Gyrate atrophy of the choroid and retina (GACR) is a rare autosomal recessive disease characterised by elevated plasma ornithine levels due to deficiency of the enzyme ornithine aminotransferase (OAT). The accumulation of this amino acid in plasma leads to the development of patches of chorioretinal atrophy in the peripheral retina extending into the macular area. Patients usually present with night blindness followed by constriction of the visual field and, finally, decreased central vision and blindness. The disease is diagnosed by the presence of the characteristic clinical picture, the presence of hyperornithinaemia in plasma and the detection of mutations in the OAT enzyme gene. There is currently no effective gene therapy and the most common therapeutic intervention mainly involves dietary modifications with arginine restriction. This article aims to summarise the pathogenesis, clinical and diagnostic findings and treatment options in patients with GACR.

OBJECTIVE: This study reports visual health during the COVID-19 pandemic in 2021 in Spain and Portugal, focusing on eye complaints and population habits.
METHODS: Cross-sectional survey through an online email invitation to patients attending ophthalmology clinics in Spain and Portugal from September to November 2021. Around 3833 participants offered valid anonymous responses in a questionnaire.
RESULTS: Sixty percent of respondents identified significant discomfort related to dry eye symptoms for increased screen time and lens fogging using facemasks. 81.6% of the participants used digital devices for more than 3 h per day and 40% for more than 8 h. In addition, 44% of participants referred to worsening near vision. The most frequent ametropias were myopia (40.2%) and astigmatism (36.7%). Parents considered eyesight the most important aspect of their children (87.2%).
CONCLUSIONS: The results show the challenges for eye practices during the initial COVID-19 pandemic. Focusing on signs and symptoms that lead to ophthalmologic conditions is an essential concern, especially in our digital society highly dependent on vision. At the same time, the excessive use of digital devices during this pandemic has aggravated dry eye and myopia.

BACKGROUND: Keratoplasty in the pediatric population has been considered a high-risk procedure.
OBJECTIVE: To know the demographic data of patients younger than 18 years treated with optical keratoplasty, indications for surgery and its results.
METHODS: A retrospective study was carried out at Conde de Valenciana Ophthalmology Institute, in which the medical records of patients younger than 18 years treated with optical keratoplasty from 2009 to 2019 were analyzed.
RESULTS: A total of 53 eyes were included. The most common diagnosis and procedure were keratoconus and penetrating keratoplasty, respectively. Mean initial visual acuity was 2.05 ± 0.99 logMAR, and 0.82 ± 1.33 at last visit. Survival time was 130.34 months. Four failures were recorded. As for associated surgeries, corneal wound closure was recorded in seven eyes prior to transplant; during the keratoplasty procedure, anterior vitrectomy in two eyes, and after the transplant, Ahmed valve implantation and re-suture. A total of eight eyes had glaucoma, five of them diagnosed prior to transplantation. Regarding rejection, 15 eyes had an episode during follow-up, and mean time to transplant rejection was 10.8 months.
CONCLUSIONS: Prolonged graft survival can be achieved with better knowledge and management of different associated factors.
BACKGROUND: La queratoplastia en población pediátrica ha sido considerada un procedimiento de alto riesgo.
OBJECTIVE: Conocer datos demográficos de pacientes menores de 18 años tratados con queratoplastia óptica, indicaciones de la cirugía y sus resultados.
UNASSIGNED: Se realizó un estudio retrospectivo en el Instituto de Oftalmología Conde de Valenciana, en el que se revisaron expedientes de pacientes menores de 18 años tratados con queratoplastia óptica de 2009 a 2019.
RESULTS: Un total de 53 ojos fueron incluidos. El diagnóstico y el procedimiento más comunes fueron el queratocono y la queratoplastia penetrante. El promedio de agudeza visual inicial fue de 2.05 ± 0.99 logMAR y en la última visita, 0.82 ± 1.33. El tiempo de supervivencia del injerto fue de 130.34 meses. Se registraron cuatro fallas. En cuanto a las cirugías asociadas, antes del trasplante se registró cierre de herida corneal en siete ojos y durante la queratoplastia, vitrectomía anterior en dos ojos y después del trasplante, implante de válvula de Ahmed y resutura. Un total de ocho ojos tuvieron glaucoma, cinco con diagnóstico antes del trasplante. Respecto al rechazo, 15 ojos tuvieron un episodio durante el seguimiento y el tiempo promedio de rechazo al trasplante fue de 10.8 meses.
UNASSIGNED: Se puede lograr una supervivencia prolongada del injerto con el mejor conocimiento y manejo de los diferentes factores asociados.

