Retrolental Fibroplasia Mechanism, Symptoms, Causes, Treatment

Retrolental fibroplasia (RLF) also known as Terry syndrome or retinopathy of prematurity (ROP), is an eye illness that affects premature newborns who are under neonatal critical care and oxygen therapy due to their premature lungs. This disease mostly affects the retinas of premature newborns.

Retinas are the light-sensitive inside lining of the eyes. The retinas of premature babies are not developed fully because the blood arteries that nourish them are not grown properly.

Retrolental Fibroplasia leads to many abnormalities among new-borns such as retinal detachment, retinal scarring, blindness, and hemorrhage. Some of the abnormalities vanish on their own in premature infants. But, infants with Retrolental Fibroplasia develop strabismus, near-sightedness, or such kinds of abnormalities in the future. 

Retrolental Fibroplasia can be minor and resolve on its own, but in severe cases, it might result in blindness. Retrolental Fibroplasia affects all preterm newborns. The low body mass of the infant also contributes to the development of Retrolental Fibroplasia. The other factors that lead to the formation of retrolental fibroplasia are relative hypoxia and oxygen toxicity.

Retrolental Fibroplasia Mechanism

The mechanism of Retrolental Fibroplasia is as follows:

1. After birth, all traces of the tunica vasculosa lentis vanish.
2. The normal duration is between 2 and 3 weeks.
3. The retinal arteries become convoluted and engorged in the third or fourth week of pregnancy. This shift affects the entire fundus or only a small portion of it.
4. In the extreme periphery of the retina, little grey spots of retinal edema emerge. Hemorrhages or opaque bands develop into it.
5. The vitreous becomes hazy, especially in the area of retinal edema patches. Hemorrhages or opaque bands develop into it.
6. Retinal detachments become obvious in the affected areas. At first, these detachments are rather superficial, but over time, they gradually become more severe and more widespread.
7. The posterior surface of the lens becomes vascularized due to the encroachment of the membrane.
8. Development of Posterior synechiae takes place.
9.  The working of the eye is affected by the membrane.

Thus, the process of retrolental fibroplasia becomes stationary at certain phases if detected timely but if not diagnosed at the early stages this process leads to the end stage which is complete blindness.

Retrolental Fibroplasia Symptoms

Premature newborns with retinopathy of prematurity (ROP) have an abnormal and uncontrolled formation of new blood vessels mostly in the posterior of the eye (the retina). The retina is the deepest tissue layer in the back of the eye where images are focused. It (retina) contains nerve tissues through which the light images are transformed into nerve impulses and transmitted to the brain.

The retina of the eye works properly with the help of the blood vessels at the periphery of the retina. These blood vessels emerge from the center of the retina (i.e., around the optic nerve) during fetal development. The blood vessels are developed completely around 38 to 42 weeks. This is the period required for the proper formation of the blood vessels that helps in the working of the retina. Thus, Retrolental Fibroplasia is caused by aberrant blood vessel formation, which includes disorderly budding of retinal blood vessels and irregular interconnections.

Retrolental Fibroplasia Causes

The following are considered the risk factors for Retrolental Fibroplasia:

1. The improper development of retinal blood vessels in preterm newborns.
2. Lack of retinal neuronal and vascular growth.
3.  Lower gestational ages and birth weights.
4. Postnatal hazards such as hyperglycemia, sepsis, excessive or fluctuating oxygen levels, and the need for blood transfusions.
5. Premature babies whose weight is 3 pounds are prone to retrolental fibroplasia.
6. Hyperoxia (high oxygen concentrations) during the newborn period, which is, unfortunately, necessary to keep the newborn alive because of the premature lungs’ inability to exchange oxygen efficiently, appears to increase the risk of retrolental fibroplasia.
7. Abrupt occurrences of unregulated electrical activity within the brain.
8. Repeated occurrences of a heart rate that are significantly slower than normal.

Retrolental Fibroplasia Treatment

Retrolental fibroplasia is treated with the help of medical professionals, ophthalmologists, pediatricians, health care professionals, and neonatologists. The following treatment options are available for the disease:

1. Cryotherapy.
2. Laser therapy.

Both therapies damage the retina’s outer portions, which may result in some side vision loss, although this is a minor drawback compared to the substantial loss of vision. The other new therapy is utilized for severe cases of Retrolental Fibroplasia. This new therapy is known as pharmacological therapy. It consists of Intravitreal injections of anti-vascular endothelial growth factor. The additional procedures are also used to treat retrolental fibroplasia in certain circumstances such as surgery and corrective glasses.