Acknowledgement: Aniridia Associated With Congenital Aphakia and Secondary Glaucoma

July - August 2009
Brief Communications
contact with the milky latex. There is burning sensation, pain,

photophobia and lacrimation which may worsen over hours
even after copious irrigation. At rst, there is mild diminution
of vision, but may diminish further to 20/200 or counting ngers
to hand movements within 24 h as Case 2 in this report. On
initial examination, the corneal epithelium may be intact or with
mild punctate epitheliopathy, but eventually it may show frank
epithelial defect on the next day.[10] It takes around four to seven
days for the epithelium to heal completely. There is stromal edema
with Descemets fold which decreases with time. The degree of
anterior uveitis is variable and is particularly marked with certain
species as in Case 1 and Case 3 in this report.[3] The degree of
ocular inammation may also vary with the amount of sap that
enters the eye. Neglected cases can progress to blindness due to
corneal scarring, complicated uveitis, and anterior staphyloma.[3,4]
The species of Euphorbia causing ocular toxicity reported
earlier were mostly with E. royaleana, E. lathyris and
E. tirucalli.[4,5,11,12] Only one case of ocular toxicity with E. trigona
was reported earlier by Scott et al.[5] and they reported only corneal
epithelial defect without edema and anterior chamber reaction.
But in our Case 1, there was gross corneal edema with moderate
anterior uveitis and secondary elevated IOP. This was possibly
due to a greater amount of sap entering into the RE in our case.
There was only one case report on E. milii by Eke et al.[6] and the
patient presented with corneal epithelial defect and edema with
mild anterior uveitis which was similar to our third case. To the
best of our knowledge which includes MEDLINE search, we
could not nd any case report of ocular toxicity by the sap of E.
neriifolia (Indian Spurge tree). If the patient presents early within
24 h, the treatment is antibiotic eye drops, topical corticosteroids,
cycloplegics, tears substitute and IOP-lowering medications if
necessary. No patching is required. With appropriate supportive
therapy and close daily observation, the condition generally
resolves completely within 10-15 days. In case of suspected
bacterial infection and in the presence of a hypopyon, topical
corticosteroids may be started later once the epithelial defect
gets healed.[10]
In conclusion, the clinical course may be aected by particular
species of Euphorbia, the amount of sap exposure, the time between
exposure and irrigation, and host factors. Ophthalmologists
managing Euphorbia keratouveitis should warn the patient that
Aniridia associated with congenital

aphakia and secondary glaucoma
Mayur Moreker1, Rajul Parikh2, Shefali R Parikh1,
Ravi Thomas1,3
313
vision may get worse on the next day before it improves. It is

always advisable to ask the patient to bring a sample of the plant
for identication. People who work with Euphorbia species should
wear protective goggles while handling the plant.
Acknowledgement
We acknowledge the contribution of Mr. Volker Buddensiek,
Editor, Euphorbia World, and Mr. Tim Marshall, Seed Bank
Secretary, International Euphorbia Society, 17 High Street,
Wighton, Norfolk, NR23 1AL, UK, for identication of Euphorbia
neriifolia plant.
References
1.
Webster GL. Irritant plant in Spurge family (Euphorbiaceae). Clin

Dermatol 1966;4:36-45.
2.
Elder S Duke. System of Ophthalmology. Vol XIV. London: Kimpton;

1972, p. 1185.
3.
Grant WM, Schuman JS. Toxicology of the eye. In: Charles C Thomas
editor 4th ed. Springeld, IL: Thomas Publishers; 1993, p. 680-2.
4.
Sofat BK, Sood GC, Chandel RD, Mehrotra SK. Euphorbia royaleana
latex keratitis. Am J Ophthalmol 1972:74;634-7.
5.
Scott IU, Karp CL. Euphorbia sap keratitis: Four cases and possible
pathologic mechanism. Br J Ophthalmol 1996:80;823-6.
6.
Eke T, Al-Husainy S, Raynor MK. The spectrum of ocular inammation

caused by Euphorbia plant sap. Arch Ophthalmol 2000;118:13-6.
7.
Available from: http://www.desert-tropicals.com/Plants/

Euphorbiaceae/Euphorbia.html. [last accessed on 2008 Jun 22].
8.
Available from: http://en.wikipedia.org/wiki/List_of_Euphorbia_

species. [last accessed on 2008 Jun 22].
9.
Available from: http://www.euphorbia-international.org/. [last

accessed on 2008 Jun 22].
10. Merani R, Sa-Ngiampornpanit T, Kerdraon Y, Billson F, Mc Clellan KA.

