Surgical interventions for bilateral congenital cataract in children aged two years and under.

BACKGROUND: Congenital cataracts are lens opacities in one or both eyes of babies or children present at birth. These may cause a reduction in vision severe enough to require surgery. Cataracts are proportionally the most treatable cause of visual loss in childhood, and are a particular problem in low-income countries, where early intervention may not be possible. Paediatric cataracts provide different challenges to those in adults. Intense inflammation, amblyopia (vision is obstructed by cataract from birth which prevents normal development of the visual system), posterior capsule opacification and uncertainty about the final trajectory of ocular growth parameters can affect results of treatment. Two options currently considered for children under 2 years of age with bilateral congenital cataracts are: (i) intraocular lens (IOL) implantation; or (ii) leaving a child with primary aphakia (no lens in the eye), necessitating the need for contact lenses or aphakic glasses. Other important considerations regarding surgery include the prevention of visual axis opacification (VAO), glaucoma and the route used to perform lensectomy. OBJECTIVES: To assess the effectiveness of infant cataract surgery or lensectomy to no surgery for bilateral congenital cataracts in children aged 2 years and under. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL; which contains the Cochrane Eyes and Vision Trials Register; 2022, Issue 1); Ovid MEDLINE; Ovid Embase; the ISRCTN registry; ClinicalTrials.gov and the WHO ICTRP. The date of the search was 25 January 2022. SELECTION CRITERIA: We included all randomised controlled trials (RCTs) that compared infant cataract surgery or lensectomy to no surgery, in children with bilateral congenital cataracts aged 2 years and younger. This update (of a review published in 2001 and updated in 2006) does not include children over 2 years of age because they have a wider variety of aetiologies, and are therefore managed differently, and have contrasting outcomes. DATA COLLECTION AND ANALYSIS: We used standard methods expected by Cochrane. Two review authors extracted data independently. We assessed the risk of bias of included studies using RoB 1 and assessed the certainty of the evidence using GRADE. MAIN RESULTS: We identified three RCTs that met our inclusion criteria with each trial comparing a different aspect of surgical intervention for this condition. The trials included a total of 79 participants under 2 years of age, were conducted in India and follow-up ranged from 1 to 5 years. Study participants and outcome assessors were not masked in these trials. One study (60 children) compared primary IOL implantation with primary aphakia. The results from this study suggest that there may be little or no difference in visual acuity at 5 years comparing children with pseudophakia (mean logMAR 0.50) and aphakia (mean logMAR 0.59) (mean difference (MD) -0.09 logMAR, 95% confidence intervals (CIs) -0.24 to 0.06; 54 participants; very low-certainty evidence), but the evidence is very uncertain. The evidence is very uncertain as to the effect of IOL implantation compared with aphakia on visual axis opacification (VAO) (risk ratio (RR) 1.29, 95% CI 0.23 to 7.13; 54 participants; very low-certainty evidence). The trial investigators did not report on the cases of amblyopia. There was little evidence of a difference betwen the two groups in cases of glaucoma at 5 years follow-up (RR 0.86, 95% CI 0.24 to 3.10; 54 participants; very low-certainty evidence). Cases of retinal detachment and reoperation rates were not reported. The impact of IOL implantation on adverse effects is very uncertain because of the sparse data available: of the children who were pseudophakic, 1/29  needed a trabeculectomy and 8/29 developed posterior synechiae. In comparison, no trabeculectomies were needed in the aphakic group and 2/25 children had posterior synechiae (54 participants; very low-certainty evidence).  The second study (14 eyes of 7 children under 2 years of age) compared posterior optic capture of IOL without vitrectomy versus endocapsular implantations with anterior vitrectomy (commonly called 'in-the-bag surgery'). The authors did not report on visual acuity, amblyopia, glaucoma and reoperation rate. They had no cases of VAO in either group. The evidence is very uncertain as to the effect of in-the-bag implantation in children aged under 1 year. There was a higher incidence of inflammatory sequelae: 4/7 in-the-bag implantation eyes and 1/7 in optic capture eyes (P = 0.04, 7 participants; very low-certainty evidence). We graded the certainty of evidence as low or very low for imprecision in all outcomes because their statistical analysis reported that a sample size of 13 was needed in each group to achieve a power of 80%, whereas their subset of children under the age of 1 year had only 7 eyes in each group. The third study (24 eyes of 12 children) compared a transcorneal versus pars plana route using a 25-gauge transconjunctival sutureless vitrectomy system. The evidence is very uncertain as to the effect of the route chosen on the incidence of VAO, with no cases reported at 1 year follow-up in either group. The investigators did not report on visual acuity, amblyopia, glaucoma, retinal detachment and reoperation rate. The pars plana route had the adverse effects of posterior capsule rupture in 2/12 eyes, and 1/12 eyes needing sutures. Conversely, 1/12 eyes operated on by the transcorneal route needed sutures. We graded the outcomes with very low-certainty because of the small sample size and the absence of a priori sample size calculation. AUTHORS' CONCLUSIONS: There is no high level evidence for the effectiveness of one type of surgery for bilateral congenital cataracts over another, or whether surgery itself is better than primary aphakia. Further RCTs are required to inform modern practice about concerns, including the timing of surgery, age at which surgery should be undertaken, age for implantation of an IOL and development of complications, such as reoperations, glaucoma and retinal detachment. Standardising the methods used to measure visual function, along with objective monitoring of compliance with the use of aphakic glasses/contact lenses would greatly improve the quality of study data and enable more reliable interpretation of outcomes.

