Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
FIG. 2. A, Hematoxylin- and eosin-stained paraffin section showing the typical wiry/ropey collagen (arrows). B, Collections of mature adipocytes between the collagen (arrows); (C) bland wavy spindle cells (arrows); (D) occasional mast cell present (arrow); and (E) immunohistochemistry showing strong brown positive staining for CD34.
Orbital Sarcoid-Like Granulomatosis After Inhibition of Tumor Necrosis Factor-α Edward J. Wladis, M.D., F.A.C.S.*, Ashley J. Tarasen, M.D.†, Zachary J. Roth, M.D.‡, Martha G. Farber, M.D.†‡, Jeffrey Ross, M.D.†, and Victoria M. Michaels, M.D.§ Abstract: Pharmacologic inhibition of tumor necrosis factor-alpha (TNF-α) has been used in the management of a variety of inflammatory conditions. Recently, reports on the development of sarcoid-like granulomatous disease *Ophthalmic Plastic Surgery, Department of Ophthalmology, Lions Eye Institute, †Department of Pathology, ‡Department of Ophthalmology, Lions Eye Institute, and §Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, New York, U.S.A. Accepted for publication March 18, 2014. The authors disclose no financial or conflicts of interest. The information presented in this manuscript has not been presented elsewhere and is not under consideration for publication elsewhere. Address correspondence and reprint requests to Edward J. Wladis, M.D., F.A.C.S., 1220 New Scotland Rd, Suite 302, Slingerlands, NY 12159. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000200
e30
at multiple systemic sites after treatment with TNFα inhibitors have emerged, although, to the authors’ knowledge, orbital manifestations of this problem have not been previously described. A 48-year-old woman who received injections of adalimumab for the treatment of psoriatic arthritis developed right-sided orbital pain and inflammation. Orbital biopsy of a focal lesion demonstrated sarcoid-like granulomatosis, and a workup for other causes of this problem was noncontributory. This report represents the first documented case of this phenomenon in the orbit, and possible mechanisms are discussed in this presentation. Given the expanding role of TNF-α inhibitors and the increased frequency of their use, clinicians should be aware of this possible side effect.
I
nhibition of tumor necrosis factor-alpha (TNF-α) has emerged as an important modality in the management of multiple systemic inflammatory conditions.1 Currently, 3 TNF-αantagonists are commercially available, including etanercept, adalimumab, and infliximab, and these agents have been previously used to treat various ophthalmic and orbital conditions, including uveitis, scleritis, myositis, idiopathic orbital inflammation, and thyroid eye disease.2–5 While these medications are generally
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
CASE REPORT
FIG. 1. External photograph of the patient’s modest periorbital edema and erythema.
FIG. 2. Coronal MRI demonstrating a focal inferior orbital mass.
A 48-year-old woman was referred to the Oculoplastic Surgery service at Albany Medical College for the evaluation of a 1-month history of a painful mass along the inferior aspect of her right orbit. This lesion began acutely and was not associated with diplopia or vision loss. Reportedly, the patient had recently initiated a course of oral prednisone (40 mg daily) with modest relief of her discomfort. Her medical history was significant for psoriatic arthritis, for which she was previously treated with a several-week course of adalimumab (40-mg subcutaneous injections every other week), and the patient reported that the orbital lesion developed roughly 1 month after her most recent injection. On initial examination, the patient’s visual acuity was 20/20 with correction in the OD, and her extraocular motility was reduced by 50% with infraduction. There was no evidence of an afferent pupillary defect. Digital palpation of the orbit demonstrated a firm, tender, fixed mass along the central portion of the orbital floor with mild overlying cutaneous edema and erythema (Fig. 1). While the patient had 1.5 mm of ipsilateral proptosis, the remainder of her ophthalmic examination was noncontributory. An orbital MRI scan demonstrated a 1.8 × 2.3 × 1.3 cm focal, relatively homogeneous right-sided lesion that was immediately adjacent to the inferior rectus (Fig. 2). To further evaluate this problem, the patient underwent a transconjunctival anterior orbitotomy; intraoperatively, the mass appeared as a whitish-yellow firm nodule, and several biopsies were taken from the core of the lesion. Histopathologic evaluation of these biopsies demonstrated non-necrotizing granulomatous inflammation without evidence of malignancy or infection (Fig. 3). Staining for acid-fast bacilli was negative, as was assessment with Gomori methenamine silver staining. In addition to microscopic evaluation of the lesion, a thorough evaluation for other explanations for these granulomas was noncontributory; a chest x-ray did not reveal any active disease, and angiotensin-converting enzyme and lysozyme levels were within normal limits. Based on these findings, the patient discontinued adalimumab and began a several-week course of oral prednisone. Oral methotrexate was thus initiated to suppress the inflammation associated with both the patient’s orbital lesion and her psoriatic arthritis.
