Educational paper

Retinopathy of prematurity (ROP) is a proliferative retinal vascular disease affecting the premature infant with an incompletely vascularized retina. The spectrum of ophthalmological findings in ROP exists from minimal sequelae, which do not affect vision, to bilateral retinal detachment and total blindness. With the increased survival of very small infants, retinopathy of prematurity has become one of the leading causes of childhood blindness. Over the past two decades, major advances have been made in understanding the pathogenesis of ROP, to a large extent as a result of changes in clinical risk factors (oxygen and non-oxygen related) and characteristics observed in ROP cases. This article provides a literature review on the evolution in clinical characteristics, classification and treatment modalities and indications of ROP. Special attention is hereby paid to the neonatal factors influencing the development of ROP and to the necessity for everyone caring for premature babies to have a well-defined screening and treatment protocol for ROP. Such screening protocol needs to be based on a unit-specific ROP risk profile and, consequently, may vary between different European regions. Conclusion: Retinopathy of prematurity is an important cause of ocular morbidity and blindness in children. With better understanding of the pathogenesis, screening and treatment guidelines have changed over time and are unit specific.     

早产儿视网膜病1082例筛查报告及诊治分析

目的 探讨早产儿视网膜病(ROP)的早期诊治方法,分析其筛查结果.方法 由眼底病专科医生应用双目间接眼底镜对本院新生儿科2004年7月至2009年6月收治的胎龄＜34周或出生体质量＜2000 g的住院早产儿进行ROP筛查.首次筛查时间为纠正胎龄32～34周或生后4～6周,对检出的阈值期或阈值前期1型ROP(重症)患儿全部给予眼底激光光凝术,对视网膜血管未发育成熟、1～2期或阈值前期2型(轻度)ROP患儿进行密切随访,直至视网膜血管发育至锯齿缘或发展成为重症.对所有的临床资料进行回顾性分析.结果 5年共收治早产儿2 295例,符合筛查标准的早产儿1 082例,占47.14%;检出ROP总阳性病例154例,占筛查对象的14.23%(154/1082);其轻度ROP86例,占7.94%(86/1 082);重症ROP 68例,占6.28%(68/1 082).68例重症ROP患儿中,有6例出院后随访期间发现进展为重症ROP而再入院,有2例放弃治疗1年后证实全部失明.66例(132只眼)接受各种治疗,其中63例单用光凝术治疗;3例急进性后极ROP中2例采用玻璃体腔内注入血管内皮生长因子拮抗剂(Avastin)联合光凝术治疗,1例单用光凝术治疗者治疗后仍出现部分视网膜脱离,经玻璃体视网膜手术后仍失明;随访结果65例成功的保存了视力,成功率98.48%(65/66).在观察期间未达到光凝治疗条件,因原发病恶化死亡10例,经光凝治疗后的患儿未出现死亡.结论 ROP筛查是防止ROP病情发展的有效措施,对重症ROP及时给予光凝术治疗是安全有效的方法,对急进性后极ROP可用玻璃体腔内注入血管内皮生长因子拮抗剂联合光凝术治疗抢救视力.     

Retinopathy of prematurity: the disease process, classifications, screening, treatment, and outcomes

Retinopathy of prematurity (ROP) is the cessation of normal eye development and subsequent abnormal vessel growth that occurs exclusively in premature infants. ROP was first discovered in the 1940s and was for two decades the leading cause of blindness in children. Currently, the disease causes about 500 new cases of blindness per year. The severity of the disease increases with decreasing gestational age. The pathogenesis of ROP involves disruption of normal retinal vascularization. Vessel endothelial growth factor, insulin-like growth factor, and oxygen play important roles in its development. ROP is classified using an international classification system that provides direction for screening and treatment of premature infants. Examinations are performed by ophthalmologists, who identify the scope of vascularization, the degree of abnormal vessel growth, and the amount of the eye that is affected. Treatment modalities include cryosurgery and laser photocoagulation. Long-term outcomes include both structural and functional vision problems.     

