Applicability and attachment findings of the Strange Situation Paradigm in infants with visual impairment.

Attachment studies with diverse populations enrich the understanding of infants' socioemotional development by documenting both universal and idiosyncratic aspects of attachment. Given the effects of attachment in children's socioemotional outcomes, such studies are necessary to investigate the impact of children's sensory impairments on attachment development. Yet, very little attachment research has focused on infants with visual impairment (VI infants), a population in which infant-caregiver emotional exchanges through visual means are reduced/absent. We investigated the applicability of the Strange Situation Paradigm (SSP), with added instructions to compensate for degraded visual input, in 20 VI infants (with no additional disabilities and who were receiving developmental counseling). In all but 1 of the SSPs coded, VI infants displayed observable attachment behavior that was classifiable. Nineteen VI infants showed attachment by 12 months of age. Across the ages tested (fractional age range = 0.9-2.33 months), most VI infants' attachment patterns were classified as secure and organized.

Los estudios sobre la afectividad con grupos diversos enriquecen la comprensión acerca del desarrollo socio-emocional de los infantes ya que documentan tanto los aspectos universales como idiosincráticos de la afectividad. Dados los efectos de la afectividad en los resultados socio-emocionales de los niños, tales estudios son necesarios para investigar el impacto que los impedimentos sensoriales de los niños tienen en el desarrollo de la afectividad. Aun así, poca investigación sobre la afectividad se ha enfocado en infantes con impedimentos visuales (infantes VI), un grupo en el cual los intercambios emocionales por medios visuales entre el infante y quien le cuida son reducidos o no existen. Nosotros investigamos la aplicabilidad del Paradigma de la Situación Extraña (SSP), con instrucciones adicionales para compensar por la debilitada interacción visual, en 20 infantes VI (que no presentaban ninguna discapacidad adicional y quienes recibían consejería sobre el desarrollo). En todos los casos codificados como SSP, excepto en uno, los infantes VI mostraron una conducta de afectividad observable la cual era clasificable. Diecinueve infantes VI mostraron afectividad para los 12 meses de edad. A lo largo de la gama de edades examinadas (promedio de edad fraccional = 0.9-2.33), la mayoría de los patrones de afectividad de los infantes VI fueron clasificados como seguros y organizados.

Les études sur l'attachement avec des populations diverses enrichissent la compréhension du développement socio-émotionnel des nourrissons en documentant à la fois les aspects universels et les aspects idiosyncratiques de l'attachement. Au vu des effets de l'attachement dans les résultats socio-émotionnels des enfants, de telles études sont nécessaires afin d'enquêter sur l'impact des déficiences sensorielles des enfants sur le développement de l'attachement. Cependant très peu de recherches sur l'attachement ont porté sur le handicap visuel des nourrissons (nourrissons VI), une population chez qui l'échange émotionnel nourrisson-personne s'occupant du nourrisson au travers de moyens visuels est réduit/absent. Nos recherches ont porté sur l'applicabilité du Paradigme de Situation Etrange (SSP), avec des directives ajoutées pour compenser la saisie visuelle dégradée, chez 20 nourrissons (sans aucun autre handicap et qui étaient en consultation pour leur développement). Dans tous sauf 1 des points SSP codés, les nourrissons VI ont fait état d'un comportement d'attachement observable qui était classifiable. Dix-neuf des nourrissons CI ont fait preuve d'attachement à l'âge de 12 mois. Au travers des âges testés (étendue fractionnelle d'âge = 0,9-2,33), la plupart des patterns d'attachement des nourrissons ont été classifiés comme étant sécures et organisés.

[National protocol for diagnosis and care of congenital aniridia: Summary for the attending physician]. / Protocole national de diagnostic et de soins (PNDS) de l'aniridie congénitale : synthèse pour le médecin traitant.

