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134 Indian Journal of Ophthalmology Volume 66 Issue 1

References oedema and keratic precipitates. Clin Exp Ophthalmol


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1. Kaufman  HE. Adenovirus advances: New diagnostic and 7. Souza  PM, Holland  EJ, Huang  AJ. Bilateral herpetic
therapeutic options. Curr Opin Ophthalmol 2011;22:290‑3. keratoconjunctivitis. Ophthalmology 2003;110:493‑6.
2. Jhanji  V, Chan  TC, Li  EY, Agarwal  K, Vajpayee  RB. Adenoviral 8. Koizumi N, Inatomi T, Suzuki T, Shiraishi A, Ohashi Y, Kandori M,
keratoconjunctivitis. Surv Ophthalmol 2015;60:435‑43. et al. Clinical features and management of cytomegalovirus
3. Meyer‑Rüsenberg B, Loderstädt U, Richard  G, Kaulfers  PM, corneal endotheliitis: Analysis of 106 cases from the Japan corneal
Gesser C. Epidemic keratoconjunctivitis: The current situation and endotheliitis study. Br J Ophthalmol 2015;99:54‑8.
recommendations for prevention and treatment. Dtsch Arztebl Int 9. Laibson  PR, Dhiri  S, Oconer  J, Ortolan  G. Corneal infiltrates
2011;108:475‑80. in epidemic keratoconjunctivitis. Response to double‑blind
4. Trousdale MD, Goldschmidt PL, Nóbrega R. Activity of ganciclovir corticosteroid therapy. Arch Ophthalmol 1970;84:36‑40.
against human adenovirus type‑5 infection in cell culture and 10. Asena  L, Şıngar Özdemir E, Burcu  A, Ercan  E, Çolak M,
cotton rat eyes. Cornea 1994;13:435‑9. Altınörs DD, et al. Comparison of clinical outcome with different
5. Huang  J, Kadonosono  K, Uchio  E. Antiadenoviral effects treatment regimens in acute adenoviral keratoconjunctivitis.
of ganciclovir in types inducing keratoconjunctivitis by Eye (Lond) 2017;31:781‑7.
quantitative polymerase chain reaction methods. Clin Ophthalmol 11. Maychuk DY, Vasil’eva OA, Russu LI, Mezentseva MV. Clinical
2014;8:315‑20. and immunological comparisons of therapeutic regimens for
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Adenoviral keratoconjunctivitis associated with stromal Vestn Oftalmol 2015;131:49‑55.

Bilateral idiopathic spontaneous Spontaneous subconjunctival filtering bleb is a rare occurrence


and has been observed with ocular or systemic abnormalities
filtering bleb with ectopia lentis: such as Axenfeld syndrome,[1] Terrien marginal degeneration,[2,3]
A case report and review of literature familial craniofacial dysmorphism,[4,5] scleritis,[6] and connective
tissue diseases.[7] To the best of our knowledge, only few cases
have been reported till date, and we report a 26‑year‑old patient
Premanand Chandran, Anjali S Khairnar, with bilateral spontaneous filtering bleb.
Nabeed Aboobacker, Ganesh V Raman
Case Report
A 26‑year‑old male presented with superior filtering bleb with A 26‑year‑old male presented with defective vision in both
scleral thinning, dislocated lens, and hypotony in both the eyes  (BEs) since childhood. The patient had no history of
eyes. His cornea was normal without any sign of ectasia, and previous ocular surgery, trauma, or systemic illness. His
there was no history of recurrent redness, trauma, or surgery best‑corrected visual acuity was 6/18 with + 11 + 1.5 × 90 in
in either eye. Anterior segment optical coherence tomography the right eye  (RE) and 6/12 with  +  11.5  +  2.5  ×  90 in the left
did not reveal communicating fistula between the anterior eye (LE). Slit‑lamp examination showed a diffuse conjunctival
chamber and subconjunctival space. Physical examination and bleb near the limbus extending from 10 to 4 o’clock in the
blood investigations did not reveal any systemic association. RE [Fig. 1a and b] and 8 to 1 o’clock in the LE [Fig. 1d and e]
He was diagnosed to have spontaneous filtering bleb, with superior scleral thinning in BEs. Seidel’s test was negative
which is a rare condition observed with ocular or systemic in BE. RE showed clear cornea with deep anterior chamber
abnormalities. and inferonasal dislocated lens. LE showed iridocorneal
adhesion in the paracentral zone with corneal scar, iris
Key words: Hypotony, lens dislocation, spontaneous filtering atrophy, and aphakic lens status. Dilated examination revealed
bleb microphakic‑dislocated lens in the anterior chamber in the
RE and inferonasal dislocated lens in the LE [Fig. 1c and f].
Access this article online Anteriorly dislocated lens in the RE was reposited into the
Quick Response Code: posterior chamber in the supine posture. His intraocular
Website:
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pressure was 6 and 3 mmHg in the RE and LE, respectively.
On gonioscopy, angles were closed in BEs without evidence of
DOI: ostium or cyclodialysis cleft. Fundus examination showed a cup
10.4103/ijo.IJO_630_17