Euphorbia lactea sap keratouveitis: Case report and review of the
literature. Cornea 2007;26:749-52.
11. Crowder JI, Sexton RR. Keratoconjunctivitis resulting from the sap of
candelabra cactus and the pencil tree. Arch Ophthalmol 1964:72:47684.
12. Sood GC, Sofat BK, Chandel RD. Injury to the eye by the sap of
Euphorbia royaleana. Br J Ophthalmol 1971;55:856-7.
We report a case of aniridia associated with congenital aphakia

and secondary glaucoma. A 35-year-old male presented with
aniridia, congenital aphakia and secondary glaucoma in both
eyes. After an unsuccessful medical management, he underwent
trabeculectomy with mitomycin C and anterior vitrectomy under
local anesthesia in his left eye. Postoperatively, at the end of six
months, intraocular pressure (IOP) in his left eye was controlled
without medications. This case highlights the rare association of
aniridia with congenital aphakia and secondary glaucoma.
VST Center for Glaucoma Care, L.V. Prasad Eye Institute, Hyderabad,
Bombay City Eye Institute and Research Center, Mumbai, India.
3
Queensland Eye Institute, Brisbane, Australia, 4University of
Queensland, Brisbane, Australia
Key words: Aniridia, congenital aphakia, secondary glaucoma
Correspondence to Dr. Rajul Parikh, Bombay City Eye Institute and

Research Center, 5, Babulnath Road, Mumbai - 400 007, Maharashtra,
India. E-mail: [email protected]
DOI: 10.4103/0301-4738.53061
Manuscript received: 03.02.08; Revision accepted: 28.08.08
Indian J Ophthalmol: 2009;57:313-314
Aniridia is a phenotypically heterogeneous condition that

can be inherited as an autosomal dominant disorder or as
314
IndianJournalofOphthalmology
Vol. 57 No. 4
part of several systemic syndromes. It has been linked to

Chromosomes 1 and 2 and associated with the deletion of the
p-13 band of Chromosome 11.[1] Aniridia involves not only
the iris, but also the retina, optic nerve, lens and cornea.[2]
Visual acuity deteriorates as a result of nystagmus, glaucoma,
cataract, corneal opacities and retinal hypoplasia. There are
numerous reports of association of aniridia with congenital
cataract but there is no report in literature showing association
of aniridia and congenital aphakia. We report a patient with
aniridia, congenital aphakia and secondary glaucoma.
Case Report
A 35-year-old male presented with complaints of decreased
vision in both eyes for 15 years. There was no history of
intraocular surgery in either eye. His best-corrected visual
acuity was no perception of light in the right eye and 20/400
in the left eye with + 9.0 diopter sphere (Dsph). Horizontal
pendular nystagmus was noted in both eyes. Examination of
the right eye revealed corneal stromal edema and an intercalary
staphyloma. The cornea in the left eye had mild corneal haze.
Both eyes had aniridia and were aphakic. The view in the
left eye was clearer and showed aphakia with total absence
of zonules [Fig. 1]. Intraocular pressure (IOP) measured by
Goldmann applanation tonometry was 28mm Hg and 36mm
Hg in the right and left eye respectively. Corneal edema
obscured visualization of the angle in the right eye. Gonioscopy
in the left eye with a four-mirror lens showed open angles
up to the cilliary body inferiorly; the stump of the iris had
formed peripheral anterior synechia (PAS) superiorly. Fundus
details were not clear in the right eye but a total glaucomatous
optic atrophy was noted. Fundus examination of the left eye
showed a near total glaucomatous optic atrophy (vertical disc
diameter of 2.1 mm, 0.9 : 1 cup disc ratio with bipolar notch)
as well as foveal hypoplasia. As the IOP was uncontrolled with
topical 0.5% timolol maleate eye drops and 0.15% brimonidine
tartarate eye drops, the patient underwent trabeculectomy
with Mitomycin C under local anesthesia in his left eye. Partial
anterior vitrectomy was performed at the same time.
At ve weeks postoperatively the patient maintained a
best-corrected visual acuity of 20/400 (using + 9 Dsph and 1
diopter cylinder (D cyl) at 180 degree) in the left eye [Fig. 2].
At the three-month follow-up the vision remained the same;
there was a diuse bleb and the IOP was 7mm Hg without
any anti-glaucoma medications. When last seen (six months
postoperatively), the best-corrected visual acuity of 20/400 was
maintained; the IOP was 6 mm Hg without any anti-glaucoma
medications.
Discussion
The visual function in aniridia varies from near normal to
blindness. The more serious cases where blindness occurs are
not due to the aniridia but due to associated conditions like
cataract, glaucoma, foveal hypoplasia, corneal dystrophy, and
nystagmus. Deletion or mutations involving the PAX6 gene
have been implicated in the pathogenesis of various anterior
segment anomalies including congenital aphakia.[3] However,
Figure 1: Left eye of the patient with aphakia and aniridia. The patients
left cornea has mild haze. Aniridia and aphakia can be noted
Figure 2: Left eye of the patient with diffuse bleb (postoperatively)
to our knowledge there is no reported case in literature of

