Fundus Changes in the Offspring of Mothers With Confirmed Zika Virus Infection During Pregnancy in French Guiana, Guadeloupe, and Martinique, French West Indies.

Importance: Most ocular lesions have been described for children with congenital Zika syndrome. The frequency of finding ocular abnormalities is unknown among children exposed to Zika virus (ZIKV) during pregnancy. This study was conducted on newborns whose mothers were positive for ZIKV, confirmed with reverse-transcription polymerase chain reaction (RT-PCR) testing. Objective: To report ocular fundus manifestations in newborns with congenital ZIKV exposure in French Guiana, Martinique, and Guadeloupe, French West Indies, to assess its prevalence. Risk factors, such as the presence of extraocular fetopathies and the gestational term at infection, were sought. Design, Setting, and Participants: This was a cross-sectional multicentric study, conducted from August 1, 2016, to April 30, 2019, for which data were collected prospectively. The study inception was at the beginning of 2016 from the onset of the ZIKV epidemic in the French West Indies. Newborns whose mothers tested positive (by RT-PCR) for ZIKV during pregnancy were included. Interventions: Fundus examination was performed using widefield retinal imaging after pupil dilation. Infection date, delivery mode, and newborn measurements were collected. Main Outcomes and Measures: Anomalies of the vitreous, choroid, retina, and optic disc. Results: A total of 330 children (mean [SD] age, 68 [IQR, 22-440] days; 170 girls [51.5%]) were included. Eleven children (3.3%) had perivascular retinal hemorrhages, and 3 (0.9%) had lesions compatible with congenital ZIKV infection: 1 child had torpedo maculopathy, 1 child had a chorioretinal scar with iris and lens coloboma, and 1 child had a chorioretinal scar. Retinal hemorrhages were found at childbirth during early screening. Lesions compatible with congenital ZIKV infection were not associated with the presence of extraocular fetopathy. Microcephaly was not associated with lesions compatible with congenital ZIKV infection (odds ratio [OR], 9.1; 95% CI, 0.8-105.3; P = .08), but severe microcephaly was associated with an OR of 81 (95% CI, 5.1-1297.8; P = .002). Conclusions and Relevance: Results of this cross-sectional study suggest that the ocular anomalies found may be associated with ZIKV in 0.9% of the exposed population. Ocular lesions were rare, affected mostly the choroid and retina, and seemed to be associated with choroiditis-related scarring that developed during fetal growth.

Case Report: Two Cases of Keratoconjunctivitis Tied to Sargassum Algae Emanations.

Since 2011, considerable amounts of Sargassum algae regularly end up on beaches in the Gulf of Mexico, the Caribbean, and in the French overseas departments. We report observations of two bilateral keratoconjunctivitis associated with important functional symptomatology. There was a conjunctival hyperemia and superficial punctate keratitis. The ocular impairment would repeat at every algae ashore landing. Clinical examination, history, and time line of symptomatology onset allowed us to eliminate the classic etiologies of bilateral keratoconjunctivitis and to suggest an irritant toxic origin tied to hydrogen sulfide. This is the first description of ocular impairment tied to Sargassum algae decomposition. Their decomposition, through H2S emission, can be at the origin of bilateral keratoconjunctivitis. Ocular impairment is often at the forefront of complaints made by individuals exposed to H2S.

Ocular manifestations in Congenital Zika syndrome: About a case of torpedo maculopathy.

PURPOSE: To describe pertinent imaging studies and clinical features of a torpedo maculopathy presumably associated with congenital Zika syndrome. OBSERVATION: A 23-month-old child, with no prematurity or microcephaly at birth, was examined in the Ophthalmology department of the University Hospital of Fort-de-France (Martinique, French West Indies), as part of a systematic screening of malformations in children suspected of maternal-fetal exposure to Zika virus. Zika infection was confirmed in the mother's serum by Reverse Transcriptase Polymerase Chain Reaction during the third trimester of pregnancy. Fundus examination found a unilateral hypopigmented retinal lesion, temporal to the macula, with an apex pointing to the fovea. Explorations in spectral-domain optical coherence tomography showed a subretinal cleft with broadening and attenuation of the interdigitation zone, elevation of the outer limiting membrane and the ellipsoid zone, without thinning of the outer retinal layers. CONCLUSION AND IMPORTANCE: There is a proven risk of congenital eye defects after Zika infection during pregnancy. We report here the first case of torpedo maculopathy without microcephaly, in a child suspected of maternal-fetal exposure to Zika.