DISCUSSION
FIG. 3. Photomicrograph (×20 magnification) from the patient’s orbital biopsy, documenting diffuse sarcoid-like granulomas.
efficacious and well-tolerated, several recent case reports have documented the development of sarcoid-like granulomatous processes in the skin,6,7 lungs,1,8 hypopharynx,9 and lymph nodes10 during treatment with TNF-α inhibitors. In this report, the first case of orbital sarcoid-like granulomatosis secondary to therapy with TNF-α antagonists is reported. This investigation was compliant with the Health Insurance Portability and Accountability Act and adhered to the tenets of the Declaration of Helsinki.
Sarcoid-like granulomatosis has been reported at several other anatomical sites during TNF-α inhibition.1–10 Classically, TNF-α has been associated with the progression of sarcoidosis and the formation of granulomas and treatment with TNF-α inhibitors has been successfully used in the management of sarcoidosis,11 making the association between antagonism of this molecule and this phenomenon counterintuitive. Compared with etanercept, adalimumab has been reported to have a more favorable treatment profile in the management of sarcoidosis.12,13 This report represents the first documentation of this process in the orbit; PubMed and Medline searches for “TNF-α,” “sarcoid,” and “orbit” did not reveal any similar descriptions. Several hypotheses have been proposed to explain this side effect. First, partial suppression of TNF-α may induce a selective redistribution of this molecule, thereby inducing it to travel sites with lower internal concentrations that are distal to the anatomical locale of the initial pathology.7 As a result, while the total systemic concentration of TNF-α decreases, a relative increase of TNF-α in the orbit (at the expense of the skin) could ultimately lead to granulomatosis.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
Alternatively, TNF-α selectively suppresses autoreactive T cells, and inhibition of this suppressive effect may ultimately lead to enhanced T-cell reactivity, changes in the molecular milieu, and the development of granulomas.7 Previous investigations have indicated increases in interferon-γ–producing T cells during treatment with TNF-α inhibitors, and interferon-γ has been implicated in granuloma formation.1 These subtle immunologic aberrancies may ultimately explain this side effect. Certainly, the induction of sarcoid-like orbital lesions after chemotherapy has been documented with other agents. Hwang and Gausas14 and Morley and et al.15 reported cases of sarcoid-related masses after interferon treatments. As in the present case, these authors implicated an enhanced Th1 response in these reports. As the uses of and indications for TNF-α inhibitors become more widespread, clinicians should be aware of this potential side effect. Furthermore, the dramatic clinical effects associated with this subtle change in the molecular biologic milieu of the orbit offer tremendous potential for enhanced comprehension of orbital disease; future investigations should explore the molecular homeostatic regulation of normal orbital physiology and the effects of aberrations from it.
