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Transcript
Residents Day Case Submission for the American Academy of Optometry
Author: Jo Pongsatien, OD
Jack C. Montgomery VAMC Resident
I. Case History
CC/HPI: 23 year old Navajo male reports distance vision is progressively worsening
over the past year with poor night vision. Patient is unsure if night vision has progressed
but states that it has been that way for a long time. Reports the change is in OU, but feels its
worse in OD. Also reports floaters in his vision. Denies injury, infection, allergies, dry eye
symptoms, or having any other systemic conditions.
Social History:
Smoking: Negative
Alcohol: Negative
Occupation: Cashier at McDonalds
Family History:
No history of any blindness in the family
Medical History:
Unremarkable
Ocular History (per exam 1 year ago):
1. Large Refractive error
2. Good ocular health
II. Pertinent Findings
a. Entrance testing:
-Entering ccVA’s:
OD: 20/50 ph 20/25
OS: 20/50 ph 20/30
-EOM’s: SAFE
-Confrontational Fields: mild constriction to finger counting 360 OD, OS
-Pupils: PERRL (-) APD
b. Manifest Refraction:
OD: -0.50 -3.50 x027
20/25+
OS: -0.50 -3.75 x169
20/20c. Anterior Segment:
-IOPs: 14/13
-all findings unremarkable except
Lens OD: 2+ PSC
OS: Clear
d. Posterior segment:
Vitreous:
OD: Grade 2 vitreous cells and floaters
OS: trace vitreous cells and floaters
Optic disc: Pink healthy rim tissue OU, no pallor
C/D:
OD: 0.1
OS: 0.1
Retina:
Macula OU: Perimacular ERM
Periphery OU:
Mottled/irregular RPE with washed out grayish color, most evident below
inferior arcades.
e. Macular OCT:
OU: shows perimacular ERM superiorly and inferiorly, good foveal contour, no
edema
f. HVF 30-2 SITA Fast:
OD: shows dense ring scotoma
OS: shows dense arcuating superior field loss and significantly reduced nasal and
temporal quadrants.
OU: general reduced sensitivity in both eyes at all points. Patient does not meet
peripheral vision requirements for driving in NM and Arizona.
III. Differential Diagnosis
 Retinits Pigmentosa variant
 Choroideremia
 Gyrate atrophy
 Fundus Albipunctatus
 Retinitis puncta albescens
 Stargardt’s Disease
 Congenital stationary night blindness
IV. Diagnosis and Discussion
 Diagnosis: Retinal degeneration most likely an RP variant
 Two types of RP variants have been identified in the Navajo population. One shows a
gray granular surface of exposed choroidal tissue, due to RPE loss. This is known as
the recessive form and blindness is common by age 30. The dominant form will
manifest with the classic RP presentation of bone spicules and pigment clumping.
Progression to blindness is usually around age 55. However in both forms, fundus
findings are apparent within the first decade.
 Both forms combined is estimated to affect over 1:1878 Navajos.
 Due to the subtle presentation of symptoms, it is likely that this condition was
missed at the previous eye exam. Given his high astigmatism and mildly reduced
BCVA, refractive amblyopia is a possible misleading diagnosis. Furthermore, he did
not voice symptoms like night blindness and floaters until further questioned late in
the exam.
V. Treatment and Management
 Informed patient he is not suited to operate vehicles due to his condition.
 Recommended vision rehabilitation services in the area, in case his condition
progresses
 Advised patient to ask other family members if they have experienced similar visual
symptoms
 There is insufficient evidence to support nutritional supplements as a method of
slowing down RP progression
 Electrodiagnostics testing would be beneficial in narrowing down the true
diagnosis.
VI. Conclusion
 This case is an example of why we must dilate and must evaluate the peripheral
retina in seemingly normal young patients. Even if patients were dilated the year
before, it is prudent to re-dilate them the following year.
References
 Frederich, R. Eye Diseases in the Navajo Indians. Annals of Ophthalmology. 1982
 Heckinglively, John. Retinitis Pigmentosa in the Navajo. Michigan. Metabolic and
Pediatric Ophthalmology; 1981.
 Massof, Robert. How Strong is Evidence That Nutritional Supplement Slows
Progression of RP? Baltimore MD. Arch Opthalmology; 2010
 Rotenstreich, Ygal. Treatment with 9-cis Beta-Carotene Rich Powder in Patients
with Retinitis Pigmentosa. Tel-Hashoner, Israel. JAMA Ophthalmology; 2012