Congenital aniridia is a rare panocular disease defined by a national diagnostic and care protocol (PNDS) validated by the HAS. In most cases, it is due to an abnormality in the PAX6 gene, located at 11p13. Aniridia is a potentially blinding autosomal dominant disease with high penetrance. The prevalence varies from 1/40,000 births to 1/96,000 births. Approximately one third of cases are sporadic. Ocular involvement includes complete or partial absence of iris tissue, corneal opacification with neovascularization, glaucoma, cataract, foveal hypoplasia, optic disc hypoplasia and ptosis. These ocular disorders coexist to varying degrees and progress with age. Congenital aniridia manifests in the first months of life as nystagmus, visual impairment and photophobia. A syndromic form such as WAGR syndrome, WAGRO syndrome (due to the risk of renal Wilms tumor) or Gillespie syndrome (cerebellar ataxia) must be ruled out. Systemic associations may include diabetes, due to expression of the PAX6 gene in the pancreas, as well as other extraocular manifestations. Initial assessment is best carried out in a referral center specialized in rare ophthalmologic diseases, with annual follow-up. The management of progressive ocular involvement must be both proactive and responsive, with medical and surgical management. Visual impairment and photophobia result in disability, leading to difficulties in mobility, movement, communication, learning, fine motor skills, and autonomy, with consequences in personal, school, professional, socio-cultural and athletic life. Medico-socio-educational care involves a multidisciplinary team. Disability rehabilitation must be implemented to prevent and limit situations of handicap in activities of daily living, relying on the Commission for the Rights and Autonomy of People with Disabilities (CDAPH) within the Departmental House of People with Disabilities (MDPH). The general practitioner coordinates multidisciplinary medical and paramedical care.

[Neuro-ophthalmological conditions: Study of the clinical care pathway]. / Les affections neuro-ophtalmologiques : étude du parcours de soins du patient.

INTRODUCTION: Neuro-ophthalmologic conditions require specialized multidisciplinary management, both medical and surgical, for patients affected by visual loss due to nervous system disease. The primary goal of this study is to define the specificity of neuro-ophthalmology within the realm of visual health. The secondary goal is to review clinical care pathways by studying the organization of management, in terms of accessibility to care and personalization of the care pathway. PATIENTS AND METHODS: A field study was carried out from February to June 2015, within the ophthalmology service of the Pitié-Salpêtrière University Medical Center in Paris. A 30-minute interview with the patient before or after his or her neuro-ophthalmology consultation was performed, so as to describe the clinical care pathway. The medical records of interviewed patients were also analyzed. RESULTS: Seventeen care pathways (10 women and 7 men) were reviewed. The mean age at appearance of visual involvement was 44.5 years (±8.4 years). If we exclude 3 patients over 66 years and retired, 35.71% were active, 35.71% were disabled, and 28.57% were on sick leave. Ten patients (58.82%) met the criteria for admission to long-term care. The first step had been carried out by local private practitioners. The first physician seen was the general medicine physician (59%), then the private ophthalmologist on an emergency basis (17%). On average, patients went through 8 steps during their care pathway (from 6 to 10 steps) and 14 medical departments were involved. The study showed collaboration with the other services of the University Hospital Department of Vision and Disabilities (notably with the Fondation Rothschild, the Quinze-Vingts National Ophthalmology Hospital and the Fondation Sainte-Marie). In addition to rehabilitation services, health care professionals participating in the outpatient care of the patients included an orthoptist (11.7%), a psychologist (11.7%), and an optician specializing in low vision for visual aids. And finally, patient support groups, AFM-Téléthon (myasthenia) and the ARIBa Association (visual disability) were solicited by 2 patients for their involvement. A disturbance in activities of daily living leading to disabilities with psychological repercussions was noted by a number of patients. The most frequent complaints involve mobility (29.41%) and reading (23.52%). In total, 77% of patients state that their well-being has been affected and they are disturbed by the progression of their disease. DISCUSSION AND CONCLUSIONS: The review of the clinical care pathway of patients affected by neuro-ophthalmological conditions shows that these pathologies are, on the one hand, often poorly understood, and on the other hand complex, leading to an often significant number of steps for the patient. This also emphasizes the necessity of a care network, specialized and structured to improve the efficiency of this management. Finally, these results demonstrate the existence of a very frequent disability, which may affect all aspects of the patients' lives, highlighting the importance of rehabilitation services and individuals participating in the follow-up of these patients beyond their acute care.