REFERENCES 1. Massara A, Cavazzini L, La Corte R, et al. Sarcoidosis appearing during anti-tumor necrosis factor alpha therapy: a new “class effect” paradoxical phenomenon. Two case reports and literature review. Semin Arthritis Rheum 2010;39:313–9. 2. Sahlin S, Lignell B, Williams M, et al. Treatment of idiopathic sclerosing inflammation of the orbit (myositis) with infliximab. Acta Ophthalmol 2009;87:906–8. 3. Durrani OM, Reuser TQ, Murray PI. Infliximab: a novel treatment for sight-threatening thyroid associated ophthalmopathy. Orbit 2005;24:117–9. 4. Verma S, Kroeker KI, Fedorak RN. Adalimumab for orbital myositis in a patient with Crohn’s disease who discontinued infliximab: a case report and review of the literature. BMC Gastroenterol 2013;13:59. 5. Osborne SF, Sims JL, Rosser PM. Short-term use of Infliximab in a case of recalcitrant idiopathic orbital inflammatory disease. Clin Experiment Ophthalmol 2009;37:897–900. 6. Unterstell N, Bressan AL, Serpa LA, et al. Systemic sarcoidosis induced by etanercept: first Brazilian case report. An Bras Dermatol 2013;88(6 suppl 1):197–9. 7. Lamrock E, Brown P. Development of cutaneous sarcoidosis during treatment with tumour necrosis alpha factor antagonists. Australas J Dermatol 2012;53:e87–90. 8. Bhamra K, Stevens R. Pulmonary sarcoidosis following etanercept treatment. Case Rep Rheumatol 2012;2012:724013. 9. Christoforidou A, Goudakos J, Bobos M, et al. Sarcoidosis-like granulomatosis of the hypopharynx as a complication of anti-TNF therapy. Am J Otolaryngol 2013;34:268–72. 10. Kanellopoulou T, Filiotou A, Kranidioti H, et al. Sarcoid-like granulomatosis in patients treated with anti-TNFα factors. A case report and review of the literature. Clin Rheumatol 2011;30:581–3. 11. González-López MA, Blanco R, González-Vela MC, et al. Development of sarcoidosis during etanercept therapy. Arthritis Rheum 2006;55:817–20. 12. Burns AM, Green PJ, Pasternak S. Etanercept-induced cutaneous and pulmonary sarcoid-like granulomas resolving with adalimumab. J Cutan Pathol 2012;39:289–93. 13. Dragnev D, Barr D, Kulshrestha M, et al. Sarcoid panuveitis associated with etanercept treatment, resolving with adalimumab. BMJ Case Rep 2013;2013:pii: bcr2013200552. 14. Hwang CJ, Gausas RE. Sarcoid-like granulomatous orbital in flammation induced by interferon-alpha treatment. Ophthal Plast Reconstr Surg 2008;24:311–3. 15. Morley AM, O’Sullivan E, Thaung C, et al. Sarcoid-related dacryoadenitis following treatment with interferon alpha and ribavarin for hepatitis C. Orbit 2011;30:27–9.
e32
Pseudoadenomatous Hyperplasia of the Inferior Forniceal Conjunctiva Due To Prosthetic Irritation in an Anophthalmic Socket Frederick A. Jakobiec, M.D., D.Sc.*†, Alia Rashid, M.B.Ch.B.*†, Vicky Massoud, M.D.†‡, and Aaron Fay, M.D.†‡ Abstract: Secondary complications in an anophthalmic socket can include late appearing shrinkage due to scarring and squamous cell carcinoma. This article reports a 51-yearold man who 27 years after an enucleation developed an inability to retain his ocular prosthesis due to an acquired multilobular fleshy mass in his inferior fornix. The patient had worn his prosthesis without removal for years at a time. Microscopic evaluation of the excised lesion disclosed a pseudoadenomatous (pseudoglandular) hyperplasia of the conjunctival epithelium with myriad goblet cells and accompanying chronic inflammation. In cross section, these structures microscopically resembled an adenoma but were found to display multifocal origins from the surface epithelium resembling exaggerated pseudoglands of Henle. Simple excision without recurrence 6 months later has permitted a new prosthesis to be comfortably worn with stability.
