A B
2.5
Figure 2. Echocardiogram and color Doppler imaging. A, Echocardiogram shows prominent trabeculations in the left ventricle. Red arrow indicates intertrabecular
recesses; yellow arrow, trabeculae. B, Color Doppler image shows the presence of intraventricular cavity blood flow up to the depth of the intertrabecular
recesses. Green arrows indicate blood flow in the intertrabecular recesses; red arrow, noncompacted zone; and purple arrow, compacted zone.
ventricular wall (Figure 2A). Color flow imaging dem-
onstrated blood flow within the deep recess between the
trabeculations (Figure 2B). Severe mitral regurgitation due
to grade 1 anterior mitral valve prolapse was also present.
Findings on the carotid Doppler study were normal, and
no signs of carotid stenosis or plaques were observed.
A provisional diagnosis of noncompaction cardiomy-
opathy was made. Cardiac magnetic resonance imaging
was recommended to further evaluate the cardiomyo-
pathy, but the patient declined. Long-term oral warfarin
sodium treatment was commenced to reduce the risk of
systemic embolization. At 1 month after the attack, his
visual acuity remained similar in the right eye and im-
proved to 20/200 OS.
Comment. The embryonic arrest of compaction of myo-
cardial fibers seen in noncompaction cardiomyopathy is
most frequently observed in the left ventricle.3 The car-
diomyopathy is diagnosed by echocardiography or mag-
netic resonance imaging.
Echocardiography shows trabeculations and deep in-
tertrabecular recesses. Blood flow can be observed within
the deep intertrabecular recesses, and the flow is in con-
tinuity with the left ventricular cavity. Noncompaction
cardiomyopathy is diagnosed echocardiographically when
the ratio of trabeculations to the thickness of the under-
lying ventricular wall is more than 2.
Magnetic resonance imaging shows a 2-layered wall
structure comprising a thin compacted epicardium and
a thick noncompacted myocardium. Our patient’s echo-
cardiograms are consistent with the diagnosis of non-
compaction cardiomyopathy.
Strokes have been reported as a systemic thrombo-
embolism that occurs in patients with noncompaction
cardiomyopathy.4,5 The bilateral retinal artery occlu-
sion seen in our patient is likely of a thromboembolic na-
ture. We postulated that the microembolus observed in
the left retinal arteriole originated from the heart. The
noncompaction cardiomyopathy with relative blood sta-
sis in the intertrabecular recess explains the most prob-
able cause of this phenomenon.4 Thus, it is extremely im-
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265
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37
cm/s
37
portant to highlight this rare cause of retinal artery
occlusion that has resulted in devastating vision loss.
Tan Jin-Poi, MBBChBAO
Ismail Shatriah, MD
Seng Loong Ng, MD
Yusof Zurkurnai, MD
Rohaizan Yunus, MD
Author Affiliations: Departments of Ophthalmology (Drs
Jin-Poi and Shatriah), Medicine (Drs Ng and Zurkur-
nai), and Radiology (Dr Yunus), School of Medical
Sciences, Universiti Sains Malaysia, Kubang Kerian,
Malaysia.
Correspondence: Dr Shatriah, Department of Ophthal-
mology, School of Medical Sciences, Universiti Sains Ma-
laysia, Kubang Kerian 16150, Malaysia (shatriah@kck
.usm.my).
Conflict of Interest Disclosures: None reported.
1. Pignatelli RH, McMahon CJ, Dreyer WJ, et al. Clinical characterization of left
ventricular noncompaction in children: a relatively common form of
cardiomyopathy. Circulation. 2003;108(21):2672-2678.
2. Ergul Y, Nisli K, Demirel A, et al. Left ventricular non-compaction in chil-
dren and adolescents: clinical features, treatment and follow-up. Cardiol J.
2011;18(2):176-184.
3. Espinola-Zavaleta N, Soto ME, Castellanos LM, Játiva-Chávez S, Keirns C.
Non-compacted cardiomyopathy: clinical-echocardiographic study. Cardio-
vasc Ultrasound. 2006;4:35.
4. Mageshkumar S, Patil D, Samuel D, Muthukumar D. Unusual case of iso-
lated biventricular non-compaction presenting with stroke. J Postgrad Med.
2011;57(3):211-213.
5. Jiménez-Caballero PE. Juvenile stroke as the presenting symptom of a non-
compaction cardiomyopathy [in Spanish]. Rev Neurol. 2010;51(8):509-510.
Multiply Recurrent Solitary Fibrous Tumor
of the Orbit Without Malignant
Degeneration: A 45-Year
Clinicopathologic Case Study
S olitary fibrous tumor (SFT) is a rare mesenchy-
mal spindle cell neoplasm originally described in
the pleura and subsequently identified in a num-
ber of extrathoracic sites. Orbital SFT was first de-
WWW.JAMAOPHTH.COM
 scribed in 1994; since then, more than 100 cases have
been reported or reclassified with that diagnosis.1,2 Only
4 patients were younger than 10 years when first diag-
nosed as having orbital SFT. While most reports de-
scribe a benign clinical course, rare cases of primary ma-
lignant orbital SFT have also been documented. This led
the World Health Organization to classify SFT as a neo-
plasm of intermediate biological potential (locally ag-
gressive, rarely metastasizing).3,4 Increased cellular atypia
or mitotic activity in recurrent lesions following pri-
mary excision of orbital SFTs has also been described,
suggesting that complete initial resection is a critical prog-
nostic factor in preventing malignant degeneration.1,4-7
We describe a patient with orbital SFT whose proptosis
was first recognized at age 9 years and who underwent
surgical excision at various institutions at ages 12, 22,
and 52 years. To our knowledge, this represents the lon-
gest histopathologically documented follow-up of a pa-
tient with orbital SFT.
Report of a Case. In 1967, a 9-year-old girl with pain-
less left proptosis underwent an exploratory crani-
otomy in which no tumor was found. With a presump-
tive diagnosis of orbital hemangioma, she was treated with
external beam radiation. In 1970, she underwent an or-
bitotomy with removal of a 4.0⫻ 2.5-cm lobulated, red-
dish-blue mass, described as a hemangioma. Recurrent
proptosis was noted in 1980, and a lateral orbitotomy with
bone flap was performed with removal of a 5-cm lesion.
At that time, review at the Armed Forces Institute of Pa-
thology of the 1970 and 1980 specimens yielded a diag-
nosis of fibrous histiocytoma.

