[Paramedian frontal clefts or superior orbital clefts]. / Les fentes frontales paramédianes ou orbitaires supérieures.

Superior orbital frontal clefts are one of the rare craniofacial clefts described by Tessier in 1976, and occur most often sporadically. They are numbered 9, 10 and 11 in this classification, and are located respectively laterally, in the middle and medially to the upper part of the orbit. Their clinical expression is variable on soft tissue and bone, with possible dissociation of involvement. They range from a simple aesthetic defect to an eyes functional prognosis. CT scans are systematically required in this context. Their management must be adapted to the polymorphism of the damage, and is based on multidisciplinary approach. In case of ocular risk, the eyelid reconstruction is an emergency. In all other cases, treatment is deferred, but must be carried out at an early stage to ensure the child's healthy development.

[Combined vitrectomy-lensectomy surgery with bag-in-the-lens or lens in the bag implantation: comparison of final visual acuity]. / Chirurgie combinée vitrectomie et phakoéxérèse avec implantation bag-in-the-lens (BIL) ou de sac : comparaison de l'acuité visuelle finale.

INTRODUCTION: Combined vitrectomy-lensectomy surgery is a safe and effective procedure. Nevertheless, it is frequently complicated by posterior capsule opacification and the formation of posterior synechiae. These complications can be avoided by placing a "bag in the lens" (BIL) implant. The objective of this study is to compare the visual acuity gain (VA) after combined vitrectomy-lensectomy surgery between a group implanted with the BIL technique and a group with implantation in the bag (LIB). MATERIAL AND METHODS: We included in the study all vitrectomy-lensectomy procedures for epiretinal membrane and vitreomacular traction performed between May 2013 and July 2016 at the Hospital and University Center of Caen. We compared the VA gain between the BIL group and the LIB group six months after surgery. RESULTS: A total of 33 patients were included in the study, consisting of 28 eyes in the BIL group and 8 eyes in the LIB group. The mean VA gain in the BIL group was -0.52 LogMAR (P<0.0001) and -0.56 LogMAR (P=0.0047) for the LIB group. The difference between the two groups was not significant (P=0.74). CONCLUSION: The use of the BIL technique during vitrectomy-lensectomy allows visual recovery as good as implantation within the capsular bag. In addition, this implant has the advantage of significantly reducing the occurrence of posterior synechiae and preventing anterior and posterior capsular proliferation.

Sclerotic scatter.

Sclerotic scatter involves the scattering of incident light by the limbal sclera followed by entry of part of the scattered light into the cornea, where some of the light travels through total internal reflection to the other side, where it scatters a second time in the limbal sclera. It is then visible in the form of a limbal scleral arc of light. Sclerotic scatter has been used for decades to spot and delineate corneal opacities, which disrupt and scatter the light travelling through total internal reflection. To implement the technique, the slit beam and the binoculars of the slit lamp should be dissociated so that the limbal sclera is illuminated, while the binoculars are centered on the cornea. The technique does not provide any information as to the depth of corneal opacities and therefore needs to be complemented by direct illumination. The second sclerotic scatter may also be used clinically, for instance for diode cycloablation, the posterior part of the arc of light projecting 0.5mm behind the scleral spur. This article aims to describe the phenomenon of sclerotic scatter, explaining how the slit-lamp should be set to use this technique, describing its clinical applications (in the opacified cornea and in the normal sclera), showing that the limbal scleral arc of light of sclerotic scatter may be seen under certain circumstances in daily life with the naked eye and, finally, explaining how the arc of light differs from peripheral light focusing ("Coroneo effect").

Beyond-the-edge proliferation after relaxing retinectomy.

PURPOSE: To report beyond-the-edge proliferation (BTEP) after relaxing retinectomies (RR) i.e. fibrous sheets stretched between the RR edge and the far periphery; to evaluate the tractional potential and report the long-term course of BTEP. METHODS: Retrospective review of the medical records of 83 patients having undergone a RR between January 2009 and December 2014 to identify patients with BTEP. RESULTS: Six patients aged 31 to 76 were identified. Retinectomy had been performed for traumatic retinal incarceration in one case and anterior PVR in 5 cases. BTEP occurred within weeks of the RR (earliest: 5 weeks). It was discovered intraoperatively in two patients with silicone oil tamponade, at 7 weeks and 6 months respectively after RR. It recurred over a few months after excision in 5 patients, causing inferior tractional retinoschisis in 4 patients and inferior tractional retinal detachment in two patients. CONCLUSIONS: BTEP is an unusual form of proliferative vitreoretinopathy developing despite the absence of the usual vitreo-retinal support (excised during RR), probably through compartmentalization and cell migration along the inferior interface between silicone oil or gas and the aqueous humour. BTEP can cause serious retinal traction, develops over weeks after the RR and recurs frequently a few months after excision.

[Neurotrophic keratitis after vitrectomy and circumferential endophotocoagulation for retinal detachment]. / Kératite neurotrophique après chirurgie du décollement de rétine par vitrectomie et endophotocoagulation circonférentielle.

