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connection to the vitreous cavity without any
structural deformity of other parts of the
globe.
Comment
Peripapillary staphyloma denotes deep fun-
dus excavation surrounding a relatively nor-
mal appearing optic disc with normal retinal
vasculature and without any retinochoroidal
coloboma.
16
The pathogenesis remains spec-
ulative. It is thought to be the result of a
developmental failure of the posterior sclera
from neural crest cells occurring near the
fifth month of gestation.
17
Peripapillary
staphyloma is generally unilateral with only
four bilateral cases reported previously.
4–6
The
staphylomatous depth varies in size,
ranging up to about 10 mm with the fovea
often involved by the excavation.
6
Peripapillary staphyloma is often confused
with other excavated congenital optic disc
anomalies. In an optic disc coloboma, the
optic disc itself is excavated while surround-
ing inferonasal retinochoroidal or iris tissue
defects may also be present. In morning glory
disc anomaly, the excavation may be shal-
lower, cone-shaped and containing a cen-
trally located peripapillary glial tuft. In
addition, the optic disc may be anomalously.
As opposed to the central origin of vessels in
peripapillary staphyloma, in morning glory
syndrome, the vessels radiate more distinctly
from the disc periphery. Other vascular
anomalies such as moyamoya disease affect-
ing the carotid arteries, as well as pituitary
hormone insufficiencies may be present in
morning glory syndrome.
8–10
Our patient had been referred for evalua-
tion of a suspected optic nerve mass noted on
MRI. Detailed examination of the fundi,
however, revealed a bilateral peripapillary
staphyloma. Furthermore, MRI findings,
including the posterior scleral location and
connection of the presumed ‘‘mass’’ with the
vitreous cavity, associated with sharp internal
and external margins, with absence of
pathological enhancement all highlighted a
peripapillary staphyloma.
Eyes with peripapillary staphyloma may
sometimes achieve visual improvement by
occlusion therapy, but compliance and visual
outcome is often poor.
6
Often macular invol-
vement or ‘‘capture’’ by the staphyloma is
present, limiting final visual outcome. A few
reports of retinal detachment associated with
peripapillary staphyloma have been pub-
lished.
26
A complete ophthalmic examination
and regular follow ups are therefore neces-
sary because of associated ocular disease and
refractive errors. Unlike in patients with
morning glory anomaly, however, MRI is
unnecessary.
M S Sanjari, K Ghasemi Falavarjani,
M B Kashkouli
Eye Research Center, Rassoul Akram Hospital, Iran
University of Medical Sciences, Tehran, Iran
Correspondence to: Mostafa Soltan Sanjari, MD, Eye
Department, Rassoul Akram Hospital, Sattarkhan-
Niayesh Street, Tehran 14455-364, Iran;
msoltansanjari@yahoo.com
doi: 10.1136/bjo.2006.096362
References
1 Brown G, Tasman W. Congenital anomalies of
the optic disc. New York: Grune & Stratton,
1983:178–83.
2 Gottlieb JL, Prieto DM, Vander JF, et al.
Peripapillary staphyloma. Am J Ophthalmol
1997;124:249–51.
3 Blair MP, Blair NP, Rheinstrom SD, et al. A case of
peripapillary staphyloma. Arch Ophthalmol
2000;118:1138–9.
4 Hodgkins P, Lees M, Lawson J, et al. Optic disc
anomalies and frontonasal dysplasia.
Br J Ophthalmol 1998;82:290–3.
5 Caldwell JB, Sears ML, Gilman M. Bilateral
peripapillary staphyloma with normal vision.
Am J Ophthalmol 1971;71:423–5.
6 Kim SH, Choi MY, Yu YS, et al. Peripapillary
staphyloma clinical features and visual
outcome in 19 cases. Arch Ophthalmol
2005;123:1371–6.
7 Pollock S. The morning glory disc anomaly:
contractile movement, classification, and
embryogenesis. Doc Ophthalmol
1987;65:439–60.
8 Brodsky MC. Congenital optic disk anomalies.
Surv Ophthalmol. 1994;39: 89–112, [published
correction appears in Surv Ophthalmol
1995;40:172.].