[Expert consensus on prevention and treatment of amblyopia in children].

Amblyopia is caused by vision deprivation and/or abnormal binocular interaction during visual immaturity. According to different etiologies, it is divided into strabismic, ametropic, anisometropic and visual deprivation amblyopia. The key to the treatment of amblyopia is early screening and timely intervention. The managements include eliminating the factors of visual deprivation, optical correction, patching and penalization. Following the publication of the Expert Consensus on Diagnosis of Amblyopia in 2011, the Chinese Association for Pediatric Ophthalmology and Strabismus organized experts in this field again to discuss and formulate the Expert Consensus on Prevention and Treatment of Amblyopia in Children based on the research results of evidence-based medicine. The purpose is to provide clinical guidance of the diagnosis and treatment of amblyopia for ophthalmologists and to improve clinical service. (Chin J Ophthalmol, 2021, 57: 336-340).

Evaluation of the Spot Vision Screener in School-Aged Children.

PURPOSE: To determine the accuracy of the Spot Vision Screener (Welch Allyn, Skaneateles Falls, NY) in children 6 years and older and recommend device thresholds to improve its accuracy for the detection of refractive error. METHODS: The Spot Vision Screener results were compared with three gold standard conditions of increasingly narrow refractive error criterion. The sensitivity, specificity, positive predictive value, and negative predictive value of the Spot Vision Screener in detecting each gold standard criterion were calculated. The most accurate threshold setting for each parameter was identified by calculating the area under the curve receiver operating characteristic. RESULTS: The Spot Vision Screener was able to successfully evaluate 313 of 330 children (95%). The sensitivity of the Spot Vision Screener to detect American Association for Pediatric Ophthalmology and Strabismus guidelines for amblyopia risk factors was 89.5% and the specificity was 76.7%. The sensitivity decreased to 80% and the specificity increased to 75.3% with narrower refractive criteria. The sensitivity in detecting refractive criteria improved with the proposed optimized device thresholds. Estimates for the general population indicate that the positive predictive value is reasonable at 52.3% to 61.8%, depending on the stringency of the criteria, with excellent negative predictive values. CONCLUSIONS: In school-aged children, the primary screening focus shifts from preventing amblyopia to detecting visual disturbances, including refractive error, that may interfere with academic performance. In this age group, the Spot Vision Screener was an acceptable method of detecting significant refractive error with improved sensitivity with threshold optimization. [J Pediatr Ophthalmol Strabismus. 2020;57(3):146-153.].

Anterior lamellar keratoplasty for the management of a central dermoid tumour in a baby. / Queratoplastia lamelar anterior en el manejo de un tumor dermoide central en un bebé.

