The large Hellenic Study of Uveitis: epidemiology, etiologic factors and classification.

PURPOSE: To analyse the demography, etiology, and classification of uveitis at a tertiary academic referral center. METHODS: An observational study was conducted on the archives of uveitic patients at the Ocular Inflammation Service of the Department of Ophthalmology at the University Hospital of Ioannina (Greece) from 1991 to 2020. This study aimed to investigate the epidemiological profile of patients, including their demographics and the main etiologic factors of uveitis. RESULTS: Out of 6191 cases with uveitis, 1925 were infectious, 4125 were non-infectious, and an overall of 141 masquerade syndromes were recorded. Among these cases, 5950 patients were adults, with a slight female predominance, while 241 were children (< 18 years old). Interestingly, 24.2% of cases (1500 patients) were associated with 4 specific microorganisms. Herpetic uveitis (HSV-1 and VZV/HZV) was the most common cause of infectious uveitis (14.87%), followed by toxoplasmosis (6.6%) and tuberculosis (2.74%). In 49.2% of non-infectious uveitis cases, no systematic correlation was found. The most frequent causes of non-infectious uveitis included sarcoidosis, white dot syndromes, ankylosing spondylitis, lens-induced uveitis, Adamantiades-Behçet disease, and idiopathic juvenile arthritis. Infectious uveitis was more common in the rural population, whereas non-infectious uveitis was more frequently recorded in the urban population CONCLUSIONS: Although our study was conducted on a predominantly white Caucasian population, it also reflects the effect of increasing immigration, improvements of diagnostic techniques, changes in referral patterns, and various actual changes in disease incidence.

Inner retinal layers' alterations of the microvasculature in early stages of Parkinson's disease: a cross sectional study.

PURPOSE: To investigate microcirculation characteristics of the inner retinal layers at the macula and the peripapillary area using Optical Coherence Tomography Angiography (OCT-A) of patients in early stages of Parkinson's disease (PD). METHODS: 32 PD patients and 46 age- and gender-matched healthy controls were included in this cross sectional study. OCT-A imaging was performed to analyze microcirculation characteristics at each separate macular region (fovea, parafovea, and perifovea) and the peripapillary area of the inner retinal layers. RESULTS: Individuals with PD had significantly lower parafoveal, perifoveal and total vessel density (VD) in the superficial capillary plexus (SCP) than controls (all p < 0.001), while foveal VD was higher in PD eyes than that of controls, though not statistically significant. Similarly, individuals with PD had significantly lower parafoveal, perifoveal and total perfusion in the SCP than control eyes (all p < 0.001), while foveal perfusion was significantly higher in PD eyes than that of controls (p = 0.008). PD eyes had significantly smaller FAZ area and perimeter accompanied by decreased circularity at the SCP as compared to controls (all p < 0.001). Concerning the peripapillary area, individuals with PD had significantly lower radial peripapillary capillary perfusion density and flux index at the SCP than controls (all p < 0.001). All p values remained statistically significant even after using the Bonferroni correction for multiple comparisons, except for that of foveal perfusion. CONCLUSIONS: Our study indicates alterations of the inner retinal layers at the macula and the peripapillary area at the preliminary stages of PD. OCT-A parameters could potentially comprise imaging biomarkers for PD screening and improve the diagnostic algorithms.

The Large Hellenic Study of Uveitis: Diagnostic and Therapeutic Algorithms, Complications, and Final Outcome.

PURPOSE: The purpose of this study is to present the diagnostic and therapeutic algorithms, complications, and final outcome in the management of uveitic patients at a tertiary academic referral center. DESIGN: Observational study. METHODS: Analysis of the archives of 6191 uveitic patients at the Ocular Inflammation Service of the Department of Ophthalmology of the University Hospital of Ioannina in Greece from 1991 to 2020. RESULTS: During the 30 years of the study, the diagnostic ability climbed from 45.43% (1991-1995) to 73.4% (2016-2020). This improvement was linked to several factors including the increase in the number of diagnostic paracenteses for the analysis of intraocular fluids, the range and quality of laboratory blood tests, the multimodal ophthalmic imaging, the proper use of nonophthalmic imaging, and the multidisciplinary approach. The degree of uveitis-related complications was related to the severity and cause of inflammation, the recurrence rate, inappropriate treatment, and the prolonged or initially inactive inflammation. The 3 most common complications included cataract, macular edema, and glaucoma. Apart from the modern treatments and surgical techniques, the 3-month preoperative control of inflammation played a critical role in the surgical outcomes. The percentage of patients with a successful outcome increased from 72% (2001-2005) to 90.50% (2016-2020). The center's experience, prompt referral, patient's compliance, and regular follow-ups are associated with a better outcome. The analysis of the results allowed the development of diagnostic and therapeutic algorithms. CONCLUSIONS: Developing diagnostic and therapeutic algorithms allows for the efficient management of uveitis, leading to better visual outcome and therefore a better quality of life.