Incidence and risk factors for retinopathy of prematurity in the West Black Sea region, Turkey

The objective of this study was to determine the incidence, risk factors and severity of retinopathy of prematurity (ROP) and to establish screening criteria for our region. Data on 330 infants with gestational age at birth < or = 34 weeks were analyzed retrospectively for a ROP diagnosis and risk factors. Infants with type 1 ROP were treated with argon laser photocoagulation. ROP was detected in 106 of 330 infants; 18 infants had type 1 ROP and were treated. Two infants with ROP that progressed to stage 4 disease required surgery. No treatment was needed in infants born after 32 weeks of gestation. Respiratory distress syndrome and low gestational age were the most important risk factors for type 1 ROP. In the West Black Sea region of Turkey, screening all premature infants with a gestational age < or = 32 weeks or a birth weight < or = 1900 g appears to be appropriate.     

Severe retinopathy of prematurity in infants from the southern regions of Sweden

We studied children born in 1986–1989 with severe retinopathy of prematurity (ROP) defined as stage 3 "plus" or more. Sixteen children from the southern and central areas of Sweden were identified and 15 of these were referred to Orebro Medical Center Hospital for surgical treatment of ROP. The incidence of severe ROP was estimated as 0.8 per 10000 newborns per year. All children were born before 29 weeks' gestation and weighed less than 1310 g; they also needed ventilatory support for a long time. The overall neonatal morbidity was high. A model for eye examination in premature newborns is suggested.     

Fundus fluorescein angiography in the screening for and management of retinopathy of prematurity

OBJECTIVES: To describe the characteristics of fundus fluorescein angiography in retinopathy of prematurity (ROP) and to explore the possible benefits of fundus fluorescein angiography over conventional indirect ophthalmoscopy in the screening for and management of ROP. PATIENTS AND METHODS: Beginning in January 2003, 23 consecutive patients were recruited for a nonrandomized, investigational trial. Fifty-one sessions of fundus fluorescein angiography were performed as part of ROP screening. RESULTS: Fundus fluorescein angiography caused no adverse effect. Clear angiograms were easily obtained. There was delayed arm-to-eye fluorescein transit. Leakage of fluorescein was observed in all stage 2 and stage 3 ROP. The leakage resolved soon after treatment. Arteriovenous tufts were found far posterior to the ROP ridge and were a feature of severe ROP. CONCLUSIONS: Some vascular pathology observed on angiograms cannot be seen on indirect ophthalmoscopy. There was delayed arm-to-eye transit and fluorescein transit within the eyes. Fundus fluorescein angiography allows more objective assessment of disease stage and zone. Regression of ROP is clearly observed on fundus fluorescein angiography.     

An Update on Retinopathy of Prematurity (ROP)

Retinopathy of prematurity (ROP) is a proliferative retinal vascular disease affecting the retina of premature infants. The clinical spectrum of ROP varies from spontaneous regression to bilateral retinal detachment and total blindness. Between these two extremes lies the form of ROP, which is amenable to treatment with laser photocoagulation, anti-vascular endothelial growth factor drugs or surgery. Increasing rates of preterm births coupled with better survival rates but lack of uniform quality of neonatal care and delays in diagnosis have led to increasing ROP blindness. Atypical forms of Aggressive Posterior ROP are seen in heavier birth weight babies in developing countries. Prevention of ROP by following stringent protocols for supplemental oxygen, prevention of sepsis, timely screening and laser treatment by a concerted and collaborative effort of neonatologists and ophthalmologists are required to fight the blindness from ROP.     

Risk factors of threshold retinopathy of prematurity

OBJECTIVE: To determine the risk factors which predispose to the development of threshold retinopathy of prematurity among patients of retinopathy of prematurity. METHODS: The ROP clinic records of a 3 year period were retrospectively studied to identify babies with threshold ROP (T-ROP) and sub-threshold ROP (ST-ROP). Various antenatal and perinatal risk factors, neonatal morbidity and therapeutic interventions were compared between the 2 groups. RESULTS: Of the total of 108 babies, 55 had T-ROP and 53 had ST-ROP. On univariate analysis, packed cell transfusions for anemia, double volume exchange transfusions (DVET), number of DVET, ventilation, gestational age 相似文献   

Retinopathy of prematurity: Past,present and future

Retinopathy of prematurity (ROP) is a vasoproliferative disorder of the retina occurring principally in new born preterm infants. It is an avoidable cause of childhood blindness. With the increase in the survival of preterm babies, ROP has become the leading cause of preventable childhood blindness throughout the world. A simple screening test done within a few weeks after birth by an ophthalmologist can avoid this preventable blindness. Although screening guidelines and protocols are strictly followed in the developed nations, it lacks in developing economies like India and China, which have the highest number of preterm deliveries in the world. The burden of this blindness in these countries is set to increase tremendously in the future, if corrective steps are not taken immediately. ROP first emerged in 1940s and 1950s, when it was called retrolental fibroplasia. Several epidemics of this disease were and are still occurring in different regions of the world and since then a lot of research has been done on this disease. However, till date very few comprehensive review articles covering all the aspects of ROP are published. This review highlights the past, present and future strategies in managing this disease. It would help the pediatricians to update their current knowledge on ROP.     