PMID:
This is an open access article distributed under the terms of the Creative
*** Commons Attribution‑NonCommercial‑ShareAlike 3.0 License, which allows
others to remix, tweak, and build upon the work non‑commercially, as long as the
author is credited and the new creations are licensed under the identical terms.
Glaucoma Department, Aravind Eye Hospital, Coimbatore,
Tamil Nadu, India
For reprints contact: reprints@medknow.com
Correspondence to: Dr. Premanand Chandran, Glaucoma Department,
Aravind Eye Hospital, Avinashi Road, Coimbatore  ‑  641  014, Cite this article as: Chandran P, Khairnar AS, Aboobacker N, Raman GV.
Tamil Nadu, India. E‑mail: drcgprem@yahoo.co.in Bilateral idiopathic spontaneous filtering bleb with ectopia lentis: A case report
and review of literature. Indian J Ophthalmol 2018;66:134-6.
Manuscript received: 22.07.17; Revision accepted: 03.10.17
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January 2018 Case Reports 135

to disc ratio of 0.5:1 with healthy neuroretinal rim, choroidal within normal limits. Antinuclear antibody test was negative.
folds, and attached retina in BE. Electrocardiogram and echocardiogram were normal.
Corneal topography and pachymetry  (Orbscan, Bausch Discussion
and Lomb, Orbtek Inc., UT, USA) were suggestive of
oblique astigmatism in BE without evidence of corneal First case of spontaneous filtering bleb was reported in a patient
ectasia [Fig.  2a and b]. Anterior segment optical coherence with Axenfeld syndrome by Nemet et  al.[1] The patient had
tomography  (DRI OCT Triton plus, Topcon, Tokyo, Japan) prominent Schwalbe line and iridocorneal adhesion in both
showed aqueous pockets in subconjunctival and intrascleral the eyes. Authors have postulated that increased IOP leads to
spaces in BEs [Fig. 3a and b]. There was no communicating rupture of the corneoscleral junction resulting in the formation
fistula between the anterior chamber and subconjunctival of filtering bleb. Our patient had iridocorneal adhesion only in
space. the LE and did not have anterior embryotoxon in both the eyes.
Spontaneous filtering bleb formation due to corneal pathologies
Physical examination did not reveal joint or skin such as Terrien and pellucid marginal degeneration have been
hyperextensibility, brachydactyly, facial dysmorphic reported by Soong et al.,[2] Munro et al.,[3] and Toriyama et al.[8]
features, or marfanoid habitus. Blood investigations including Mechanism proposed was the extreme distortion and stretching
rheumatoid factor, homocysteine, and lysine level were of the peripheral cornea resulting in rupture of the Descemet’s

a b c

d e f
Figure 1: Slit‑lamp photograph of the right eye showing inferonasal dislocated lens, superonasal diffuse bleb with scleral thinning (a and b) and
anterior dislocated microphakic lens on dilatation (c). Left eye showing iridocorneal adhesion and aphakic lens status, superonasal diffuse bleb
with scleral thinning (d and e) and inferonasal dislocated lens on dilatation (f)

a b
Figure 2: Corneal topography image of the right (a) and left (b) eye showing oblique astigmatism
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136 Indian Journal of Ophthalmology Volume 66 Issue 1

