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| == Retinopathy of Prematurity (ROP): Understanding a Neonatal Eye Disorder ==
| | {{SI}} |
| | | {{Infobox medical condition |
| [[File:NEI Animation- Retinopathy of Prematurity (ROP)harf-size.gif|thumb|right|500px|A retinal image showing the effects of Retinopathy of Prematurity (ROP).]] | | | name = Retinopathy of prematurity |
| | | | image = [[File:Eye_exam.jpg|250px]] |
| '''Retinopathy of Prematurity (ROP)''', also known as '''retrolental fibroplasia (RLF)''' and '''Terry syndrome''', is a medical condition affecting the eyes of prematurely born infants, particularly those who have received neonatal intensive care, including oxygen therapy for underdeveloped lungs. ROP is characterized by the disorganized growth of retinal blood vessels, which can lead to scarring and retinal detachment. This comprehensive article explores the definition, causes, risk factors, clinical manifestations, diagnosis, treatment, and preventative measures related to ROP.
| | | alt = |
| | | | caption = Eye examination for retinopathy of prematurity |
| === Understanding Retinopathy of Prematurity === | | | synonyms = ROP |
| ROP is a significant eye disorder primarily affecting premature infants.
| | | pronounce = |
| | | | specialty = [[Ophthalmology]] |
| == Causes and Risk Factors == | | | symptoms = Abnormal blood vessel development in the [[retina]] |
| The development of ROP is influenced by several factors:
| | | onset = Premature infants |
| | | | duration = |
| === Premature Birth === | | | types = |
| Prematurely born infants, especially those with very low birth weight, are at heightened risk for ROP due to the incomplete development of their retinas.
| | | causes = [[Premature birth]] |
| | | | risks = Low birth weight, early gestational age |
| | | diagnosis = [[Eye examination]] |
| | | differential = |
| | | prevention = |
| | | treatment = [[Laser therapy]], [[cryotherapy]], [[anti-VEGF therapy]] |
| | | medication = |
| | | prognosis = Varies; can lead to [[blindness]] if untreated |
| | | frequency = |
| | | deaths = |
| | }} |
| | {{Short description|A disease of the eye affecting premature infants}} |
| [[File:Human Infant in Incubator.jpg|thumb|left|250px|Premature birth is a significant risk factor for the development of ROP.]] | | [[File:Human Infant in Incubator.jpg|thumb|left|250px|Premature birth is a significant risk factor for the development of ROP.]] |
| | | [[File:Human eye cross section detached retina.svg|thumb|left}|alt=Diagram of an eye, in cross-section. |The retina (red) is detached at the top of the eye.]] |
| === Neonatal Intensive Care ===
| | [[Image:ROP zones.jpg|250px|thumb|left]] |
| Neonatal intensive care, often involving oxygen therapy to support lung development, is a common setting where ROP can occur.
| | [[File:Human eye cross section scleral buckle.svg|thumb|left|alt=Diagram of an eye with a scleral buckle, in cross-section. |The silicone band ([[scleral buckle]], blue) is placed around the eye. This brings the wall of the eye into contact with the detached retina, allowing the retina to re-attach.]] |
| | | [[File:NEI Animation- Retinopathy of Prematurity (ROP)harf-size.gif|thumb|left|500px|A retinal image showing the effects of Retinopathy of Prematurity (ROP).]] |
| === Oxygen Toxicity and Hypoxia ===
| | '''Retinopathy of prematurity''' ('''ROP''') is a potentially blinding eye disorder that primarily affects premature infants. It is characterized by abnormal development of [[retina|retinal]] blood vessels. The condition can lead to [[retinal detachment]] and [[blindness]] if not properly managed. |
| The balance between oxygen toxicity and relative hypoxia plays a critical role in the development of ROP.
