Two unusual osteogenic orbital tumors: presumed parosteal osteosarcomas of the orbit

OBJECTIVE: To report two cases of suspected parosteal osteosarcoma of the orbit, with dedifferentiation into a high-grade liposarcoma occurring in one patient. DESIGN: Two retrospective case reports. METHODS: The clinical, radiologic, and pathologic records of two patients with suspected orbital parosteal osteosarcoma were retrospectively reviewed. MAIN OUTCOME MEASURES: Histologic evaluation and clinical follow-up were measured. RESULTS: The first patient was a 47-year-old male presenting with a 5-month history of painless right lower lid swelling; excision biopsy suggested a well-differentiated parosteal osteosarcoma of the orbital floor, which recurred 3 years later. Six months after excision of the recurrence, the mass demonstrated accelerated growth, and a lid-sparing exenteration was performed; histologic examination showed a high-grade liposarcoma. The patient remains disease-free at 4 years. The second patient, a 40-year-old male, presented with an 8-year history of proptosis and a right superotemporal orbital mass. The mass was excised completely at lateral orbitotomy; histologic examination suggested a well-differentiated parosteal osteosarcoma. The patient remains well 9 months postoperatively. CONCLUSIONS: Parosteal osteosarcoma is an uncommon tumor, usually affecting long bones, that is extremely rare in the orbit. It is a low-grade sarcoma that tends to recur locally after excision but has a favorable prognosis. Dedifferentiation into a high-grade sarcoma occasionally occurs in parosteal osteosarcoma, but transformation into liposarcoma does not seem to have been previously reported. It is important to recognize dedifferentiated parosteal osteosarcoma, because the prognosis is poor, and radical treatment may be required.     

Two cases of retinal detachment following laser in Situ keratomileusis repaired by scleral buckling surgery

BACKGROUND: As laser in situ keratomileusis (LASIK) becomes the treatment of choice in the field of refractive surgery, postoperative rhegmatogenous retinal detachment has also begun to occur. However, since it has not been reported yet here in Japan, we report two cases of retinal detachment following LASIK with suggestions based on our experience. CASES: A 34-year-old male(Case 1) and a 26-year-old male(Case 2) suffered from retinal detachment one month and eight months after LASIK, respectively. Contributing retinal breaks were a horseshoe tear in Case 1 and atrophic holes in the lattice degeneration in Case 2. Each patient underwent partial scleral buckling with no intraoperative complication, resulting in successful retinal reattachment. Postoperatively, myopic changes developed and persisted one month in Case 1 and several months in Case 2. CONCLUSIONS: Great attention should be paid to the possibility of postoperative myopia after a repair of retinal detachment following LASIK. Thus, we stress the necessity of close cooperation between LASIK surgeons and vitreoretinal surgeons to settle the matter.     

CHARGE association, hyper-immunoglobulin M syndrome, and conjunctival MALT lymphoma

PURPOSE: To report a case of a 5-year-old child with CHARGE association and bilateral conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma treated with topical interferon-alpha. METHODS: Case report. RESULTS: A 5-year-old girl, diagnosed with nonclassical CHARGE association and hyper-immunoglobulin M (IgM) syndrome, was referred with a 2-month history of suspected purulent bilateral streptococcal conjunctivitis. Clinical symptoms did not resolve despite multiple antibiotic treatments, and her clinical course was attributed to underlying immunodeficiency. Biomicroscopy showed salmon-colored, nodular lesions occupying both fornices and caruncles. Inferior conjunctival biopsy was performed, and MALT lymphoma was diagnosed. Bilateral treatment was initiated with topical interferon-alpha, applied 3 times a day at a concentration of 5 x 10 U/m/d, for 4 months. Complete regression of symptoms and conjunctival lesions was achieved. No recurrences have been observed after 1-year follow-up. CONCLUSIONS: We report the use of topical interferon-alpha as treatment for bilateral conjunctival MALT lymphoma in a young child with CHARGE association and hyper-IgM syndrome.     