A 3-month-old baby presented with a whitish lesion over the right cornea since birth. Due to the clinical and ultrasonic characteristics of the lesion, a manual anterior lamellar keratoplasty was performed. Histopathological examination showed it to be a simple grade II epibulbar choristoma. Although dermoid tumours are usually considered as benign, some of them, depending on their location, can seriously affect the vision of a paediatric patient. Due to the high probability of amblyopia in these cases, a surgical procedure is mandatory. Anterior lamellar keratoplasty is recommended when the visual axis is compromised without intraocular implications.

Management of a Large Congenital Hemangioma Obstructing Visual Axis: A Case Report and Review of Literature.

Congenital hemangiomas comprise a subset of vascular tumors with clinicopathologic features that are distinct from the more common infantile hemangioma. The authors present a patient with a large congenital hemangioma involving the forehead and brow which obstructed the visual axis and created significant risk for deprivational amblyopia. Management of the congenital hemangioma involved customized headgear to clear the visual axis and early vascular embolization of feeder vessels with the subsequent successful surgical resection at 23 days of life.A large amblyogenic congenital hemangioma required a multidisciplinary approach involving early vascular embolization of feeder vessels and subsequent surgical resection at 23 days of life.

Comportamiento clínico quirúrgico de la exotropía de gran ángulo / Clinical and surgical behavior of large-angle exotropia

RESUMEN Objetivos: Describir algunas características clínicas de la exotropía de gran ángulo y determinar su resultado quirúrgico. Métodos: Se realizó un estudio descriptivo retrospectivo de 35 pacientes atendidos en el Hospital Oftalmológico "Amistad Cuba-Argelia", en la Wilaya de Ouargla, Argelia, en el período enero 2014 - diciembre 2017. Se analizó el comportamiento de algunas variables relacionadas con la exotropía, como fueron la edad del paciente, el sexo, el color de la piel, las alteraciones oftalmológicas asociadas, el grado de ambliopía, el defecto refractivo asociado, el ángulo de desviación preoperatorio y posoperatorio y el tipo de cirugía realizada. Resultados: Predominó el grupo etario entre 24 y 29 años de edad con el 25,7 por ciento; el 51,4 por ciento representó al sexo femenino; el color de la piel negra fue el más frecuente con 48,6 por ciento; la ptosis palpebral correspondió al 11,4 por ciento de las alteraciones oftalmológicas asociadas y al 68,6 por ciento sin alteraciones; la ambliopía moderada se registró en el 42,9 por ciento de los casos; el defecto refractivo más observado fue el astigmatismo miópico con el 37,1 por ciento; el ángulo de desviación preoperatorio más significativo se encontró en el grupo de 71-80 dioptrías con 37,2 por ciento. A los tres meses de la cirugía el 91,4 por ciento de los pacientes se encontró en ortoforia (± 8 dioptrías) y al año de la cirugía el 94,3 por ciento se mantenía en ortoforia. El retroceso de ambos músculos rectos laterales (10,0 mm) más la resección de un músculo recto medio (7 mm) se realizó en el 37,2 por ciento de los pacientes. Conclusiones: La casi totalidad de los pacientes con exotropía de gran ángulo logran el alineamiento ocular y consiguen una mejoría en la calidad visual(AU)

ABSTRACT Objectives: Describe some clinical characteristics of large-angle exotropia and determine its surgical outcome. Methods: A retrospective descriptive study was conducted of 35 patients attending Cuba-Algeria Friendship Ophthalmological Hospital in the wilayah of Ouargla, Algeria, from January 2014 to December 2017. Analysis was carried out of variables related to exotropia, such as the patients' age, sex, skin color, associated ophthalmological alterations, degree of amblyopia, associated refractive defect, pre- and post-operative angle of deviation, and type of surgery performed. Results: The 24-29 year age group prevailed with 25.7 percent; 51.4 percent of the patients were female; black skin color was the most common with 48.6 percent; eyelid ptosis represented 11.4 percent of the associated ophthalmological alterations and 68.6 percent without alteration; moderate amblyopia was present in 42.9 percent of the cases; the most frequent refractive defect was myopic astigmatism with 37.1 percent; the most significant preoperative angle of deviation was found in the 71-80 diopter group with 37.2 percent. Three months after surgery, 91.4 percent of the patients were orthophoria (± 8 diopters) and at one year 94.3 percent remained orthophoria. Bilateral lateral rectus muscle recession (10.0 mm) plus resection of a medial rectus muscle (7 mm) was achieved in 37.2 percent of the patients. Conclusions: Practically all patients achieved ocular alignment and improved their visual quality(AU)

The impact of vision screening in preschool children on visual function in the Swedish adult population.

