Retinopathy of prematurity
From Wikipedia, the free encyclopedia
| ICD-10 | H35.1 |
|---|---|
| ICD-9 | 362.21 |
Retinopathy of prematurity (ROP), previously known as retrolental fibroplasia (RLF), is a disease of the eye that affects prematurely born babies. It is thought to be caused by disorganised growth of retinal blood vessels resulting in scarring and retinal detachment. ROP can be mild and may resolve spontaneously, but may lead to blindness in serious cases. Prematurity is a direct risk factor for ROP. As such, all preterm babies are at high risk for ROP, and very low birth weight is an additional risk factor. High oxygen concentration is known to contribute to the development of ROP.
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[edit] Pathophysiology
Normally, maturation of the retina proceeds in-utero and at term, the mature infant has fully vascularised retina. However, in preterm infants, the retina is often not fully vascularised. ROP occurs when the development of the retinal vasculature is arrested and then proceeds abnormally. The key disease element is fibrovascular proliferation. This is growth of abnormal new vessels that may regress, but frequently progress. Associated with the growth of these new vessels is fibrous tissue (scar tissue) that may contract to cause retinal detachment. Multiple factors can determine how fast a patient progresses through the stages, including overall health, birth weight, the stage of ROP at initial diagnosis, and the presence or absence of "plus disease".
Patients with ROP are at greater risk for squint, glaucoma, cataracts and myopia later in life, and should be examined yearly to help prevent and treat these conditions.
[edit] Diagnosis
The examination of the retina of a premature infant using indirect ophthalmoscopy shows that the retinal blood vessels have not extended to the ora serrata. These vessels may or may not proliferate to form fibrovasular tissue, which is deletrious to the retina. Normal maturation of the retina proceeds in-utero and at term, the mature infant has fully vascularised retina. However, in premature babies developing ROP, the retinal examination shows extraretinal fibrovascular tissue in various stages. These stages of disease have been defined by the International Classification of Retinopathy of Prematurity (ICROP). 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.
[edit] Differential diagnosis
The most difficult aspect of the differential diagnosis may arise from the similarity of two other diseases:
- Familal Exudative Vitreoretinopathy which is a genetic disorder that also disrupts the retinal vascularization in full-term infants.
- Persistent Fetal Vascular Syndrome also known as Persistent Hyperplastic Primary Vitreous that can cause a traction retinal detachment difficult to differentiate but typically unilateral.
[edit] International Classification of Retinopathy of Prematurity (ICROP)
The system used for described the findings of active ROP is entitled The International Classification of Retinopathy of Prematurity (ICROP) (1). ICROP uses a number of parameters to describe the disease. They are location of the disease into zones (1, 2, and 3), the circumferential extent of the disease based on the clock hours (1-12), the severity of the disease (stage 1-5) 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 has been revised in 2005 (2)
The zones are centered on the optic nerve. Zone 1 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 2 is an annulus with the inner border defined by zone 1 and the outer border defined by the radius defined as the distance from the optic nerve to the nasal ora serrata. Zone 3 is the residual temporal crescent of the retina.
The circumferential extent of the disease is described in segments as if the top of the eye were 12 on the face of a clock. For example one might report that there is stage 1 disease for 3 clock hours from 4 to 7 o'clock. (The extent is a bit less important since the treatment indications from the Early Treatment for ROP (3))
The Stages describe the ophthalmoscopic findings at the junction between the vascularized and avascular retina.
- Stage 1 is a faint demarcation line.
- Stage 2 is an elevated ridge.
- Stage 3 is extraretinal fibrovascular tissue.
- Stage 4 is sub-total retinal detachment.
- Stage 5 is total retinal detachment.
In addition, Plus disease may be present at any stage. It describes a significant level of vascular dilation and tortuosity observed at the posterior retinal vessels. This reflects the increase of blood flow through the retina. [1]
[edit] Prognosis
Stages 1 and 2 do not lead to blindness. However, they can progress to stage 3. Stage 3, also called threshold disease. It may regress but if it progresses to stage 4 (partial retinal detachment), and then to stage 5 (total retinal detachment), these stages 4 and 5 will lead to substantial loss of vision for the infant.
[edit] Monitoring
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 gms or under 28 (32 tentative till I reference the new guidelines) weeks gestation in most cases.
[edit] Treatment
- Peripheral Retinal Ablation is the mainstay of ROP treatment. The destruction of the avascular retina is performed with a solid state laser photocoagulation, as they are easily portable around the neonatal ICU. Cryotherapy used to be performed and has been evaluated in multi-center clinical trials as an effective modality for prevention and treatment of ROP. However, it is no longer prefered for routine retinal coagulation, due to tremendous amounts of inflammation and lid swelling accompanying cryotherapy in premature babies.
- Scleral buckling and/or Vitrectomy surgery may be considered for severe ROP (stage 4 and 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.
[edit] History
A significant time in the history of the disease was between 1941-1953, when a worldwide epidemic of ROP was seen. Over 12,000 babies worldwide were not only born with the disease but blinded by it - the musician Stevie Wonder is a famous victim of the disease. The first case of the epidemic was seen on St Valentine's Day in 1941, where 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 were 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.
[edit] External link
[edit] References
1.An international classification of retinopathy of prematurity. The Committee for the Classification of Retinopathy of Prematurity. Arch Ophthalmol. 1984 Aug;102(8):1130-4.
2. The International Classification of Retinopathy of Prematurity revisited. Arch Ophthalmol. 2005 Jul;123(7):991-9.
3. Early Treatment for Retinopathy of Prematurity Cooperative Group. . Revised indications for the treatment of retinopathy of prematurity: results of the early treatment for retinopathy of prematurity randomized trial. Arch Ophthalmol. 2003;121:1684–1696
4. Medicine's Strangest Cases - Michael O'Donnell
