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3De Becker I, Walter M, Noel LP. Phenotypic
variations in patients with a 1630 A.T point
mutation in the PAX6 gene. Can J Ophthalmol
2004;39:272–8.
4Ritch R, Forbes M, Hetherington J Jr, et al.
Congenital ectropion uveae with glaucoma.
Ophthalmology 1984;91:326–31.
5Futterweit W, Ritch R, Teekhasaenee C, et al.
Coexistence of Prader-Willi syndrome, congenital
ectropion uveae with glaucoma, and factor XI
deficiency. JAMA 1986;255:3280–2.
6Trovo-Marqui AB, Goloni-Bertollo EM,
Teixeira MF, et al. Presence of the R1748X
mutation in the NF1 gene in a Brazilian patient
with ectropion uveae. Ophthalmic Res
2004;36:349–52.
7Azuma N, Yamada M. Missense mutation at the C
terminus of the PAX6 gene in ocular anterior
segment anomalies. Invest Ophthalmol Vis Sci
1998;39:828–30.
8Mirzayans F, Pearce WG, MacDonald IM, et al.
Mutation of the PAX6 gene in patients with
autosomal dominant keratitis. Am J Hum Genet
1995;57:539–48.
9Nelson LB, Spaeth GL, Nowinski TS, et al.
Aniridia. A review. Surv Ophthalmol
1984;28:621–42.
Fourth time lucky: a case of
multiple recurrence of a macular
hole
We present the case of a patient who has
undergone four macular hole operations for
late multiple recurrences. The same experi-
enced ophthalmologist confirmed the pre-
sence of each macular hole and performed
each operation.
Case report
A 55 year old female myope, –3.75 D right eye
and 23.5 D left eye, was referred to the
vitreoretinal clinic with a 2 month history of
right visual disturbance. On presentation, her
visual acuities were 6/9 right eye and 6/60 left
eye. Fundal examination and fluorescein
angiography showed a right stage I macular
hole and a left stage IV macular hole. As the left
macular hole was long standing, it was decided
not to operate on this eye. Two months later the
right visual acuity was reduced further to 6/36
because of progression to a stage II macular
hole and she underwent a right vitrectomy and
18% C
3
F
8
gas tamponade. At the time of
surgery, the posterior hyaloid was detached
and the absence of an epiretinal membrane
was confirmed but no inner limiting mem-
brane (ILM) peel was performed. Following
this and each subsequent vitrectomy, the
patient postured face down for 2 weeks.
Postoperatively, right visual acuity improved
to 6/6 but 8 months later the hole reopened,
and her right visual acuity dropped to 6/18. A
further vitrectomy and 20% C
3
F
8
gas tampo-
nade was carried out. During this, a silicon
tipped aspiration needle was used to confirm
the absence of vitreous at the macula and a
barbed blade used to show that no epiretinal
membrane was present. The hole closed and
the vision improved to 6/12. Two years later the
patient underwent uneventful right phacoe-
mulsification cataract surgery with posterior
chamber intraocular lens implantation and her
acuity was restored to 6/9. After another
2 years right visual acuity fell to 6/36, the hole
had reopened. The patient underwent a third
macular hole operation consisting of ILM peel
and 18% C
3
F
8
tamponade. The hole remained
closed and the vision improved to 6/9 for a
further 3 years when the hole reopened and the
vision decreased to 6/24. A fourth procedure
was performed consisting of trypan blue
assisted ILM peel to two disc diameters from
the hole and 18% C
3
F
8
gas tamponade.
When last seen, 16 months after surgery and
9 years after presentation, the right visual
acuity was 6/12 and the macula hole confirmed
as closed on ocular coherence tomography.
Comment
Previous studies have shown that repeat
macular hole surgery is successful in closing
recurrent macular holes and improving
vision.
12
Late reopening of successfully closed
macular holes has been reported to occur in
between 4.8% and 6.9% of eyes.
34
The success
of repeat surgery did not depend on whether
the hole reopened early or late as in this
case.
12
A third recurrence of a macular hole is
a less common event. The results of repeat
macular hole surgery are better in those eyes
in which one of the previous operations had
successfully closed the macula hole.
1
In those
eyes that had previous successful surgery the
mean final visual acuity was 20/40. By
comparison, in those eyes in which surgery
failed to close the macula hole, the success of
final closure rate was 56% and the mean final
visual acuity was 20/100.
5
The pathophysiology for failure of closure
and reopening remain unclear. Early failure is
thought to result from the lack of formation
of a stable glial plug that is essential for hole
closure.
6
The causes for late reopening remain
unclear as in this case, it may be a sponta-
neous event.
27
Some authors have suggested
that chronic cystoid macular oedema, catar-
act surgery, or the development of an
epiretinal membranes resulting in tractional
forces may be involved.