9 Taskintuna I, Oz O, Teke MY, et al. Morning
glory syndrome: association with moyamoya
disease, midline cranial defects, central nervous
system anomalies, and persistent hyaloid artery
remnant. Retina 2003;23:400–2.
10 Pierre-Filho Pde T, Limeira-Soares PH,
Marcondes AM. Morning glory syndrome
associated with posterior pituitary ectopia and
hypopituitarism. Acta Ophthalmol Scand
2004;82:89–92.
Recurrent hypopyon in chronic
anterior uveitis of pauciarticular
juvenile idiopathic arthritis
The typical ocular manifestation of pauciarti-
cular juvenile idiopathic arthritis (JIA) is an
insidious, non-granulomatous chronic ante-
rior uveitis (CAU). The main risk factors for
the development of uveitis in JIA are being
female, antinuclear antibody (ANA) positiv-
ity, and oligoarticular disease at onset.
12
Even during acute exacerbations of JIA
associated CAU with severe anterior chamber
(AC) cellular reaction, it is rare for patients to
have symptoms and the eye is invariably
white.
12
This is in distinct contrast to the
acutely symptomatic red eye of acute anterior
uveitis (AAU). Hypopyon can form within the
AC in severe forms of anterior uveitis,
particularly HLA-B27 associated AAU.
3–5
We
report a case of CAU in a patient with
pauciarticular JIA, which was complicated
by the development of recurrent hypopyon.
Case report
A 4 year old white girl first presented with
blurred vision in her right eye and was found
to have a unilateral, non-granulomatous CAU
with extensive posterior synechiae and early
band keratopathy. She was initially treated
with intensive topical corticosteroids and
mydriatics. She was ANA positive and had
an ankle effusion, resulting in the diagnosis
of pauciarticular JIA associated CAU.
Repeated orbital floor steroid injections and
systemic low dose methotrexate were
required for the control of persistent anterior
uveitis activity. Control of AC inflammatory
activity was then maintained with regular
topical prednisolone and oral methotrexate
treatment.
At a routine follow up, 6 months after her
initial presentation with CAU, the patient
was found to have an asymptomatic inflam-
matory hypopyon and +4 cells in the AC of
the right eye without conjunctival injection.
There was no history of ocular surgery or
trauma and the patient was well systemically.
Topical steroids were increased to hourly
leading to a significant improvement in the
anterior uveitis activity with resolution of the
hypopyon and the achievement of a quiet AC
with no cells. Further investigations revealed
that the patient was HLA-B27 positive and
the angiotensin converting enzyme level was
not elevated. There were no clinical features
of a seronegative spondyloarthropathy. Two
months later, the hypopyon recurred upon
attempted tapering of topical steroid treat-
ment, once again occurring in a painless
‘‘white’’ eye of CAU (fig 1). The hypopyon
again demonstrated therapeutic response to
hourly topical steroids and was completely
resolved within 1 week. A quiet eye was
eventually achieved and maintained with
systemic methotrexate and regular topical
prednisolone therapy, with last follow up
being 9 months after the resolution of the
recurrent hypopyon.
Comment
JIA is by far the most common identifiable
clinical association of uveitis in children.
There have been no previous reports describ-
ing the development of hypopyon in the
typical clinical phenotype of CAU associated
with pauciarticular JIA.
1256
Our case exhib-
ited all of the characteristic clinical features
of non-granulomatous CAU of ANA positive
pauciarticular JIA, including its insidious
onset and asymptomatic chronic disease
course requiring long term anti-inflammatory
treatment for uveitis control.
Although our patient was also found to be
HLA-B27 positive, her clinical features were
not consistent with HLA-B27 associated
AAU, which includes an abrupt onset of red
and painful anterior uveitis that is typically of
short duration, predominantly occurring in
boys who are ANA negative.
36
HLA-B27
antigen is of clinical significance in AAU;
however, in contrast, HLA-B27 positivity does
not occur more frequently in CAU than in the
general healthy population.
