Download Patel, Devina

A Case of Something More Than What Meets the Clinical Observer’s Eye – Devina Patel, OD
ABSTRACT:
29yof h/o POHS, 20/20 OD/OS and h/o OS parafoveal scar, presents sudden VA loss to CF
OS. Exam positive for APDOS, unchanged scar. IRCSLO, Autofluorescence and SDOCT
identify new outer retinal pathologic process not evident on funduscopy. Multimodal imaging
reveals new diagnosis.
I.
CASE HISTORY
a. Patient Demographics: 29 year old female born and raised in Texas
b. Chief Complaint: Sudden unilateral vision loss accompanied with pain at onset
one month ago. Patient woke up with head pain graded as a 7/10 (dull, over left
brow). Vision disturbance initially presented “as though everything was 3-D” per
history. Patient covered each individually to discover that she was unable to see
anything out of her left eye. Patient denied photopsias or other visual
disturbances.
c. Ocular History: Patient has history of Presumed Ocular Histoplasmosis
diagnosed in 2005. At time of diagnosis patient also reports having had an
intravitreal injection she believes was steroidal in nature. At a subsequent followup visit patient was noted to have an IOP of 46 in the same eye, consistent with a
steroid response. Patient was treated with Alphagan bid for about two months.
No recurrence or impact on visual outcome at that time.
d. Medical History: Unremarkable
e. Medications: Lipo-6 weight loss supplement
II.
PERTINENT FINDINGS
a. Clinical:
i. Best corrected visual acuity OD: 20/20 OS: CF @ 3 ft. with eccentric
fixation (where OS was previously 20/20)
ii. Refractive error: Moderate myopia – OD: -3.00 sph OS: -3.25 sph
iii. FCF full OD & restricted OS 360
iv. Pupils: OS>OD; sluggish response OS; (+) 1+/2 APD OS
v. EOM’s: FESA
vi. Amsler grid: testing revealed no abnormalities in the right eye, whereas
there was a complete scotoma OS sparing ~4 degrees infero-temporally.
vii. Color Vision (Ishihara): OD: 10/10; OS: unable to see plates due to
central scotoma
viii. Anterior Segment:
1. Unremarkable: no endothelial keratic precipitates OU; no
anterior chamber reaction OU
ix. Intraocular Pressure by Goldmann Applanation Tonometry:
1. OD: 13
2. OS: 12 @ 9:25 AM
x. Dilated Fundus Examination:
1. Vitreous: clear OD & OS; no vitritis OU
2. OD: distinct ONH margins; C/D: 0.60hx0.55v c temporal PPA;
no pallor, no disc edema
3. OS: distinct ONH margins; C/D: 0.50x.50 c temporal PPA; no
pallor, no disc edema
b.
c.
d.
e.
4. Posterior Pole:
 OD: very small punched out parafoveal hypopigmented
lesions
 OS: parafoveal macular scar ~1/3 DD in size; no
subretinal fluid; no hemorrhages; additional punched out
lesion superior to macula – hyperpigmented with whitish
ring; small circular hypopigmented circular lesion
inferonasal to ONH
5. Periphery:
 OD: lattice degeneration inferiorly
 OS: lattice degeneration inferiorly and superotemporally
Physical: Review of Systems: Within Normal Limits
Laboratory Studies:
i. Histoplasmosis antibody test: not performed
Radiological Studies:
i. Recent MRI performed for this problem: negative for multiple sclerosis
plaques; positive for mild sinusitis
Other:
i. Humphrey Visual Field Testing (OSx2)
1. OD: clean, reliable, repeatable
2. OS: central scotoma, scattered disorganized defects with no
repeatable pattern
ii. Goldmann Visual Field Testing
1. No restrictions OD & OS
iii. Confocal Scanning Laser Ophthalmoscope:
1. Infra red CSLO shows patchy irregular signal OS; Short
wavelength (488nm) CSLO and short wavelength
autofluorescence reveal irregular deep retinal reflectance pattern
change compared to scans 1 year ago.
iv. IVFA
1. No leakage indicative of CNVM OU
v. High Resolution Spectral Domain OCT:
1. OD: Scan through small parafoveal punched out lesions revealed
no significant extension or damage to fovea ; CMT: 189
2. OS: Scan through punched out macular histo spot revealed
discontinuity of ISOS line at FAZ and throughout posterior pole,
photoreceptor atrophy at fovea & throughout clinically defined
macula, outer retinal disorganization as well as accumulation of
material in outer neurosensory retina beneath histo spot that does
not show autofluorescent properties; CMT: 197
vi. Standard Digital Fundus Photography: Photography performed 1 year
apart shows no observable change in macular lesion OS.