T
he inferior and superior conjunctival fornices are preferred sites for the development of a small number of disorders, including lymphoid lesions,1 giant fornix syndrome,2 and mucus fishing syndrome.3 Pseudoadenomatous (pseudoglandular) hyperplasia of the fornices is also a distinctive lesion preferentially arising in the fornices.4 This article describes for the first time an example of this condition in an anophthalmic socket. This study was conducted under the auspices of the Massachusetts Eye and Ear Infirmary Institutional Review Board, in compliance with the rules and regulations of the Health Insurance Portability and Accountability Act, and in adherence to the Declaration of Helsinki and all other relevant federal and state laws.
CLINICAL HISTORY A 51-year-old man presented to the Oculoplastics clinic complaining of a growth in his OS socket that had been noticed over several months. He experienced a traumatic globe rupture and subsequent enucleation in 1986 and had been fitted with a scleral prosthesis. At presentation, he was using the same prosthesis fitted in 1986 and admitted to poor hygiene care of his prosthesis and eye socket. There was no pain, discharge, or bleeding, but the growth prevented the prosthesis from fitting securely in the socket, so it often dislocated. On examination, the right visual acuity was 20/20 and the remainder of the examination parameters were within normal limits. The left anophthalmic socket was lined by healthy, glistening conjunctiva, with diffuse mild injection and no evidence of infection or inflammation. A multilobular mass was *David G. Cogan Laboratory of Ophthalmic Pathology; †Harvard Medical School; and ‡Ophthalmic Plastic Surgery Service, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A. Accepted for publication March 26, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Frederick A. Jakobiec, M.D., D.Sc., David G. Cogan Ophthalmic Pathology Laboratory, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Suite 328, Boston, MA 02114. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000208
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Case Reports
FIG. 2. A, Hematoxylin- and eosin-stained paraffin section showing the typical wiry/ropey collagen (arrows). B, Collections of mature adipocytes between the collagen (arrows); (C) bland wavy spindle cells (arrows); (D) occasional mast cell present (arrow); and (E) immunohistochemistry showing strong brown positive staining for CD34.
Orbital Sarcoid-Like Granulomatosis After Inhibition of Tumor Necrosis Factor-α Edward J. Wladis, M.D., F.A.C.S.*, Ashley J. Tarasen, M.D.†, Zachary J. Roth, M.D.‡, Martha G. Farber, M.D.†‡, Jeffrey Ross, M.D.†, and Victoria M. Michaels, M.D.§ Abstract: Pharmacologic inhibition of tumor necrosis factor-alpha (TNF-α) has been used in the management of a variety of inflammatory conditions. Recently, reports on the development of sarcoid-like granulomatous disease *Ophthalmic Plastic Surgery, Department of Ophthalmology, Lions Eye Institute, †Department of Pathology, ‡Department of Ophthalmology, Lions Eye Institute, and §Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, New York, U.S.A. Accepted for publication March 18, 2014. The authors disclose no financial or conflicts of interest. The information presented in this manuscript has not been presented elsewhere and is not under consideration for publication elsewhere. Address correspondence and reprint requests to Edward J. Wladis, M.D., F.A.C.S., 1220 New Scotland Rd, Suite 302, Slingerlands, NY 12159. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000200
e30
at multiple systemic sites after treatment with TNFα inhibitors have emerged, although, to the authors’ knowledge, orbital manifestations of this problem have not been previously described. A 48-year-old woman who received injections of adalimumab for the treatment of psoriatic arthritis developed right-sided orbital pain and inflammation. Orbital biopsy of a focal lesion demonstrated sarcoid-like granulomatosis, and a workup for other causes of this problem was noncontributory. This report represents the first documented case of this phenomenon in the orbit, and possible mechanisms are discussed in this presentation. Given the expanding role of TNF-α inhibitors and the increased frequency of their use, clinicians should be aware of this possible side effect.