BACKGROUND: Amblyopia is the most common monocular visual impairment in Scandinavia. A visual screening programme for preschool children was introduced in Sweden in the 1970s to reduce the prevalence of amblyopia. The purpose of this study was to investigate the impact of this visual screening programme in adult men recruited to the armed forces in Sweden. METHODS: The prevalence of amblyopia was compared in recruits born in 1956-1957 (n = 1500), before the introduction of the visual screening programme (unscreened), and those born in 1977-1979 (n = 2626), after the visual screening programme was introduced (screened). Amblyopia was defined as bilateral, if the bilateral best corrected visual acuity (BCVA) was <0.5 (20/40), and unilateral if there was a ≥2-line interocular difference and BCVA was <0.625 (20/32) in the worse eye. Subgroup analysis was performed on recruits with hyperopia, myopia and anisometropia. RESULTS: The prevalence of amblyopia was significantly lower after the introduction of the Swedish visual screening programme. Forty-seven (3.3%) of the unscreened and 23 (0.9%) of the screened recruits had unilateral amblyopia (p < 0.0001, OR = 0.26 (95%CI 0.16-0.43)). The risk for developing amblyopia was markedly reduced by screening in recruits with hyperopia (≥2D) (p < 0.0001 and OR 0.034 (95% CI 0.003-0.207)) and anisometropia (≥1.5D interocular difference) (p < 0.01 and OR 0.20 (95% CI 0.08-0.66)). Only a few recruits with myopia had amblyopia. CONCLUSIONS: The results demonstrate the value of the Swedish visual screening programme for preschool children in preventing amblyopia. Anisometropia or high hyperopic refractive errors can be identified by screening and corrected to prevent amblyopia.

[Impact of timing of surgery on outcome in children with bilateral congenital cataract]. / Bilaterale kongenitale Katarakt: Klinische und funktionelle Ergebnisse in Abhängigkeit vom Operationszeitpunkt.

BACKGROUND: The optimal time for surgical intervention in bilateral congenital cataract is still a matter of controversy. OBJECTIVES: Herein, we evaluated clinical and functional results after bilateral congenital cataract surgery and performed subgroup analysis based on the age at the time of surgery. MATERIALS AND METHODS: We retrospectively reviewed the records of 52 eyes of 26 children who underwent surgery for bilateral congenital cataract without intraocular lens implantation within the first 12 months of life; 16 eyes underwent phacoaspiration within the first 10 weeks of life (group A) and 26 eyes had cataract extraction at an age of >10 weeks (group B). We defined the primary outcome measure to be the prevalence of mild, moderate, and severe amblyopia in relationship to age-dependent visual acuity norms after a mean follow-up of 59.7 ± 43.2 months. Secondary outcome measures were interocular difference of visual acuity and the presence of strabismus, nystagmus, posterior capsule opacification, and aphakic glaucoma. RESULTS: The prevalence of amblyopia was not statistically different between the two age groups. Of all children, 62.5 % (A) and 61.1 % (B) developed age-dependent normal visual acuity or mild amblyopia. However, the younger cohort developed significantly less strabismus than the older cohort (P = 0.03). There was a strong relationship between cataract surgery within the first 14 weeks of life and the development of aphakic glaucoma. All children developing secondary cataract formation underwent cataract surgery in the first 20 weeks of life. CONCLUSIONS: Our results suggest that long-term prevalence of mild, moderate, and severe amblyopia is similar between the two cohorts of bilateral congenital cataract eyes based on the age ≤ or >10 weeks at the time of surgery. Because there is a strong relationship between postoperative complications and a young age at the time of cataract surgery, close postoperative follow-up is essential. Strabismus is less likely to develop in infants after bilateral cataract surgery within the first 10 weeks of life.

Topical timolol maleate 0.5% solution for the management of deep periocular infantile hemangiomas.

