjunction with this year's glaucoma week, we have also performed our bit to screen and treat for glaucoma.

Dr. Syed Shoeb Ahmad 

Ahmed Glaucoma Valve for NVG in PDR vs. CRVO

Is there any difference in the outcomes after Ahmed Glaucoma Valve (AGV) implantation in patients who develop neovascular glaucoma (NGV) following proliferative diabetic retinopathy (PDR), as compared to NGV following central retinal vein occlusion (CRVO)?

Kanra et al, performed a study in which AGV implantation was performed in NGV patients who developed following PDR (n=28), and CRVO (n=18). All AGV implantation surgeries were done by the same surgeon.

Surgical success was defined as ≥20% intraocular pressure (IOP) reduction from baseline and IOP ≤ 21 mm Hg without additional glaucoma surgery or vision loss to no light perception. 

The study found that the mean surgical success duration was longer in PDR (45.87 mo) than in CRVO (38.68 mo).

One-year, 2-year, and 3-year success rates were 95.5%, 90.4%, and 90.4% in PDR, compared with 92.3%, 64.6%, and 55.4% in CRVO. 

Early complications, such as hyphema, were more frequent in PDR but not statistically significant. 

Tube exposure was observed in only one case (2.1% of total cases), which was in the CRVO group. 

Tube explantation was performed in 2 patients (4.2% of total cases) which included the patient with tube exposure . 

The study found that older age was a significant risk factor for failure (HR = 1.066, P = 0.049).

The study concluded that, AGV implantation provides favorable long-term outcomes for NVG secondary to PDR and CRVO, but with higher success rates in PDR.

REFERENCE:

Kanra, Ayşe Yağmur; Dursun Yilmazşamli, Tuğçe; Altinel, Meltem Güzin; İmamoğlu, Serhat. Surgical Outcomes of Ahmed Glaucoma Valve in Neovascular Glaucoma Secondary to Diabetic Retinopathy Versus Central Retinal Vein Occlusion. Journal of Glaucoma 35(3):p 190-197, March 2026. | DOI: 10.1097/IJG.0000000000002680

DOES GLAUCOMA PROGRESS AFTER REFRACTIVE SURGERY?

Glaucoma is not a contraindication for refractive surgery performed to remove glasses. However, is there any association between these surgeries and glaucoma progression?

A retrospective observational cohort study by Sujin and Rim, included 65 eyes of 65 patients with primary open angle glaucoma (POAG) who underwent refractive corneal surgery (RCS). 

Glaucoma progression was determined based on structural changes in optic disc/retinal nerve fiber layer (RNFL) photographs and/or visual field (VF) deterioration. 

Cox proportional hazards analysis was used to identify risk factors for disease progression. 

VF mean deviation (MD) and RNFL thickness progression rates obtained using a linear mixed-effects model were compared across tertile groups based on axial length (AXL) and central corneal thickness (CCT), respectively.

Over the follow-up period (mean: 9.1±2.9 y), 23 eyes (35%) exhibited glaucomatous progression. 

The progression group had significantly longer AXL (P<0.001), thinner CCT (P=0.009) compared with those in the stable group. 

Multivariate analysis identified longer AXL [hazard ratio (HR): 1.507, P=0.037] and thinner CCT (HR: 0.988, P=0.037) as significant predictors of glaucoma progression. 

VF MD declined faster in the middle and highest AXL tertile groups, whereas RNFL thinning was the most pronounced in the highest AXL tertile group. 

The lowest CCT tertile group exhibited the fastest VF MD decline and RNFL thinning.

Conclusion:

Patients with POAG and a history of RCS who present with longer axial length and thinner central corneal thickness, are at significantly higher risk of glaucomatous progression, highlighting the importance of vigilant long-term monitoring in these eyes.

REFERENCE:

Yeo S, Sung KR. Factors Associated With Glaucomatous Progression in Eyes With Prior Refractive Corneal Surgery. J Glaucoma. 2026 Mar 1;35(3):157-165. doi: 10.1097/IJG.0000000000002682. Epub 2025 Dec 17. PMID: 41474867.