Retinopathy of prematurity in VLBW and extreme LBW babies

Objective : This is a hospital-based, prospective clinical study to determine the incidence, risk factors, and outcome of extreme low birth weight and very low birth weight pre-term babies with retinopathy of prematurity (ROP) at the Sultan Qaboos University Hospital, Oman.Methods : All babies with a birth weight=/ <1500 g and gestational age =/ < 32 weeks admitted in the Neonatal Unit, were screened for ROP between 4 to 6 weeks of age and staged according to the international classification and were followed up until complete vascularization of the retina. Fifty nine babies formed the study group.Results : The overall incidence of ROP was 25.4% (15 out of 59), of which 6 babies had severe ROP and underwent cryotherapy/laser. All babies with ROP had a birth weight <1250 g and were born before 31 weeks of gestation.Conclusion: ROP is a multifactorial disease, the immature retina of the pre-term baby being the primary factor. Incidence and severity was inversely proportional to birth weight and gestational age. Multiple logistic regression analysis showed that sepsis and total parenteral nutrition to be highly significant risk factors. Repeated blood transfusions, hypotension and congenital heart disease with left to right shunt were seen to be considerably associated with the development of ROP. A decrease in overall incidence and severity of ROP was observed in this study.     

Retinopathy of prematurity: a global perspective of the epidemics, population of babies at risk and implications for control

Globally at least 50,000 children are blind from retinopathy of prematurity (ROP) which is now a significant cause of blindness in many middle income countries in Latin American and Eastern Europe. Retinopathy of prematurity is also being reported from the emerging economies of India and China. The characteristics of babies developing severe disease varies, with babies in middle and low income countries having a much wider range of birth weights and gestational ages than is currently the case in industrialized countries. Rates of disease requiring treatment also tend to be higher in middle and low income countries suggesting that babies are being exposed to risk factors which are, to a large extent, being controlled in industrialised countries. The reasons for this "third epidemic" of ROP are discussed as well as strategies for control, including the need for locally relevant, evidence based criteria which ensure that all babies at risk are examined.     

Retinopathy of prematurity: Mutations in the Norrie disease gene and the risk of progression to advanced stages

BACKGROUND: Retinopathy of prematurity (ROP) is a retinal vascular disease that occurs in infants with short gestational age and low birth weight and may lead to retinal detachment and blindness. Missense mutations in the Norrie disease (ND) gene have been associated with the risk of progression to advanced stages in cases of ROP from the US and also in clinically similar ND and familial exudative vitreoretinopathy. METHODS: We have screened two ND gene mutations, namely A105T and Val60Glu, by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) and allele-specific PCR methods, respectively, in 210 Kuwaiti premature newborns to replicate these findings in a different ethnic group. RESULTS: In the Kuwaiti premature newborn cohort, 115 of 210 babies had no eye problems and served as controls, while 95 were cases of ROP. In 71 of 95 ROP cases, the disease regressed spontaneously on or before stage 3, while in 24 of 95 ROP cases the disease progressed to advanced stages 4 and 5. In case of missense mutation (A105T), the AA genotype was detected in 96% of controls compared with 87% of ROP cases (NS); similarly no significant difference was found between spontaneously regressed ROP cases and those who progressed to advanced stages. For the Val60Glu mutation, no significant association was detected between the genotype and progression of ROP to advanced stages. CONCLUSIONS: Unlike data from the US, our findings from a Kuwaiti cohort of ROP cases and controls suggest a lack of association between the two ND gene mutations (A105T and Val60Glu) and ROP and the risk of progression of the disease to advanced stages.     