This retrospective, consecutive, clinical case series examined the use of topical timolol in the treatment of 5 children with deep, periocular infantile hemangiomas that caused astigmatism, change in head posture, or ptosis. All patients were treated with timolol maleate solution 0.5% twice daily until the lesions had regressed. All 5 children showed regression of the lesion and improvement in amblyogenic risk factors within 2 weeks.

Evaluating the evidence for and against the use of IOLs in infants and young children.

Congenital cataracts account for 5-20% of childhood blindness worldwide. In the US, the prevalence of visually significant infantile cataracts is anywhere from 3-4 per 10,000 live births. Infantile cataracts need to be removed early in life in order to prevent the onset of deprivation amblyopia. As a result, cataract surgery is usually performed between age 4-8 weeks depending on the laterality and severity of the cataract. Given advances in the field, pediatric cataract surgery is now a safe and effective intervention for infants, but good visual outcomes require occlusion therapy and optical correction. This review will address current perspectives on the use of intraocular lenses to optically correct infants and young children after cataract surgery, as well as novel designs for intraocular lenses and directions for future research.

Uveitis in Children: Diagnosis and Management.

Uveitis is a term used to describe inflammation of uvea, which is the middle layer of eye. It is an important cause of blindness in children in both developed and developing countries. Delayed diagnosis, inadequate treatment and risk of amblyopia are some of the factors that are unique to childhood uveitis and are responsible for significant morbidity seen with this disease.

Congenital Cataract: Progress in Surgical Treatment and Postoperative Recovery of Visual Function.

Congenital cataract is a common cause of childhood low vision or blindness worldwide. Early surgery should be performed in sensitive vision development period to avoid vision deprivation in cases of strabismus and nystagmus development. Postoperative recovery of visual function is of great significance for these patients and should include proper postoperative aphakia correction by the application of a contact lens and secondary implantation of an intraocular lens (IOL). Patients should receive amblyopic training after surgery to improve their postoperative visual acuity. Although recent advances in surgery techniques and materials have brought better postoperative visual acuity and less complications, a few postoperative complications can still hamper vision condition and vision development, including posterior capsule opacification and secondary glaucoma. Surgery in early infancy are risk factors for these two complications. Life-long follow-up is essential for these patients. Further study will continue to address the surgery timing issue and methods that prevent postoperative complications.

Customised simple limbal epithelial transplantation for recurrent limbal stem cell deficiency.

A 4-year-old boy developed unilateral, total limbal stem cell deficiency (LSCD) following an acid injury. A stable ocular surface was achieved with autologous simple limbal epithelial transplantation (SLET), performed 4 months after the injury. Focal areas of conjunctivalisation were noted in the postoperative period. These were addressed using a novel modification of SLET. A very small piece of limbus from the healthy eye was split into multiple pieces, and applied to the bare corneal stroma with fibrin glue after pannus resection. The surface was covered with amniotic membrane. This resulted in a stable surface, and visual acuity improved to 20/50. The donor remained healthy despite two biopsies being harvested. This case demonstrates that early ocular surface reconstruction may be considered in children with LSCD if amblyopia is a concern. Customised SLET allows surgeons to tackle focal recurrences of LSCD effectively, using minimal limbal tissue from the donor site.

[Current aspects of eyelid, lacrimal and orbital surgery in childhood]. / Aktuelle Aspekte zur Lid-, Tränenwegs- und Orbitachirurgie im Kindesalter.

BACKGROUND: During childhood the spectrum of disorders of the eyelid, the lacrimal drainage system and the orbit as well as the subsequent therapeutic management differ from those in adults. OBJECTIVES: This review outlines the clinical picture and treatment of the most common eyelid, lacrimal drainage and orbital diseases in childhood. METHODS: The study comprises a PubMed literature review and own clinical results. RESULTS: The most common eyelid disorders in childhood include congenital malpositions, such as ptosis or entropium, which may require fast surgical correction in order to prevent amblyopia. For connatal dacryostenosis a step-by-step therapeutic approach is recommended: (1) conservative treatment using lacrimal sac massage, astringent eye and nose drops, (2) irrigation and probing of the nasolacrimal system and (3) irrigation, probing and silastic tube intubation under general anesthesia. Benign orbital lesions such as dermoid cysts can be removed at pre-school age. Using systemic beta blockers is a novel conservative approach in the treatment of capillary hemangioma, which should precede surgical interventions. CONCLUSION: Eyelid and orbital diseases during childhood may require fast surgical intervention to prevent amblyopia. Initial conservative treatment is recommended for connatal dacryostenosis and capillary hemangioma.

