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Purpose To report the first two observations of bilateral paracentral acute middle maculopathy in the early postoperative period after cardiopulmonary bypass procedures. Methods Comprehensive ophthalmic examinations were performed, including best-corrected visual acuity, Humphrey visual field testing, dilated fundus examination, and optical coherence tomography. Results Two patients complained of unilateral vision loss after cardiac surgery involving cardiopulmonary bypass. Unilateral optic disc edema consistent with ischemic optic neuropathy was observed in the symptomatic eye of each patient, whereas scattered cotton-wool spots were identified bilaterally. In addition, optical coherence tomography imaging revealed bilateral paracentral acute middle maculopathy, correlating with visual field defects detected on automated perimetry in the asymptomatic eyes. Conclusion Although symptomatic postoperative vision loss is a rare complication of cardiac surgery, intraoperative retinal microvascular nonperfusion as a consequence of extracorporeal circulation of blood may be a more common and under-recognized occurrence.

Oculomotor synkinesis is the involuntary movement of the eyes or eyelids with a voluntary attempt at a different movement. The chemokine receptor CXCR4 and its ligand CXCL12 regulate oculomotor nerve development; mice with loss of either molecule have oculomotor synkinesis. In a consanguineous family with congenital ptosis and elevation of the ptotic eyelid with ipsilateral abduction, we identified a co-segregating homozygous missense variant (c.772G>A) in ACKR3, which encodes an atypical chemokine receptor that binds CXCL12 and functions as a scavenger receptor, regulating levels of CXCL12 available for CXCR4 signaling. The mutant protein (p.V258M) is expressed and traffics to the cell surface but has a lower binding affinity for CXCL12. Mice with loss of Ackr3 have variable phenotypes that include misrouting of the oculomotor and abducens nerves. All embryos show oculomotor nerve misrouting, ranging from complete misprojection in the midbrain, to aberrant peripheral branching, to a thin nerve, which aberrantly innervates the lateral rectus (as seen in Duane syndrome). The abducens nerve phenotype ranges from complete absence, to aberrant projections within the orbit, to a normal trajectory. Loss of ACKR3 in the midbrain leads to downregulation of CXCR4 protein, consistent with reports that excess CXCL12 causes ligand-induced degradation of CXCR4. Correspondingly, excess CXCL12 applied to ex vivo oculomotor slices causes axon misrouting, similar to inhibition of CXCR4. Thus, ACKR3, through its regulation of CXCL12 levels, is an important regulator of axon guidance in the oculomotor system; complete loss causes oculomotor synkinesis in mice, while reduced function causes oculomotor synkinesis in humans.

Importance Amyotrophic lateral sclerosis (ALS) is a fatal, rapidly progressive neurodegenerative disease that primarily affects motor neurons. Recently, three causative genes have been implicated in both ALS and glaucoma. However, it is still uncertain whether patients with ALS have neurodegeneration in their retinas. If so, retinal thickness measurements might be a useful biomarker for ALS progression. Previous work in this area has been inconclusive, as it has not taken into account the effect of ophthalmic diseases on retinal thinning. Objective To determine whether there are differences in retinal neurons in ALS patients utilizing spectral-domain optical coherence tomography (SD-OCT). We tested the hypothesis that ALS patients exhibit retinal neurodegeneration that is not associated with ophthalmic diseases. Design, settings and participants Observational, comparative, cross-sectional study performed on patients recruited from the Duke University Medical Center ALS clinic. Patients underwent a comprehensive ophthalmologic examination to rule out ocular pathology. 21 patients met inclusion criteria. Two eyes with ocular pathology were excluded, leading to a total of 40 eyes of 21 patients included in the study. Retinal neurodegeneration was assessed by retinal nerve fiber layer (RNFL) thickness measurement using SD-OCT (Spectralis; Heidelberg Engineering). Main outcomes and measures ALS disease severity, determined through the ALS Functional Rating Scale (ALSFRS-R); mean and six sector RNFL thickness values compared to age-adjusted values in the normative database provided by Heidelberg Engineering; RNFL thickness correlation with ALSFRS-R, ALSFRS-R progression rate, forced vital capacity (FVC), and visual acuity. Results ALSFRS-R mean score was 30+/-10. Mean RNFL thickness in ALS patients was 88.95 +/- 10.8 microns, significantly thinner than values in the normative database (95.81 +/- 0.8). These RNFL thickness values did not demonstrate correlation to ALSFRS-R score, ALSFRS-R progression rate, FVC, intraocular pressure, or visual acuity. Conclusions Using SD-OCT, our study shows that ALS patients without ocular pathology exhibit thinned retinal layers. Future studies are warranted to clarify the clinical relationship between retinal thinning and motor neuron loss in ALS.