Screening for retinopathy of prematurity in developing countries

Improved survival of low birth weight, premature babies in India has increased the incidence of retinopathy of prematurity. Western reports describe screening criteria to pick up babies most at risk. However, our population of at-risk neonates is likely to be different, as most nurseries in India are not very well equipped. Our aim was to develop a screening strategy appropriate for our conditions. Ophthalmic records of 60 neonates with gestational age < or =35 weeks and/or birth weight < or =1500 g, born over a 1-year period, were retrospectively reviewed. Laterality, location and stage of retinopathy of prematurity were recorded. Age at detection, at threshold disease and at maximum stage was recorded, and progression or regression of retinopathy noted. The incidence of retinopathy was 13/60 (21.7%) and of threshold disease was 3/60 (5.0%). Threshold disease was never seen before 5.5 weeks PNA. Zone I disease invariably, zone II disease in 12.5% cases and zone III disease never progressed to threshold stage. Most (10/13; 76.9%) cases regressed without treatment. Screening for retinopathy should commence at 4 weeks PNA (post-neonatal age). Screening time, discomfort to the baby and complications can be reduced by examining temporal retina first. If normal, the nasal retina need not be examined. Also, babies with zone III disease need not be followed up to complete visualization. Retinal vascular dilatation, resistance to pupillary dilation and persistence of tunica vasculosa lentis can be indicators of intensive screening.     

Retinopathy of prematurity-current diagnosis and management

Despite advances in ophthalmological care of premature infants, retinopathy of prematurity (ROP) remains a still unsolved problem for paediatricians as well as ophthalmologists. A survey of the current literature concerning drug therapy and surgical management as related to the different stages of ROP is given. The classification system for ROP according to the International Committee is presented as well as our screening policy in relation to the literature. The effectiveness in preventing severe cases of ROP and the toxicity of vitamin E supplementation in high-risk premature infants is still disputed and no recommendations can be given. Cryotherapy is recommended in symmetric cases of stage 3+ ROP. Nevertheless, quite a number of eyes still progress to more severe stages of ROP. Scleral buckling procedures and vitrectomy may lead to anatomical success in a few cases of retinal detachment, however, the visual outcome of such an operation is usually very poor despite reattachment of the cental retina.     

Retinopathy of prematurity: overview of new global problem

Retinopathy of prematurity (ROP) is a disease characterized by abnormal retinal vasculature in preterm infants. It is an important cause of visual disability in premature infants and although the incidence varies among different countries it is increasing as advances in neonatal care result in improved survival. Oxygen, growth factors like vascular endothelial growth factor, and poor postnatal growth play a significant role in the pathogenesis of ROP. Targeting lower oxygen saturation is associated with a reduction in ROP, but with increased mortality. Screening for ROP varies between centres and countries but generally it includes preterm infants (less than 32 weeks’ gestation) and/or those with a birth weight of less than 1500g. ROP has been recently reclassified as type-1-needing treatment and type-2 ROP needing observation, based on the benefits and treatment efficacy. Laser therapy and anti-VEGF are the two main treatments. Recent reports suggest that anti-VEGF therapy may have better visual outcomes (myopia) and a better safety profile. ROP is a global disease of prematurity and understanding the pathogenesis, course of ROP, preventive strategies, treatment options and outcomes are essential for all healthcare professionals caring for preterm babies. This short article describes the evidence for screening, prevention and treatment options and looks ahead to possible advances in the near future.     

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??Abstract??Objective??To evaluate the clinical features of retinopathy of prematurity ??ROP?? and the long-term therapeutic effect. Methods??The clinical data ?? ROP stage ?? the therapy and long-term prognosis of 107 preterm infants with ROP were retrospectively analyzed??who were admitted to Children’s Hospital of Fudan University between January 1?? 2004 to July 31?? 2009. Results??Among 64 preterm infants with Stage 1 or 2 ROP?? 6 infants with Stage 2 ROP developed to type I threshold ROP and received the laser therapy. All the follow-up infants except the death did not need special treatment without the vision affected later and the lesions of ROP were self-limiting. Fifteen infants were detected with Stage 3 ROP?? of whom 14 infants had threshold ROP and received the laser therapy. Another one infant who did not develop to the threshold ROP had no treatment. Eleven follow-up infants had no blindness ?? but three infants’ vision was affected severely after the treatment and 8 infants had the normal vision. There were 28 Stage 4 or 5 ROP infants??but 18 infants were followed up completely. In follow-up infants ?? there was only one infant with the normal post-operative vision??5.6%???? 12 ones had blindness after treatment ??66.7%???? and the remaining five ones had poor eyesight and were light-sensitive ??27.7%??. Conclusion??It’s a key step to screening and intervention in time at the early stage of ROP. Otherwise the outcome is very poor when developed to the late stage with retinal detachment.     