I
nhibition of tumor necrosis factor-alpha (TNF-α) has emerged as an important modality in the management of multiple systemic inflammatory conditions.1 Currently, 3 TNF-αantagonists are commercially available, including etanercept, adalimumab, and infliximab, and these agents have been previously used to treat various ophthalmic and orbital conditions, including uveitis, scleritis, myositis, idiopathic orbital inflammation, and thyroid eye disease.2–5 While these medications are generally
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
CASE REPORT
FIG. 1. External photograph of the patient’s modest periorbital edema and erythema.
FIG. 2. Coronal MRI demonstrating a focal inferior orbital mass.
A 48-year-old woman was referred to the Oculoplastic Surgery service at Albany Medical College for the evaluation of a 1-month history of a painful mass along the inferior aspect of her right orbit. This lesion began acutely and was not associated with diplopia or vision loss. Reportedly, the patient had recently initiated a course of oral prednisone (40 mg daily) with modest relief of her discomfort. Her medical history was significant for psoriatic arthritis, for which she was previously treated with a several-week course of adalimumab (40-mg subcutaneous injections every other week), and the patient reported that the orbital lesion developed roughly 1 month after her most recent injection. On initial examination, the patient’s visual acuity was 20/20 with correction in the OD, and her extraocular motility was reduced by 50% with infraduction. There was no evidence of an afferent pupillary defect. Digital palpation of the orbit demonstrated a firm, tender, fixed mass along the central portion of the orbital floor with mild overlying cutaneous edema and erythema (Fig. 1). While the patient had 1.5 mm of ipsilateral proptosis, the remainder of her ophthalmic examination was noncontributory. An orbital MRI scan demonstrated a 1.8 × 2.3 × 1.3 cm focal, relatively homogeneous right-sided lesion that was immediately adjacent to the inferior rectus (Fig. 2). To further evaluate this problem, the patient underwent a transconjunctival anterior orbitotomy; intraoperatively, the mass appeared as a whitish-yellow firm nodule, and several biopsies were taken from the core of the lesion. Histopathologic evaluation of these biopsies demonstrated non-necrotizing granulomatous inflammation without evidence of malignancy or infection (Fig. 3). Staining for acid-fast bacilli was negative, as was assessment with Gomori methenamine silver staining. In addition to microscopic evaluation of the lesion, a thorough evaluation for other explanations for these granulomas was noncontributory; a chest x-ray did not reveal any active disease, and angiotensin-converting enzyme and lysozyme levels were within normal limits. Based on these findings, the patient discontinued adalimumab and began a several-week course of oral prednisone. Oral methotrexate was thus initiated to suppress the inflammation associated with both the patient’s orbital lesion and her psoriatic arthritis.
DISCUSSION
FIG. 3. Photomicrograph (×20 magnification) from the patient’s orbital biopsy, documenting diffuse sarcoid-like granulomas.
efficacious and well-tolerated, several recent case reports have documented the development of sarcoid-like granulomatous processes in the skin,6,7 lungs,1,8 hypopharynx,9 and lymph nodes10 during treatment with TNF-α inhibitors. In this report, the first case of orbital sarcoid-like granulomatosis secondary to therapy with TNF-α antagonists is reported. This investigation was compliant with the Health Insurance Portability and Accountability Act and adhered to the tenets of the Declaration of Helsinki.