formation would be seeping of aqueous through the area of


scleral thinning.
Declaration of patient consent
a The authors certify that they have obtained all appropriate
patient consent forms. In the form, the patient has given his
consent for his images and other clinical information to be
reported in the journal. The patient understands that his name
and initial will not be published and due efforts will be made
b
to conceal their identity, but anonymity cannot be guaranteed.
Figure 3: Anterior segment optical coherence tomography of the Financial support and sponsorship
right (a) and left eye (b) showing aqueous pockets in subconjunctival Nil.
and intrascleral spaces
Conflicts of interest
membrane and bleb formation. Our patient had normal cornea There are no conflicts of interest.
without signs of thinning or degeneration.
References
Shawaf et  al.[4] and Haddad et  al.[5] have reported two
1. Nemet  P, Bracha  R, Lazar  M. Spontaneous filtering blebs in
unrelated families of Lebanese Druze community with axenfeld syndrome. Am J Ophthalmol 1973;76:590‑1.
syndrome of ectopia lentis, spontaneous filtering bleb, and
2. Soong  HK, Fitzgerald  J, Boruchoff  SA, Sugar  A, Meyer  RF,
craniofacial dysmorphism. Dysmorphic features were dental
Gabel  MG, et al. Corneal hydrops in Terrien’s marginal
crowding, large beaked nose, and antimongoloid slanting of degeneration. Ophthalmology 1986;93:340‑3.
the palpebral fissures. Authors concluded that the syndrome
3. Munro M, McWhae J, Romanchuk K, Crichton A, Carter G, Ball A,
is due to abnormality in the connective tissue development et al. Two cases of spontaneous filtering blebs, one idiopathic
and it is inherited in an autosomal recessive manner. Pasquale and one associated with Terrien marginal degeneration. Cornea
et  al.[9] have reported spontaneous filtering bleb in a patient 2014;33:752‑4.
with microspherophakia and dysmorphic features in the form 4. Shawaf  S, Noureddin  B, Khouri A, Traboulsi  EI. A  family with
of malar hypoplasia, frontal bossing, and septal deviation. Our a syndrome of ectopia lentis, spontaneous filtering blebs, and
patient had filtering bleb and microphakic‑dislocated lens, but craniofacial dysmorphism. Ophthalmic Genet 1995;16:163‑9.
there was no facial dysmorphic features. 5. Haddad R, Uwaydat S, Dakroub R, Traboulsi EI. Confirmation of
the autosomal recessive syndrome of ectopia lentis and distinctive
Mantravadi and Stock[6] have reported spontaneous filtering
craniofacial appearance. Am J Med Genet 2001;99:185‑9.
bleb with scleral thinning as a consequence of scleritis in a female
with systemic lupus erythematosus. Gerke et al.[7] have reported 6. Mantravadi  AV, Stock  EL. Spontaneous filtration bleb as a
consequence of scleritis. Arch Ophthalmol 2007;125:1578‑9.
spontaneous filtering bleb, ocular hypotension, inferior oblique
and superior rectus paralysis, and madarosis in a patient with 7. Gerke E, Meyer‑Schwickerath G, Joussen F. [Sclerodermie en coup
de sabre with spontaneous filtering bleb  (author’s transl)]. Klin
scleroderma. There were no signs of systemic connective tissue
Monbl Augenheilkd 1976;168:426‑8.
abnormality in our patient. Two patients with spontaneous
filtering bleb without any other ocular or systemic abnormalities 8. Toriyama K, Inoue T, Suzuki T, Higashiura R, Maeda N, Ohashi Y,
et al. Spontaneous bleb formation in a presumed pellucid marginal
have been reported by Munro et al.[3] and Tatham et al.[10] corneal degeneration with acute hydrops. Cornea 2013;32:839‑41.
Conclusion 9. Pasquale  LR, Smith  SG, Traboulsi  E, Jampel  H. Spontaneous
filtration blebs in a patient with microspherophakia. Am J
Our patient had bilateral superior filtering bleb with scleral Ophthalmol 1991;112:350‑2.
thinning, dislocated lens, and hypotony for which the cause 10. Tatham A, Karwatowski W, Kumar P. Spontaneous bleb formation.
could not be elucidated and the probable mechanism of bleb Clin Exp Ophthalmol 2011;39:712.

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