Giant nodular posterior scleritis simulating choroidal melanoma. Dhananjay Shukla, MS;Ramasamy Kim, DNB. A 45-year-old woman, complaining of sudden ...
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onset hematic cyst without any history of previous trauma or surgery. Simple aspiration of these cysts may result in recurrence,1 as happened in our patient. However, surgical excision of the cyst with lamellar patch grafting proved to be an effective modality of treatment. The young age of presentation of our patient, along with the absence of any history of trauma or surgery, points to a developmental origin of the cyst. Although no connection with the anterior chamber or Schlemm’s canal was noted, a defect in the perilimbal sclera strengthens the possibilty of sequestration of conjunctival epithelium within the scleral defect as the possible origin of the cyst. The presence of a fluid level within the cyst, has been noted by various authors.1,2,4 This has been described as desquamated debris that settles down. Presence of brownish looking tissue within the cyst which was histopathologically proven to be lacrimal tissue, has also been reported.2 In our case, presence of ghost cells on histopathology, suggests bleeding within the cyst. The possible origin of the bleeding is from a break in an adjoining conjunctival blood vessel. Spontaneous onset intracorneal hematoma has been described by Kachi and Hirano5 in an elderly lady with ghost vessels and corneal opacity. The causes of intracorneal hematoma as proposed by Muenzler6 include intraocular surgery, ocular trauma, inflammatory corneal disease like corneal ulcer and contact lens wear. None of these factors were present in our patient. UBM is a useful tool in the management of these cystic lesions. It may help in assessing the posterior extent, as well as the contents of the cyst. The typical description on UBM, is of a cystic lesion with high reflective wall, with low reflective internal echoes.7 Presence of blood within the cyst can give rise to high reflective internal echoes.5 Visual impairment and unacceptable cosmesis form the main indications for treatment. As it presents in the early years of life, it may be amblyogenic, when it involves the visual axis, or causes significant alterations of the corneal curvature. Although spontaneous resolution of a corneal cyst by spontaneous drainage of the cyst into the anterior chamber, has been described, 1 the cysts usually require surgical
Giant nodular posterior scleritis simulating choroidal melanoma Dhananjay Shukla, MS; Ramasamy Kim, DNB A 45-year-old woman, complaining of sudden visual loss in the right eye (best-corrected visual acuity: 17/200), was suspected of having a malignant melanoma of the choroid. Fundus Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, 1, Anna Nagar, Madurai - 625 020, Tamil Nadu, India Correspondence to Dhananjay Shukla, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, 1, Anna Nagar, Madurai - 625 020, Tamil Nadu, India. E-mail: Manuscript received: 29.10.04; Revision accepted: 2.2.06
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intervention. Aspiration of the cyst contents has been known to fail.1,4 Reed and Dohlman1 tried aspiration in two cases, both of which recurred within a short period of time. Other surgical modalities like incision on the anterior cyst wall,1,8 excision of the cyst wall with or without chemical or electrocautery,1 has been tried with variable success. In their series of three cases, Rao et al2 have excised the anterior wall, cauterized the posterior wall with 20% Tricholoracetic acid and reported no recurrence at the end of 6 months. Sano et al3 have tried excision of the cyst with lamellar keratoplasty with frozen corneal tissue and also did not report any recurrence. Aspiration of the cyst contents failed in our case too and the cyst recurred within one month. Excision of the cyst with a patch graft to cover the scleral defect and amniotic membrane grafting without any chemical cautery, was effective in preventing recurrence in our case. Use of an amniotic membrane in this case was to enhance epithelial healing and reduce fibrosis and need not be used in all cases.
References 1.
Reed JW, Dohlman CH. Corneal cysts. Arch Ophthalmol 1971;86:648-52.
2.
Rao SK, Fogla R, Biswas J, Padmanabhan P. Corneoscleral epithelial cysts: Evidence of developmental etiology. Cornea 1998;17:446-50.
3.
Sano Y, Okamoto S, Nishida K, Sotozono C, Kinoshita S. Peripheral lamellar keratoplasty for corneoscleral cyst: Three case reports. Cornea 1999;18:233-6. Liakos GM. Intracorneal and sclerocorneal cysts. Br J Ophthalmol 1978;62:155-8. Kachi S, Hirano K. Ultrasonographic biomicroscopic study of an intracorneal hematoma. Cornea 2002;21:421-3. Muenzler WS. Intracorneal hemorrhage. Am J Ophthalmol 1973;76:440-2. Rao SK, Padmanabhan P, Fogla R, Bhende MP. Ultrasound biomicroscopy of an intracorneal epithelial cyst. Cornea 2000;19:249-50. Ruedemann AD Jr. A corneoscleral epithelial inclusion cyst, a rarely described clinieopathological entity. Am J Ophthalmol 1956;41:316-7.