INTRODUCTION: Circumferential (360°) endophotocoagulation is frequently implemented during vitrectomies for retinal detachment. This photocoagulation may result in neurotrophic keratitis by damaging the ciliary nerves in the suprachoroidal space on their way to the pupil. We report a series of 4 cases of neurotrophic keratitis following a circumferential endophotocoagulation. PATIENTS AND METHODS: A retrospective observational case series of 4 non-diabetic patients having presented with a neurotrophic keratitis following a retinal detachment treated with vitrectomy and circumferential endophotocoagulation (532 nm) at Caen University Hospital. We report the various forms of corneal lesions and the diagnostic criteria allowing for the diagnosis of neurotrophic keratitis. DISCUSSION: Neurotrophic keratitis is caused by lesions occurring at various levels of corneal innervation. Endophotocoagulation may cause a neurotrophic keratitis by damaging the short and long ciliary nerves on their way to the pupil in the suprachoroidal space. The sequelae of this condition can limit visual recovery. Hence, it is probably advisable to screen for corneal anesthesia or severe hypesthesia following a retinal detachment treated with vitrectomy and circumferential endophotocoagulation and to implement prophylactic treatment (intensive lubricant therapy; preservative-free eye drops) if needed. CONCLUSION: The risk of neurotrophic keratitis should be weighed against the dose of laser retinopexy necessary and sufficient to obtain a sustained retinal reattachment. If circumferential endophotocoagulation is implemented, it is probably sensible to monitor corneal sensitivity and to adapt postoperative treatment if necessary.

[Are the ophthalmology items of the French health record realistic?]. / Les items d'ophtalmologie du carnet de santé sont-ils réalistes ?

PURPOSE: Since 2006, French general practitioners have had to carry out an ophthalmological screening for children, using a methodology contained within the child's health record (carnet de santé). The purpose of this screening is to allow an early diagnosis of sight-threatening as well as potentially life-threatening conditions (e.g., retinoblastoma). The aim of the present study was to evaluate the theoretical knowledge and the clinical skills of practitioners implementing the screening, with regards to two specific items of the health record screening schedule, by means of a questionnaire. MATERIAL AND METHODS: From December 2013 to September 2014, 42 practitioners (16 general practitioners, 14 pediatricians, 12 resident physicians) from four distinct locations in Normandy, France, were questioned using a questionnaire on two ophthalmological items of the French health record: one on red reflex and one on eyeball size. RESULTS: Only four of 42 practitioners provided correct answers on the red reflex color. Thirty of 42 practitioners reported difficulties performing the red reflex test, mostly because they did not know the normality criteria and the purpose served by this test. Thirty-three of 42 practitioners declared that they found it difficult to know whether or not eyeball sizes were normal, mostly because they were unaware of the normality criteria. None of the practitioners provided the correct (to within ± 1 mm) mean horizontal cornea diameter in full-term newborns and adults. CONCLUSION: This study demonstrates that there is poor theoretical knowledge and a low level of clinical skill for practitioners performing the ophthalmological screening based on the French health record schedule. This schedule therefore does not seem to meet its purpose.

[Serious adverse side effects after pupillary dilation in preterm infants]. / Effets indésirables graves liés à la dilatation pupillaire chez le prématuré.

PURPOSE: This study aimed at investigating serious side effects of the pupillary dilation protocol used in Caen University Hospital for the screening of retinopathy of prematurity. This protocol includes one drop of phenylephrine 5% and two drops of tropicamide 0.5% instilled at a 5-minute interval. PATIENTS AND METHODS: This retrospective study included all premature infants with a birth weight less than or equal to 1500 g and/or a gestational age less than or equal to 30 gestational weeks, hospitalized in the neonatal intensive care unit of Caen University Medical Center, having ocular fundus examinations for retinopathy of prematurity screening between 2009 and 2014. The medical records of patients who died or developed necrotizing enterocolitis were reviewed to analyze the imputability of the two eye drops used for pupil dilation. RESULTS: Five-hundred and twelve infants were included, corresponding to 1033 ocular fundus examinations. No case of death could be ascribed to the use of eye drops. Two cases of necrotizing enterocolitis could be ascribed to the use of tropicamide with a doubtful and plausible intrinsic imputability according to French imputability criteria. CONCLUSION: The pupillary dilation protocol used in Caen University Hospital for screening of retinopathy of prematurity might be implicated in two cases of necrotizing enterocolitis with an uncertain imputability of tropicamide 0.5% eye drops. No serious side effect could be ascribed to the use of phenylephrine 5% eye drops in this study.