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| ==Causes==
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| By the fourth month of pregnancy, the fetal retina has begun to develop vascularization. Such formation of blood vessels appears to be very sensitive to the amount of oxygen supplied, either naturally or artificially. In rare cases ROP has been found in some patients with a mutation in the NDP gene, which is normally associated with the more formidable [[Norrie disease]].<ref name="pmid9152134">{{cite journal|last=Shastry|first=BS|author2=Pendergast, SD |author3=Hartzer, MK |author4=Liu, X |author5= Trese, MT |title=Identification of missense mutations in the Norrie disease gene associated with advanced retinopathy of prematurity.|journal=Archives of Ophthalmology|date=May 1997|volume=115|issue=5|pages=651–5|pmid=9152134|doi=10.1001/archopht.1997.01100150653015}}</ref><ref name="pmid16970763">{{cite journal|last=Dickinson|first=JL |author2=Sale, MM |author3=Passmore, A |author4=FitzGerald, LM |author5=Wheatley, CM |author6=Burdon, KP |author7=Craig, JE |author8=Tengtrisorn, S |author9=Carden, SM |author10=Maclean, H |author11=Mackey, DA|title=Mutations in the NDP gene: contribution to Norrie disease, familial exudative vitreoretinopathy and retinopathy of prematurity.|journal=Clinical & Experimental Ophthalmology|date=Sep–Oct 2006|volume=34|issue=7|pages=682–8|pmid=16970763|doi=10.1111/j.1442-9071.2006.01314.x}}</ref><ref name="pmid20738858">{{cite journal|last=Shastry|first=Barkur S|title=Genetic susceptibility to advanced retinopathy of prematurity (ROP)|journal=Journal of Biomedical Science|date=1 January 2010|volume=17|issue=1|pages=69|doi=10.1186/1423-0127-17-69|pmid=20738858|pmc=2933676}}</ref>
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| ===Risk factors===
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| Various risk factors contribute to the development of ROP. They are:
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| * Prematurity<ref>{{Cite journal | last1 = Karna | first1 = P. | last2 = Muttineni | first2 = J. | last3 = Angell | first3 = L. | last4 = Karmaus | first4 = W. | title = Retinopathy of prematurity and risk factors: A prospective cohort study | journal = BMC Pediatrics | volume = 5 | issue = 1 | pages = 18 | doi = 10.1186/1471-2431-5-18 | year = 2005 | pmid = 15985170 | pmc =1175091 }}</ref>
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| * High exposure to oxygen
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| * Low birth weight
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| * Various types of infections
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| * Cardiac defects
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| ==Pathophysiology== | | ==Pathophysiology== |
| | | ROP occurs when the normal development of the retinal blood vessels is disrupted. In a full-term infant, the blood vessels of the retina finish developing in the last few weeks of pregnancy. However, in premature infants, this process is incomplete. The abnormal growth of these vessels can lead to scarring and pulling on the retina, potentially causing [[retinal detachment]]. |
| During development, blood vessels grow from the central part of the retina outwards. This process is completed a few weeks before the normal time of delivery. However, in [[premature babies]] it is incomplete. If blood vessels grow normally, ROP does not occur. If the vessels grow and branch abnormally the baby develops ROP. These abnormal blood vessels may grow up from the plane of the retina and may bleed inside the eye. When the blood and abnormal vessels are reabsorbed, it may give rise to multiple ''band like'' membranes which can pull up the retina, causing detachment of the retina and eventually blindness before 6 months.
| | ==Risk Factors== |
| | | Several factors increase the risk of developing ROP, including: |
| Normally, maturation of the retina proceeds ''in utero'', and at term, the [[Human anatomical terms#Anatomical directions|medial]] portion (Nasal retina) of the [[retina]] is fully vascularized, while the [[Lateral (anatomy)|lateral]] portion (Temporal retina) is only incompletely vascularized.<ref name=RobbinsPath>{{cite book|last=Kumar|first=Vinay|title=Robbins basic pathology|year=2007|publisher=Saunders/Elsevier|location=Philadelphia|isbn=978-1416029731|chapter=Chapter 29: Eye, Retina and Vitreous, Retinal Vascular Disease|edition=8th}}</ref> The normal growth of the blood vessels is directed to relatively low-oxygen areas of the retina, but the vessels remain in the plane of the retina and do not grow into the [[vitreous humor]]. If excess oxygen is given, normal blood vessels degrade and cease to develop. When the excess oxygen environment is removed, the blood vessels rapidly begin forming again and grow into the vitreous humor of the eye from the retina.<ref name=RobbinsPath /><ref>{{cite book |last1=Guyton |first1=Arthur |last2=Hall |first2=John |editor1-first=Rebecca |editor1-last=Gruliow |title=Textbook of Medical Physiology |type=Book |edition=11th |year=2006 |publisher=Elsevier Inc. |location=Philadelphia, Pennsylvania |isbn=978-0-7216-0240-0 |page=200 |chapter=Chapter 17: Local and Humoral Control of Blood Flow by the Tissues}}</ref>
| | * Premature birth, particularly before 31 weeks of gestation |
| | | * Low birth weight, especially less than 1500 grams |
| The key disease element in ROP is fibrovascular proliferation. This is growth of abnormal new vessels; this may regress, but frequently progresses. Associated with the growth of these new vessels is fibrous tissue (scar tissue) that may contract to cause retinal detachment. Multiple factors can determine whether the disease progresses, including overall health, birth weight, the stage of ROP at initial diagnosis, and the presence or absence of "plus disease". Supplemental [[oxygen]] exposure, while a [[risk factor]], is not the main risk factor for development of this disease. Restricting supplemental oxygen use reduces the rate of ROP, but may raise the risk of other [[Hypoxia (medical)|hypoxia]]-related systemic complications, including death.<ref name="Stenson et al. Oxygen Saturation and Outcomes in Preterm Infants. NEJM 2013">{{cite journal|last=Stenson|author2=BOOST ll Cooperative Groups |title=Oxygen Saturation and Outcomes in Preterm Infants|journal=New England Journal of Medicine|year=2013|volume=368|issue=22 |pages=2094–2104|doi=10.1056/nejmoa1302298|pmid=23642047 |url=http://espace.library.uq.edu.au/view/UQ:319530/UQ319530_OA.pdf }}</ref>
| | * High levels of [[oxygen therapy]] |
| | | * [[Sepsis]] |
| Patients with ROP, particularly those who have developed severe disease needing treatment are at greater risk for [[strabismus]], [[glaucoma]], [[cataracts]] and shortsightedness ([[myopia]]) later in life and should be examined yearly to help prevent or detect and treat these conditions.