Sarcoid-like granulomatosis has been reported at several other anatomical sites during TNF-α inhibition.1–10 Classically, TNF-α has been associated with the progression of sarcoidosis and the formation of granulomas and treatment with TNF-α inhibitors has been successfully used in the management of sarcoidosis,11 making the association between antagonism of this molecule and this phenomenon counterintuitive. Compared with etanercept, adalimumab has been reported to have a more favorable treatment profile in the management of sarcoidosis.12,13 This report represents the first documentation of this process in the orbit; PubMed and Medline searches for “TNF-α,” “sarcoid,” and “orbit” did not reveal any similar descriptions. Several hypotheses have been proposed to explain this side effect. First, partial suppression of TNF-α may induce a selective redistribution of this molecule, thereby inducing it to travel sites with lower internal concentrations that are distal to the anatomical locale of the initial pathology.7 As a result, while the total systemic concentration of TNF-α decreases, a relative increase of TNF-α in the orbit (at the expense of the skin) could ultimately lead to granulomatosis.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e31
Ophthal Plast Reconstr Surg, Vol. 32, No. 2, 2016
Case Reports
Alternatively, TNF-α selectively suppresses autoreactive T cells, and inhibition of this suppressive effect may ultimately lead to enhanced T-cell reactivity, changes in the molecular milieu, and the development of granulomas.7 Previous investigations have indicated increases in interferon-γ–producing T cells during treatment with TNF-α inhibitors, and interferon-γ has been implicated in granuloma formation.1 These subtle immunologic aberrancies may ultimately explain this side effect. Certainly, the induction of sarcoid-like orbital lesions after chemotherapy has been documented with other agents. Hwang and Gausas14 and Morley and et al.15 reported cases of sarcoid-related masses after interferon treatments. As in the present case, these authors implicated an enhanced Th1 response in these reports. As the uses of and indications for TNF-α inhibitors become more widespread, clinicians should be aware of this potential side effect. Furthermore, the dramatic clinical effects associated with this subtle change in the molecular biologic milieu of the orbit offer tremendous potential for enhanced comprehension of orbital disease; future investigations should explore the molecular homeostatic regulation of normal orbital physiology and the effects of aberrations from it.
REFERENCES 1. Massara A, Cavazzini L, La Corte R, et al. Sarcoidosis appearing during anti-tumor necrosis factor alpha therapy: a new “class effect” paradoxical phenomenon. Two case reports and literature review. Semin Arthritis Rheum 2010;39:313–9. 2. Sahlin S, Lignell B, Williams M, et al. Treatment of idiopathic sclerosing inflammation of the orbit (myositis) with infliximab. Acta Ophthalmol 2009;87:906–8. 3. Durrani OM, Reuser TQ, Murray PI. Infliximab: a novel treatment for sight-threatening thyroid associated ophthalmopathy. Orbit 2005;24:117–9. 4. Verma S, Kroeker KI, Fedorak RN. Adalimumab for orbital myositis in a patient with Crohn’s disease who discontinued infliximab: a case report and review of the literature. BMC Gastroenterol 2013;13:59. 5. Osborne SF, Sims JL, Rosser PM. Short-term use of Infliximab in a case of recalcitrant idiopathic orbital inflammatory disease. Clin Experiment Ophthalmol 2009;37:897–900. 6. Unterstell N, Bressan AL, Serpa LA, et al. Systemic sarcoidosis induced by etanercept: first Brazilian case report. An Bras Dermatol 2013;88(6 suppl 1):197–9. 7. Lamrock E, Brown P. Development of cutaneous sarcoidosis during treatment with tumour necrosis alpha factor antagonists. Australas J Dermatol 2012;53:e87–90. 8. Bhamra K, Stevens R. Pulmonary sarcoidosis following etanercept treatment. Case Rep Rheumatol 2012;2012:724013. 9. Christoforidou A, Goudakos J, Bobos M, et al. Sarcoidosis-like granulomatosis of the hypopharynx as a complication of anti-TNF therapy. Am J Otolaryngol 2013;34:268–72. 10. Kanellopoulou T, Filiotou A, Kranidioti H, et al. Sarcoid-like granulomatosis in patients treated with anti-TNFα factors. A case report and review of the literature. Clin Rheumatol 2011;30:581–3. 11. González-López MA, Blanco R, González-Vela MC, et al. Development of sarcoidosis during etanercept therapy. Arthritis Rheum 2006;55:817–20. 12. Burns AM, Green PJ, Pasternak S. Etanercept-induced cutaneous and pulmonary sarcoid-like granulomas resolving with adalimumab. J Cutan Pathol 2012;39:289–93. 13. Dragnev D, Barr D, Kulshrestha M, et al. Sarcoid panuveitis associated with etanercept treatment, resolving with adalimumab. BMJ Case Rep 2013;2013:pii: bcr2013200552. 14. Hwang CJ, Gausas RE. Sarcoid-like granulomatous orbital in flammation induced by interferon-alpha treatment. Ophthal Plast Reconstr Surg 2008;24:311–3. 15. Morley AM, O’Sullivan E, Thaung C, et al. Sarcoid-related dacryoadenitis following treatment with interferon alpha and ribavarin for hepatitis C. Orbit 2011;30:27–9.