Assessment of Maternal Retinal Microvasculature in Preterm Pregnancy Using OCT-Angiography: a Cross-Sectional Study.

Background: Pregnancy induces a cascade of hemodynamic changes that are likely to affect the maternal systemic and ocular circulation. Objective:The current study aimed to investigate retinal microvasculature alterations in women with preterm and full-term pregnancy using optical coherence tomography angiography (OCT-A). Design:This was a cross-sectional, comparative, single-center study. Methods:The present study included 21 women with preterm pregnancy, who were in the early postpartum period, and 18 controls with full-term pregnancy. Optical coherence tomography angiography imaging was performed to analyze macular microvasculature characteristics at the retinal superficial capillary plexus (SCP) and the choriocapillaris (CC). Results:In females with preterm pregnancy, the mean values of vessel density (VD) at the SCP of the total macular area were significantly higher than those in females with full-term pregnancy (p=0.001), and the mean values of foveal, parafoveal and perifoveal VD at the SCP were higher, though not statistically significant. Similarly, the mean values of perfusion at the SCP of the total macular area were significantly higher in females with preterm pregnancy than controls (p=0.023), while the mean values of foveal, parafoveal and perifoveal perfusion were higher, though not statistically significant. The mean values of foveal avascular zone (FAZ) parameters (area, perimeter, circularity) at the SCP in females with preterm pregnancy did not have any significant differences from those of controls. Concerning the measurements of choroidal parameters, there were no statistically significant differences in subfoveal choroidal thickness, as well as the CC OCT-A characteristics between females with preterm and full-term pregnancy. Conclusions:Our study indicates that retinal blood flow alterations may be present in the early postpartum period in women with preterm pregnancy. Increased macular vasculature may reflect the systemic perfusion changes compensating for placental insufficiency.

A novel case of Raoultella planticola - associated acute dacryocystitis.

This study aims to report an acute dacryocystitis associated with Raoultella planticola infection. The patient's medical records and laboratory were thoroughly assessed. A thorough literature search was performed in PubMed database. An additional search was made in Google Scholar to complete the collected items. This is the first recorded case of acute dacryocystitis attributed to Raoultella planticola infection. A limited number of studies has related this pathogen with conjunctivitis. Acute dacryocystitis on rare occasions can also be caused by atypical organisms. Swabs must be obtained and patients must be started on empirical treatment while waiting for the laboratory results.

Retinal microcirculation characteristics in obstructive sleep apnea/hypopnea syndrome evaluated by OCT-angiography: a literature review.

PURPOSE: The retina may provide a window to estimate systemic vascular status; therefore, there has been interest in identifying microcirculation characteristics that possibly reflect hypoxic alterations in obstructive sleep apnea/hypopnea syndrome (OSAS). Emerging evidence has suggested that retinal microvasculature investigation holds the potential to characterize the pathophysiology involved in ocular manifestations of OSAS. The advent of optical coherence tomography angiography (OCT-A) has attracted significant attention as this technique offers detailed analysis of the retinal capillary plexus. METHODS: A detailed literature search was performed in PubMed database until December 2021. We identified and reviewed all papers referring to the alterations of the retinal capillary plexus in OSAS using OCT-A. RESULTS: A comprehensive update indicates that microcirculation alterations of the retinal capillary plexus utilizing OCT-A may differ with severity of OSAS and imply the potential underlying pathophysiology of ocular manifestations. The reviewed series have revealed variability concerning microvasculature characteristics at the macular and the peripapillary area. Further studies are warranted to establish the OCT-A parameters as biomarkers regarding the evaluation of OSAS in clinical practice. CONCLUSION: Retinal capillary plexus characteristics as seen on OCT-A reflect microvasculature alterations, potentially leading to concomitant ocular comorbidity in the context of OSAS. The reviewed literature may confirm the diagnostic and prognostic value of OCT-A in the assessment of the pathophysiology of ocular manifestations in OSAS and highlight unmet needs to be addressed by future research.