眼睑皮肤松弛综合征临床研究进展

眼睑皮肤松弛综合征是一种较少见的眼睑疾病,表现为青少年时期特发性局限性眼睑皮肤反复间断性无痛性血管神经性水肿,反复水肿破坏眼睑组织结构导致眼睑皮肤松弛变薄,毛细血管增多迂曲扩张以及后期上睑下垂、眶脂肪脱垂、泪腺脱垂等多种并发症。眼睑皮肤松弛综合征的发病机制尚不明确,可能存在遗传等因素的参与;组织病理学研究发现IgA沉积和基质金属蛋白酶活性表达增强,提示免疫反应参与弹性纤维的降解,后者进一步引起局部淋巴系统结构功能的改变;这些因素共同参与了本病的发生发展。眼睑皮肤松弛综合征的治疗分为急性水肿期和稳定期的治疗。     

A case of orbital dirofilariasis in Northern Turkey

A 29-year-old male patient presented with complaints of a localized mass behind his right lower eyelid that had been present for the last 6 months. Ophthalmologic examination was completely normal except for a 2 cm x 2 cm painless mass localized on the inferior orbital rim behind the right lower eyelid. An excisional biopsy was planned, and an anterior orbitotomy was performed by a lateral canthotomy and inferior cantholysis. Premature rupture of the cyst occurred during dissection, disclosing a thin white parasite surrounded by copious purulent material. The parasite and surrounding cyst were submitted to laboratory for evaluation. The parasite was typed as Dirofilaria repens (13 cm, female). The patient remained symptom-free over the 4 months of postoperative follow-up. Detection of the first human case of orbital dirofilariasis in Northern Turkey, in a patient with no history of travel to any endemic areas, has raised questions about the parasite's zoonotic characteristics.     

Spontaneous regression of orbital Langerhans cell granulomatosis in a three-year-old girl.

PURPOSE: To report a case of spontaneous regression of orbital Langerhans cell granulomatosis. METHOD: Case report. A 3-year-old girl was initially examined with a 5-week history of slowly progressive blepharoptosis and periorbital swelling of the left eye. RESULTS: Computed tomographic scan showed a mass in the left orbit eroding into the left frontal bone; fine-needle aspiration confirmed diagnosis of Langerhans cell granulomatosis. After initial biopsy, the patient was treated by close observation alone. Six months after initial examination, the monostotic lesion had completely resolved. CONCLUSIONS: In some cases of monostotic Langerhans cell granulomatosis, initial biopsy followed by observation alone may allow for the spontaneous regression of the lesion. This conservative approach to treatment is an important therapeutic option that may spare the patient the adverse effects of surgical resection, radiation, or chemotherapy.     

Bilateral multifocal choroidal carcinoid: long-term follow-up without treatment

Purpose: To report a minimally symptomatic patient with bilateral carcinoid tumors metastatic to the choroid who was followed up for 42 months without any treatment.Method: Case report.Results: A 54-year-old white man with a history of bronchial carcinoid tumor had bilateral multiple carcinoid tumors metastatic to the choroid. Follow-up for 42 months without treatment demonstrated no growth of the choroidal tumors, and corrected visual acuity remained RE, 20/20 and LE, 20/25. Systemic status also remained stable.Conclusions: Carcinoid tumors metastatic to the choroid may remain stationary for an extended period. Treatment may be deferred until tumor growth, exudative retinal detachment, or substantial impairment of vision develops.     

Retinopathy associated with pegylated interferon and ribavirin treatment for chronic hepatitis C

Background

Retinopathy is associated with the use of interferon and ribavirin for the treatment of chronic hepatitis C. Common ocular complications include cotton wool spots, retinal hemorrhages, and macular edema.

Case Report

A 53-year-old black man with a history of drug and alcohol abuse was admitted to the Department of Veterans Affairs clinic for substance abuse treatment. Results of a liver biopsy showed chronic hepatitis C, and treatment with interferon and ribavirin was initiated. A comprehensive eye examination was performed just before the initiation of treatment, and the patient was then periodically monitored for the presence of retinopathy. No retinopathy was found at the initial visit or at his 1-month or 2-month follow-up examinations. His 4-month retinal evaluation found cotton wool spots in both eyes and retinal hemorrhages in the left eye. The retinopathy persisted for several months but resolved in both eyes before his treatment was discontinued. Throughout his treatment period there were no visual complaints or visual acuity changes.