OBJECTIVE: Digital evolution represents an opportunity for ophthalmology to adapt to new care models. This study aimed to find out how the pandemic has modified the clinical practice and training activities of the ophthalmologist specialised in ocular surface, as well as to analyse emerging trends and needs.
METHODS: This study was carried out through an online survey. A committee of 3 specialists developed a questionnaire of 25 questions structured in: 1) Participant profile; 2) Impact of the pandemic on patient management and professional activities; 3) Trends and needs.
RESULTS: 68 clinical ophthalmologists participated. There was a high degree of agreement (90%) that the pandemic has delayed ophthalmological follow-up visits and diagnosis. The participants agreed that the frequency of patients with dry eye disease (75%), stye/chalazion (62%) and blepharitis (60%) has increased. According to 28%, remote monitoring of pathologies such as dry eye, glaucoma, diabetes, conjunctivitis, hyposphagmas, styes, etc., will be common, especially in the young population. This will be especially relevant in chronic or mild pathologies of the ocular surface, and in the follow-up of patients after cataract and diabetic retinopathy interventions.
CONCLUSIONS: During the pandemic, an increase in the incidence of certain ocular surface diseases has been perceived. The telematic follow-up of chronic or mild pathologies of the ocular surface entails the need to provide specific training for both the patient and the healthcare professional, in addition to screening and referral protocols that would optimise the flow of care.

OBJECTIVE: To identify clinical and epidemiological characteristics of patients with peripheral facial palsy (PFP) at a tertiary care hospital.
METHODS: This is a retrospective observational study of patients with PFP treated at a tertiary medical center. We gathered demographic data, etiology, laterality, recurrence, recovery, clinical ophthalmology, severity according to the House-Brackmann (HB) scale, electrophysiological tests, medical services attended, medical and surgical treatment.
RESULTS: Two hundred and eighty-three PFP were included, 135 (48%) were men and 148 (52%) were women p = 0.47). All patients had unilateral involvement. The mean age was 54 ± 20 years. The main etiology was idiopathic in 215 (76%) patients. Median recovery time was 7 weeks. Recovery was complete in 190 (67%) patients. One hundred and seventy (84%) patients with idiopathic PFP had complete recovery, versus 30 (16%) patients with non-idiopathic PFP (p < 0.01). The 84% of patients with HB grade II, recovered completely, while with HB grade VI only 17% recovered (p = 0.003). Two hundred and twenty-nine patients (81%) had lagophthalmos. The majority received ocular surface care treatment in 271 (96%) patients and of these 249 (88%) patients received oral corticosteroid therapy. Thirteen patients (5%) required ophthalmologic surgery.
CONCLUSIONS: PFP affects all age ranges, without predilection for sex and unilateral. Its main cause is idiopathic. Recovery is complete in most cases, being more favorable in mild and idiopathic affections. Most only require medical treatment.

Ophthalmological complications are uncommon in dermatologic surgery. Nonetheless, all surgeons should know the basics of recognizing, preventing, and treating the 4 complications addressed in this article from the series \'Safety in Dermatologic Procedures\'. The first complication that surgeons should be familiar with is eye damage due to chemical irritants. This is a common complication in operating rooms given the presence of irritant substances and the performance of procedures in the eyebrow and eyelid region. The second complication is laser-induced eye damage. In this case, eye protection with safety glasses or eye caps is crucial. The third complication is accidental eyeball perforation, which can occur during certain surgical procedures. The fourth and final complication is retinal artery vasospasm or embolism due to drugs or filler materials. This complication is rare but important to recognize, as early treatment can prevent permanent blindness.

