Stage 5 retinopathy of prematurity: The silent epidemic blinding our smallest babies
Year: 2024
Type: article
Abstract: India had the highest number of preterm births (3.02 million) in the world in 2020, accounting for over 20% of all preterm births worldwide.[1] Retinopathy of prematurity (ROP) is the most common cause of childhood blindness seen in preterm and low-birth-weight babies, and the third “ROP epidemic” is severely affecting low- and middle-income countries. With timely ROP screening, severe ROP can be detected and treated early with intravitreal anti-VEGF injections, laser therapy, or microincision lens-sparing surgery with excellent outcomes. Stage 5 ROP is the last stage with total retinal detachment, which leads to irreversible blindness.[2] As ROP affects both eyes, it can lead to bilateral blindness. With the rising incidence of assisted reproduction techniques and multiple births, all the siblings might be affected by blinding stage 5 ROP, which is particularly devastating to families. Many babies develop aggressive ROP (AROP), a severe variant that rapidly progresses to stage 5 blinding ROP. Ocular auto-stimulation is also commonly observed (78.5%) in stage 5 ROP-affected babies, leading to enophthalmos, corneal opacity, and posterior synechiae, which is further cosmetically disabling.[3] Often these preterm babies are labeled as blind from birth, but it is well known that ROP develops after birth due to multiple systemic risk factors and poor NICU care. The common underlying story is that the sick preterm baby (who braved multiple systemic life-threatening disorders and a prolonged NICU stay) was never screened for ROP. Parents notice bilateral leukocoria or nystagmus and visit an eye surgeon who diagnoses it as stage 5 ROP, which comes as a huge shock to parents. This has led to rising numbers of ROP blindness-related medicolegal cases, which is resulting in large payouts,[4] and this is just the beginning as the public is becoming more aware of ROP and its consequent blindness. Lack of screening is the most important reason that babies develop stage 5 ROP.[5,6] It is alarming that 32.5% (230 eyes of 115 babies) of ROP cases presenting to a single center in a year had stage 5 ROP, of which 109 (89.8%) babies had never been screened for ROP.[5] This is even though ROP is a decades-old disease, and well-established Indian national ROP screening guidelines are available.[7] It is notable that these guidelines differ from other Asian countries[8] and US guidelines[9] as bigger babies develop ROP in our country. Surprisingly, many pediatricians are not well aware of ROP and its blindness implications.[10,11] It is also difficult to fathom the huge number of babies that might have gone ROP-blind due to a lack of screening during the COVID-19 pandemic lockdowns.[12] Surgical outcomes are poor in stage 5 ROP, yet many ophthalmic surgeons operate these eyes in the hope of providing navigable vision.[13,14] There are still very few ophthalmic centers with advanced surgical setups, trained vitreoretinal surgeons, pediatric anesthetists, and good NICU backup. Complex surgery with poor outcomes demotivates retinal surgeons, and parents too are reluctant to provide consent for surgery. Therefore, timely treatment with laser, anti-VEGF injections, or surgery must be done for the best outcomes, but the only way to detect and refer treatable ROP in time is by effective ROP screening programs. Government programs such as Janani Shishu Suraksha Karyakram provide much-needed monetary support making these expensive anti-VEGF injections and treatment services accessible to these babies. Stage 5 ROP blind babies must have access to rehabilitation services to get support regarding orientation and mobility training, activities of daily living, visual aids, braille education, special school admission, and disability certification. It empowers the child to be self-reliant, provides support to the family, and enables these babies to grow up as independent individuals reducing the burden on society. However, visual rehabilitation is challenging, and very few centers have trained technicians who can provide this care, and there is a need to increase capacity. Counseling parents regarding challenges associated with bringing up a blind child needs sensitization of the treating doctors and support teams. It is time to strike the root cause of the problem and prevent ROP.[15] In a tertiary hospital, of all referred ROP cases in 1 year, 22.6% (486 eyes) of out-born babies had stage 5 ROP, while no stage 5 ROP was seen in in-born babies,[16] highlighting that high-quality NICU care can prevent severe ROP.[17] There might never be enough ophthalmologists and treatment services to deal with this rising epidemic. Therefore, it is best to prevent ROP by implementing best practices such as optimal use of oxygen, use of oxygen blenders, judicious blood transfusions, management of anemia and hypothermia, preventing sepsis, early breastfeeding, delayed cord clamping, and antenatal corticosteroids for preterm births.[18] Mandatory establishment of ROP screening programs in every NICU, regular audit of NICU protocols, and central monitoring of ROP incidence data in NICUs with feedback and quality improvement are necessary. The establishment of telescreening programs with public-private partnerships,[19] neonatology-led screening,[20] and AI-assisted screening can ensure wider ROP screening coverage in the community. Sensitization and training of rural health workers, neonatal nurses, and obstetricians to prevent preterm birth, follow best delivery/NICU protocols, and ensure referral for ROP screening is essential. There should be clear ROP referral/treatment protocols once treatment-warranted ROP is detected. No preterm baby eligible for ROP screening should go unscreened. It is time all stakeholders recognize the urgency to prevent stage 5 ROP blindness and deal with the silent epidemic blinding our smallest babies.About the authorProf. (Dr.) Parijat Chandra is a Professor of Ophthalmology at Dr. R. P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences (AIIMS), New Delhi, India. He is actively engaged in research and management of paediatric vitreoretinal diseases, especially retinopathy of prematurity (ROP) for the last 25 years. He has authored 150+ indexed peer-reviewed publications. He has conducted nationwide ROP training workshops, instruction courses and was Co-PI of the Sightsavers-supported National ROP Project in India. He was organizing secretary of the 2nd World ROP Congress in New Delhi (India) and the scientific program coordinator of the 3rd World ROP Congress in Shanghai (China). He is member of the National ROP Task Force under the Ministry of Health and Family Welfare, Government of India.
Source: Indian Journal of Ophthalmology
Author: Parijat Chandra
Institution: All India Institute of Medical Sciences
Language: English
Subfield: Radiology, Nuclear Medicine and Imaging
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4404839462