III.
DIFFERENTIAL DIAGNOSES
a. Primary:
i. Punctate Inner Choroidopathy
ii. POHS Reactivation
b. Other:
i. Subretinal Fibrosis & Uveitis Syndrome
ii. Multifocal Choroiditis with Panuveitis
iii. Acute Macular Neuroretinopathy
iv. Retrobulbar Optic Neuritis
IV.
DIAGNOSES & DISCUSSION
a. Elaboration on the condition: Ocular histoplasmosis presents as a triad of
punched out chorioretinal histo spots, peripapillary atrophy and macular
choroidal neovascular membranes predominantly in females 20-50 years of age.
Disseminated lesions present initially, with macular involvement following many
years later. In this case, a 29 year old myopic female is diagnosed with POHS
and maintains a BCVA of 20/20 until 4 years after diagnosis. There is no
clinically observable change in the macular lesion that has been stable since time
of diagnosis. IVFA shows no sign of active CNVM. In the absence of vitritis or
uveitis, a White Dot Syndromes such as Punctate Inner Chorioretinopathy should
be considered. PIC is characterized by development of subretinal fibrosis formed
by coalescence and evolution of acute lesions that greatly mimic histo spots. In
this case, high resolution spectral domain OCT shows significant outer retinal
disorganization and photoreceptor atrophy in a patchy distribution throughout the
macular area. There is a generalized discontinuity of the IS/OS line in both eyes.
OCT findings are supported by corresponding regional changes in CSLO
imaging features to long and short wavelengths. Further diagnostic testing
should include multifocal ERG.
b. Expound on unique features: Initial diagnosis of POHS was made in 2005, at
which time macular scar in the left eye was documented and patient was treated
with intravitreal steroid injection. Best corrected visual acuity remained 20/20
OS up until 2008. When patient presented in 2009 with complain of sudden
unilateral vision loss, no evidence of CNVM was noted and macular scar OS
appeared to be unchanged and consistent with prior year’s photodocumentation.
Fundus appearance remained unchanged to conventional examination but
imaging with advanced diagnostic instrumentation modifies the original
diagnosis.
V.
TREATMENT, MANAGEMENT
a. Treatment and response to Tx: Pending definitive diagnosis– no treatment at
present time due to lack of active CNVM.
b. References to Literature:
i. The Macular Photocoagulation Study: showed that argon or krypton laser
Tx reduced vision loss in POHS patients with juxtafoveal or extrafoveal
CNVM. There is no benefit of prophylactic treatment in absence of an
active membrane.
ii. A study by Rivers et al. retrospectively reviewed 208 patients with
already diagnosed POHS. It focused on cases of 7 individuals, with
existing POHS scars, who presented with new symptoms of decreased
vision or metamorphopsia. IVFA performed on all aforementioned
patients was not conclusive due to CNVM growing within the margins of
a longstanding atrophic scar.
iii. Watzke initially described the clinical entity of Punctate Inner
Chorioretinopathy in 1984. The condition is characterized by blurred
vision or central scotoma at initial presentation most commonly in
young, moderately myopic females. Additional presenting symptoms
may include flashes, floaters and photopsias. Although symptoms are
typically unilateral, fundusopic examination more often than not reveals
bilateral lesions that are described as multiple discrete, flat, yellow,
round, punched-out spots at the level of the RPE and choroid. PIC is
distinguished from other white dot syndromes by the absence of anterior
and posterior segment inflammation. The most threatening aspect of PIC
is the development of a choroidal neovascular membrane, which if
present, significantly diminishes visual prognosis. It is estimated that 1740% of patients with PIC will develop CNVM.
c. Bibliography:
i. Alexander LJ. Primary Care of the Posterior Segment. 3rd Edition: 146154. McGraw Hill. 2002.
ii. Amaro MH, Muccioli C, Abreu MT. “Ocular histoplasmosis-like
syndrome: a report from a nonendemic area”. Arq Bras Oftalmol.