| | * [[Respiratory distress syndrome]] |
| | | ==Stages== |
| | ROP is classified into five stages, ranging from mild (Stage 1) to severe (Stage 5): |
| | * '''Stage 1''': Mildly abnormal blood vessel growth. |
| | * '''Stage 2''': Moderately abnormal blood vessel growth. |
| | * '''Stage 3''': Severely abnormal blood vessel growth. |
| | * '''Stage 4''': Partial retinal detachment. |
| | * '''Stage 5''': Total retinal detachment. |
| ==Diagnosis== | | ==Diagnosis== |
| | | ROP is diagnosed through a comprehensive eye examination by an ophthalmologist. The examination involves dilating the infant's pupils and using an ophthalmoscope to view the retina. Regular screenings are recommended for at-risk infants. |
| The stages of ROP disease have been defined by the International Classification of Retinopathy of Prematurity (ICROP).
| | ==Treatment== |
| | | Treatment for ROP depends on the severity of the condition. Options include: |
| In older patients, the appearance of the disease is less well described but includes the residua of the ICROP stages as well as secondary retinal responses.
| | * '''Laser therapy''': Used to stop the abnormal growth of blood vessels. |
| | | * '''Cryotherapy''': Freezing treatment to prevent further retinal damage. |
| ===International classification===
| | * '''Anti-VEGF injections''': Medications injected into the eye to inhibit the growth of abnormal blood vessels. |
| | | * '''Surgery''': In advanced cases, surgery may be necessary to reattach the retina. |
| The system used for describing the findings of active ROP is entitled ''The International Classification of Retinopathy of Prematurity (ICROP)''.<ref>{{cite journal| author = Committee for the Classification of Retinopathy of Prematurity| title = An international classification of retinopathy of prematurity | journal = Arch. Ophthalmol. | date = Aug 1984| volume = 102| issue = 8| pages = 1130–1134| pmid = 6547831| doi = 10.1001/archopht.1984.01040030908011}}</ref> ICROP uses a number of parameters to describe the disease. They are location (zone) of the disease, the circumferential extent of the disease based on the clock hours, the severity (stage) of the disease and the presence or absence of "Plus Disease". Each aspect of the classification has a technical definition. This classification was used for the major clinical trials. It was revised in 2005.<ref>{{cite journal| author = Committee for the Classification of Retinopathy of Prematurity| title = The International Classification of Retinopathy of Prematurity revisited| journal = Arch. Ophthalmol. | date = Jul 2005| volume = 123| issue = 7| pages = 991–999| doi = 10.1001/archopht.123.7.991| pmid = 16009843| doi-access = free}}</ref>
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| [[Image:ROP zones.jpg|250px|thumb|left|Zones of the retina in ROP]]The zones are centered on the optic nerve. Zone{{nbsp}}I is the posterior zone of the retina, defined as the circle with a radius extending from the optic nerve to double the distance to the macula. Zone{{nbsp}}II is an annulus with the inner border defined by zone{{nbsp}}I and the outer border defined by the radius defined as the distance from the optic nerve to the nasal ora serrata. Zone{{nbsp}}III is the residual temporal crescent of the retina.