Macular microcirculation characteristics in Parkinson's disease evaluated by OCT-Angiography: a literature review.

PURPOSE: Given the fact that retina may provide a window into the central nervous system, there has been interest in identifying retinal biomarkers as predicting factors of pathological processes in neurodegenerative disorders. Emerging evidence has suggested that macular microcirculation changes in Parkinson disease (PD) may indicate the alterations of cerebral microvasculature. The use of Optical Coherence Tomography Angiography (OCT-A) has attracted significant attention in recent years as this technique offers a detailed analysis of the existence of changes at the macular capillary plexus. METHODS: A detailed review of the literature was performed in PubMed until June 2021. We identified all papers referring to the alterations of the macular capillary plexus in PD using OCT-A. RESULTS: A comprehensive update indicates that microvasculature alterations of the macular capillary plexus utilizing OCT-A may comprise useful biomarkers regarding the cerebral vasculature in PD. Since the available evidence is limited, additional studies are warranted to establish the OCT-A parameters as predicting factors in clinical practice. CONCLUSIONS: A review of the existing literature sheds light on the microvasculature changes of the macular capillary plexus as seen on OCT-A in PD patients. The current article discusses notable aspects of key publications on the topic, highlights the importance of the potential long-term effectiveness of OCT-A biomarkers in PD and proposes the need for further future research.

A Narrative Review of the Ocular Manifestations in Noonan Syndrome.

PURPOSE: The purpose of this review is to provide an update on ophthalmological manifestations of Noonan Syndrome (NS). Emerging evidence has suggested that NS patients may present with a wide spectrum of ocular characteristics. Detailed investigation of genotype has revealed the diversity of related gene mutations. The potential association of genetic basis with clinical expressivity of phenotype remains a challenging aspect of this issue. METHODS: A literature search was performed in PubMed; we have analyzed prospective and retrospective cohort studies, case reports, and reference lists of retrieved articles until February 2021. We identified all papers referring to NS ocular manifestations referring to genotype and phenotype characteristics. RESULTS: A comprehensive update on ocular manifestations of NS patients indicates significant evidence for variability of genotype and phenotype features. Ophthalmologic features of NS are characterized by a wide spectrum of abnormalities; external ocular malformations, distortions of refraction, alignment, motilily, anterior and posterior ocular segment and visual impairment. Currently, a variety of pathogenic mutations in patients with NS have been investigated, while the nature of the genetic variants may determine expressivity. Albeit it remains a clinical diagnosis with variation in the progress of each NS phenotype. Although the available evidence that is needed to establish genotype-phenotype correlation as predicting factor in clinical practice is limited, it could aid risk assessment and patient management. CONCLUSION: A review of the existing literature sheds light on the ocular characteristics of NS. The current article discusses notable aspects of key publications on the topic, highlights the importance of the potential genotype-phenotype correlation in clinical practice, and proposes the need for further future research.

Spirochetal uveitis: Spectrum of clinical manifestations, diagnostic and therapeutic approach, final outcome and epidemiological data.

PURPOSE: Analysis of cases with spirochetal uveitis related to spirochetes in a tertiary referral academic center. METHODS: Retrospective study of patients diagnosed with uveitis attributed to Treponema pallidum, Leptospira spp. and Borrelia burgdorferi from June 1991 until December 2019. RESULTS: A total of 57 cases of spirochetal uveitis (22 patients with T. pallidum, 26 with Leptospira spp., and 9 with B. burgdorferi) that consisted 1% of the overall number of uveitics were recorded. All these cases presented with a wide spectrum of clinical presentations (anterior uveitis, posterior uveitis, panuveitis, vasculitis, papillitis, and in some rare cases concomitant posterior scleritis). The treatment included mainly penicillin or doxycycline, while corticosteroids were administered systematically in some cases with Borrelia or Leptospira infection. The final visual outcome was favorable (> 6/10 in Snellen visual acuity) in approximately 76% of our patients. CONCLUSION: Despite being rare, spirochetal uveitis can be detrimental for the vision and must always be included in the differential diagnosis.

The contribution of multifocal visual evoked potentials in patients with optic neuritis and multiple sclerosis: a review.

