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Discussion Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The disease can be either “localized” to the skin or “systemic” involving other organs in addition to the skin. Localized scleroderma can be further divided into linear and morphea (localized or generalized patches of atrophic and sclerotic skin lesions).[1] Linear scleroderma is characterized by localized fibrosis of skin, blood vessels, subcutaneous fat, muscle, and sometimes bone. It primarily affects the pediatric population in the first and second decade.[2] When linear scleroderma occurs on the fronto‑parietal scalp, it is referred to as “‘en coup de sabre,” given the resemblance of the skin lesion to the stroke of a sabre.[3] Specific neurological and ophthalmic associations of craniofacial scleroderma have been reported in literature that were all ruled out in our patient. The ophthalmic manifestations in scleroderma may include ptosis, lid induration or tightening, lash loss, motility disorder, keratoconjunctivitis sicca, conjunctival injection and vascular sludging, fornix shortening, iritis, heterochromia iridis, papillitis, retrobulbar pain, enophthalmos, displacement of outer canthus from resorption of orbital bone, and retinal hemorrhages.[2‑5]
Conclusion
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Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
References 1. Kirkali PA, Kansu T, Sanac AS. Unilateral enophthalmos in systemic scleroderma. J Clin Neuroophthalmol 1991;11:43‑4. 2. Fernando BS, Cannon PS, Tumuluri K, Cook AE. Linear scleroderma as a rare cause of enophthalmos: A case report. J Med Case Rep 2007;1:179. 3. Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet BA, et al. Linear scleroderma en coup de sabre w i t h a s s o c i a t e d n e u r o l o g i c a b n o r m a l i t i e s . Pe d i a t r i c s 2006;117:e132‑6.
Scleroderma is a rare cause of enophthalmos after ruling out other likely differentials of orbital fracture and orbital metastatic breast cancer. The linear scar on the forehead “en coup de sabre” should not be discarded as an innocuous finding rather should aid the examiner in making the correct diagnosis.
4. West RH, Barnett AJ. Ocular involvement in scleroderma. Br J Ophthalmol 1979;63:845‑7.
Unilateral corneal edema in young: A diagnostic dilemma
Key words: Congenital hereditary corneal dystrophy, corneal edema, Harboyan syndrome, polymegathism, polymorphism, specular microscopy
Dewang Angmo, Harathy Selvan, Aswini Kumar Behera, Pramod Kumar Suman
A 27‑year‑old male was referred to us as a case of unilateral glaucoma with corneal haze. Elaborate history taking revealed painless diminution in vision OS for past 18 months and presence of hearing impairment since childhood. Family history was not contributory [Fig. 1].
Access this article online Quick Response Code:
Website: www.ijo.in DOI: 10.4103/ijo.IJO_564_18 PMID: *****
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India Correspondence to: Dr. Dewang Angmo, Room No. 374, Third Floor, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India. E‑mail:
[email protected] Manuscript received: 19.04.18; Revision accepted: 16.08.18
5. Hock LE, Kontzialis M, Szewka AJ. Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. Neurology 2016;87:1741‑2.
The best‑corrected visual acuity OD was 6/6 and OS 1/60. Intraocular pressure (IOP) was OD: 12 and OS: 18 mmHg (on two topical medications) and both eyes had normal corneal sensations. Slit lamp showed OD to be apparently normal while OS demonstrated diffuse corneal haze [Fig. 2]. There
This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact:
[email protected] Cite this article as: Angmo D, Selvan H, Behera AK, Suman PK. Unilateral corneal edema in young: A diagnostic dilemma. Indian J Ophthalmol 2018;66:1612-4.