Figure 1. Axial (A) and coronal (B) computed tomographic images

demonstrating a left 2.7×1.7×1.6-cm mass isodense to muscle (A) and with
homogeneous intense contrast enhancement (B). Figure 2. Gross tumor following excision.

A B C

Figure 3. Short intersecting fascicles of cytologically bland spindle cells arranged in a “patternless pattern” (hematoxylin-eosin) (A), dilated hemangiopericytic
thin-walled vessels (hematoxylin-eosin) (B), and positive CD34 immunostaining (C) (original magnification ×200).

JAMA OPHTHALMOL/ VOL 131 (NO. 2), FEB 2013 WWW.JAMAOPHTH.COM

266

©2013 American Medical Association. All rights reserved.

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 In 2010, the patient was referred to our institution with
a history of gradually recurrent proptosis and recent-
onset diplopia. Orbital imaging demonstrated a large, ho-
mogeneously enhancing mass that filled the inferotem-
poral extraconal and intraconal left orbit (Figure 1). A
lower fornix-approach orbitotomy was performed with
removal of all gross tumor (Figure 2). Histologic sec-
tions showed a proliferation of spindle cells with eosino-
philic cytoplasm, poorly defined cytoplasmic borders, and
elongated nuclei with small eosinophilic nucleoli, ar-
ranged in short intersecting fascicles in a “patternless pat-
tern” (Figure 3A). Occasional hemangiopericytic thin-
walled vessels were present (Figure 3B). Rare mitotic
figures were seen, with no atypical forms, areas of ne-
crosis, or cytologic atypia identified. Immunohistochemi-
cal staining revealed that neoplastic cells were strongly
and diffusely positive for CD34 (Figure 3C), CD99, and
Bcl-2 and negative for smooth muscle actin, muscle-
specific actin, desmin, S-100 protein, and AE1/AE3 cy-
tokeratins, supporting the diagnosis of SFT. The patient
did well following gross tumor excision and remained
symptom free at 1 year.
Comment. We are aware of 4 cases of recurrent SFT that
showed increased cellularity, atypia, or mitotic rate com-
pared with the primary lesions, suggesting malignant trans-
formation with time.4-7 Such cases have led to the conclu-
sion that the most important prognostic factor is not the
initial histologic appearance but rather complete primary
resection.1 In those reports, the interval between initial tu-
mor excision and final recurrent tumor excision ranged from
6 months to 7 years. Our case provides further insight into
the heterogeneous histologic and clinical behavior of these
tumors. After an interval that included initial recognition
of proptosis at age 9 years and multiple surgical resections
as late as age 52 years, there were no histopathologic fea-
tures to suggest malignant transformation. A limitation of
this study is that the 1970 and 1980 outside histopatho-
logic material is no longer available to document a uni-
form diagnosis throughout the 45-year course. However,
a 1980 Armed Forces Institute of Pathology review of that
material did yield a diagnosis of orbital fibrous histiocy-
toma, an entity that has been more recently reclassified as
orbital SFT in many cases.2 Our case suggests that incom-
pletely excised, long-standing SFTs do not necessarily in-
crease in biological aggressiveness.
Gregory J. Griepentrog, MD
Gerald J. Harris, MD
Eduardo V. Zambrano, MD
Author Affiliations: Section of Oculofacial and Orbital
Surgery, Department of Ophthalmology (Drs Griepen-
trog and Harris) and Section of Musculoskeletal Pathol-
ogy, Department of Pathology (Dr Zambrano), Medical
College of Wisconsin, Milwaukee.
Correspondence: Dr Griepentrog, Section of Oculofa-
cial and Orbital Surgery, Department of Ophthalmol-