We investigated the impact of perioperative complications on patients' health-related quality of life (HRQL) and intermediate-term survival after cardiac surgery. Improved results for cardiac surgery are well demonstrated in low rates of operative mortality and morbidity. However, the association between perioperative morbid events, HRQL at 1 year, and survival is unclear. We performed a prospective study among 836 elective cardiac surgery patients to assess the impact of perioperative outcomes on survival and HRQL at 1 year. Health-related quality of life was generated using the 20-item short-form questionnaire. Kaplan-Meier and life-table estimates were used to examine the relationship of HRQL at 1-year and intermediate-term survival. All HRQL domains were statistically improved at 1 year, with the highest gains in general health perception (+19.4%) and the least gains in social (+5.1%) and mental (+5.1%) domains. Patients experiencing 1 or more perioperative complication or increased length of stay reported similar HRQL scores as patients with no complications. Patients with negative changes in social (94.5% vs 99.2%, P < .001) and general health perception (99.4% vs 95.5%, P < .001) domains showed a reduced survival compared with patients with positive HRQL gains at 2 to 5 years after surgery. Perioperative complications had minimal or no effect on HRQL at 1 year after cardiac surgery.

We analyzed 3 calcified silicone IOLs from 2 patients with a history of asteroid hyalosis. In both cases, posterior capsule opacification had been diagnosed and a laser capsulotomy performed before referral. Subsequently, both IOLs were exchanged with hydrophobic acrylic IOLs that were placed in the sulcus. The IOL exchange was challenging due to the open capsule. Light microscopy demonstrated that the white deposits on the explanted IOLs formed an almost confluent crust in some areas, interspersed with clear areas on the posterior optic surfaces. The findings in our cases and in other reported cases may affect the choice of IOL in the presence of asteroid hyalosis. Also, ophthalmologists may consider deferring laser posterior capsulotomy treatment in a calcified silicone IOL to facilitate an IOL exchange procedure. Financial Disclosure No author has a financial or proprietary interest in any material or method mentioned.

Idiopathic intracranial hypertension (IIH) is a disorder of elevated intracranial pressure due to an unknown cause. In most cases, IIH can be managed with medical therapy and weight reduction. Surgical treatment of IIH is reserved for patients who cannot tolerate medical therapy, are nonadherent to medical therapy, develop progressive symptoms despite maximal medical therapy, or present with fulminant visual loss. To date, there has been no randomized controlled trial to evaluate the surgical treatment of IIH, and our current knowledge of the efficacy and complications of these procedures is based on retrospective and observational studies. This review discusses the indications for surgical intervention in IIH and provides an overview of the recently published data on the efficacy and complications of these interventions. A surgical management algorithm is also presented to guide the clinician when evaluating a patient with IIH.

The optic nerve is a white matter tract of the brain and its sheath is composed of pia mater, arachnoid mater, and dura mater. The subarachnoid space (SAS) of the optic nerve sheath, which contains cerebrospinal fluid, is contiguous with the SAS of the brain. Elevated intracranial pressure is transmitted to the optic nerve head through the SAS resulting in papilledema. Optic nerve sheath fenestration (ONSF) can be an effective surgical technique to preserve vision in patients with progressive optic neuropathy due to papilledema caused by idiopathic intracranial hypertension (IIH); however, a dreaded complication of ONSF is visual loss. This article will discuss the rationale, indications, and complications of ONSF. A variety of surgical techniques can be employed to perform an ONSF. In particular, the medial transconjuctival approach will be highlighted.