Screening for retinopathy of prematurity: results of a retrospective 3-year study of 502 infants

PURPOSE: To describe the surveillance, results of screening, and treatment of retinopathy of prematurity (ROP) in a university hospital setting in southeast France. PATIENTS AND METHODS: Five hundred two premature infants were included in the screening protocol between January 1997 and December 1999. Criteria for inclusion in the study were a gestational age of 32 weeks or younger, a birth weight of less than 1,501 g, or both. The first fundus examination was performed between 4 and 6 weeks of life. Thereafter, fundus examination was performed in the absence of ROP every 2 weeks until complete retinal vasculature developed, gestational age of 50 weeks, or death. Examination was weekly in cases of retinopathy, biweekly if progression was ascertained, and less frequent only if regression was evident. Hospital records were reviewed to assess the presence or absence and eventual degree of ROP. RESULTS: Stage 1 was observed in 32 infants, and stage 2 in 11 infants; all of these cases regressed. Three cases of bilateral stage 3 (two threshold and one prethreshold) disease underwent diode laser peripheral retinal ablation and regressed. One infant with bilateral stage 3 disease who underwent peripheral cryoablative surgery progressed to stage 4A in one eye and 4B in the other eye and then underwent scleral buckling surgery in the second eye. CONCLUSIONS: Despite survival increasing with improved neonatal intensive care, the incidence of ROP does not appear to be increasing. In our center, the incidence appears to be lower than previously reported.     

Screening for retinopathy of prematurity in a tertiary care newborn unit in Turkey: frequency, outcomes, and risk factor analysis

PURPOSE: To report the frequency, risk factors, and outcomes of screening for retinopathy of prematurity (ROP). METHODS: Data of neonates with a gestational age of 34 weeks or less were analyzed and the predictors on the development of ROP were determined by using logistic regression analysis. RESULTS: Of the 318 neonates, the frequency of ROP was 37.1% for any stage and 7.2% for stage 3 or greater. Treatment was needed in 16.1% of neonates with ROP. No treatment was required in neonates with a gestational age of greater than 32 weeks. Oxygen therapy, sepsis, gestational age of 32 weeks or less, and birth weight of less than 1,250 g were determined as the independent risk factors. CONCLUSIONS: Although frequency of ROP in Turkey is similar to that in the United States, the rate of severe ROP necessitating treatment seems to be higher in Turkey. Neonates with a gestational age of 32 weeks or less, a birth weight of less than 1,250 g, sepsis, and oxygen therapy may have a greater risk of developing ROP and screening should be intensified in the presence of these risk factors.     

Retinopathy of prematurity screening and follow-up with Retcam120: expertise of a team of neonatologists concerning 145 patients]

OBJECTIVES: The high incidence of retinopathy in very premature infants requires strict evaluation and follow-up in neonatal intensive care. The strict organization required in each center, under the responsibility of ophthalmologists, is sometimes puzzling. Therefore, we tested the hypothesis that the introduction of the Retcam allows the neonatologist under the control of ophthalmologist to diagnose retinopathy of prematurity then preventing sequelae, by comparison of pictures interpretations between neonatologists and ophthalmologists. METHODS: The Retcam gives a 120 degrees picture of the retina which is captured digitally. Then, the interpretation of the neonatologist can be reviewed by the ophthalmologist. We screened premature babies less than 32 weeks of gestation and less than 1500 g, during 1 year, including learning experience. We compared pictures interpretation by neonatologists and ophthalmologists of Retcam recordings. RESULTS: One hundred and forty-five patients were included. Eight cases of retinopathy were diagnosed with an exact correlation : 3 grade III in zone 2 form plus disease, 1 stage III zone 2 unilateral, 1 stage II in zone 3, 2 stage II en zone 2, 1 grade I zone 3 on at least 5 h contiguous. We had neither false positive, nor false negative. Five infants were treated without significant sequelae. CONCLUSIONS: Retcam 120 allows an easy diagnosis and follow-up for the retinopathy of prematurity by the neonatologist. We advocate to spread Retcam to the wards where the screening of retinopathy is difficult for the ophthalmology department. As every case requiring therapy is diagnosed, prevention of severe visual handicap is completed. The cost of this apparatus is equivalent to the cost of the care for a congenital blindness.