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| The circumferential extent of the disease is described in segments as if the top of the eye were 12 on the face of an analog clock, e.g. stage{{nbsp}}1 from 4:00 to 7:00. (The extent is a bit less important since the treatment indications from the Early Treatment for ROP.)<ref>{{cite journal| author = Early Treatment for Retinopathy of Prematurity Cooperative Group| title = Revised indications for the treatment of retinopathy of prematurity: results of the early treatment for retinopathy of prematurity randomized trial| journal = Arch. Ophthalmol. | year = 2003| volume = 121| pages = 1684–1696| doi = 10.1001/archopht.121.12.1684| pmid = 14662586| issue = 12| doi-access = free}}</ref>
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| The Stages describe the ophthalmoscopic findings at the junction between the vascularized and avascular retina.
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| * Stage 1 is a faint demarcation line.
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| * Stage 2 is an elevated ridge.
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| * Stage 3 is extraretinal fibrovascular tissue.
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| * Stage 4 is sub-total retinal detachment.
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| * Stage 5 is total retinal detachment.
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| ===Plus disease===
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| Plus disease can be present as a major complicating factor at any stage. It is characterised by:
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| * Significant level of vascular dilation and [[tortuosity]] observed at the posterior retinal arterioles. This reflects the increase of blood flow through the retina.<ref name="emedicine">{{eMedicine|oph|413|Retinopathy of Prematurity}}</ref>
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| * Vitreous haze and anterior chamber haze<ref name="emedicine" />
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| * Iris vascular engorgement<ref name="emedicine" />
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| * [[Persistent tunica vasculosa lentis]] or immature blood vessels growing over the lens which also restrict the dilatation of the pupils.<ref name="emedicine" />
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| ===Differential diagnosis===
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| The most difficult aspect of the differential diagnosis may arise from the similarity of two other diseases:
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| * [[Familial exudative vitreoretinopathy]] which is a genetic disorder that also disrupts the retinal vascularization in full-term infants.
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| * [[Persistent fetal vasculature syndrome]] also known as [[persistent hyperplastic primary vitreous]] that can cause a traction [[retinal detachment]] difficult to differentiate but typically unilateral.
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| ==Screening==
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| Almost all infants with ROP have a gestational age of 31 weeks or less (regardless of birth weight) or a birth weight of 1250 g (2.76 lbs) or less; these indications are generally used to decide whether a baby should be screened for ROP, but some centres, especially in developing countries [http://utsaveyeclinic.com/ROP-screening-guidelines/] extend birth weight screening criteria to 1500 g (3.3 lbs).<ref name="ROP Screening Reccom">{{cite journal|last=Jefferies|first=AL|author2=Canadian Paediatric Society |author3=Fetus and Newborn Committee |title=Retinopathy of prematurity: Recommendations for screening|journal=Paediatrics & Child Health|date=1 December 2010|volume=15|issue=10|pages=667–0|url=http://www.cps.ca/en/documents/position/retinopathy-of-prematurity-screening|accessdate=9 March 2013|pmc=3006218|pmid=22131866|doi=10.1093/pch/15.10.667}}</ref>
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| Any premature baby with severe illness in perinatal period (Respiratory distress syndrome, sepsis, blood transfusion, Intra ventricular haemorrhage, apnoeic episodes, etc.) may also be offered ROP screening.
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| ===Timing===
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| Retinal examination with scleral depression is generally recommended for patients born before 30–32 weeks [[gestation]], or 4–6 weeks of life, whichever is later. It is then repeated every 1–3 weeks until vascularization is complete (or until disease progression mandates treatment).
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| ===Procedure===
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| Following [[pupillary dilation]] using eye drops, the [[retina]] is examined using a special lighted instrument (an [[Ophthalmoscope|indirect ophthalmoscope]]). The peripheral portions of the retina are sometimes pushed into view using [[scleral depression]]. Examination of the retina of a premature infant is performed to determine how far the retinal blood vessels have grown (the zone), and whether or not the vessels are growing flat along the wall of the eye (the stage). Once the vessels have grown into zone{{nbsp}}III (see below) it is usually safe to discharge the child from further screening for ROP. The stage of ROP refers to the character of the leading edge of growing retinal blood vessels (at the vascular-avascular border).
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| ===Monitoring=== | |
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| In order to allow timely intervention, a system of monitoring is undertaken for infants at risk of developing ROP. These monitoring protocols differ geographically because the definition of high-risk is not uniform or perfectly defined. In the USA the consensus statement of experts is informed by data derived by clinical trials and published in Pediatrics 2006. They included infants with birthweights under 1500 grams or under 30 weeks gestation in most cases. The first examination should take place within the first 4 weeks of birth, and regular, weekly examination is required until it is clear that the eyes are not going to develop disease needing treatment, or one or both eyes develop disease requiring treatment. Treatment should be administered within a 48 hours, as the condition can progress rapidly.
