Penetrating keratoplasty in children: results of surgical treatment

Penetrating keratoplasty (PK) in children is a complex and ultifacetedproblem that has been experiencing pediatric ophthalmology for several decades With all its radicality and with all its therapeutic potential, most surgeons prefer, nevertheless, to refuse to transplant the cornea in childhood, and transfer it to a later date, especially in infants (up to a year). In the absence of alternative interest in this operation did not fade, the practice of corneal transplantation in children continued and improved. The desirability of PK in corneal opacities (CO) in children is no longer discussed as such. On the agenda is another question: how to make this operation a truly effective way of treatment. This is not an easy task in itself, with many problems. Among them – the complexity of communication with a young patient, a special plasticity of the tissues of the child’s eye, heavy, as a rule, the combined nature of the pathology, predisposition to violent inflammatory reactions, etc. In many years of practice, for the most part, by trial and error, these problems are gradually finding their solution. An empirical experience always precedes the understanding of the problem and the search for its solution, no matter how unsuccessful it may seem at the very beginning. This work is devoted to generalization of such experience.Objective. To Evaluate the immediate and long-term results ofpenetrating keratoplasty in children. Material and methods. Retrospectively and prospectively analyzed the medical history and hospital records of children operated in the Department of pathology of eyes in children, Moscow Helmholtz Research Institute of Eye Diseases in the period from 1997 to 2017. The total sample consisted of 208 cases of the PK, was performed in 185 children on 208 eyes. By the nature of the disease, all observations were divided into congenital and acquired CO. Among the latter, turbidity of traumatic and non-traumatic nature was distinguished. Biological and functional results of PK were evaluated. The biological result of the operation was evaluated in terms of graft survival (Kaplan-Mayer model). The functional result was estimated approximately: by tracking the child’s toys from a certain distance and by the method of pr eferred gaze.Results. The first 6 months after the surgery, the transplants, with rare exceptions, remained transparent. By the end of the 1st year, 72% of transplants remained transparent, by the end of the 2nd year not less than 65%, by the end of the 3rd year-not less than 55%, by the end of the 5th year not less than 45% of transplants. In search of a more rigorous determination of the results, PK assessed the impact of particular clinical circumstances on the engraftment of transplants. It turned out that regardless of the etiology of the disease graft survival is significantly lower if keratoplasty is carried out in a vascularized couch, if simultaneously with corneal transplantation are other optical-reconstructive surgery: cataract extraction, vitrectomy, plastic iris, if the diameter of the transplant >8 mm, in the eyes with glaucoma in history. Transparency of transplants is significantly reduced in complicated postoperative course: recurrent rejection crises, increased IOP or the appearance of synechiae. In at least 80% of patients, corneal transplants have resulted in improved visual aquity (VA). In most of these cases, VA increased from light perception to 0.1-0.3. Satisfactory results, when VA after surgery reached 0.6-0.8, were in patients with keratoconus and congenital hereditary corneal  dystrophy. Among those who have measured hundredth of VA dominated children with severe congenital malformations of the cornea and anterior segment of the eye.Conclusion. PK in children today is a very successful surgical intervention, the therapeutic potential of which can be realized with proper consideration of risk factors, impeccable technique and careful postoperative monitoring

Current issues of differential diagnosis and treatment of congenital corneal opacities in children

Purpose. To analyze the literature on the topic of congenital corneal opacities, their diagnosis, differential diagnosis, as well as the choice of optimal treatment tactics.Congenital corneal opacities (CCO), when effective treatment is delayed, usually lead to permanent loss of visual function. This diagnosis requires urgent measures. On the other hand, the rarity of neonatal corneal pathology and difficulties associated with evaluation of newborns yield the situation when more or less defined clinical practice patterns in such cases remain unclear for most pediatric ophthalmologists.Based on our own experience and the literature data, we describe the main issues of differential diagnosis and management of CCO in newborns. A broad range of developmental anomalies, congenital hereditary corneal dystrophies and metabolic diseases as the main causes of CCO are reviewed. In addition to this diagnostic paradigm, the prognosis in every particular pathology as well as treatment recommendations are given.Conclusion. Based on the presented review, the prognosis and therapeutic recommendations for a particular congenital pathology of the cornea are given