Fovea Plana in a 9-Year-Old Boy Presenting with Decreased Vision in the Left Eye.

BACKGROUND Foveal hypoplasia (FH) is a congenital disorder, generally associated with other conditions. CASE REPORT A 9-year-old boy presented with moderately decreased vision in the left eye. Fundus examination showed an absence of macular reflection and no foveal pit was seen on optical coherence tomography. Fluorescein angiography demonstrated the absence of a foveal avascular zone. CONCLUSIONS This is a rare case of a unilateral fovea plana associated with a visual impairment.

Temporal trends in Human T-Lymphotropic virus 1 (HTLV-1) associated myelopathy/tropical spastic paraparesis (HAM/TSP) incidence in Martinique over 25 years (1986-2010).

BACKGROUND: Human T-lymphotropic virus type 1 (HTLV-1) has been discovered in 1980 and has been linked to tropical spastic paraparesis (HAM/TSP) in 1985 in Martinique. There is no data on HAM/TSP incidence trends. We report, in the present work, the temporal trends incidence of HAM/TSP in Martinique over 25 years. METHODS: Martinique is a Caribbean French West Indies island deserved by a unique Neurology Department involved in HAM/TSP diagnosis and management. A registry has been set up since 1986 and patients diagnosed for a HAM/TSP were prospectively registered. Only patients with a definite HAM/TSP onset between 1986 and 2010 were included in the present study. The 25-year study time was stratified in five-year periods. Crude incidence rates with 95% confidence interval (95%CI) were calculated using Poisson distribution for each period. Age-standardized rates were calculated using the direct method and the Martinique population census of 1990 as reference. Standardized incidence rate ratios with 95% CIs and P trends were assessed from simple Poisson regression models. Number of HTLV-1 infection among first-time blood donors was retrospectively collected from the central computer data system of the Martinique blood bank. The HTLV-1 seroprevalence into this population has been calculated for four 5-year periods between 1996 and 2015. RESULTS: Overall, 153 patients were identified (mean age at onset, 53+/-13.1 years; female:male ratio, 4:1). Crude HAM/TSP incidence rates per 100,000 per 5 years (95%CI) in 1986-1990, 1991-1995, 1996-2000, 2001-2005 and 2006-2010 periods were 10.01 (6.78-13.28), 13.02 (9.34-16.70), 11.54 (8.13-14.95), 4.27 (2.24-6.28) and 2.03 (0.62-3.43). Age-standardized 5-year incidence rates significantly decreased by 69% and 87% in 2001-2005 and 2006-2010 study periods. Patients characteristics did not differ regarding 1986-2000 and 2001-2010 onset periods. Between 1996-2000 and 2011-2015 study periods, the HTLV-1 seroprevalence significantly decreased by 63%. CONCLUSION: Martinique faces a sudden and rapid decline of HAM/TSP incidence from 2001 in comparison to 1986-2000 periods. Reduction of HTLV-1 seroprevalence, that may result from transmission prevention strategy, could account for HAM/TSP incidence decrease.

Martinique Crinkled Retinal Pigment Epitheliopathy: Clinical Stages and Pathophysiologic Insights.

