Effect of Lens Status on the Outcomes of Glaucoma Drainage Device Implantation.

PURPOSE: To assess the effect of lens status and cataract surgery on glaucoma drainage device (GDD) efficacy. DESIGN: Retrospective cohort study. PARTICIPANTS: Two hundred and forty-three eyes of 216 patients that underwent GDD implantation with ≥1 follow-up visit within 3 years postoperatively. Exclusion criteria included GDD combined with other ophthalmic procedures. 90%-94% of GDDs were Ahmed implants; 83%-90% had adjunctive mitomycin-C. METHODS: Outcomes were compared between phakic eyes (group A), eyes phakic at time of implantation but subsequently underwent cataract surgery within 3 years (group B), and pseudophakic eyes (group C). Outcomes were measured at 1, 3, 6, 12, 24, and 36 months after tube shunt implantation. Multivariable regression models were performed, adjusting for baseline characteristics. MAIN OUTCOME MEASURES: Intraocular pressure (IOP) after GDD implantation. Secondary outcomes included change in visual acuity (VA), number of glaucoma eye drops, and rate of failure, defined as additional glaucoma surgery, vision decrease to no light perception, or IOP persistently ≤ 5 mmHg or > 21 mmHg or not reduced from baseline by 20%. RESULTS: There were 65 eyes in group A, 52 in group B, and 126 in group C. Within group B, cataract surgery was performed at a mean of 1.3 ± 0.7 years after GDD implantation. There were no statistically significant differences in mean IOP or medications between the 3 groups at all time points up to 3 years postoperatively. Significant improvement in VA was noted in groups A and B compared to group C at 6 months, 1 year, and 2 years after implantation; however, by postoperative year 3, change in VA was similar across groups. There were no significant differences in the failure rate amongst groups (P = 0.68). IOP and medications up to 12 months after cataract surgery were similar compared to preoperative baseline. Group B had significantly more short-term (P = 0.02) and long-term (P < 0.001) postoperative complications than groups A or C, driven primarily by hypotony. CONCLUSIONS: There were no differences in IOP, glaucoma medications, or rate of failure 3 years after GDD implantation based on lens status or after undergoing subsequent cataract surgery. These results may inform the management of patients with co-existing glaucoma and cataract. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Superior oblique palsy after facial feminization surgery.

Facial feminization surgery (FFS), or gender-affirming facial surgery, is a common procedure for patients with gender dysphoria. One goal of FFS involves extensive contouring of the frontal and nasal bones to reduce supraorbital bossing. Ophthalmic complications after FFS have been rarely reported. We report 2 cases of superior oblique palsy after FFS producing persistent vertical and torsional diplopia. One case was successfully treated with prism spectacles; the other required surgical management. Both cases likely involved surgical trauma to or disinsertion of the trochlea during orbital bony reshaping.

Efficacy of Ahmed and Baerveldt glaucoma drainage device implantation in the pediatric population: A systematic review and meta-analysis.

Glaucoma drainage devices (GDD) are increasingly utilized in the management of childhood glaucoma. This systematic review and meta-analysis assesses the efficacy of first-time Ahmed or Baerveldt implantation in children. PubMed, Embase, and Cochrane Library were searched for relevant English-language, peer-reviewed literature. Postoperative outcomes were pooled using random effects regression models with restricted maximum likelihood estimation. Thirty-two studies (1,221 eyes, 885 children) were included. Mean ± standard deviation preoperative IOP was 31.8 ± 3.4 mm Hg. Pooled mean IOP at 12 and 24 months postoperatively were 16.5 mm Hg (95% CI, 15.5-17.6) and 17.6 mm Hg (95% CI, 16.4-18.7), respectively. Pooled proportions of success were 0.87 (95% CI, 0.83-0.91) at 12 months, 0.77 (95% CI, 0.71-0.83) at 24 months, 0.54 (95% CI, 0.44-0.65) at 48 months, 0.60 (95% CI, 0.48-0.71) at 60 months, and 0.37 (95% CI, 0.32-0.42) at 120 months. There were no differences in proportion of success at 12 and 24 months among eyes that received Ahmed and Baerveldt tube shunts, nor between eyes with primary glaucoma, glaucoma following cataract surgery, or other secondary glaucoma. Our findings show that Ahmed and Baerveldt shunts substantially reduced IOP for at least 24 months in childhood glaucoma, with similar findings among device types and glaucoma etiologies.

Retinopathy and optic atrophy in a case of COQ2-related primary coenzyme Q10 deficiency.

PURPOSE: To describe a case of primary coenzyme Q10 deficiency in a child manifesting as early-onset renal failure, retinal dystrophy, and optic atrophy leading to progressive vision loss. METHODS: Clinical presentation and workup including visual fields, electroretinogram, and optical coherence tomography are presented. Genetic testing was performed. RESULTS: An eight-year-old female with nephropathy requiring renal transplantation subsequently developed progressive cone-rod dystrophy and optic atrophy. The patient had negative results on a targeted next-generation sequencing retinal dystrophy panel but whole-exome sequencing revealed two variants in COQ2 (likely biallelic), consistent with a diagnosis of primary coenzyme Q10 deficiency. CONCLUSIONS: Primary coenzyme Q10 deficiency is a rare disorder with variable systemic and ocular findings; there is also genetic heterogeneity. Genetic testing aids in the diagnosis of this condition, and variants in the COQ2 and PDSS1 genes appear to have the strongest association with ocular manifestations. Oral supplementation of coenzyme Q10 may slow progression of disease. This case highlights the utility of whole-exome sequencing in the diagnosis of a rare syndromic form of ocular disease and reports a novel phenotypic association for this condition.

Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3).

Purpose: To describe a case of incomitant divergence insufficiency esotropia in the setting of Machado-Joseph disease (spinocerebellar ataxia type 3) that recurred completely within one week after augmented bilateral medial rectus recession. Observations: A 53-year-old female with a history of Machado-Joseph disease presented with horizontal diplopia primarily at distance consistent with divergence insufficiency esotropia. Augmented bilateral medial rectus recessions were performed which initially produced orthotropia, but recurrence of the esodeviation to the full preoperative amount occurred by post-operative week one. The patient subsequently underwent bilateral lateral rectus resections with excellent result. Conclusions and importance: Divergence insufficiency is common in the spinocerebellar ataxia variants and is thought to be secondary to atrophy of brainstem structures involved in the control of ocular vergence. Strabismus surgery in these patients may be complicated by limited response or even rapid regression despite augmented surgery as suggested for divergence insufficiency in the setting of neurologic disease. Patients should be counseled on these risks as well as the potential for multiple procedures in order to achieve surgical success.

Plexiform Neurofibroma With Activating KRAS Mutation and Segmental Presentation Involving the Unilateral Eyelid.

Plexiform neurofibromas are classically thought to be pathognomonic for neurofibromatosis type 1. However, isolated forms may occur, particularly as a manifestation of segmental neurofibromatosis related to postzygotic mosaicism in the NF1 gene. Most cases occur on the head and neck, trunk, and extremities with very few cases reported in the periorbital area. The authors report a case of plexiform neurofibroma with perineuriomatous features of the right upper eyelid in a patient with no other stigmata of neurofibromatosis. While suggestive of segmental neurofibromatosis, genetic analysis revealed activating KRAS mutation and inactivating mutation in PHF6 with no evidence of NF1 mutation in germline or tumor tissue. Neither KRAS nor PHF6 have been previously reported in association with neurofibroma.

Massive periorbital edema following hematopoietic stem cell transplantation.

Purpose: To describe a case of severe, bilateral periorbital edema after hematopoietic stem cell transplantation. Observations: A three-year old girl with metastatic neuroblastoma underwent the second of two tandem autologous peripheral blood stem cell transplants, complicated by engraftment syndrome. On post-engraftment day 11, she developed acute onset of severe periorbital edema. She was soon thereafter diagnosed with transplant-associated thrombotic microangiopathy with significant volume overload requiring treatment with eculizumab and etanercept. Periorbital edema resolved after four days with concurrent treatment of her underlying condition. Conclusions and Importance: We report an ocular manifestation related to complications of hematopoietic stem cell transplantation. This highlights a non-infectious etiology of eyelid swelling in the post-transplant, immunocompromised population.

Spontaneous hyphema in the setting of COVID-19 pneumonia.

PURPOSE: To report a challenging case of spontaneous hyphema in the setting of prone positioning for COVID-19 pneumonia. OBSERVATIONS: A previously healthy patient was concomitantly diagnosed with acute myelogenous leukemia (AML) and COVID-19 infection. During his hospitalization he required intubation and prone positioning. Following change from prone to supine positioning, he was noted to have developed a large unilateral spontaneous hyphema. CONCLUSIONS AND IMPORTANCE: We present a challenging case of spontaneous hyphema due to a hematologic malignancy in the setting of prone positioning for COVID-19 pneumonia.

Treatment Patterns and Clinical Outcomes for Central Retinal Vein Occlusion in the Antivascular Endothelial Growth Factor Era.

Purpose: This article describes treatment patterns and visual outcomes for central retinal vein occlusion (CRVO) in the antivascular endothelial growth factor (anti-VEGF) era. Methods: A retrospective cohort study of eyes diagnosed with CRVO between 2009 and 2016 was conducted. Treatment history and visual acuity (VA) measurements were abstracted from medical records and analyzed. Results: A total of 476 eyes of 476 patients (median age 67 years, median follow-up 25.4 months) were included. Optical coherence tomography was obtained in 93.9% and fluorescein angiography in 80% of cases on presentation. Mean VA at presentation and final visit was 20/60 and 20/94, respectively, for eyes with nonischemic CRVO, whereas that of ischemic cases remained worse than 20/800 at final follow-up. Intravitreal bevacizumab was the most common first treatment (42.2%). Intravitreal steroid was the first treatment in 3.6% and ultimately administered in 11.3% of eyes. In the first year, an average of 5.2 ± 3.6 and 2.2 ± 3.4 anti-VEGF injections were given in treatment-naive and nontreatment-naive eyes, respectively. Conclusions: In our real-world cohort, anti-VEGF injection burden and frequency are lower than in published clinical trials. Visual outcomes in both ischemic and nonischemic eyes with CRVO are poorer than expected and worse than those recorded in controlled trial settings.