Оценка морфометрических параметров диска зрительного нерва и сетчатки при врожденной глаукоме у недоношенных детей

PURPOSE: To study the morphometric parameters of the optic nerve head and retina in premature infants with congenital glaucoma.METHODS: Children with congenital glaucoma (21 children) with favorable outcomes of 1-3 stages of retinopathy of prematurity (RP) aged from 6 months to 12 years were examined (group I). The control group consisted of 32 fullterm children with congenital glaucoma (group II). In addition to the standard methods of examination, Heidelberg retinal tomography (HRT) and optical coherence tomography (Spectralis-OCT) of optic nerve head (ONH) and retina were used.RESULTS: According to clinical manifestations of glaucoma, there were no significant differences between the groups. Both children with 1-2 stage of RP and congenital glaucoma and full-term infants with congenital glaucoma revealed the same pronounced morphometric changes in structures of the disk and peripapillary zone of the retina, which were characteristic for the development of glaucomatous optic neuropathy and intensified in the course of glaucoma progression. ONH changes interpretation in RP infants was hampered by traction deformation of the ONH and displacement of the central vessel trunk, especially in patients with stage 3 RP. We have not revealed a clear link between the changes in the optic nerve disk and IOP level.CONCLUSION: Morphometric parameters measurement and a number of pathognomonic symptoms of optic nerve and peripapillary retina damage in congenital glaucoma in premature infants identified in this paper are necessary for studying the pathogenesis of glaucomatous process in RP, clarifying the diagnosis, determining the disease prognosis, assessing glaucoma progression and developing treatment tacticsЦЕЛЬ. Изучить морфометрические особенности зрительного нерва и сетчатки у недоношенных детей с врожденной глаукомой.МЕТОДЫ. Обследованы дети с врожденной глаукомой (21 ребенок) с благоприятными исходами ретинопатии недоношенных (РН) 1-3 степени в возрасте от 6 мес до 12 лет (группа 1). Группу контроля составили 32 доношенных ребенка с врожденной глаукомой (группа 2).Помимо стандартных методов обследования применяли гейдельбергскую ретинальную томографию (НRТ) и оптическую когерентную томографию диска зрительного нерва (ДЗН) и сетчатки (Spectralis-OCT).РЕЗУЛЬТАТЫ. По клиническим проявлениям глаукомы существенных различий между группами не было. При врожденной глаукоме у детей с 1-2 степенью РН, как и у доношенных детей с врожденной глаукомой, выявлены одинаково выраженные морфометрические изменения структур всех зон ДЗН и зон перипиллярной сетчатки, характерные для развития глаукомной оптической нейропатии и усугубляющиеся по мере прогрессирования глаукоматозного процесса. При РН интерпретация изменений ДЗН затруднена вследствие тракционной деформации ДЗН и смещения сосудистых пучков, особенно при 3 степени РН. Четкой связи изменений ДЗН с показателями ВГД нами не выявлено.ЗАКЛЮЧЕНИЕ. Измерения морфометрических параметров и ряд выявленных в данной работе патогномоничных симптомов поражения зрительного нерва и перипапиллярной сетчатки при врожденной глаукоме у недоношенных детей необходимы для изучения патогенеза глаукоматозного процесса при РН, уточнения диагноза, определения прогноза заболевания, оценки стабилизации глаукоматозного процесса и выработки тактики лечения

