-
What is
optic nerve hypoplasia? How is it acquired?
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How is this condition diagnosed? Are there any imagings necessary to
confirm the diagnosis?
-
What is the
visual prognosis associated with it?
-
Are there
any systemic conditions associated with it?
-
Is there any treatment
available?
-
How do I get
more information?
What is optic
nerve hypoplasia? How is it acquired?
Optic nerve hypoplasia is the most common optic disc
anomaly. It is due to a supranormal regression of the optic nerve axons that
starts before birth. It is non-progressive and may affect one or both optic
discs. The spectrum of its clinical presentation is broad. Most bilateral
cases will present with ophthalmic abnormalities such as poor vision and wobbly
eyes (nystagmus). Unilateral cases present with strabismus because the affected
eye tends to drift. Others would present with neurologic deficits or endocrine
dysfunction. Although it may be sporadic, several teratogens have been
implicated, including phenytoin, quinine, LSD, phencyclidine, alcohol,
intrauterine infection, cytomegalovirus, maternal diabetes.
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How is this condition diagnosed? Are there any imagings necessary to confirm
the diagnosis?
The gold standard for its diagnosis is thru indirect
ophthalmoscopy. The optic disc appears small and is surrounded by a ring of
hypopigmentation, constituting the “double ring sign” that is the hallmark of
this condition. Neuroimaging is recommended, particularly MRI, to document the
presence of associated abnormalities in the brain. The presence of these
abnormalities may aid in identifying patients that are more prone to hormonal
imbalance. These children should be co-managed by a pediatrician once
identified.
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What is the visual
prognosis associated with it?
The visual potential is determined by the integrity of the
central retinal axons and does not necessarily correlate with the size of the
optic disc.
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Are there any
systemic conditions associated with it?
Optic nerve hypoplasia, particularly the bilateral form, is
associated with multiple brain malformations, including absence of the septum
pellucidum, hypoplasia or agenesis of the corpus callosum, dysplasia of the
anterior third ventricle, and anomalies of the hypothalamic-pituitary axis.
Adrenal crisis and hypopituitarism may lead to sudden death.
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Is there any treatment available?
The endocrine abnormalities associated with optic nerve
hypoplasia are treatable, and an endocrine work-up, including provocative tests,
should be obtained. Patients with optic nerve hypoplasia and hypopituitarism
may require supplemental corticoteroids during times of stress. However, there
is no specific treatment for the optic nerve anomaly.
References:
Birkebaek et al. Optic nerve
size evaluated by magnetic resonance imaging in children with optic nerve
hypoplasia, multiple pituitary hormone deficiency, isolated growth hormone
deficiency, and idiopathic short stature. J Pediatr; October 2004; 145:
536-541.
Birkebaek et al. Endocrine
status in patients with optic nerve hypoplasia: relationship to midline central
nervous system abnormalities and appearance of the hypothalamic-pituitary axis
on magnetic resonance imaging. J Clin Endocrinol Metab; November 2003; 88:
5281-5286.
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EYE REPUBLIC
Ophthalmology Atlas
CLINIC INFORMATION
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SMS, text LUK4 EYEREPUBLIC
(send to 2851 for Globe and Sun Cellular, and 2951 for Smart). |
EYE REPUBLIC Ophthalmology Clinic
Manila
3/F Don Santiago Building Units 309-310
1344 Taft Avenue, Ermita
Manila, 1000 Philippines
Direct and Fax: (632) 536-2398
Trunk Line: (632) 523-8271 to 79 local 30
Mobile: (63917) 899-2020
Map and directions
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EYE REPUBLIC
Ophthalmology Clinic
Asian Hospital
and Medical Center
5/F Medical Office
Building (MOB) Suite 509
2205 Civic Drive,
Filinvest, Alabang
Muntinlupa City,
1781 Philippines
Direct:
(632) 771-9253
Direct and Fax:
(632) 771-9254
Mobile: (63917) 795-2020
Map and Directions
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EYE REPUBLIC Ophthalmology Clinic
Medical City
6/F Medical Arts Tower Inc (MATI) Suite 602
MERALCO Compound, Ortigas Avenue
Pasig City, 1604, Philippines
Direct and Fax: (632) 632-7846
Mobile: (63917) 537-2020
Map and directions
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EYE REPUBLIC
Ophthalmology Clinic
St. Luke's
Medical Center
6/F
Cathedral Heights Building Complex (CHBC)
North Tower Suite 614
279 E. Rodriguez
Sr. Boulevard
Quezon City, 1102
Philippines
Direct and Fax:
(632) 407-3883
Mobile: (63917) 855-2020
Map and directions
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CLINIC HOURS
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Monday to Saturday 9:00 AM to 6:00 PM
All clinics are closed on Sundays and Holidays
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OPTIC NERVE HYPOPLASIA information compiled by
Dr. Barbara L. Roque
and initially uploaded on August 23, 2005.
Last updated on
September 13, 2007. |