Conclusions

Although this patient was visually asymptomatic, and his retinopathy resolved during treatment, we chose to monitor the patient monthly during treatment because of the risk of serious (although rare) ocular complications.     

Oral cimetidine (Tagamet) for recalcitrant, diffuse conjunctival papillomatosis.

PURPOSE: To report the efficacy of cimetidine for treatment of conjunctival papillomatosis. METHODS: An 11-year-old boy had an 8-year history of diffuse conjunctival papillomas, treated previously with standard measures of excisional biopsy and cryotherapy. He developed spontaneous conjunctival bleeding and diffuse tumor recurrence over the entire conjunctival surface. Oral cimetidine liquid (30 mg/kg/day) was prescribed. RESULTS: Within 2 months, dramatic tumor regression was noted, with nearly complete resolution by 4 months. The patient had no local or systemic side effects from the medication. CONCLUSIONS: Oral cimetidine possesses immune modulation properties and may be useful in some cases of virus-induced conjunctival papillomatosis.     

Sebaceous cell carcinoma of the eyelid and the human immunodeficiency virus

PURPOSE: Patients infected with the human immunodeficiency virus (HIV) are at increased risk for developing malignancies, which are acquired at a younger age and are more aggressive. Sebaceous cell carcinoma is a rare eyelid tumor typically occurring in the seventh decade of life. We report two cases of sebaceous cell carcinoma in young HIV-infected patients. METHODS: Case series and review of the literature. We describe two HIV-infected patients with sebaceous cell carcinoma of the eyelid and caruncle. The first patient was a 36-year-old woman with a 9-month history of an enlarging right lower eyelid mass. The second patient was a 34-year-old man with a 6-month history of an enlarging right caruncular mass. RESULTS: Biopsy showed both masses to be sebaceous cell carcinoma. The first patient underwent Mohs' micrographic excision of the lesion followed by reconstruction of the full-thickness eyelid defect with a combination of tarsoconjunctival and myocutaneous advancement flaps. The second patient underwent exenteration because of orbital involvement. CONCLUSION: Sebaceous cell carcinoma should be considered for any suspicious eyelid lesion in young HIV-infected patients.     

A case of infected scleral buckle with Mycobacterium chelonae associated with chronic intraocular inflammation

PURPOSE: To describe a unique case of chronic intraocular inflammation secondary to scleral buckle infection with Mycobacterium chelonae that was successfully treated with buckle explantation. METHODS: Case report. RESULTS: A 59-year-old male with a history of retinal detachment repair at the age of 41 presented with chronic, recurrent intraocular inflammation responsive to topical corticosteroids. Conjunctival erosion with exposure of the scleral buckle occurred five months after initial presentation. The scleral buckle was removed and cultured. After three weeks of postoperative topical tobramycin and dexamethasone treatment, the patient has remained symptom-free without medications. The explanted material grew acid-fast bacilli later identified as M. chelonae. CONCLUSIONS: This case describes a new finding of chronic intraocular inflammation associated with a scleral buckle infected with M. chelonae and the successful resolution of extraocular infection and intraocular inflammation after buckle removal.     

Late corneal perforation after photorefractive keratectomy associated with topical diclofenac: involvement of matrix metalloproteinases