24
This was not the
case in this patient.
In summary, this case suggests that the
visual outcome can be favourable following
multiple repeat macular hole operations
especially if there has been previous success-
ful closure.
H M Hussin, R H Y Asaria, N E Knox
Cartwright, R H B Grey
Bristol Eye Hospital, Lower Maudlin Street, Bristol BS1
2LX, UK
Correspondence to: Mr Hussin M Hussin, Bristol Eye
Hospital, Lower Maudlin Street, Bristol BS1 2LX, UK;
h.m.hussin@bristol.ac.uk
doi: 10.1136/bjo.2006.079012
References
1Smiddy WE, Sjaarda RN, Glaser BM, et al.
Reoperation after failed macular hole surgery.
Retina 1996;16:13–18.
2Paques M, Massin P, Blain P, et al. Long-term
incidence of reopening of macular holes.
Ophthalmology 2000;107:760–5.
3Duker JS, Wendel R, Patel AC, et al. Late re-
opening of macular holes after initially successful
treatment with vitreous surgery. Ophthalmology
1994;101:1373–8.
4Christmas NJ, Smiddy WE, Flynn HW Jr.
Reopening of macular holes after initially
successful repair. Ophthalmology
1998;105:1835–8.
5Thompson JT, Sjaarda RN. Results of macular
hole surgery in patients over 80 years of age.
Retina 2000;20:433–8.
6Funata M, Wendel RT, de la CZ, et al.
Clinicopathologic study of bilateral macular holes
treated with pars plana vitrectomy and gas
tamponade. Retina 1992;12:289–98.
7Thompson JT, Sjaarda RN. Surgical treatment of
macular holes with multiple recurrences.
Ophthalmology 2000;107:1073–7.
Vision restoration therapy and
raising red flags too early
Vision restoration therapy (VRT) has been
the subject of considerable controversy,
1–3
and
with Horton’s latest editorial
3
it has now
turned rather personal. The editorial is filled
with numerous inaccuracies and misrepre-
sentations and this cannot go unanswered.
Just as a scientific debate should not focus on
fiction but on facts, comments by critics must
be evaluated with the same rigor as the
original work itself. Only by keeping track of
all of the scientific evidence rather than by a
personal and limited review of some selected
facts will we get closer to the truth.
Firstly, it is a fundamental misrepresenta-
tion by Horton that we are trying to ‘‘salvage
neurons’’ at the fringe of a lesion. Rather, we
believe that VRT activates surviving neurons
in areas of residual vision (ARVs)—that is,
areas that are partially damaged.
4–7
Repetitive
use through daily training increases neuronal
activation,
8
which is greatly enhanced by
attentional processes.
9
No neuron is ‘‘sal-
vaged.’’
It is correct that at the time Horton wrote
his editorials no one had published studies on
eye movements. But since then the first eye
movement studies have been published in
abstract form. It actually turns out that eye
movements, when measured with an eye
tracker, are not altered after VRT.
10
There is
also other indirect evidence, which is incom-
patible with the hypothesis that eye move-
ments explain visual field improvements:
NFixation performance in standard auto-
mated perimetry is an accepted quality
control measure and our patients typically
show .90% stable fixation, which is
measured by counting the number of hits
at fixation. Because this does not change
after VRT (it usually improves slightly, see
Kasten et al,
4
Poggel et al,
9
Reinhard et al,
11
Mueller et al,
12
and Sabel et al
13
)itis
unlikely that patients moved their eyes
more after VRT.
NHorton erroneously believes that temporal
field loss prevents blind spot measure-
ments. This is true only for one eye. In our
studies, the position of the blind spot
remains unchanged in monocular mea-
surement after VRT.
13
Thus, neither
eccentric fixation occurred after VRT nor
are there more large saccades after VRT.
10
The position of the blind spot is a reliable
parameter of eccentric fixation.
14
NIf eye movements were responsible for the
visual field shifts, then the entire visual
field border should shift in parallel. In
most patients this is not the case. The
border of the visual field may shift in one
place (say the lower quadrant) and remain
invariant in another (upper quadrant).
Also, highly localised border shifts are
induced by attention cues,
9
and horizontal
border shifts may occur in the absence of
border shifts at fixation. All of these
observations are not compatible with the
proposal that eye movements cause the
visual field border shifts. In fact, in the
periphery the border shifts tend to be more
pronounced, which is explained by the
cortical magnification factor—that is, by
visual cortex architecture.
Accepted for publication 30 January 2006
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doi: 10.1136/bjo.2006.079012
2006 90: 659Br J Ophthalmol
H M Hussin, R H Y Asaria, N E Knox Cartwright, et al.
recurrence of a macular hole
Fourth time lucky: a case of multiple
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