PURPOSE: To review the evidence on the usefulness of the multifocal visual evoked potential (mfVEP) test in patients with optic neuritis (ON) and/or multiple sclerosis (MS). METHODS: We critically review key published evidence on the use of mfVEP in ON/MS patients and its association with other functional and structural tests. RESULTS: Multifocal VEP tests are useful in detecting abnormality in patients with ON/MS and monitor the progression of lesions (remyelination, atrophy). In addition, mfVEP has good correlation with conventional visual evoked potential (VEP), standard automated perimetry, optical coherence tomography and magnetic resonance imaging. In patients with ON, mfVEP might be useful in predicting the risk of conversion to MS.

Four common diseases causing sudden blindness or death in the eye emergency department.

Neuro-ophthalmological emergency disorders typically present with symptoms of visual loss, diplopia, ocular motility impairment or anisocoria. The ocular manifestations of these disorders are sometimes indicative of a more serious global neurology disease rather than an isolated ocular disease. The aim of this review is to highlight four important neuro-ophthalmological emergency disorders that must not be missed by an ophthalmologist. These include acute painful Horner's syndrome, painful cranial nerve III palsy, giant cell arteritis and transient ischaemic attack with amaurosis fugax. The delayed diagnosis of these clinical entities puts the patient at risk of blindness or death. Therefore, prompt diagnosis and management of these conditions are essential. This can be acquired from understanding the main signs and symptoms of the disease presentation together with a high index of suspicion while working at a busy eye emergency department.

Recurrent Vitreomacular Traction in a Patient Treated with Ocriplasmin: A Case Report.

INTRODUCTION: To describe a case of recurrent vitreomacular traction and macular edema that appeared both before and after the intravitreal injection of ocriplasmin. CASE REPORT: An 82-year-old monocular man presented with metamorphopsia and reduced vision of 1-week duration. The patient's general medical history was unremarkable. His ophthalmic history was significant for severe ocular trauma in the right eye in childhood that caused phthisis. The left eye had undergone uncomplicated phacoemulsification 3 months earlier and the 1-month postoperative best corrected visual acuity (BCVA) was logarithmic mean angle of resolution (logMAR) 0.0. There was no history of other ocular conditions. At presentation, BCVA was logMAR 0.2 and optical coherence tomography (OCT) revealed the presence of cystoid macular edema caused by vitreomacular traction (VMT). The patient was scheduled for intravitreal ocriplasmin injection. Prior to treatment, the vision improved spontaneously to logMAR 0.1, and no VMT could be detected with spectral domain (SD)-OCT. The ocriplasmin injection was deferred but 3 weeks later the patient presented again with metamorphopsia, while VMT was again evident on SD-OCT. Ocriplasmin was injected and 1 month later the BCVA reached logMAR 0.1 without VMT. However, at 2 months post injection the VMT reappeared and a conservative approach with observation and topical nepafenac administration was decided. At the 3-month post-injection visit there was no VMT. More than 3 years after the ocriplasmin injection there is still no evidence of VMT, the patient is free of metamorphopsia, and his BCVA is logMAR 0.0. CONCLUSION: Separation of consecutive layers of the vitreous cortex (vitreoschisis) may account for recurrent VMT.

Safety and Efficacy of YAG Laser Vitreolysis for the Treatment of Vitreous Floaters: An Overview.

Emerging evidence has suggested that the entoptic phenomena associated with vitreous opacities (i.e. vitreous floaters) are more bothersome than previously believed. In addition, the prevalence of vitreous floaters is likely increasing due to the evolving global pandemic of myopia. The use of YAG laser vitreolysis for the treatment of annoying vitreous floaters has attracted significant attention in recent years as the technique offers a number of potential advantages. Unfortunately, the currently available evidence that is needed to guide clinical practice is both very limited and contradictory. As a consequence, the technique remains highly controversial. A review of the existing literature sheds light on patient- and treatment-related factors that may significantly affect both the effectiveness and the safety of the procedure. The current article discusses important aspects of key publications on the topic, offers suggestions for clinical practice, and highlights unmet needs that should be addressed by future research.

Successful treatment with rituximab of IgG4-related disease coexisting with adult-onset asthma and periocular xanthogranuloma.