37
JIA associated
uveitis that is positive for both ANA and
HLA-B27 appears to be a relatively rare
clinical subgroup and whether these repre-
sent a distinct clinical entity remains
unclear.
268
Three patients with ANA positive
CAU in early childhood who later developed
HLA-B27 associated recurrent attacks of AAU
during adolescence or adulthood have been
Accepted for publication 15 May 2006
The authors have no financial relationship with the
manufacturer of any product discussed here.
Figure 1 Slit lamp biomicroscopy examination
demonstrating a hypopyon in a painless
‘‘white’’ eye with chronic anterior uveitis that is
associated with ANA positive pauciarticular
JIA. Note the lack of ciliary conjunctival
injection.
PostScript 1327
www.bjophthalmol.com
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reported.
8
In one of these reported patients,
who were positive for both ANA and HLA-
B27, hypopyon had been described in asso-
ciation with the recurrent episodes of AAU
that developed during adulthood. The hypop-
yon described in that case occurred in the
context of HLA-B27 associated AAU in an
adult, distinct from and temporally following,
clinical features of childhood ANA positive
CAU.
8
In contrast, our reported patient
developed recurrent spontaneous hypopyon
(at the age of 5) in the context of the typical,
insidious CAU without any clinical features
of HLA-B27 associated AAU.
It remains unclear whether the presence of
HLA-B27 antigen in our patient is a coin-
cidence or whether it is of pathogenic
significance. HLA-B27 antigen may represent
a risk factor for the development of hypopyon
in anterior uveitis, even in those that do not
display the typical phenotype of HLA-B27
associated AAU. It would be of interest to
follow the clinical course of our patient into
her adulthood to determine whether she later
develops ocular or systemic features of HLA-
B27 associated AAU. Further studies of a
cohort of ANA and HLA-B27 positive children
with uveitis are indicated to investigate their
clinical phenotype and the potential interac-
tion between ANA and HLA-B27.
J H Chang, P J McCluskey
Department of Ophthalmology, St Vincent’s Hospital,
Sydney, and Laboratory of Ocular Immunology,
School of Medical Sciences, University of NSW,
Sydney, Australia
J R Grigg
Department of Ophthalmology, Sydney Eye Hospital,
Sydney, and Children’s Hospital at Westmead,
Sydney, Australia
Correspondence to: Dr John H Chang, Department of
Ophthalmology, St Vincent’s Clinic, 1004/438
Victoria Street, Darlinghurst, NSW 2010, Australia;
jhchang@optusnet.com.au
doi: 10.1136/bjo.2006.096313
References
1 Kanski JJ. Juvenile arthritis and uveitis. Surv
Ophthalmol 1990;34:253–67.
2 Kotaniemi K, Savolainen A, Karma A, et al.
Recent advances in uveitis of juvenile idiopathic
arthritis. Surv Ophthalmol 2003;48:489–502.
3 Chang JH, McCluskey PJ, Wakefield D. Acute
anterior uveitis and HLA-B27. Surv Ophthalmol
2005;50:364–88.
4 D’Alessandro LP, Forster DJ, Rao NA. Anterior
uveitis and hypopyon. Am J Ophthalmol
1991;112:317–21.
5 Ramsay A, Lightman S. Hypopyon uveitis. Surv
Ophthalmol 2001;46:1–18.
6 Kanski JJ. Anterior uveitis in juvenile rheumatoid
arthritis. Arch Ophthalmol 1977;95:1794–7.
7 Huhtinen M, Karma A. HLA-B27 typing in the
categorisation of uveitis in a HLA-B27 rich
population. Br J Ophthalmol 2000;84:413–16.
8 Bosch-Driessen EH, Lardy NM, Rothova A.
Antinuclear antibody and HLA-B27 positive
uveitis: combination of two diseases?
Br J Ophthalmol 1997;81:771–3.
A simple model for teaching
indirect ophthalmoscopy
Indirect ophthalmoscopy is a useful techni-
que to allow a wide angle view of the fundus,
to screen for retinal disease, and to examine
the peripheral retina. It provides a better view
of the fundus in patients with lens opacities
than is allowed by direct ophthalmoscopy. As
the threshold at which cataracts are operated
in developing countries is changing we have
found it important that junior doctors and
other eye health workers who select cataract
patients for operation can use the instru-
ment.