aniridia associated with congenital aphakia. Such an association
could be expected to occur by a chance alone in approximately
one in 490,000,000 live births. (Chance associations calculated
based on approximate prevalence of aniridia in one in 70,000
live births and approximate prevalence of congenital aphakia
in one in 70,000 live births.)
This case highlights the rare association of aniridia with
aphakia and secondary glaucoma.
References
1.
Nelson LB, Spaeth GL, Nowinski TS, Margo CE, Jackson L.

Aniridia: A review. Surv Ophthalmol 1984;28:621-42.
2.
Ivanov I, Shuper A, Shohat M, Snir M, Weitz R. Aniridia: Recent

achievements in paediatric practice. Eur J Pediatr 1995;154:795-800.
3.
Johnson BL, Cheng KP. Congenital aphakia: A clinicopathologic

report of three cases. J Pediatr Ophthalmol Strabismus
1997;34:35-9.

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July - August 2009

contact with the milky latex. There is burning sensation, pain,

Aniridia associated with congenital

vision may get worse on the next day before it improves. It is

Webster GL. Irritant plant in Spurge family (Euphorbiaceae). Clin

Elder S Duke. System of Ophthalmology. Vol XIV. London: Kimpton;

Eke T, Al-Husainy S, Raynor MK. The spectrum of ocular inammation

Available from: http://www.desert-tropicals.com/Plants/

Available from: http://en.wikipedia.org/wiki/List_of_Euphorbia_

Available from: http://www.euphorbia-international.org/. [last

10. Merani R, Sa-Ngiampornpanit T, Kerdraon Y, Billson F, Mc Clellan KA.

We report a case of aniridia associated with congenital aphakia

Key words: Aniridia, congenital aphakia, secondary glaucoma

Correspondence to Dr. Rajul Parikh, Bombay City Eye Institute and

Manuscript received: 03.02.08; Revision accepted: 28.08.08

Indian J Ophthalmol: 2009;57:313-314

Aniridia is a phenotypically heterogeneous condition that

part of several systemic syndromes. It has been linked to

Figure 2: Left eye of the patient with diffuse bleb (postoperatively)

to our knowledge there is no reported case in literature of

Nelson LB, Spaeth GL, Nowinski TS, Margo CE, Jackson L.

Ivanov I, Shuper A, Shohat M, Snir M, Weitz R. Aniridia: Recent

Johnson BL, Cheng KP. Congenital aphakia: A clinicopathologic

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