PURPOSE: To reappraise the autosomal dominant Martinique crinkled retinal pigment epitheliopathy (MCRPE) in light of the knowledge of its associated mutated gene mitogen-activated protein kinase-activated protein kinase 3 (MAPKAPK3), an actor in the p38 mitogen-activated protein kinase pathway. DESIGN: Clinical and molecular study. PARTICIPANTS: A total of 45 patients from 3 generations belonging to a family originating from Martinique with an autosomal dominant MCRPE were examined. METHODS: Best-corrected visual acuity, fundus photographs, and spectral-domain optical coherence tomography (SD OCT) of all clinically affected patients and carriers for the causal mutation were reviewed at the initial visit and 4 years later for 10 of them. Histologic retinal lesions of Mapkapk3(-/-) mice were compared with those of the human disease. MAIN OUTCOME MEASURES: The MCRPE natural history in view of MAPKAPK3 function and Mapkapk3(-/-) mouse retinal lesions. RESULTS: Eighteen patients had the c.518T>C mutation. One heterozygous woman aged 20 years was asymptomatic with normal fundus and SD OCT (stage 0). All c.518T>C heterozygous patients older than 30 years of age had the characteristic dried-out soil fundus pattern (stages 1 and 2). Complications (stage 3) were observed in 7 cases, including polypoidal choroidal vasculopathy (PCV) and macular fibrosis or atrophy. One patient was homozygous and had a form with severe Bruch's membrane (BM) thickening and macular exudation with a dried-out soil pattern in the peripheral retina. The oldest heterozygous patient, who was legally blind, had peripheral nummular pigmentary changes (stage 4). After 4 years, visual acuity was unchanged in 6 of 10 patients. The dried-out soil elementary lesions radically enlarged in patients with a preferential macular extension and confluence. These findings are in line with the progressive thickening of BM noted with age in the mouse model. During follow-up, there was no occurrence of PCV. CONCLUSIONS: MCRPE is an autosomal dominant, fully penetrant retinal dystrophy with a preclinical stage, an onset after the age of 30 years, and a preserved visual acuity until occurrence of macular complications. The natural history of MCRPE is in relation to the role of MAPKAPK3 in BM modeling, vascular endothelial growth factor activity, retinal pigment epithelial responses to aging, and oxidative stress.

Martinique (West Indies) crinkled retinal pigment epitheliopathy: clinical description.

PURPOSE: To report a previously undescribed pattern of crinkled retinal pigment epithelium (RPE), observed in a family of black patients originating from Martinique, an island in the French West Indies. METHODS: Three generations were examined by visual acuity measurement and fundus photography. Autofluorescence photography, fluorescein and indocyanine green angiography, visual field testing, electrophysiology, and spectral domain optical coherence tomography were performed in certain patients. RESULTS: One 86-year-old grandmother, her 7 children, her nephew, and 18 of her 22 grandchildren were examined. Nine patients were affected: five children, one nephew, and three grandchildren. An unrelated patient originating from the same area was also affected. In the third generation, fundus findings were whitish deep lines located in the posterior pole. Optical coherence tomography showed a crinkled pattern of a slightly elevated RPE. In the second generation, a scalloped crinkled RPE was observed in the posterior pole and midperiphery, giving an image of dry desert land in fluorescein and indocyanine green angiography. Optical coherence tomography showed that the RPE formed ripples, giving it a crinkled appearance. Complications were observed in six cases: they included RPE atrophy (one case), subretinal and sub-RPE hemorrhages because of polypoidal choroidal vasculopathy (four cases), and fibrovascular scarring (one case). The grandmother's fundi were characterized by peripheral pigmentary changes, with severe visual loss. CONCLUSION: The observed pattern appeared different from previously described dystrophies and could be referred to as Martinique crinkled retinal pigment epitheliopathy.

Outbreak of keratomycosis attributable to Fusarium solani in the French West Indies.

PURPOSE: We report an unusual increase in the number of cases of keratomycosis attributable to Fusarium solani in contact lens wearers. DESIGN: Observational case series. METHODS: Between November 2005 and May 2006, 14 corneal abscesses were reported at the University Hospital Center of Fort-de-France. Twelve patients were contact lens wearers, and 10 used Bausch & Lomb (Rochester, New York, USA) ReNu with MoistureLoc. Corneal scrapings were collected for microbiological analyses. RESULTS: In six months, five abscesses attributable to Fusarium solani in contact lens wearers, using Bausch & Lomb ReNu with MoistureLoc as contact lens solution, were reported. CONCLUSIONS: This study reports an epidemic of keratomycosis attributable to Fusarium solani among contact lens wearers in the Caribbean, during the same timeframe as a similar epidemic in Asia and North America. We are unaware of previous report of this phenomenon and could find no reference to it in a computerized search utilizing MEDLINE.