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| ==Management==
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| ===Treatment===
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| [[File:Human eye cross section detached retina.svg|thumb|right}|alt=Diagram of an eye, in cross-section. |The retina (red) is detached at the top of the eye.]]
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| [[File:Human eye cross section scleral buckle.svg|thumb|right|alt=Diagram of an eye with a scleral buckle, in cross-section. |The silicone band ([[scleral buckle]], blue) is placed around the eye. This brings the wall of the eye into contact with the detached retina, allowing the retina to re-attach.]]
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| * Peripheral retinal ablation is the mainstay of ROP treatment. The destruction of the avascular retina is performed with a solid state [[laser photocoagulation]] device, as these are easily portable to the operating room or [[neonatal]] [[Intensive care unit|ICU]]. [[Cryotherapy]], an earlier technique in which regional retinal destruction was done using a probe to freeze the desired areas, has also been evaluated in multi-center clinical trials as an effective modality for prevention and treatment of ROP. However, when laser treatment is available, cryotherapy is no longer preferred for routine avascular retinal ablation in premature babies, due to the side effects of inflammation and lid swelling. Further more recent trials have shown that treatment at an earlier stage of the disease gives better results.<ref>{{Cite journal | last1 = Dobson | first1 = V. | last2 = Quinn | first2 = G. E. | last3 = Summers | first3 = C. G. | last4 = Hardy | first4 = R. J. | last5 = Tung | first5 = B. | last6 = Good | first6 = W. V. | last7 = Good | first7 = W. V. | doi = 10.1001/archophthalmol.2011.143 | title = Grating Visual Acuity Results in the Early Treatment for Retinopathy of Prematurity Study | journal = Archives of Ophthalmology | volume = 129 | issue = 7 | pages = 840–846 | year = 2011 | pmid = 21746974 | pmc = 4374597}}</ref> | |
| * [[Scleral buckle|Scleral buckling]] and/or [[vitrectomy]] surgery may be considered for severe ROP (stages{{nbsp}}4 and{{nbsp}}5) for eyes that progress to [[retinal detachment]]. Few centers in the world specialize in this surgery, because of its attendant surgical risks and generally poor outcomes.
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| * Intravitreal injection of [[bevacizumab]] ([[Avastin]]) has been reported as a supportive measure in aggressive posterior retinopathy of prematurity.<ref>{{cite journal |vauthors=Shah PK, Narendran V, Tawansy KA, Raghuram A, Narendran K |title=Intravitreal bevacizumab (Avastin) for post laser anterior segment ischemia in aggressive posterior retinopathy of prematurity|journal=Indian Journal of Ophthalmology |volume=55 |issue=1 |pages=75–76 |year=2007 |pmid=17189897 |doi=10.4103/0301-4738.29505|doi-access=free }}</ref> In a 2011 clinical trial comparing bevacizumab with conventional laser therapy, intravitreal bevacizumab monotherapy showed a significant benefit for zone{{nbsp}}I but not zone{{nbsp}}II disease when used to treat infants with stage{{nbsp}}3+ retinopathy of prematurity.<ref>{{cite journal|pmid=21323540|year=2011|last1=Mintz-Hittner|first1=HA|last2=Kennedy|first2=KA|last3=Chuang|first3=AZ|last4=Beat-Rop Cooperative|first4=Group|title=Efficacy of intravitreal bevacizumab for stage{{nbsp}}3+ retinopathy of prematurity|volume=364|issue=7|pages=603–15|doi=10.1056/NEJMoa1007374|pmc=3119530|journal=The New England Journal of Medicine}}</ref> Potential benefits of intravitreal Avastin injection over laser therapy include: reduction in level of anesthesia required, preservation of viable peripheral retina, and, possibly, reduced incidence of subsequent high refractive error. However, the safety of this new treatment has not yet been established in terms of ocular complications as well as systemic complications. The latter are theoretically possible, as the active ingredient of bevacizumab not only blocks the development of abnormal blood vessels in the eye but may also prevent the normal development ofother tissues such as the lung and kidney. A 2018 Cochrane review also examined the effectiveness of [[Anti–vascular endothelial growth factor therapy|Anti-vascular Endothelial Growth Factor]] Drugs and their use for ROP.<ref>{{Cite journal|last=Sankar|first=Mari Jeeva|last2=Sankar|first2=Jhuma|last3=Chandra|first3=Parijat|date=8 January 2018|title=Anti-vascular endothelial growth factor (VEGF) drugs for treatment of retinopathy of prematurity|url=https://www.ncbi.nlm.nih.gov/pubmed/29308602|journal=The Cochrane Database of Systematic Reviews|volume=1|pages=CD009734|doi=10.1002/14651858.CD009734.pub3|issn=1469-493X|pmc=6491066|pmid=29308602|via=}}</ref> | |
