Optic Nerve Hypoplasia - Overcoming Visual Impairment
Villa Medica

Optic Nerve Hypoplasia – Overcoming Visual Impairment

Cell Therapy For Optic Nerve Hypoplasia

Overview

Optic Nerve Hypoplasia (also known as ONH) is a condition that affects the optic nerves, which are responsible for transmitting visual information between the eyes and brain.

The prevalence of ONH as a major cause of vision impairment has rapidly been increasing over the years. For instance, in Sweden, ONH quadrupled between 1980 and 1999 as all the other causes of vision loss diminished.

Villa Medica - Optic Nerve Hypoplasia (ONH) Statistics Infographic

Optic Nerve Hypoplasia ONH Statistics : The prevalence of ONH in Sweden quadrupled between 1980 and 1999 to 7.1 per 100,000 while all other causes of childhood blindness declined. In 2006, a report from England described the prevalence of ONH as 10.9 per 100,000 children.

Persons with ONH often experience visual acuity, sensitivity to bright light, and peripheral field loss. As a result, they have trouble recognizing faces and facial expressions.

Additionally, they have challenges accessing near and detailed information.

Could Cell Therapy Help With Optic Nerve Hypoplasia?

There is hope for people living with optic nerve hypoplasia. Physicians have discovered that accessing innovative cellular-based treatment can slow down vision loss.
Chances are that cell therapy could also provide new healing possibilities as compared to traditional methods of treatment.

Villa Medica - Cell Therapy Explained - Infographic

Cell Therapy is an old treatment modality which involves implantation of living cells for treatment of diseases.

Extensive research on how live cell therapy can treat severe eye conditions is still ongoing. For over a decade now, the cellular therapies have played a big role in the treatment of ONH as the cells improve vision based on their unique characteristics and differences.

Mechanism

Cell Therapy has the ability to stimulate regeneration of neural cells and secrete their neurotrophic factors that help in suppressing the inflammation associated with visual problems. In turn, the visual impairment is improved since the optic nerve regenerates to preserve vision.

What is Optic Nerve Hypoplasia?

Optic Nerve Hypoplasia (ONH) is a condition that affects the optic disc and is characterized by small disc size, thinning of the nerve fiber, peripapillary double ring sign, and anomalous and tortuous retinal vessels.

ONH might occur by itself or alongside other facial abnormalities, ocular defects such as nystagmus and strabismus, and cranial anomalies (midline anomalies of the brain and agenesis of septum pellucidum).

Villa Medica - Optic Nerve Hypoplasia (ONH) Diagnosis, Risk Factors and Types infographic

Optic Nerve Hypoplasia (ONH) is a congenital anormaly of the optic disc that might result in moderate to severe vision loss in children. ONH is a pervasive disease of child neurodevelopment associated with anatomic abnormalities and overall miswiring of the brain that result in visual impairment and profound systemic and functional morbidity.

In addition, brain and hormonal anomalies are prevalent in persons with nystagmus and bilateral severe vision loss, and is less prevalent in instances where vision loss is minor or unilateral.

ONH, often taken as a more diffused syndrome is classified into three clinical subtypes namely, Type I (Optic Hypoplasia Simplex), Type II (Septo-optic dysplasia) and Type III (Septo-optic-pituitary dysplasia).

How is ONH Diagnosed ?

Diagnosis of ONH is majorly clinical. Currently, laboratory or radiographic examinations to establish the diagnosis of ONH are non-existent. ONH diagnosis is through ophthalmic verification of a small optic disc that is pale or grayish. The disc size is measured by taking the distance from the optic nerve to the macula.

ONH is diagnosed if four or more normal optic disc size (DD) can be placed between the macula and the optic nerve head (DM). Eyes with ONH have characteristically increased ratio of DM/DD as compared to normal eyes. Additionally, a pigmented double ring is observed.

ONH may also be accompanied by tortuous retinal vessels that may affect both venules and arterioles. Alternatively, the vessels might be unusually straight with reduced branching. However, in mild cases of ONH, the double ring sign might not be seen and the optical disc appears normal. As a result, MRI of the intracranial visual pathways is a better clinical diagnostic tool.

Treatment Options

Currently, no known cure is available for ONH. However, in some cases, occlusion of one eye with better vision may result in visual improvement in the other eye. Additionally, children with major bilateral vision impairment might benefit from early supportive attention from low vision professionals.

Although there is no cure for ONH, early intervention programs and early vision stimulation programs are often undertaken in order to reduce the effect of the loss of vision in general growth of persons with ONH.

Causes And Risk Factors

There is no known cause of ONH. However, in a number of cases, various risk factors have been associated with ONH. These include maternal diabetes, maternal alcohol abuse and use of anti-epileptic drugs. Others risk factors include fetal or maternal infections such as Hepatitis B, young maternal age and preterm birth.

Physicians are usually observant for any signs of hypothalamic dysfunction together with any vision complications. For instance, all children with neonatal jaundice and repeated hypoglycemia ought to have ophthalmoscopy examination, particularly, if the child experiences related temperature instability.

Moreover, if a child exhibits hormonal deficiencies or congenital anomaly, MRI scan should be taken in order to detect intracranial abnormalities and rule out treatable conditions.
Growth of children with ONH should also be monitored and their sexual development observed in order to anticipate delayed sexual growth. They should also frequently undergo physical, occupational and speech therapy.

Optic Nerve Hypoplasia Symptoms

Patients diagnosed with optic nerve hypoplasia show a wide range of visual function ranging from normal visual acuity to inability to perceive light. Furthermore, they exhibit a loss in visual field capability ranging from generalized loss of in depth visualization in both central and outlying fields to refined peripheral fields.

Most children with ONH have nystagmus, that is, they experience rapid, involuntary, rhythmic eye movements. In some cases, mild sensitivity to light (photophobia) might occur. Additionally, many children with ONH have sensory integration difficulties. For instance, they might be sensitive to certain sights, smells, or sounds, and have a high tolerance to pain thus endangering themselves.

ONH is, however, a stable condition, as visual ability does not worsen with time. In some instances, visual function improves slightly with maturation processes, which might also result in reduced nystagmus.

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Further Reading – Quality Articles From Third Party Websites

    1. [1] ONH Consulting
    2. [2] American Association for Pediatric Ophthalmology and Strabismus
    3. [3] NCBI – National Center for Biotechnology Information