We use a simple model to teach the
technique of indirect ophthalmoscopy. An
hour or two with this model gets the
beginner ‘‘over the hump’’ of learning how
to position and move the light and lens.
When the first real patient is examined, the
learner can immediately concentrate on the
fundus, minimising frustration and time
spent shining an annoying light into a
patient’s eye.
The model is shown in the figure 1. The
‘‘eyeball’’ is a clear glass marble, set into
anything that will keep it from rolling, such
as a small bottle cap or a hole made in a piece
of Styrofoam. Behind the marble, moulded
around it, a piece of paper is placed with the
smallest print available—the package inserts
from prescription medicines work well.
Finally, a hole is punched in a scrap of paper
to use as a pupil. The optics of the system are
excellent for practising indirect ophthalmo-
scopy and the student can be tested by how
many words, letters, or lines he can see
compared to the instructor.
Other models have been described before,
which are more sophisticated or allow for
scleral indentation but the materials may be
hard to obtain.
1–3
A model equally simple to
make as that described here has been
proposed
4
; it does not require a glass marble
and is good for helping the beginner get
oriented, but the marble model provides more
of a challenge at learning to view the retina
outside the posterior pole.
Correspondence to: Susan Lewallen, Kilimanjaro
Centre for community Ophthalmology, Tumaini
University/ KCMC, Moshi, Tanzania;
slewallen@kcco.net
References
1 Dodaro NR, Maxwell DP. An eye for an eye. A
simplified model for teaching. Arch Ophthalmol
1995;113:824–6.
2 Chew D, Gray RH. A model eye to practice
indentation during indirect ophthalmoscopy. Eye
1993;7:599–600.
3 Bartner H, Paton D. An improved model for
instruction in binocular indirect ophthalmoscopy.
Arch Ophthalmol 1971;85:530–3.
4 Ing EB, Ing TGE. A method of teaching indirect
ophthalmoscopy to beginning residents.
Can J Ophthlamol 1992;27:166–7.
In vivo measurement of opacified
H60M intraocular lenses using
Scheimpflug photography
The Scheimpflug videophotographic camera
is a modified slit lamp camera that uses a
narrow slit beam and Scheimpflug optics to
increase apparent depth of field (enabling
simultaneous focusing of the entire anterior
segment). Area densitometry measurement
of scattered light intensity gives a cross
sectional image of the anterior segment. In
1995, Lasa et al demonstrated the use of
Scheimpflug photography
1
to assess posterior
capsular opacification (PCO). Subsequent
studies have correlated the values obtained
with visual acuity.
23
The system is efficient
and reliable for PCO evaluation with good
intraobserver and interobserver reproducibil-
ity.
45
This has been verified by comparison
with histological findings.
6
Delayed opacifi-
cation of Hydroview (H60M) intraocular
implant lenses (Bausch & Lomb, Rochester,
New York, USA) has been reported.
7
In our
patient series we find this to give rise to
progressive visual loss and symptoms of
glare.
We demonstrate the use of Scheimpflug
video-photography (Pentacam, Oculus) for
assessment of three cases of opacified H60M
intraocular lenses (IOLs). All had uncompli-
cated cataract surgery with insertion of
Hydroview R hydrogel lenses.
Case 1
An 89 year old woman had a +21.50 dioptre
IOL in January 2001, for nuclear sclerotic
cataract giving Snellen visual acuity of 6/18.
Accepted for publication 18 April 2006
Figure 1 Model for practising indirect ophthalmoscopy.
Thanks to Dr Paul Meyer, Addenbrookes Hospital,
Cambridge, UK, for this idea.
doi: 10.1136/bjo.2006.096784
Accepted for publication 15 May 2006
1328 PostScript
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doi: 10.1136/bjo.2006.096313
2006 90: 1327-1328Br J Ophthalmol
J H Chang, P J McCluskey and J R Grigg
of pauciarticular juvenile idiopathic arthritis
Recurrent hypopyon in chronic anterior uveitis
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