Natural history of the visual impairment of relapsing neuromyelitis optica.

PURPOSE: To describe the clinical features and natural history of the visual impairment of relapsing neuromyelitis optica. DESIGN: Prospective observational case series. PARTICIPANTS: Thirty patients of Afro-Caribbean origin with neuromyelitis optica and 47 patients with multiple sclerosis. METHODS: A complete ophthalmologic examination was performed with each ocular attack and during a remission period at least 6 months after the last attack. MAIN OUTCOME MEASURES: Incidence of unilateral and bilateral optic neuritis and incidence of unilateral and bilateral severe visual loss, defined as visual acuity (VA) < or = 20/200. RESULTS: Twenty-eight patients (93%) with neuromyelitis optica were female and 2 (7%) were male. The mean age of onset was 30+/-10.5 years. Mean disease duration was 9.5+/-5.4 years. Neuromyelitis optica first manifested by an episode of optic neuritis in 23 cases (76.6%), bilateral in 4. The average number of ocular attacks per patient was 2.7+/-1.6. Twenty-one patients (70%) showed impairment in both eyes. Median times from onset to unilateral optic neuritis, bilateral optic neuritis, mono-ocular severe visual loss, and binocular severe visual loss were 0+/-0.08, 1+/-1.6, 2+/-0.8, and 13+/-3 years, respectively. Fifteen patients (50%) experienced severe visual loss in both eyes and 6 (20%) in one eye. Severe visual loss occurred in as few as 2 attacks. CONCLUSION: This study is the largest series of relapsing neuromyelitis optica in a population of African descent. Neuromyelitis optica's visual impairment is very severe; it contrasts drastically with that typically observed in multiple sclerosis. We confirmed that the prognosis for optic neuritis in patients with neuromyelitis optica is worse than the prognosis in patients with multiple sclerosis.

Natural history of human T-lymphotropic virus 1-associated myelopathy: a 14-year follow-up study.

BACKGROUND: The progression of neurological disability in human T-lymphotropic virus 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) remains undefined. OBJECTIVES: To determine the time course of disability scores and to identify predictors of outcome among patients with HAM/TSP. DESIGN: Clinical 14-year follow-up study. SETTING: University hospital. Patients One hundred twenty-three patients with HAM/TSP. MAIN OUTCOME MEASURES: We determined time from onset to the following 4 Kurtzke Disability Status Scale (DSS) end points: scores of 6 (unilateral aid required), 6.5 (bilateral aid required), 8 (wheelchair confinement), and 10 (death related to the disease). Times to reach selected DSS scores were estimated using the Kaplan-Meier method. Univariate and multivariate analyses identified variables related to the rate of progression to DSS 8. The HTLV-1 proviral loads were also assessed. RESULTS: The disability of the cohort progressed throughout the follow-up period. The median times from onset to DSS 6, 6.5, and 8 were 6, 13, and 21 years, respectively. The median time from DSS 6 to DSS 8 was 8 years; DSS 10 was reached by one fourth of the patients within 20 years. Age at onset of 50 years or older and high HTLV-1 proviral load were associated with a shorter time to DSS 8 (P = .01 and P = .02, respectively). A shorter time to DSS 6 significantly adversely affected the time to progression from DSS 6 to DSS 8. CONCLUSIONS: Human T-lymphotropic virus 1-associated myelopathy/tropical spastic paraparesis is a rapidly disabling disease. Monitoring for HTLV-1 proviral load is recommended in future therapeutic trials.

Occipital infarction revealed by quadranopsia following snakebite by Bothrops lanceolatus.

We report a case of snakebite in which envenomation was manifested through impairment of the visual field. The patient, a 46-year-old man, was bitten on the right thumb by Bothrops lanceolatus. Treatment with a specific equine antivenom (Bothrofav) was administered one hour after the bite. With the exception of fang marks, the results of a clinical examination, particularly the neurologic component, were normal. The day after the bite, the patient developed an inferior left lateral homonymous quadranopsia with macular epargne. T2 magnetic resonance imaging showed a right occipital infarction. His condition improved clinically and biologically. This observation of snakebite is the first in which envenomation was accompanied exclusively by an impairment of the visual field. Envenomation by B. lanceolatus is distinct in its incidence of significant thrombotic complications at a distance from the site of the bite.