ogy, Medical College of Wisconsin, 925 N 87th St, Mil-
waukee, WI 53226 (ggriepentrog@mcw.edu).
Author Contributions: Drs Griepentrog and Harris had
full access to all the data in the study and take respon-
JAMA OPHTHALMOL/ VOL 131 (NO. 2), FEB 2013
267
©2013 American Medical Association. All rights reserved.
Downloaded From: https://jamanetwork.com/ on 08/02/2023
sibility for the integrity of the data and the accuracy of
the data analysis.
Conflict of Interest Disclosures: None reported.
Funding/Support: This work was supported in part by an
unrestricted grant from Research to Prevent Blindness.
1. Krishnakumar S, Subramanian N, Mohan ER, Mahesh L, Biswas J, Rao NA.
Solitary fibrous tumor of the orbit: a clinicopathologic study of six cases with
review of the literature. Surv Ophthalmol. 2003;48(5):544-554.
2. Furusato E, Valenzuela IA, Fanburg-Smith JC, et al. Orbital solitary fibrous
tumor: encompassing terminology for hemangiopericytoma, giant cell angio-
fibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases. Hum
Pathol. 2011;42(1):120-128.
3. Fletcher CDM, Unni KK, Mertens F, eds. Pathology and Genetics of Tumours
of Soft Tissue and Bone. Lyon, France: IARC Press; 2002.
4. Manousaridis K, Stropahl G, Guthoff RF. Recurrent malignant solitary fi-
brous tumor of the orbit [in German]. Ophthalmologe. 2011;108(3):260-264.
5. Dorfman DM, To K, Dickersin GR, Rosenberg AE, Pilch BZ. Solitary fibrous
tumor of the orbit. Am J Surg Pathol. 1994;18(3):281-287.
6. Romer M, Bode B, Schuknecht B, Schmid S, Holzmann D. Solitary fibrous tu-
mor of the orbit: two cases and a review of the literature. Eur Arch
Otorhinolaryngol. 2005;262(2):81-88.
7. Hayashi S, Kurihara H, Hirato J, Sasaki T. Solitary fibrous tumor of the orbit
with extraorbital extension: case report. Neurosurgery. 2001;49(5):1241-
1245.
COMMENTS AND OPINIONS
␻-3 Intake in Patients With Retinitis
Pigmentosa Receiving Vitamin A
B erson and colleagues1 claim that visual acuity loss
is slower in adults with retinitis pigmentosa re-
ceiving vitamin A who also consume a diet rich
in ␻-3 fatty acids. They previously reported findings of
a clinical trial of nutritional supplementation for pa-
tients with retinitis pigmentosa.2 They were criticized in
2 subsequent publications3,4 for overstating the strength
of evidence for their clinical recommendations. The same
can be said for this article.
A suggestive fragment of data taken from an epide-
miological study, not a randomized trial, is offered as a
basis for clinical therapy. No biological mechanism is of-
fered as motivation or interpretation of this research. The
groups compared are self-selected in their dietary choices,
not randomly assigned. Such studies are ordinarily
thought of as starting points for clinical research. If other
nutritional factors in addition to ␻-3 intake were evalu-
ated for association with visual acuity changes, tests of
significance should be modified.
The authors point out that the cut points for high and
low ␻-3 fatty acid intake were taken from a previous trial
with different treatments and visual outcomes, and they
reassure us that the high- and low-intake groups in this
article are balanced. No such assurance is offered for the
median and quartile data. A regression analysis to see
whether a trend exists would be helpful.
Ophthalmologists need to be cautioned not to allow
this article to influence their management of patients with
retinitis pigmentosa, despite the strong conclusions of-
fered by these investigators.
Daniel Seigel, ScD
Olivier Richoz, MD
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