Ophthalmological complications are uncommon in dermatologic surgery. Nonetheless, all surgeons should know the basics of recognizing, preventing, and treating the 4 complications addressed in this article from the series \'Safety in Dermatologic Procedures\'. The first complication that surgeons should be familiar with is eye damage due to chemical irritants. This is a common complication in operating rooms given the presence of irritant substances and the performance of procedures in the eyebrow and eyelid region. The second complication is laser-induced eye damage. In this case, eye protection with safety glasses or eye caps is crucial. The third complication is accidental eyeball perforation, which can occur during certain surgical procedures. The fourth and final complication is retinal artery vasospasm or embolism due to drugs or filler materials. This complication is rare but important to recognize, as early treatment can prevent permanent blindness.

OBJECTIVE: In 2013 we implemented an asynchronous telemedicine circuit for the diagnosis of eyelid diseases (tele-eyelid), connecting the outpatient primary healthcare with the hospital\'s specialists. The purpose of this study is to assess the use of telemedicine in the diagnosis of eyelid diseases by primary care teams, to evaluate its usefulness and to analyse the epidemiology of the pathological conditions referred to the tertiary level hospital, as well as the need for surgery.
METHODS: This study was carried out in the Spanish public health system, in a mainly rural area assisted by the Institut Català de la Salut (ICS) and Althaia Xarxa Assistencial Universitària de Manresa. This is a retrospective, descriptive analysis of the telematic consultations undertaken between 2013 and 2019. The consultations between 2018 and 2019 underwent a further descriptive retrospective-prospective analysis to assess the conditions referred to the hospital.
RESULTS: Unnecessary referrals were avoided in 72% of telematic consultations. More than 50% of primary care practitioners used tele-eyelid. Up to 68% of the referrals were due to eyelid tumours, 50% needed surgery and 18%, a biopsy. Moreover, we found a high reliability between telematic and face-to-face diagnosis.
CONCLUSIONS: Teleophthalmology applied to eyelid pathology is a useful tool to improve access to specialized care and helps solving pathological conditions. It avoids unnecessary consultations and increases efficiency, both in primary and hospital care.

Medical history of 6 patients diagnosed with Williams-Beuren Syndrome (SWB) in pediatric age was revised. All the patients presented characteristic elf facies and cardiovascular abnormalities. All presented good visual acuity, except one case of unilateral amblyopia. The most frequent refractive error was hyperopia (n = 6; 100%) and astigmatism (n = 5; 83.3%). Ocular motility alterations were found in 2 patients (1 case of exophoria with hyperfunction of right inferior oblique and another of congenital endotropia with bilateral hyperfunction of inferior oblique). On the cognitive function, 66.7% (n = 4) had visoperceptive disorders. Other findings were epicanthus (n = 6; 100%) and congenital obstruction of the nasolacrimal duct with unilateral epiphora (n = 1; 16.7%). SWB is a rare disorder with complex ophthalmological and systemic manifestations. For this reason, ophthalmological follow-up of these children is recommended.

OBJECTIVE: To identify the multidisciplinary uveitis units in which internal medicine departments participate in collaboration with ophthalmology departments in Spain.
METHODS: We conducted a multicentre, observational cross-sectional study that collected information using a structured survey sent by email to 1015 partners of the Systemic Autoimmune Diseases Workgroup of the Spanish Society of Internal Medicine (GEAS-SEMI) from the 1st to the 31st of March 2017.
RESULTS: We identified a total of 21 support units/consultations for the ophthalmology departments. Seventeen (81%) of the units were specific internal medicine-ophthalmology consultations, and 5 (24%) units had been created in the past 5 years. A median of 460 patients were assessed per unit by the end of the year.
CONCLUSIONS: This study shows, for the first time in Spain, the important and close collaboration between ophthalmologists and internists, especially in highly specialized national reference institutions.