| * Oral propranolol is being evaluated for counteracting the progression of ROP, but safety is a concern. A prospective randomized trial in which pre-term newborns were randomized to receiving oral propranolol with standard treatment or standard treatment alone found that oral propranolol showed a 48% relative risk reduction for progression to stage{{nbsp}}3, 58% reduction for progression to stage{{nbsp}}3 plus, and 100% reduction for progression to stage{{nbsp}}4. Furthermore, there was a 52% relative risk reduction for the need for laser treatment or intravitreal bevacizumab. However 19% of the newborns experienced serious adverse effects including hypotension and bradycardia.<ref>Filippi L (2013). [https://www.ncbi.nlm.nih.gov/pubmed/24054431] J Pediatr. 2013 Dec;163(6):1570-1577.e6</ref> A study in a mouse model of human ROP has shown that beta-blockade is protective against retinal angiogenesis and ameliorate blood-retinal barrier dysfunction.<ref>Ristori C (2011). [https://www.ncbi.nlm.nih.gov/pubmed/20739470] Invest Ophthalmol Vis Sci. 2011 Jan 5;52(1):155-70.</ref> | |
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| ===Follow up===
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| * Once diagnosed with ROP lifelong follow up (yearly) is performed in some centers. In others, only children treated for ROP are followed yearly.
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| * Follow up after laser or anti-VEGF treatment is individualized.
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| * Follow up of premature children (with or without ROP) is varying among centers and countries, mirroring the diverse states of health care system in different countries.
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| ==Prognosis== | | ==Prognosis== |
| | | The prognosis for infants with ROP varies. Many infants with mild ROP improve without treatment and have normal vision. However, severe ROP can lead to significant visual impairment or blindness. Early detection and treatment are crucial for improving outcomes. |
| Stages 1 and 2 do not lead to blindness. However, they can progress to the more severe stages. Threshold disease is defined as disease that has a 50% likelihood of progressing to retinal detachment. Threshold disease is considered to be present when stage{{nbsp}}3 ROP is present in either zone{{nbsp}}I or zone{{nbsp}}II, with at least five continuous or eight total clock hours of disease, and the presence of ''plus'' disease.<ref>{{cite journal| author = Phelps, D.L.| title = Retinopathy of Prematurity: History, Classification, and Pathophysiology| journal = NeoReviews| year = 2001| volume = 2| issue = 7| pages = e153–e166| doi = 10.1542/neo.2-7-e153}}</ref> Progression to stage{{nbsp}}4 (partial retinal detachment), or to stage{{nbsp}}5 (total retinal detachment), will result in substantial or total loss of vision for the infant.
| | ==Prevention== |
| | | Preventive measures focus on minimizing risk factors, such as careful monitoring of oxygen levels in premature infants and ensuring proper prenatal care to reduce the risk of premature birth. |
| * [[Refractive error]]s (most common)
| | ==See also== |
| * [[Strabismus|Squint]]
| | * [[Premature birth]] |
| * [[Amblyopia]]
| | * [[Blindness]] |
| * [[Retinal detachment]] and [[blindness]]
| | * [[Retinal detachment]] |
| * [[Glaucoma]]
| | * [[Ophthalmology]] |
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| ==Epidemiology==
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| ROP prevalence varies, from 5 to 8% in developed countries with adequate neonatological facilities, to up to 30% in middle-income developing countries.<ref name=Gergely>{{Cite journal | last1 = Gergely | first1 = K. | last2 = Gerinec | first2 = A. | title = Retinopathy of prematurity—epidemics, incidence, prevalence, blindness | journal = Bratislavske Lekarske Listy | volume = 111 | issue = 9 | pages = 514–517 | year = 2010 | pmid = 21180268}}</ref>
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| There is increasing evidence that ROP and blindness due to ROP are now public health problems in the middle income countries of Latin America, Eastern Europe and the more advanced economies in South East Asia and the Middle east region. In these countries ROP is often the most common cause of blindness in children.<ref name=Gilbert>{{Cite journal | last1 = Gilbert | first1 = C. | last2 = Fielder | first2 = A. | last3 = Gordillo | first3 = L. | last4 = Quinn | first4 = G. | last5 = Semiglia | first5 = R. | last6 = Visintin | first6 = P. | last7 = Zin | first7 = A. | last8 = International No-Rop | first8 = G. | doi = 10.1542/peds.2004-1180 | title = Characteristics of Infants with Severe Retinopathy of Prematurity in Countries with Low, Moderate, and High Levels of Development: Implications for Screening Programs | journal = Pediatrics | volume = 115 | issue = 5 | pages = e518–e525 | year = 2005 | pmid = 15805336 | pmc = | url =http://pediatrics.aappublications.org/content/115/5/e518.full| accessdate= 9 June 2013| doi-access = free }}</ref><ref>{{Cite journal | last1 = Limburg | first1 = H. | last2 = Gilbert | first2 = C. | last3 = Hon | first3 = D. N. | last4 = Dung | first4 = N. C. | last5 = Hoang | first5 = T. H. | title = Prevalence and Causes of Blindness in Children in Vietnam | doi = 10.1016/j.ophtha.2011.07.037 | journal = Ophthalmology | volume = 119 | issue = 2 | pages = 355–361 | year = 2012 | pmid = 22035577 | pmc = }}</ref> ROP is highly likely to become an increasing problem in India, China and other countries in Asia as these countries expand the provision of services for premature infants.