4. 5. 6. 7.
8.
examination revealed a massive pale-colored subretinal mass temporal to the fovea, with adjoining choroidal folds and exudative retinal detachment. Ultrasonography was suggestive of posterior scleritis. Systemic evaluation was unremarkable. The patient was treated with high-dose systemic corticosteroids. The mass lesion resolved completely, with visual recovery to 20/20. Posterior scleritis should be suspected when evaluating subretinal mass-lesions, even when large and without overt inflammation. Early treatment may improve the visual prognosis and avoid misdirected management, including enucleation. Key words: Noduler posterior scleritis, malignant melanoma.
Indian J Ophthalmol 2006;54:120-2
Posterior scleritis is a potentially blinding ocular disorder, often misdiagnosed due to its low incidence and varied clinical presentation, leading to delayed or wrong treatment.1,2
April - June 2006
Brief Reports
Nodular posterior scleritis is especially notorious for mimicking choroidal pathologies like granuloma, hemangioma and most importantly, amelanotic choroidal melanoma, which may lead to an unwarranted enucleation.2 5 We present a case of isolated giant nodular posterior scleritis, initially diagnosed as a choroidal melanoma; but successfully treated by systemic steroids, with complete resolution and visual recovery.
Case Report A healthy 45-year-old lady was referred for an acute loss of vision in the right eye, with a diagnosis of choroidal melanoma. She did not give any history of preceding or concomitant systemic or ocular disease, except mild pain in the right eye. Best-corrected visual acuity was 17/200 in the right eye and 20/20 in the left eye. Anterior segment examination was unremarkable in both eyes. Right fundus examination revealed a massive cream-colored lesion, temporal to fovea, with an exudative detachment involving the fovea and extending inferiorly. Folds were visible in the internal limiting membrane and choroid. [Figure 1a] There were no vitreous
Figure 1a: Fundus photograph of the right eye showing a large yellowish-white subretinal mass lesion temporal to the fovea with exudative detachment and retinochoroidal folds. Best-corrected Snellen acuity is 17/200.
Figure 1b: B-scan ultrasonogram showing the dome-shaped sclerochoroidal lesion with moderate internal reflectivity, adjoining retinal detachment and fluid in the subTenon’s space.
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cells or flare. Posterior segment of the left eye was normal. Fluorescein angiography (FA) of the right eye showed a late staining of the mass lesion; without the features of “double circulation.” B-scan ultrasonography revealed a prominent dome-shaped mass (9.8 mm x 10.3 mm x 4.5 mm), with moderate acoustic reflectivity at the sclerochoroidal level and with fluid in subTenon’s space [Figure 1b]. General physical examination and abdominal ultrasound were unremarkable. Laboratory and ancillary investigations including ESR, a complete blood cell count, PPD skin test, chest X-ray, urinalysis, VDRL, rheumatoid factor and antinuclear antibody were normal. The patient was treated with intravenous methyl prednisolone, 15 mg/kg/day for 3 days. The mass reduced in size, with near-total disappearance of the detachment and visual improvement to 20/60 [Figure 1c]. She was discharged on oral Prednisolone at 1 mg/kg/day, starting with 50 mg / day, tapered to 40 mg and 30 mg, at 10-day intervals. The patient returned a month later, with complete clinical and sonographic resolution of the mass lesion; vision in the right eye had improved to 20/20 [Figure 1d]. Steroids were tapered off in reducing doses of 20, 10 and 5 mg at 10-day intervals;
Figure 1c: After 3 days of intravenous methyl prednisolone, the mass has become smaller and well defined, with near-total absorption of subretinal fluid and visual improvement to 20/60.
Figure 1d: One month later, the massive lesion has resolved completely, with visual improvement to 20/20.
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she was off steroids after 2 months. Last follow-up at 15 months showed stable ocular and systemic status.
improvement on a trial of corticosteroids, it was not deemed necessary.