e32
Pseudoadenomatous Hyperplasia of the Inferior Forniceal Conjunctiva Due To Prosthetic Irritation in an Anophthalmic Socket Frederick A. Jakobiec, M.D., D.Sc.*†, Alia Rashid, M.B.Ch.B.*†, Vicky Massoud, M.D.†‡, and Aaron Fay, M.D.†‡ Abstract: Secondary complications in an anophthalmic socket can include late appearing shrinkage due to scarring and squamous cell carcinoma. This article reports a 51-yearold man who 27 years after an enucleation developed an inability to retain his ocular prosthesis due to an acquired multilobular fleshy mass in his inferior fornix. The patient had worn his prosthesis without removal for years at a time. Microscopic evaluation of the excised lesion disclosed a pseudoadenomatous (pseudoglandular) hyperplasia of the conjunctival epithelium with myriad goblet cells and accompanying chronic inflammation. In cross section, these structures microscopically resembled an adenoma but were found to display multifocal origins from the surface epithelium resembling exaggerated pseudoglands of Henle. Simple excision without recurrence 6 months later has permitted a new prosthesis to be comfortably worn with stability.
T
he inferior and superior conjunctival fornices are preferred sites for the development of a small number of disorders, including lymphoid lesions,1 giant fornix syndrome,2 and mucus fishing syndrome.3 Pseudoadenomatous (pseudoglandular) hyperplasia of the fornices is also a distinctive lesion preferentially arising in the fornices.4 This article describes for the first time an example of this condition in an anophthalmic socket. This study was conducted under the auspices of the Massachusetts Eye and Ear Infirmary Institutional Review Board, in compliance with the rules and regulations of the Health Insurance Portability and Accountability Act, and in adherence to the Declaration of Helsinki and all other relevant federal and state laws.
CLINICAL HISTORY A 51-year-old man presented to the Oculoplastics clinic complaining of a growth in his OS socket that had been noticed over several months. He experienced a traumatic globe rupture and subsequent enucleation in 1986 and had been fitted with a scleral prosthesis. At presentation, he was using the same prosthesis fitted in 1986 and admitted to poor hygiene care of his prosthesis and eye socket. There was no pain, discharge, or bleeding, but the growth prevented the prosthesis from fitting securely in the socket, so it often dislocated. On examination, the right visual acuity was 20/20 and the remainder of the examination parameters were within normal limits. The left anophthalmic socket was lined by healthy, glistening conjunctiva, with diffuse mild injection and no evidence of infection or inflammation. A multilobular mass was *David G. Cogan Laboratory of Ophthalmic Pathology; †Harvard Medical School; and ‡Ophthalmic Plastic Surgery Service, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A. Accepted for publication March 26, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Frederick A. Jakobiec, M.D., D.Sc., David G. Cogan Ophthalmic Pathology Laboratory, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Suite 328, Boston, MA 02114. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000208
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