70(4):577-580. 2007.
iii. Amin P, Cox TA. “Acute Macular Neuroretinopathy”. Archives of
Ophthalmology. 116:112-113. 1998.
iv. Foster CS, Vitale AT. Treatment, Diagnosis and Management of Uveitis.
Edition 1, Saunders. 2002.
v. Gerstenblith AT, Thorne JE, Sobrin L, et al. “Punctate Inner
Chorioretinopathy: A Survery Analysis of 77 Persons”. Ophthalmology.
114(6): 1201-1204. 2007.
vi. Hawkins BS, Alexander J, Schachat AP. Inflammatory Disease: Ocular
Histoplasmosis. Section 6, Ch. 103: 1661-1673.
vii. Jost BF, Olk RJ, Burgess DB. “Factors Related to Spontaneous Visual
Recovery in the Ocular Histoplasmosis Syndrome”. Retina, The Journal
of Retina and Vitreous. 7(1):1-8. 1987.
viii. Kedhar SJ, Thorne JE, Wittenberg S, et al. “Multifocal Choroiditis with
Panuveitis and Punctate Inner Chorioretinopathy: Comparison of Clinical
Characteristics at Presentation”. Retina, The Journal of Retina and
Vitreous. 27(9):1174-1179. 2007.
ix. Matsumoto Y, Haen SP, Spaide RF. “Clinical Practice: The White Dot
Syndromes”. Comprehensive Ophthalmology Update. 8(4): 179-200.
2007.
x. Monson BK, Greenberg PB, Greenerg E, et al. “High-speed, ultra-highresolution optical coherence tomography of acute macular
neuroretinopathy”. British Journal of Ophthalmology. 91:119-120. 2007.
xi. Morgan CM, Schatz, H. “Recurrent Multifocal Choroiditis”.
Ophthalmology. 93(9):1138-1147. 1986.
xii. Peyman GA, Lad EM, Moshfeghi DM. “Intravitreal Injections of
Therapeutic Agents”. Retina, The Journal of Retina and Vitreous
Diseases. 29(7):875-912.
xiii. Rivers MB, Pulido JS, Folk JC. “Ill-defined Choroidal
Neovascularization within Ocular Histoplasmosis Scars”. Retina, The
Journal of Retina and Vitreous. 12(2)90-95. 1992.
xiv. Sieving PA, Fishman GA, Salzano T, et al. “Acute macular
neuroretinopathy: early receptor potential change suggests photoreceptor
pathology”. British Journal of Ophthalmology. 68:229-234. 1984.
xv. Thorne JE, Wittenberg S, Kedhar SR, et al. “Optic Neuropathy
Complicating Multifocal Choroiditis and Panuveitis”. American Journal
of Ophthalmology. 143(4):721-723. 2007.
xvi. Quillen DA, Davis JB, Gottlieb JL, et al. “Perspective: The White Dot
Syndromes”. The American Journal of Ophthalmology. 137(3):538-549.
Elsevier Inc. 2004.
xvii. Watzke RC, Packer AJ, Folk JC, et. al. “Punctate Inner
Chorioretinopathy”. American Journal of Ophthalmology. 98(5):572584. 1984.
VI.
CONCLUSION
a. In conclusion, a 29 year old female with Presumed Ocular Histoplasmosis
presents with a profound, acute onset, unilateral vision loss from 20/20 to
counting fingers at 3 feet. There is no obvious sign of choroidal neovascular
membrane in the area of longstanding parafoveal scar. In the absence of any
funduscopic change, outer retinal pathology evidenced only with multimodal
imaging demonstrates critical findings that modify original diagnosis.
b. Clinical Pearls:
i. White dot syndromes such as Multifocal Choroiditis and Punctate Inner
Chorioretinopathy should be considered in unilateral cases of POHS,
especially in absence of uveitis or vitritis.
ii. Punched out lesions associated with POHS may increase in size and
number after initial dissemination, possibly on a subclinical level.
iii. Patients who present with atypical or inconsistent findings may benefit
from additional imaging beyond IVFA, such as CSLO tomography and
high resolution spectral domain OCT for key data to further elucidate
clinical status.