Discussion
We successfully treated a case of giant nodular posterior scleritis, using systemic steroids. Steroids are however not always helpful. 6 Arevalo et al reported inefficacy of steroids in their case. In view of the good vision and mild symptoms, additional immunosuppressive agents were not used. 5 Demirci et al reported a 12-year follow-up of an asymptomatic case without any treatment. 3 Biswas and colleagues reported a case of massive nodular posterior scleritis in their series, which improved from a vision of Counting fingers at 1 metre, to 20/60 on oral steroids at 7.5 months, with persisting choroidal folds.2 McCluskey and Wakefield favor an aggressive approach to severe cases of scleritis; and have reported the safety and efficacy of pulsed therapy with intravenous methyl prednisolone. 8 Patients with significant visual loss, or lesions near the posterior pole require more aggressive therapy. 1,6,8 In spite of such treatment, a third of patients with posterior scleritis suffer further loss of vision. 6 We obtained a rapid and complete meltdown of a giant-sized nodule of posterior scleritis, with visual recovery from 17/200 to 20/20 on intravenous, followed by oral regimen of corticosteroids. We recommend a higher level of suspicion during evaluation of posterior segment mass lesions, to detect this under-recognized but eminently treatable condition.
A case of giant nodular posterior scleritis, suspected to be amelanotic choroidal melanoma, was diagnosed using B-scan ultrasonography; and successfully treated with intravenous and oral corticosteroids. No systemic associations were apparent. Posterior scleritis has been reported to mimic a variety of ocular inflammatory and neoplastic conditions.2-5 Associated anterior scleritis, eventually seen in up to 60% of cases, helps in diagnosis. 6 Nodular posterior scleritis, especially when very large and associated with minimal inflammation, may closely resemble choroidal melanoma, as described in 3 reports.3-5 In spite of ultrasonic confirmation, Finger and colleagues could not rule out melanoma; and offered the options of observation, radiation, or enucleation to the patient. The eye was subsequently enucleated and the diagnosis of scleritis retrospectively confirmed by histopathological examination. 4 Demirci and associates performed a trephine biopsy for the same purpose, without any adverse effects on the ocular inflammation.3 Arevalo et al used magnetic resonance imaging (MRI) to support the diagnosis. They also used Indocyanine green angiography (ICGA); but choroidal folds were their only positive finding,5 which can easily be seen clinically, as well as on FA. A recent evidence-based update on the use of ICGA did not recommend it for anterior/posterior scleritis, as it added no useful diagnostic information; but conceded its possible value in monitoring the effect of treatment.7 In addition, computed tomography (CT) scan has also been employed as a diagnostic adjunct.2,6 However, short of invasive histological confirmation, B-scan ultrasonography is the key investigation, which provides the strongest diagnostic evidence in posterior scleritis. MRI and CT apparently have no advantage over this inexpensive investigation,1,2,6 even though they have been used by investigators as additional diagnostic tools. They may be reserved for cases where ultrasound images are unsatisfactory or inconclusive.1,6 Our patient was suspected to have scleritis on the basis of a complaint of ocular pain and the clinical finding of choroidal folds, both of which pointed against melanoma or hemangioma. However, she had a creamy-yellow opaque mass, without the other reported clinical clues of posterior scleritis: lesion color matching the background choroid, visibility of choroidal vasculature and positive trans-illumination.3,5 However, B-scan ultrasound was not suggestive of possible choroidal lesions: melanoma, granuloma or hemangioma. FA was supportive, but not definitive for diagnosis, as reported by others. 1,6 Our patient could not afford an MRI; and after rapid
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References 1.
Watson PG, Hazelman BL, Pavesio C, Green WR. The Sclera and Systemic Disorders. 2 nd ed. Butterworth-Heinmann: Edinburgh; 2004. p. 59-89.
2.
Biswas J, Mittal S, Ganesh SK, Shetty NS, Gopal L. Posterior scleritis: Clinical profile and imaging characteristics. Indian J Ophthalmol 1998;46:195-202.
3.
Demirci H, Shields CL, Honavar SG, Shields JA, Bardenstein DS. Long-term follow-up of giant nodular posterior scleritis simulating choroidal melanoma. Arch Ophthalmol 2000;118:1290-2.
4.
Finger PT, Perry HD, Packer S, Erdey RA, Weisman GD, Sibony PA. Posterior scleritis as an intraocular tumour. Br J Ophthalmol 1990;74:121-2.
5.
Arevalo JF, Shields CL, Shields JA. Giant nodular posterior scleritis simulating choroidal melanoma and birdshot retinochoroidopathy. Ophthal Surg Las Imag 2003;34:403-5.
6.
McCluskey PJ, Watson PG, Lightman S, Haybittle J, Restori M, Branley M. Posterior scleritis: Clinical features, systemic associations and outcome in a large series of patients. Ophthalmology 1999;106:2380-6.
7.
Stanga PE, Lim JI, Hamilton P. Indocyanine green angiography in chorioretinal diseases: Indications and interpretation: An evidence-based update. Ophthalmology 2003;110:15-21.
8.
McCluskey P, Wakefield D. Intravenous pulse methylprednisolone in scleritis. Arch Ophthalmol 1987;105:793-7.