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| There is also evidence that the population of premature infants at risk of severe ROP varies depending on the level of neonatal intensive care being provided.<ref name=Gilbert/> In countries with high development indices and very low neonatal mortality rates (e.g. North America, Western Europe), severe ROP is generally limited to extremely preterm infants i.e. those weighing less than 1 kg (2.2 lbs) at birth. At the other end of the development spectrum, countries with very low development indices and very high neonatal mortality rates (e.g. much of subSaharan Africa) ROP is rare as most premature babies do not have access to neonatal intensive care and so do not survive. Countries with moderate development indices are improving access to neonatal intensive care, and in these settings bigger, more mature babies are also at risk of severe ROP as neonatal care may be suboptimal. These findings have two main implications: firstly, much can be done in countries with moderate development indices to improve neonatal care, to reduce the risk of severe ROP in bigger babies and increase survival of extremely preterm infants, and secondly, in these settings bigger more mature babies need to be included in ROP programs and examined regularly so as to detect those babies developing ROP requiring treatment.
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| In 2012, the World Health Organization published data on rates of preterm birth and the number of premature babies born in different regions of the world.<ref>{{cite web|publisher=World Health Organization|year=2012|title=Born Too Soon: The Global Action Report on Preterm Birth|url=http://www.who.int/pmnch/media/news/2012/preterm_birth_report/en/|accessdate=9 June 2013}}</ref> This report contained three main findings:
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| * Premature birth has many different causes, and prevention is challenging,
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| * Prematurity is the most common cause of neonatal death in many countries, totaling as many as 1 million infants annually due to complications of preterm birth, and
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| * the number of preterm births is currently estimated to be 15 million, and increasing.
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| ==History==
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| This disease was first described in a premature baby in 1942. Between 1941–1953, over 12,000 babies worldwide were affected by it. Soul musician [[Stevie Wonder]], actor [[Tom Sullivan (singer)|Tom Sullivan]], and jazz singer [[Diane Schuur]] are a few famous people who have the disease.
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| The first case of the epidemic was seen on St. Valentine's Day in 1941 when a premature baby in Boston was diagnosed. Cases were then seen all over the world and the cause was, at that point, unknown. By 1951 a clear link between incidence and affluence became clear: many cases were seen in developed countries with organized and well-funded health care. Two British scientists suggested that it was oxygen toxicity that caused the disease. Babies born prematurely in such affluent areas were treated in incubators which had artificially high levels of oxygen. Studies on rats made this cause seem more likely, but the link was eventually confirmed by a controversial study undertaken by American pediatricians. The study involved two groups of babies. Some<ref name="Silverman1980">{{cite book|last=Silverman|first=William A.|title=Retrolental fibroplasia: a modern parable|url=https://books.google.com/books?id=mslsAAAAMAAJ|accessdate=21 September 2013|date=November 1980|publisher=Grune & Stratton}}</ref> given the usual oxygen concentrations in their incubators, while the other group had "curtailed" oxygen levels. The latter group was shown to have a lower incidence of the disease. As a result, oxygen levels in incubators were lowered and consequently, the epidemic was halted. Each case of ROP avoided by withholding oxygen "may have cost some 16 deaths".<ref>{{cite book|last=Silverman|first=William A.|title=Retrolental fibroplasia: a modern parable|url=https://books.google.com/books?id=mslsAAAAMAAJ|accessdate=21 September 2013|date=November 1980|quote=Chapter 8: "The Consequences of Oxygen Restriction"|publisher=Grune & Stratton}}</ref>
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| == Clinical Manifestations ==
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| ROP can vary in severity and presentation:
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| === Mild Cases ===
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| In mild cases, ROP may resolve spontaneously without intervention.