OBJECTIVE: To report a case of a 50-year-old man who was initially seen with a corneal perforation in his right eye 2 months after a photorefractive keratectomy (PRK) procedure and to discuss the roles of topical diclofenac and matrix metalloproteinases (MMPs). DESIGN: Case report with tissue analysis. MAIN OUTCOME MEASURES: Ocular examination, diagnostic workup, surgical treatment, and histologic, immunofluorescent, zymography, and real time-polymerase chain reaction studies on corneal button. RESULTS: Slit-lamp examination of the right eye revealed a 4-mm diameter area of central corneal thinning with a 2-mm diameter perforation at its center. Predisposing factors included prolonged postoperative topical diclofenac therapy for more than 2 months and a 10-year history of well-controlled diabetes mellitus. An extensive diagnostic workup ruled out a systemic autoimmune disease. A penetrating keratoplasty was performed. Results of immunohistochemical studies of the corneal button showed stromal accumulation of temporary type III and IV collagens, MMP-3, and MMP-9 in the anterior wounded stroma and MMP-9 in the basal corneal epithelial cells of the leading edge. Differential activity and expression of MMP-2 and MMP-9 were found between the central and peripheral corneal buttons. CONCLUSIONS: Prolonged use of diclofenac and diabetes mellitus might be responsible for the corneal perforation after PRK in our patient. MMP-9 and MMP-3 might be involved in delayed wound closure and corneal melting.     

Isolated squamous cell carcinoma of the orbital apex

A 43-year-old woman (Case 1), 63-year-old man (Case 2), and a 67-year-old man (Case 3) presented with diplopia (Cases 1, 2, and 3) and upper-eyelid ptosis (Cases 1 and 3). The cases had preceding cranial nerve V1 disturbances ranging from 3 months to 8 years. Each demonstrated complete internal ophthalmoplegia and external ophthalmoplegia. No case had a cutaneous squamous cell carcinoma (SCC) on presentation or by history. Imaging revealed isolated orbital apex masses. Tumor biopsies revealed SCCs of various differentiations. Systemic workup revealed no extraorbital malignancy. All received radiation therapy, and 2 patients underwent adjuvant chemotherapy. One patient is alive since diagnosis (49 months). In Case 2, SCC developed in the contralateral orbit, and the patient died 19 months after diagnosis; and Case 3 died 12 months after diagnosis. These isolated cases of orbital SCC may have arisen from orbital choristomatous squamous epithelium, may represent de novo or metastatic tumors, or may be manifestations of occult perineural spread.     

Treatment of ligneous conjunctivitis with amniotic membrane transplantation and topical cyclosporine

Ligneous conjunctivitis (LC) is a rare form of bilateral chronic recurrent disease in which thick membranes form on the palpebral conjunctiva and other mucosal sites. We report the clinical features and describe the management of two cases. Case 1 was an 8-month-old patient with bilateral membranous conjunctivitis. Case 2 was a 5-year-old patient with unilateral membranous conjunctivitis, esotropia, mechanical ptosis and complicated cataract, and had been treated with a number of medications. Histological investigation of the membrane in both cases showed LC. Treatments with amniotic membrane transplantation and institution of topical cyclosporine have shown good response. There has been complete resolution of the membranes with no recurrence at the end of 40- and 28-month follow-ups, respectively. No treatment related side effects were seen. Thus, it appears that amniotic membrane transplantation and topical cyclosporine are effective alternatives for the treatment of LC.     

Bilateral breast metastases from choroidal melanoma

PURPOSE: To report an unusual case of solitary sequential bilateral breast metastases from choroidal melanoma. METHOD: Case report. RESULTS: A 48-year-old woman with a large choroidal melanoma in the left eye was treated with Iodine-125 brachytherapy and responded satisfactorily with decrease in tumor thickness. Thirty-seven months after treatment, she developed a solitary, circumscribed melanoma metastasis to the right breast, and 54 months after treatment, a similar metastasis was detected in her left breast. Both breast tumors were managed with lumpectomy. Systemic examination including magnetic resonance imaging of abdomen, chest, and head have been performed regularly and have been normal. At 61 months after treatment, the patient has no clinical evidence of metastatic disease elsewhere. CONCLUSIONS: Uveal melanoma rarely metastasizes to breast tissue. A breast nodule in a patient with a history of uveal melanoma is most likely a primary breast tumor but may rarely represent a metastasis from uveal melanoma.     