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Photo Essay November 2018
were no keratic precipitates or corneal vascularization, and further clinical evaluation was not possible due to media haze. Due to diagnostic dilemma, we re‑evaluated the fellow eye to look for any clue. To our surprise, on specular reflection, the apparently normal fellow eye showed enlarged endothelial cells [Fig. 3]. Specular microscopy of OD showed 650 cells/ mm2 and co‑efficient of variation 38. Hexagonality% could not be picked up, but the picture was suggestive of increased polymorphism [Fig. 3]. The specular count of OS was not capturable due to corneal edema. Anterior segment OCT of both eyes showed increased pachymetry, OD: 690 µ and OS:
850 µ (corneal edema) [Fig. 4]. Confoscan of the fellow normal eye confirmed polymegathism [Fig. 3]. Being a bilateral endothelial disease at this young age, a diagnosis of congenital hereditary endothelial dystrophy (CHED)[1] was made. Pure tone audiometry was performed which showed moderate sensorineural hearing loss, implying the diagnosis of the “Harboyan syndrome”.[2] The patient was then advised to stop the topical glaucoma medications one by one under monitoring, even after which the IOP remained within normal range. The patient was started on topical hypertonic saline and registered for endothelial keratoplasty.
b
a Figure 1: A four generation pedigree chart computed based on the elicited family history
a
Figure 2: Slit-lamp biomicroscopic photography under diffuse illumination, (a) OD which appears apparently normal. (b) OS with diffuse corneal edema
c
b
Figure 3: Images of OD, (a) slit-lamp biomicroscopic specular reflection showing enlarged polymorphic endothelial cells. (b) Confocal microscopic section of the endothelium showing the same. (c) Specular microscopy showing, cell count 650/mm2, and co-efficient of variation 38%. The frame shows spread out, enlarged, and polymorphic endothelial cells
a
b
Figure 4: Anterior segment optical coherence tomography images, (a) of the right eye with pachymetry 690μ (b) of the left eye showing diffuse stromal edema (850 µ)
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Conclusion A comprehensive work‑up of both eyes is essential before prematurely concluding at the diagnosis, to avoid redundant patient mismanagement.
Volume 66 Issue 11
Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
References
Intraoperative assessment of corneal injuries using microscope‑integrated optical coherence tomography
epithelium were regular without any tissue corneal tissue loss. Along the center and periphery of the cornea, the corneal gaping becomes prominent [Fig. 2b].
Amar Pujari, Ritika Mukhija, Jayanand Urkude, Rashmi Singh, Divya Agarwal, Namrata Sharma Key words: Integrated optical coherence tomography, pediatric corneal injuries, surgery
A 2 ‑ ye a r ‑ o l d m a l e c h i l d p r e s e n t e d t o t h e o c u l a r emergency following penetrating injury with a metallic foreign body. Clinical examination revealed a nasal corneal perforation with iris tissue prolapse [Fig. 1, yellow arrow]. To further delineate the extent of the corneal perforation and associated anterior segment changes, integrated optical coherence tomography (iOCT) was used. It showed corneal wound gaping for entire corneal thickness from the paracentral region to the periphery of the cornea. Within the gaping, prolapsing hyperreflective tissue was made out in continuity with the iris tissue onto the surface of the epithelium, suggestive of iris tissue [Fig. 2a]. The margins from endothelium to the
1. Weiss JS, Møller HU, Aldave AJ, Seitz B, Bredrup C, Kivelä T, et al. IC3D classification of corneal dystrophies – edition 2. Cornea 2015;34:117‑59. 2. Desir J, Abramowicz M. Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome). Orphanet J Rare Dis 2008;3:28.
Following surgery, the iOCT increased corneal thickness due to stromal edema with well‑opposed margins [Fig. 2c and d]. The endothelium on either side of the wound showed a good continuity. Similarly, the stroma and the epithelium showed perfect opposition without any intervening residual iris tissue or any tissue loss.
Discussion The iOCT mainly helps in better intraoperative understanding of the corneal wound‑related problems and the cataract and
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Website: www.ijo.in DOI: 10.4103/ijo.IJO_546_18 PMID: *****
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India Correspondence to: Dr. Amar Pujari, Room No. 212, RPC-1 Hostel, AIIMS, New Delhi - 110 029, India. E-mail:
[email protected] Manuscript received: 15.04.18; Revision accepted: 27.06.18
Figure 1: Microscopic examination of the injured eye showing nasal corneal perforation with iris tissue prolapse This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact:
[email protected] Cite this article as: Pujari A, Mukhija R, Urkude J, Singh R, Agarwal D, Sharma N. Intraoperative assessment of corneal injuries using microscopeintegrated optical coherence tomography. Indian J Ophthalmol 2018;66:1614-5.