Immunoglobulin G4-related disease (IgG4RD) is a systemic fibro-inflammatory disease of unknown aetiology, which is characterized by tumefactive lymphoplasmatocytic infiltrative lesions, with a predominance of IgG4 positive plasma cells, fibrosis and obliterative phlebitis. Adult-onset asthma and periocular xanthogranuloma (AAPOX) is a rare disease of unknown aetiology characterized by violaceous or yellow cutaneous papules and nodules usually accompanied with adult-onset asthma. We report a case of IgG4RD associated with AAPOX. We also conducted a literature search with keywords including IgG4RD and AAPOX. A 36-year-old woman presented with bilateral exophthalmos and periorbital oedema. Keratoconjunctivitis sicca, painless left parotid gland and submandibular left lymph node enlargement were also noted. Two and half years ago biopsy of yellow plaques of her right lower eyelid confirmed periorbital xanthogranuloma. She underwent parotid gland biopsy which showed IgG4RD. She was treated with steroids and two cycles of rituximab with complete remission. The literature review revealed 8 articles describing 14 cases with IgG4RD and AAPOX, 9 men and 5 women (ratio M:F = 1.8:1) were affected. The age at diagnosis was greater in men compared to women. In the majority of patients, ophthalmic presentation included bilateral upper and lower eyelid swelling while systemic features were asthma, lacrimal and parotid involvement, lymphadenopathy, sclerosing pancreatitis and sclerosing cholangitis. Prednisone and rituximab were effective treatments. It has to be clarified whether the association between IgG4RD and AAPOX represents shared pathophysiology, a common underlying cause or coincidence. Rituximab added to steroids resulted in complete remission of the two entities.

Combined EGFR/ALK Expression Analysis in Laryngeal Squamous Cell Carcinoma.

BACKGROUND/AIM: Epidermal growth factor receptor (EGFR) acts as an oncogene in malignancies. Our aim was to examine the role of combined EGFR/ anaplastic lymphoma kinase (ALK) expression as molecular markers in laryngeal squamous cell carcinoma (LSCC) patients. MATERIALS AND METHODS: Fifty (n=50) tissue sections derived from twenty-five (n=25) primary LSCCs were analyzed by immunohistochemistry (IHC). RESULTS: EGFR overexpression was observed in 17/25 (68%) cases. Concerning ALK, 23/25 (92%) demonstrated low expression. EGFR expression was associated with grade (p=0.049), whereas ALK expression was correlated with stage (p=0.048). ALK overexpression was detected at advanced-stage EGFR-positive cases. A biphasic EGFR protein expression pattern was observed in five (n=5) LSCC cases, whereas ALK expression was stable in all cases. CONCLUSION: EGFR overexpression is frequently observed in LSCC combined with low ALK expression. LSCC patients with EGFR/ALK protein overexpression should be eligible for targeted therapeutic strategies.

Bartonella henselae- and quintana-associated uveitis: a case series and approach of a potentially severe disease with a broad spectrum of ocular manifestations.

PURPOSE: To evaluate the clinical manifestations of intraocular inflammation associated with Bartonella infection and describe the assessment and management of patients with cat-scratch disease (CSD). METHODS: This is a retrospective review of the clinical records of patients diagnosed with Bartonella henselae and Bartonella quintana intraocular inflammation from 2011 to 2018 in the Department of Ocular Inflammations and Infections of the University Eye Clinic of Ioannina (Greece). An analysis of the current literature concerning Bartonella-related intraocular infections was also carried out. RESULTS: This is a retrospective study of 13 patients (7 males and 6 females) with a mean age of 39.2 years that were diagnosed with unilateral intraocular inflammation, except one case with bilateral affection, attributed to Bartonella (either henselae or quintana). Twelve (12) patients (92.3%) had a positive history of traumatic cat contact. The main ocular clinical findings with regard to the type of uveitis included neuroretinitis in 5 eyes (38.5%), vasculitis in 3 eyes (23.1%), iridocyclitis in 2 eyes (15.4%), intermediate uveitis in 2 eyes (15.4%), posterior uveitis in 1 eye (7.7%), panuveitis in 2 eyes (15.4%), retinochoroiditis in 2 eyes (15.4%), vitritis in 1 eye (7.7%), peripheral choroidal granuloma in 1 eye (7.7%). Immunoglobulin (Ig) G was positive in all cases. All patients were treated with antibiotics (mainly rifampicin, doxycycline and azithromycin). The visual acuity was noted to be improved in all patients after treatment, but some of them experienced disturbing complications. CONCLUSION: CSD may manifest with various ocular pathological findings. Taking into consideration the increasing frequency of infections by B. henselae and B. quintana, clinicians should always incorporate CSD in the differential diagnosis of such presentations of uveitis. Educating vulnerable groups (children, immunosuppressed, etc.) and also general population, the appropriate preventing measures can contribute in limiting the risk of infection.