Alkali ocular burns in Martinique (French West Indies) Evaluation of the use of an amphoteric solution as the rinsing product.

PRECIS: During the 4 years of this study, we noted 66 cases of alkali ocular burns, or approximately 16 cases per year, nearly half (45.5%) of which are due to an assault. For grade 1 and 2 burns the time elapsed to reepithelialization appears to be shorter when rinsed with Diphoterine* versus physiological solution. PURPOSE: Comparison of the effectiveness of two rinsing solutions for emergency use: a physiological solution and an amphoteric solution (Diphoterine*, Laboratories Prevor, Valmondois, France). Description of the clinical and progressive characteristics of alkali burns treated at the University Hospital Center of Fort de France in Martinique (French West Indies). DESIGN: Prospective consecutive observational case series and nonrandomized comparative study. PARTICIPANTS: Sixty-six patients were included. The total number of burned eyes is 104. Forty-eight eyes (46%) were rinsed with physiological solution and 56 eyes (54%) with Diphoterine*. METHODS: All patients benefited from an ocular rinse with 500ml of physiological solution or Diphoterine*, followed by a complete ophthalmologic exam. The ocular injuries were classified according to the Roper-Hall modification of the Hughes classification system. The same standardized therapeutic protocol was applied and adapted to the seriousness of the burn. MAIN OUTCOME MEASURES: Demographic data, time to corneal reepithelialization, final best corrected visual acuity and complications were analysed. RESULTS: Twenty-eight (42.4%) patients have a unilateral burn and 38 (57.6%) patients have bilateral burns. In decreasing order of frequency, the circumstances surrounding the injury are: assaults in 45.5% of cases (n=30), work-related accidents in 32% of cases (n=31), and domestic accidents in 23% of cases (n=15). For grade 1 and 2 burns the time elapsed to reepithelialization appears to be shorter when rinsed with Diphoterine* versus physiological solution (respectively): 1.9+/-1 days versus 11.1+/-1.4 days (p=10(-7)) and 5.6+/-4.9 days versus 10+/-9.2 days (p=0.02). For grade 3 and 4 burns, there are complications in 11 cases (11.6%): 8 corneal opacities and 3 perforations. CONCLUSIONS: This study is the first conducted in humans that takes into account the type of ocular rinse product used in the progressive follow-up study of injuries. The time elapsed to reepithelialization is shorter with Diphoterine* for grade 1 and 2 burns. There are not enough cases of grade 3 and 4 burns to make a conclusion. Diphoterine* seems very effective in terms of its mechanism of action and the experimental and clinical results.

Ocular lesions in 200 patients infected by the human T-cell lymphotropic virus type 1 in martinique (French West Indies).

PURPOSE: To describe the ophthalmologic features observed in patients infected by the human T-cell lymphotropic virus, type 1 (HTLV-1) in Martinique (French West Indies). DESIGN: Prospective consecutive observational case series. METHODS: A complete ophthalmic examination was performed. PATIENTS: Of 200 patients infected by HTLV-1, 77 (38.5%) were seropositive and 123 (61.5%) had HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). RESULTS: Uveitis was found in 29 cases (14.5%). Symptoms were mild and the uveitis had little effect on visual function. Ten cases of uveitis were discovered through a systematic examination and had no ocular symptoms. Most of the uveitis was anterior or intermediate. The lesions responded to corticosteroid therapy, but tended to recur. Keratoconjunctivitis sicca existed in 74 patients (37%), accompanied by lymphoplasmocytoid infiltration of the secondary salivary glands rated 3 or 4 on the Chisholm scale in nearly 50% of cases. Corneal alterations were observed in 20 cases (10%), and alterations in the retinal pigment epithelium in 3 cases. CONCLUSION: The three types of ocular affections seen most frequently were uveitis, keratoconjunctivitis sicca, and interstitial keratitis. In patients with HAM/TSP, uveitis was more frequent among younger patients, patients with earlier onset of HAM/TSP, and patients with severe motor disability. Because uveitis is related to a high intrathecal production of immunoglobulin, it could represent a marker for severity of HTLV-1 infection with respect to the course of HAM/TSP. The sicca syndrome related to HTLV-1 virus differs from primary or secondary Sjögren syndrome, because it does not reveal any of the immunologic anomalies generally seen in this disease. Interstitial keratitis was more frequent among patients with HAM/TSP who had high proviral DNA levels.