Cerebral venous sinus thrombosis: a case series including thrombolysis.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) in children is associated with a high incidence of serious morbidity and mortality. The presenting features are variable. It can be diagnostically challenging and the optimal treatment is uncertain. AIM: To describe the features of a series of children with CVST treated in a single paediatric neurology centre and to discuss the role of local thrombolysis. METHODS: Electronic databases were searched using diagnostic labels and International Classification of Diseases (ICD) codes to identify children aged 1 month to under 17 years with CVST. Their records were reviewed. RESULTS: 21 children were identified over a period of 8.25 years with a median age of 7.1 years. The presenting symptoms included headache (15 children), vomiting (14 children) and visual disturbance (eight children). Signs found included papilloedema (16 children), fever (six children) and sixth nerve palsy (six children). The most common underlying condition was middle ear infection (13 children). All cases received unfractionated heparin and four severe cases received local pharmacological thrombolysis. 48% of cases had an adverse outcome (death, chronic intracranial hypertension, residual hemiparesis or sixth nerve palsy). DISCUSSION: CVST has non-specific presenting features and a high risk of significant morbidity. CVST is typically found in association with a predisposing condition. Although heparin is the mainstay of treatment, thrombolysis may reverse deterioration as seen in three cases in this series. However, there is insufficient evidence to recommend the routine use of thrombolysis at present.

Is hypsarrhythmia a form of non-convulsive status epilepticus in infants?

BACKGROUND: Hypsarrhythmia is generally associated with infantile spasms, a combination referred to as West syndrome. It is debatable whether hypsarrhythmia is usefully regarded as a form of non-convulsive status epilepticus (NCSE). SUMMARY POINTS: The earliest English language description of hypsarrhythmia reported an almost continuous EEG pattern, although later studies showed a degree of state dependence. Its principal features are very high amplitude and irregular slow waves with superimposed multifocal epileptiform discharges. Paroxysms of spasms are clearly overt seizure events, and there are variable EEG patterns associated with this ictus. There remains a debate about the definitional boundaries of hypsarrhythmia, and about the defining characteristics of NCSE. There is evidence that hypsarrhythmia is an age-dependent EEG pattern that evolves, sometimes independently of clinical features. Frequently, hypsarrhythmia is associated with delay in or regression of neuro-developmental skills, and recent studies have reported that a longer lead time to diagnosis and effective treatment is associated with poorer long-term neuro-developmental outcomes. Recent consensus definitions and classifications of NCSE have suggested boundaries that permit inclusion of hypsarrhythmia as an EEG pattern of NCSE. In practice, adopting the idea that hypsarrhythmia is a form of NCSE might lead to earlier appropriate investigation of infants with subtle developmental delay or regression, hence avoiding treatment delays and potentially preserving developmental potential.

Is hypsarrhythmia a form of non-convulsive status epilepticus in infants?

BACKGROUND: Hypsarrhythmia is generally associated with infantile spasms, a combination referred to as West syndrome. It is debatable whether hypsarrhythmia is usefully regarded as a form of non-convulsive status epilepticus (NCSE). SUMMARY POINTS: The earliest English language description of hypsarrhythmia reported an almost continuous EEG pattern, although later studies showed a degree of state dependence. Its principal features are very high amplitude and irregular slow waves with superimposed multifocal epileptiform discharges. Paroxysms of spasms are clearly overt seizure events, and there are variable EEG patterns associated with this ictus. There remains a debate about the definitional boundaries of hypsarrhythmia, and about the defining characteristics of NCSE. There is evidence that hypsarrhythmia is an age-dependent EEG pattern that evolves, sometimes independently of clinical features. Frequently, hypsarrhythmia is associated with delay in or regression of neuro-developmental skills, and recent studies have reported that a longer lead time to diagnosis and effective treatment is associated with poorer long-term neuro-developmental outcomes. Recent consensus definitions and classifications of NCSE have suggested boundaries that permit inclusion of hypsarrhythmia as an EEG pattern of NCSE. In practice, adopting the idea that hypsarrhythmia is a form of NCSE might lead to earlier appropriate investigation of infants with subtle developmental delay or regression, hence avoiding treatment delays and potentially preserving developmental potential.

Wheeze associated with prenatal tobacco smoke exposure: a prospective, longitudinal study. ALSPAC Study Team.

AIMS: To determine whether maternal smoking during pregnancy is a risk factor for reported wheeze in early childhood that is independent of postnatal environmental tobacco smoke (ETS) exposure and other known risk factors. METHODS: A total of 8561 mothers and infants completed questions about smoking during pregnancy, ETS exposure, and the mother's recall of wheeze during early childhood. RESULTS: A total of 1869 (21.8%) children had reported wheeze between 18 and 30 months of age, and 3496 (40.8%) had reported wheeze in one or more of the three study periods (birth to 6 months, 6-18 months, 18-30 months). The risk of wheeze between 18 and 30 months of age was higher if the mother smoked during pregnancy. This relation did not show a dose-response effect and became less obvious after adjustment for the effects of other factors. Average daily duration of ETS exposure reported at 6 months of age showed a dose-response effect and conferred a similar risk of reported wheeze. Factors associated with early childhood wheeze had the following adjusted odds ratios: maternal history of asthma 2.03 (1.74 to 2. 37); preterm delivery 1.66 (1.30 to 2.13); male sex 1.42 (1.28 to 1. 59); rented accommodation 1.29 (1.11 to 1.51); and each additional child in household 1.13 (1.04 to 1.24). CONCLUSIONS: Maternal smoking during pregnancy may be a risk factor for reported wheeze during early childhood that is independent of postnatal ETS exposure. For wheeze between 18 and 30 months of age, light smoking during the third trimester of pregnancy appears to confer the same risk as heavier smoking.