THE EXPERIENCE OF PENETRATING KERATOPLASTY IN INFANTS WITH CONGENITAL CORNEAL OPACITIES

Background. Corneal opacity (CO) in children is a severe pathology. In most cases it is characterized by a combination of leucoma with various pathologies of the entire anterior segment of the eye (microcornea, microphthalmia, cataract, adhesion of the lens with the corneal endothelium, glaucoma, atrophy of the iris, often partial or total aniridia). Congenital malformations of other organs and systems are supplements of the clinical pattern. A single method of treatment is penetrating keratoplasty (PK). For all its radicalism and with its entire curative potential, the majority of ophthalmic surgeons prefer to refuse corneal transplantation in children, and transfer it to a later period, particularly in babies aged less one year.Purpose. To evaluate the early and long-term results of penetrating keratoplasty (PKP) n children operated on the 1st year of life for congenital corneal opacities CCO.Material and methods. All patients were divided into 2 groups. The first group consisted of children with CCO, who underwent the PKP, the second group – children with CCO, where limbokeratoplasty was performed. In the first group results of 42 PKP with cadaver corneas were taken for the analysis carried out in 40 children on 42 eyes, in the second group: results of penetrating limbokeratoplasty were taken for the analysis in 8 children (8 eyes) aged from 2 to 12 months for the period 20082014. The average age was 7.2±2.1. The results of the PKP in 40 children (42 eyes) operated on in infancy were compared with the results of the PKP in 22 children who underwent the surgery for the same reason (CCO) aged over 1 year. The functional outcome was assessed roughly: according to the tracking toys by children looking at a certain distance and using the method of the preferred gaze.Results. In the first group in a comparative aspect the biological results of the PKP in newborns were slightly worse than those in older children (65% and 81%, respectively). At the same time, the functional results in case of an early (under one year) CCO treatment exceeded those that were in the postponed treatment (over one year). If in the children operated on in infancy, satisfactory results after the PKP were observed in a half of the cases, in the children operated at an older age – only in 1 case out of10. In the second group (corneal staphyloma) only 1 of 8 transplanted grafts retained the transparency one year after limbokeratoplasty, the organ-preserving result was achieved in all cases.Conclusion. Under certain conditions (a hard selection of patients for the surgery, a sparing microsurgical technique, a careful postoperative monitoring of the condition of the eyes, a timely treatment of complications) the surgical treatment of CCO in the early stages can be quite successful

Peters’s anomaly: clinic, diagnostics and results of surgical treatment

The Peters’s Anomaly (PA) is an infrequent congenital anomaly of a cornea and anterior segment of eye. The rarity of this pathology and difficulty bound to inspection of newborns generate a situation when more or less certain clinical approaches to maintaining this anomaly remain obscure for most of children’s ophthalmologists.Purpose. To study clinical implications and to estimate results of surgical treatment in children with PA.Material and methods. Under observation from 1996 for 2016 on examination and treatment there were 51 children with the PA different forms, aged from 3 months up to 10 years: 1 group – mesodermal – 20 people (37%), 2 – group the ectodermal 31 child (63%). Surgical treatment – penetrating keratoplasty (PKP) was carried out at 43 patients (69 eyes) aged from 8 up to 36 months. At other children (8 people) surgery was contraindicative because of serious somatic and eye pathology. Clinical-functional and ophthalmologic examination at all was conducted in the conditions of medication sleep. PKP carried out by a traditional technique, as donor material used a fresh cadaver cornea with prescription of a fence from death moment before operation no more than 48 hours. The biological result of surgery was estimated by a moment method (or Kaplana-Maier’s method) by means of which calculated so-called survival of a graft. Functional result – at children to 3-x years estimated approximately: on tracking of the child toys from a certain distance and method of a preferable look, at children of advanced age according to Sivtsev-Golovin’s table. Postoperative observation proceeded from 3 months to 15 years. The corneal disks removed during surgery were exposed to a histological research.Results. Within the first year after PKP a transparence of the replaced cornea remained in 1 group on 29 eyes (90%), in 2 group – at 21 patients of 26 eyes (67%). In 2 years after surgery the transparent donor cornea at children in 1 group remained at 17 patients (20 eyes, 62%); in – the second at 15 patients of 18 eyes (54%). In 5 years after surgery – at patients of 1 group a transparence of the replaced donor tissue was kept by 17 eyes (53%), in the second only 12 of 31 executed surgery (39%). In 10 years transparent were 53% and 31% of corneas, respectively. In 15 years after surgery a transparence was kept in 1 group of 15 eyes (46%), in 2 – on 9 (22%). Visual acuity (0,3 and above) was observed at 8 of 13 children (61%) after surgery about one year at children of advanced age only in 27% of cases, even at tr ansparent engraftment of a graft.Conclusion. The keratoplasty at PA at children of early age is the only chance to save the child from a blindness and disability on vision