Bilateral optic nerve hypoplasia

Bilateral Optic Nerve Hypoplasia

Optic nerve hypoplasia as seen on MRI

Bilateral optic nerve hypoplasia (BONH) is a congenital condition characterized by the underdevelopment or absence of the optic nerve in both eyes. This condition is one of the leading causes of blindness and visual impairment in children.

Etiology

The exact cause of BONH is unknown, but it is believed to be associated with various genetic and environmental factors. Some studies suggest a link with maternal drug use during pregnancy, particularly alcohol and drugs that affect the central nervous system.

Clinical Features

Patients with BONH typically present with nystagmus, strabismus, and reduced visual acuity. In severe cases, it can lead to total blindness. Other associated conditions include septo-optic dysplasia and de Morsier's syndrome.

Diagnosis

Diagnosis of BONH is typically made through a comprehensive eye examination, including fundoscopy and optical coherence tomography (OCT). Magnetic resonance imaging (MRI) can also be used to visualize the optic nerves and confirm the diagnosis.

Treatment

There is currently no cure for BONH. Treatment is focused on managing the symptoms and improving the quality of life for the patient. This may include vision therapy, low vision aids, and occupational therapy.

Prognosis

The prognosis for BONH varies widely depending on the severity of the condition and the presence of associated conditions. Some individuals may have near-normal vision, while others may be completely blind.

See Also

• Optic nerve
• Optic nerve disorders
• Septo-optic dysplasia
• de Morsier's syndrome