Transcript Optic Nerve Hypoplasia

Optic Nerve Hypoplasia
Presented by :
Julie Greenlee
Angela Howe
What is Optic Nerve Hypoplasia?
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Optic Nerve Hypoplasia
(ONH) refers to the
underdevelopment of the
optic nerve during
pregnancy.
Some people with ONH also
have an abnormal brain and
a poorly functioning pituitary
gland.
brain
optic
nerves
pituitary
gland
ONH Characteristics
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A wide spectrum of visual funtions ranging from normal
visual acuity to no light perceptionj.
Visual field may range from very subtle field loss to
depressed visual field
A high percentage of children with ONH have
nystagmaus
ONH is not progressive, inherited and can not be cured
A mild improvement in visual function may occur with
maturation
Mild light sensitivity may occur
Diagnosis by a Docotor
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An eye doctor
(ophthalmologist) can
diagnose optic nerve
hypoplasia by looking
inside the eye with an
ophthalmoscope. The
front surface of the optic
nerve (optic disc) appears
smaller than normal.
With several different
exams the doctor may
also be able to determine
the child’s vision loss.
normal
optic disc
small
optic disc
Other Health Conditions
There are several other conditions
that can occur with ONH. The
student can have all, some or
none of the following conditions.
 Abnormalities of the brain
 Pituitary glands problems
 Thyroid gland problems
 Adrenal gland problems
 Reproductive organ difficulties
Brain Abnormalities
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ONH brain abnormalities can be functional or
structural
Persons with septo-optic dysplasia or DeMorsier’s
syndrome have a problem with the formation of the
septum pellucidum
Other may have an abnormal corpus callosum
Others may have abnormally formed different parts
of the brain
Malformations can be seen through a CT scan or an
MRI
Pituitary Gland
&
Growth Hormone
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The Pituitary gland is located at the base of the
brain and is responsible for the making of many
hormones
When a person has problems making hormones in
the pituitary gland it is called hypopituitarism
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Growth hormone is made in the pituitary gland
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and controls bone growth
Lack of growth hormone can cause slower than
normal bone growth
Thyroid Gland
&
Adrenal Glands
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The thyroid glands are located in the neck and produce
the thyroid hormone, which helps with growth and energy
Lack of thyroid hormone can lead to poor growth, slowing of
mental and muscle function, weight gain, feeling cold, irregular
periods (menstrual cycle), hair loss, hoarse voice, brittle nails,
and dry, coarse skin.
The adrenal glands are located just above the kidneys
and are responsible for producing cortisol.
Cortisol is created during physical activity to help with energy
and blood sugar levels
Hypoglycemia
&
Anti-Diuretic Hormone
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Hypiglycemia is also know as low blood sugar. This can be
caused by growth hormone and or cortisol.
If the sugar level is low a person might feel tired, pale, sweaty,
confused, be unable to think, to use their muscles well, or have
a fast heart beat. If the blood sugar is very low the person might
pass out, become unconscious or have a seizure
The pituitary produces a hormone called ADH that is
responsible for keeping water in the body by
controlling the amount of urine that is created.
Without ADH the person feels very thirsty, has a dry
mouth and yet continues to make large amounts of
urine.
Sex Hormones
&
Prolactin
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The pituitary gland produces 2 hormones, FSH- follicle
stimulating hormone; and LHLeutinizing hormone, signal the
sex glands to produce sex hormaones
Without these hormones, a person’s sexual development is
delayed, a girl’s periods are absent or irregular, and it is difficult
to father a child or become pregnant.
Prolactin is another hormone made in the pituitary gland.
Children with ONH often have abnormal levels of prolactin in
their blood.
This leads to no known health problems, but it is often
measured by the endocrinologist to test pituitary function.
Treatments
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There are no treatments for ONH
There are medications for hormonal deficits
that can be prescribed by a docotor.
Educational Impact
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The student will require some medical
monitoring as well as educational
assessment
Depending on visual impairment increase or
deccrease font to meet needs of acuity
and/or visual field
Will likely need an O&M evaluation
Case study for OPN
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Born with hydrocephalus had stunt put in
immediately
Other testing to determine effects showed
septo-optic dyplasia which causes
hypopituitarism and optic nerve hypoplasia
His optic nerve was determined to be
“miniscule”- smallest they can be without
being absent
Case study contined
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With age other problems arise like no production of cortisol, seizure
activity, stunt replacement, reduced vision began to patch and wear
glasses
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At birth told he would have no vision due to his small optic nerve at six
he can see 3 to 10 feet and no field vision
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Therapist include vision therapist, physical therapist, occupational
therapist, speech therapist, play therapist
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Currently is special classes in public school. Continues to make gains
with all of his support in his development
Resources
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Video of project to raise money for ONH
http://www.onesmallvoicefoundation.org/video_loveisblind.html
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ONH news report – great explanation
http://www.onesmallvoicefoundation.org/video_kttv031505.html
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Video student with multiple issues
http://www.onesmallvoicefoundation.org/video_wgn032802.html
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Guide for ONH in Spanish
http://www.onesmallvoicefoundation.org/pdfs/hno_espanol.pdf
References
Bishop, V.E. (2005) Teaching visually impaired children. Springfield, IL: Charles C.
Smith.
Kaufman, F. R. , Kaufman, N., Borchert, M., Inlender, T. (June 22, 2007) . Optic nerve
hyperplasia (ONH)- General information. Retrieved July 3, 2007, from
http://www.magicfoundation.org/www/docs/101.116/opticnerve-hypoplasia.html
Kaufman, F. R. , Kaufman, N., Borchert, M., Inlender, T. (nd) Optic nerve hypoplasia a
guide for parents. Rertrieved July 14, 2007, from
http://www.onesmallvoicefoundation.org/images/ONH%20pamphlet_sm.pdf
Leguire, L. E. (nd) Optic nerve hyperplasia (ONH). Retrieved July 3, 2007 from
http://ohiolionseyeresearch.com/on_hypoplasia.htm
Pediatric visual diagnosis
fact sheet (January 1998) Optic nerve hyperplasia.
Retrieved July 3, 2007, from http://www.blindbabies.org/factsheet_onh.htm
Verderber, L. (nd). The doctor is in: Optic nerve hypoplasia. Retrieved July 3,
2007,
from http://www.nfb.org/Images/nfb/Publications/fr/fr1/optic.htm