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| === Serious Cases ===
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| Severe ROP can lead to retinal scarring, detachment, and vision loss, potentially resulting in blindness.
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| == Diagnosis and Assessment ==
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| Diagnosing ROP involves specialized eye examinations and assessments:
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| === Ophthalmologic Examination ===
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| Eye specialists conduct regular examinations to monitor retinal development and detect signs of ROP.
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| == Treatment Options ==
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| The management of ROP depends on its severity:
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| === Observation and Monitoring ===
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| Mild ROP may require only close observation and monitoring, with no immediate intervention.
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| [[File:Eye exam.jpg|thumb|right|250px|Regular eye examinations are crucial for monitoring and diagnosing ROP.]]
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| === Laser Therapy ===
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| In more severe cases, laser therapy may be used to treat the abnormal blood vessels and prevent further complications.
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| === Anti-VEGF Therapy ===
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| Anti-vascular endothelial growth factor (VEGF) medications can also be employed to manage ROP.
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| == Preventative Measures ==
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| Preventing ROP involves strategies to reduce risk factors, such as minimizing oxygen exposure in neonatal care.
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| == References == | |
| 1. Section on Ophthalmology, American Academy of Pediatrics, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, & American Association of Certified Orthoptists. (2013). "Screening Examination of Premature Infants for Retinopathy of Prematurity." Pediatrics, 131(1), 189-195.
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| 2. Hellström, A., et al. (2013). "Igf-1 as a Drug for Preterm Infants: A Step-Wise Clinical Development." Current Pharmaceutical Design, 19(33), 5882-5896.
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| == Conclusion ==
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| Retinopathy of Prematurity (ROP) is a significant eye disorder affecting prematurely born infants, particularly those receiving neonatal intensive care. Early diagnosis and appropriate management are crucial to preventing severe complications, including blindness. Ongoing research and advances in neonatal care continue to improve outcomes for at-risk infants.
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| For more information on related topics, please explore our [[Neonatal Medicine]] and [[Pediatric Ophthalmology]] articles.
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| == External Links ==
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| - [https://www.aao.org/eye-health/diseases/what-is-retinopathy-of-prematurity American Academy of Ophthalmology: Retinopathy of Prematurity (ROP)] - Detailed information and resources on ROP.
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| - [https://www.nei.nih.gov/learn-about-eye-health/eye-conditions-and-diseases/retinopathy-prematurity National Eye Institute (NEI): Retinopathy of Prematurity] - In-depth overview, research, and treatment options.
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| {{Medical resources | | {{Medical resources |
| | DiseasesDB = 11442 | | | DiseasesDB = 11442 |
| Line 194: |
Line 68: |
| | ICD9 = {{ICD9|362.20}}, {{ICD9|362.21}} | | | ICD9 = {{ICD9|362.20}}, {{ICD9|362.21}} |
| | ICDO = | | | ICDO = |
| | | MedlinePlus = 001618 |
| | | MeshID = D012178 |
| | OMIM = 133780 | | | OMIM = 133780 |
| | MedlinePlus = 001618 | | | Orphanet = 90050 |
| | eMedicineSubj = oph | | | eMedicineSubj = oph |
| | eMedicineTopic = 413 | | | eMedicineTopic = 413 |
| | eMedicine_mult = {{eMedicine2|ped|1998}} | | | eMedicine_mult = {{eMedicine2|ped|1998}} |
| | MeshID = D012178
| |
| | Orphanet = 90050
| |
| }} | | }} |
| * [https://web.archive.org/web/20070711113249/http://www.nei.nih.gov/health/rop/index.asp Retinopathy of Prematurity] Resource Guide from the National Eye Institute (NEI).
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| * [http://www.merckmanuals.com/home/childrens_health_issues/problems_in_newborns/retinopathy_of_prematurity_rop.html Merck Manual entry on ROP]
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| * [https://www.worldropcongress.com/ World ROP Congress] Archives of the International Conferences on ROP.
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| {{Eye pathology}} | | {{Eye pathology}} |
| | | {{stub}} |
| [[Category:Blindness]] | | [[Category:Blindness]] |
| [[Category:Disorders of choroid and retina]] | | [[Category:Disorders of choroid and retina]] |
| [[Category:Neonatology]]{{stub}} | | [[Category:Neonatology]] |
| | [[Category:Eye diseases]] |
| | [[Category:Neonatology]] |
| | [[Category:Pediatrics]] |
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