A simple technique for temporary eyelid closure in severe exposure keratopathy.

We describe a novel and simple technique for temporary lid closure in severe bilateral corneal exposure keratopathy, where a rapid method for corneal protection is required. The aim is to create a stable dressing base for secure closure of the eyelids that also allows instillation of medication and examination of the eye.

[Pay attention to patient's visual function in oculoplastic surgery for congenital blepharophimosis syndrome].

Blepharophimosis is characterized by the presence of a narrowed horizontal palpebral aperture, ptosis, epicanthus inversus, and telecanthus. Due to patient's eye deformity, both of his or her appearance and psychological development are affected. In some cases, blepharophimosis not only influence the appearance but also the visual development. Therefore, patients with blepharophimosis require to be evaluated the visual function before the surgery in order to prevent amblyopia. In this article, we conclude that we should pay close attention to patient's visual function before and after the surgery. The timing of surgery must weigh the balance between early surgery to prevent amblyopia and late surgery for more reliable ptosis measurement, which provides a better surgical outcome.

[Learn new version of Preferred Practice Pattern to further standardize the diagnosis and treatment of amblyopia].

The introduction of Preferred Practice Patterns (PPP) into China has given ophthalmologists in China more opportunities to acquaint themselves with international clinical guidelines for eye care, including its developing principles, methods and the application value. It had brought significant effects on the fast improvement of clinical eye care and standardization of diagnosis and treatment of eye diseases in China, and promoted the international academic exchanges. The 2nd Chinese version of PPPs translated by Prof. Jialiang Zhao was officially published in November, 2013. The new version of PPP for amblyopia adopted the newest standard for grading of evidence strength and recommendation assessment, and emphasizes the practicability based on evidence. New explanations of the definition of amblyopia are added according to the recent research progresses in amblyopia. The diagnostic criteria of best visual acuity for bilateral amblyopia at different ages is given with new specifications. Comprehensive and practical suggestions on the examination methods for amblyopia are provided from the qualitative assessment of visual acuity, the choice of eye chart, to the use of cycloplegic agents. In the aspect of the treatment of amblyopia, based on the findings of recent multi-central random controlled clinical trials, not only strong recommendations are provided, but also the insufficiency of evidence supporting for some choices of therapy is pointed out. The necessity of the follow-up evaluation after the cessation of the treatment of amblyopia is emphasized too. In the aspect of the prevention of amblyopia, the new amblyopia PPP points out the importance of the early-period screening of amblyopia, and that the healthcare insurance plans should cover timely screening, treatment, and monitoring for recurrence of amblyopia. This article deciphers the essential contents of the new version of Chinese PPP for amblyopia, and aims to promote the standardization of the diagnosis and treatment of amblyopia with our ophthalmic colleagues in China.

Complications of infantile hemangiomas.

Most infantile hemangiomas have a spontaneous and uneventful involution and, hence, may be treated expectantly. Others, however, will present some complication along their evolution that may require prompt therapeutic interventions. Ulceration is the most common complication, and amblyopia is frequently associated with periocular tumors. Airways hemangiomas may be life-threatening, and disfigurement can heavily impact the patient's quality of life.

Periocular hemangiomas and lymphangiomas.

Infantile hemangiomas are the most common benign tumor of childhood. Lymphangiomas are benign hamartomatous vascular tumors. Both lesions can be problematic when located in the periocular region. Pediatricians must be familiar with the characteristics of each which would necessitate referral to an ophthalmologist or other subspecialist for evaluation, including obstruction of the visual axis which can lead to amblyopia of the affected eye. Additional potential complications include proptosis, ocular motility limitation, optic nerve injury, and poor eyelid closure with or without corneal surface disease. All children with periocular hemangiomas or lymphangiomas should be referred to an ophthalmologist for further evaluation.