Intraocular penetration of toxic substances

PURPOSE: The seriousness of ocular alkali burns has been linked to the rapidity with which the alkali enters the eye. The authors report the results of an experimental study on intraocular ammonia penetration. MATERIALS AND METHODS: 23 eyes of New Zealand albino rabbits were burned by applying for one minute 100 ul of a solution titrating 15.3 percent of ammonia. A pH meter probe was inserted into the anterior chamber beforehand to permit pH measurements every 5 seconds. Experiments were carried out after 1,3,5,10 and 30 minutes. The ammonia concentration was measured at the end of the experiment by puncturing the anterior chamber. RESULTS: The pH increased 1 to 3 minutes after applying ammonia on the cornea, reaching a maximum (mean) of 10 five to six minutes later, before an exponential decrease. After 30 minutes, the pH was always more than the physiological pH. The penetration ratio of ammonia through the cornea is about 11 percent. At 30 minutes, the concentration of ammonia is low. The pH observed differs from the calculated pH. DISCUSSION: The difference between observed and calculated pH indicates increases with an intervening plateau, showing the existence of two successive acid-base chemical reactions between ammonia and two types of acid. In addition, the amount of protein destroyed by ammonia can be calculated. CONCLUSIONS: These data show for the time the possibility of calculating the density of proteins destroyed in vivo by a base penetrating the anterior chamber. This opens the possibilty of interesting research work, because it is possible to relate the density of proteins destroyed in the eye to the pK of this base, and to forecast the potential danger of a base of biological tissues.(AU)

Ocular ammonia burns: treatment

PURPOSE: The authors present the results of an experimental study to support the proposal for a delay in ocular bathing in the treatment of severe ocular burns due to ammonia. This study compares two solution of ocular wash: physiological serum and Diphoterine. MATERIALS AND METHODS: 23 eyes of New Zealand albino rabbits were burned for one minute by 100 ul of a solution containing 15.3 percent ammonia. Then, each eye was washed by an ocular wash of 250 ml of physiological serum, or of 250 ml of Diphoterine, after a delay of 1,3,5,10 and 30 minutes. The effects were appraised by measuring the anterior chamber, the concentration of ammonia in the anterior chamber, and by the cytopathological analysis of these burned corneas. RESULTS: An ocular wash with Diphoterine in the first few minutes following an ocular burn induces an inflexion of the pH graph, contrary to an ocular wash with physiological serum. On the other hand, there is no inflexion of the pH graph at 30 minutes, and the concentration of ammonia in the anterior chamber is low at that time. The cytopathological analysis shows stromal oedema with the ocular wash by physiological serum, but not when Diphoterine ocular wash is used. DISCUSSION AND CONCLUSIONS: This study proves the importance of ocular bathing in the first minutes following an ocular burn by ammonia. The efficacy of an external ocular wash by Diphoterine features; the importanve of sequelae has been linked to the initial stromal oedema.(AU)

Ocular alkali burns: limbal autograft

The authors present five patients treated with limbal autograft for serious recent ocular alkali burns, in four of whom it has permitted healing of recurrent corneal ulcers or the reduction in the period of conjunctival recovery. Functional amelioration is also important and this intervention has permitted a useful visual acuity. One case was complicated by bacterial keratitis, but our experience has confirmed the efficacy of the technique for managing surface ocular disorders. However there have been few reports hitherto of the use of limbal autografts for serious ocular burns, and our study has demonstrated its usefulness in this setting and the appropriate timing. VIDEOTAPES(U-Matic or VHS): The authors report their surgical technique of limbal autograft use in recent and severe ocular burn, a modification of Kenyon and Tseng's technique. The physical signs used as indicators for surgery, the selection of the limbal region and methods of following the graft, are presented.(AU)