Periorbital Tuberculosis in a Young Man: A Case Report

Background: Tuberculosis is an endemic disease in developing countries which may involve various organs. Soft tissue involvement including periorbital tissues is very rare, and often this involvement is accompanied by the involvement of other body organs such as lungs. Most of the patients suffering from periorbital infections have underlying diseases and diagnosis of these infections without concomitant involvement of other parts of body is often complex. Case presentation: In this paper, a periorbital tuberculous infection is described. A 17-year-old male with chronic periorbital mass underwent surgical and medical treatment and the mass recurred. Culture and microscopic studies of the specimen showed mycobacterium tuberculosis. The patient was treated for a periorbital tuberculous infection for a 6-month period with antituberculous regimen and was cured. Conclusion: Periorbital tuberculous infection may occur in the absence of underlying factors or active tuberculosis in the rest of the body organs or a history of tuberculosis infection.     

Charles Bonnet syndrome: Case presentation and literature review

BackgroundCharles Bonnet syndrome (CBS) is an under-recognized and under-reported disorder that involves visual hallucinations in visually impaired individuals. These patients have intact cognition, do not have hallucinations in any other sensory modalities, and retain insight into the unreal nature of their hallucinations.Case ReportsTwo case reports are presented. Case 1 is a 79-year-old man who presented with a 6-month history of hallucinations. Case 2 is an 86-year-old man who presented with a 2-month history of hallucinations. Both patients are legally blind from age-related macular degeneration. In both cases, the ocular examinations were otherwise unremarkable. A workup for other pathological causes of visual hallucinations was negative for both patients. They were given the diagnoses of CBS and were counseled. No other intervention was indicated at that time.ConclusionOptometrists should be aware of the potential for visual hallucinations in patients with visual impairment. Early recognition by clinicians of the symptoms of CBS, and sharing proper information, treatment, and advice, can alleviate distress or suffering experienced by patients with CBS.     

Labial adenocarcinoma after treatment with cyclosporin a in a patient with panuveitis

PURPOSE: To report a case of labial basal cell adenocarcinoma in a patient with uveitis on treatment with cyclosporin A. METHOD: Case report. A 73-year-old woman with panuveitis and retinal vasculitis presented with a lump on her lip after 52 months of treatment with cyclosporin A. RESULT: Excision biopsy showed a labial adenocarcinoma. CONCLUSION: Malignancy can occur after long-term cyclosporin A treatment for uveitis.     

Reopening filtration fistulas with transconjunctival argon laser photocoagulation

Three patients (a 15-year-old girl, a 45-year-old woman, and a 61-year-old woman) with previously successful full-thickness filtering procedures developed pigmented tissue in the fistula sites and uncontrolled intraocular pressures despite maximum tolerable medical therapy. The filtering procedures in the first and second patients spontaneously failed two and four years after surgery. The filtration bleb of the third patient, after successfully controlling intraocular pressure for six years, failed two months after cataract extraction. Argon laser therapy was applied through the conjunctiva to treat visible subconjunctival pigmented tissue within the sites of the previous surgical filtration blebs. In all three cases, there was an immediate and significant decrease in intraocular pressure associated with reestablishment of the filtration bleb (from 44 to 16 mm Hg in Case 1, from 40 to 15 mm Hg in Case 2, and from 25 to 12 mm Hg in Case 3). Long-term follow-up showed well controlled intraocular pressures without glaucoma medications (Case 1, 5 mm Hg after 18 months; Case 2, 17 mm Hg after one year; and Case 3, 9 mm Hg after five months).     

Primary orbital angiosarcoma: a case report

PURPOSE: The pathogenesis, natural history, histopathology, and recommended treatment for orbital angiosarcoma are illustrated and reviewed. METHODS: Case report. RESULTS: A 71-year-old white male presented with bluish discoloration and swelling of the left medial canthal area. A fine needle aspiration and excisional biopsy with histopathologic examination was performed, which showed angiosarcoma. Pattern of growth was demonstrated radiographically and histopathologically, confirming primary orbital angiosarcoma. Subsequent wide surgical resection was carried out, with substantial reconstruction of the left orbital and periorbital area. The patient responded well to the surgery, and was free of tumor after six years of follow-up. CONCLUSION: Angiosarcoma is a rare and highly malignant tumor of epithelial origin. The aggressive nature of this tumor usually results in a high mortality rate despite treatment. However, early diagnosis and wide surgical excision has resulted in successful treatment of these tumors.