The treatment of amblyopia: current practice and emerging trends.

PURPOSE: The purpose of this review is to provide an update on current management and recent research for amblyopia treatment. Part I will review patching, atropine penalization, and pharmacological treatments. Part II will focus on perceptual learning, video gaming, and binocular dichoptic approaches. METHODS: A literature search was performed in PubMed, ClinicalTrials.gov , Google Scholar, and reference lists of retrieved articles until December 20, 2018, for all papers containing "amblyopia treatment" or "amblyopia therapy." We have included RCTs, prospective observational studies, prospective and retrospective cohort studies, pilot studies, and review articles. RESULTS: The mainstay of treatment for amblyopia has been based on increasing visual stimulation of the amblyopic eye by occlusion, atropine, or optical penalization of the dominant eye. It has been established that refractive adaptation alone can significantly enhance visual acuity. However, the duration of optical correction varies between studies and the effectiveness of spectacle wear over early beginning of patching is still under investigation. Additionally, by means of occlusion dose monitors, it was found that adherence to occlusion affects the outcome, as a dose-response relationship exists between adherence and visual acuity. Treatment efficiency declines with age; however, recent evidence indicates cortical plasticity beyond the "critical period" and recommends that an attempt at treatment should be offered to all amblyopic children regardless of age, including those in later childhood. Novel approaches targeted to the restoration of binocular functions, such as perceptual learning, video gaming, and dichoptic training, have shown small effects on visual acuity and have failed to demonstrate non-inferiority over standard treatments. CONCLUSIONS: On review, significant evidence for the successful management of amblyopia, with occlusion therapy and atropine, has been found. However, the management of amblyopia remains challenging, mainly due to compliance issues and suboptimal treatment outcomes during occlusion and atropine penalization. Recent studies have found evidence of new ways of treating amblyopia particularly in regard to binocular treatment although these remain under investigation. Further robust clinical trials on these new treatment modalities are still warranted in order to establish their role in treating amblyopia.

Lack of Association of the rs11655081 ARSG Gene with Blepharospasm.

Blepharospasm (BSP) is a sub-phenotype of focal dystonia. A few genetic risk factors are considered to be implicated in the risk of developing BSP. There is recent evidence, based on results from GWAS and meta-analyses, to suggest that arylsulfatase G (ARSG), and more specifically rs11655081, is implicated in focal dystonia. The aim of the present study was to evaluate the effect of rs11655081 ARSG on BSP. A Greek cohort, which consisted of 206 BSP patients and an equal number of healthy controls, was genotyped for rs11655081. Only a marginal trend for the association between rs11655081 and the risk of BSP was found in the over-dominant model of inheritance [odds ratio, OR (95% confidence interval, CI): 0.64 (0.38-1.07), p = 0.088]. It is rather unlikely that rs11655081 across ARSG is a major genetic risk contributor for BSP.

Strabismus surgery for diplopia in chronic progressive external ophthalmoplegia.

BACKGROUND: To report midterm outcomes of strabismus strategy for management of diplopia in chronic progressive external ophthalmoplegia and specific surgical planning rationale. DESIGN: Retrospective interventional case series. RESULTS: Two patients, a 26-year-old male and a 36-year-old female, diagnosed with chronic progressive external ophthalmoplegia presented with blepharoptosis and intermittent diplopia. Ocular motility examination was significant for bilateral profound impairment of adduction with relative preservation of abduction, infraduction and elevation. Control of intermittent exotropia gradually worsened over 3 and 1.5 years of follow-up, respectively, in the presence of documented stability of the angle of exodeviation. Strabismus surgery involving modest amounts of bilateral medial rectus resection and lateral rectus recessions was undertaken. Surgical intervention was successful in controlling alignment in primary position and alleviating diplopia and asthenopia after 9 and 8 years of follow-up time, respectively, despite slow progression of ophthalmoplegia. CONCLUSION: Bilateral selective impairment of adduction and intermittent exotropia may be the presenting ocular motility disturbance in chronic progressive external ophthalmoplegia. Properly designed strabismus surgery may provide sustainable, in the midterm, control of alignment and symptomatic relief in selected patients with CPEO.