Post intrastromal corneal ring segments insertion complicated by Candida parapsilosis keratitis.

This case report describes the clinical and histopathologic features, including molecular confirmation, of fungal keratitis after intrastromal corneal ring segments placement for keratoconus. A 52-year-old woman underwent insertion of Intacs(®) corneal implants for treatment of keratoconus. Extrusion of the implants was noted 5 months post insertion and replaced. Three months later, monocular infiltrates and an epithelial defect were observed. The Intacs were removed and the infiltrates were treated with ofloxacin and prednisolone acetate. Microbial cultures and stains were negative. The patient demonstrated flares and exacerbation one month later. Mycoplasma and/or fungus were suspected and treated without improvement. Therapeutic keratoplasty was performed 10 months following initial placement of the corneal ring implants. The keratectomy specimen was analyzed by light microscopy and a panfungal polymerase chain reaction assay. A histopathologic diagnosis of Candida parapsilosis keratitis was made and confirmed by polymerase chain reaction. One year postoperatively, a systemic workup of the patient was done with no signs of recurrence. This rare report of fungal keratitis following Intacs insertion is the first reported case of C. parapsilosis complicating Intacs implantation.

Molecular detection of Bartonella henselae for the diagnosis of cat scratch disease and bacillary angiomatosis of the conjunctiva.

PURPOSE: The purpose of this study was to evaluate clinical cases of cat scratch disease (CSD) and bacillary angiomatosis involving the conjunctiva by special stains and transmission electron microscopy (TEM) and to compare these findings with the results from species-specific polymerase chain reaction (PCR) analysis of the same specimens. METHODS: Six potential cases of CSD and 2 possible cases of bacillary angiomatosis of the conjunctiva were analyzed by light microscopy, the Warthin-Starry technique, TEM, and PCR. DNA isolated from cultured Bartonella henselae, B. bacilliformis, B. quintana, and B. elizabethae were used as control templates for establishment of the PCR sensitivity and specificity. Cultured DNA was also used as appropriate positive controls during analysis of the clinical specimens. RESULTS: The histological studies, electron microscopy, and the PCR analysis confirmed the identification of the bacilli within the involved tissues. Furthermore, molecular diagnosis by PCR allowed for speciation of the infecting Bartonella organisms in 6 of the 8 cases and correlated with the histological findings. CONCLUSIONS: The PCR-based identification of Bartonella correlated well with the results of light microscopy and TEM and provided a simple and rapid method of diagnosis to the species level. The molecular analysis may prove to be beneficial in enhancing the current diagnostic techniques for CSD and bacillary angiomatosis.

Cat-scratch uveitis confirmed by histological, serological, and molecular diagnoses.

PURPOSE: To report a case of a cat-scratch uveitis caused by Bartonella henselae, which was confirmed by histology, serology, and polymerase chain reaction (PCR) methodology. METHODS: An iris nodule was biopsied from a 4-year-old child who was scratched by a kitten on the side of his face and developed redness of the eye associated with cervical lymphadenopathy. Sections of the iridectomy specimen were stained with hematoxylin-eosin, periodic acid-Schiff, and Warthin-Starry technique for histopathologic evaluation. Additionally, serologic tests and molecular diagnosis using B. henselae-specific PCR were performed. RESULTS: Histopathologically, sections of the iridectomy specimen showed a zonal granulomatous inflammation with a central iris necrotic abscess surrounded by a mantle of epithelioid histiocytes and more peripherally by lymphocytes and plasma cells. The Warthin-Starry stain disclosed scattered short bacilli within the necrotic abscess morphologically compatible with B. henselae. Report of serologic tests for B. henselae disclosed a negative immunoglobulin G antibody (negative: less than 12) and a positive immunoglobulin M antibody of 18 (positive: greater than 15). Other serologic studies including Toxocara, histoplasmin, blastomycin, coccidioidin, aspergillin, and Chlamydia were all negative. PCR was positive for B. henselae DNA. CONCLUSIONS: Our case showed a unilateral chronic granulomatous iritis with the histopathologic features compatible with CSD caused by B. henselae bacillus as demonstrated in the iris biopsy and confirmed by serology and PCR technique. This case is an example of a relatively rare uveal manifestation of CSD.

Ultrastructural features of posterior crocodile shagreen of the cornea.

Three elderly patients with a clinical diagnosis of posterior crocodile shagreen of the cornea underwent penetrating keratoplasty. Each of the patients had the characteristic symmetrical, polygonal opacities with indistinct edges and intervening clear spaces in the central posterior corneal stroma. Electron microscopic examination of the keratoplasty specimens disclosed the presence of vacuoles throughout the corneal stroma, many of which contained electron-dense material. The vacuoles were observed with increasing density posteriorly and were most concentrated adjacent to the anterior banded portion of Descemet's membrane. A sawtooth-like configuration of the stromal collagenous lamellae was noted in the posterior cornea in one case. We reviewed the three cases of central corneal clouding reported with histopathological findings as well as the clinical descriptions of this entity. Although a sawtooth-like lamellar configuration of the stromal collagen may be seen by electron microscopy, characteristic vacuoles appear to be the only consistent finding. We believe that posterior crocodile shagreen is most likely a degenerative disorder and that it should be distinguished from central cloudy dystrophy of François by its non-familial pattern of occurrence.

Mesenchymal chondrosarcoma of the orbit: a unique radiologic-pathologic correlation.

PURPOSE: To report a unique radiologic-histopathologic correlation of mesenchymal chondrosarcoma of the orbit in a 24-year-old Asian woman. METHODS: Clinicopathologic case review. RESULTS: The patient was examined for left-sided proptosis of several months' duration. CT showed a left orbital mass depicting a central radiolucent, nonenhancing component, and a denser peripheral enhancing portion. Histopathologic examination of the orbital mass showed a biphasic pattern of a mesenchymal chondrosarcoma exhibiting features of a high-grade sarcoma with hemangiopericytoma pattern that corresponds to the radiopaque portion of the mass and areas of chondrosarcoma that correlated with the radiolucent component of the tumor. The patient underwent exenteration of the left orbit followed by radiotherapy and chemotherapy. The last follow-up (8 years, 1 month) disclosed no evidence of recurrence or metastatic disease. CONCLUSIONS: In this case, a unique CT-histopathologic correlation of the mass was established. The authors believe that recognizing the different radiologic features of the orbital tumor can help clinicians in establishing the correct preoperative diagnosis of this potentially lethal neoplasm. To the best of the authors' knowledge, this diagnostic correlation has not been previously reported.

Primary adenoid cystic carcinoma of the conjunctiva arising from the accessory lacrimal glands: a clinicopathologic study of three cases.

PURPOSE: To report 3 cases of primary adenoid cystic carcinoma originating from the accessory and/or ectopic lacrimal glands of the conjunctiva. METHODS: We examined 3 conjunctival tumors histopathologically. The specimens were fixed in 10% formalin. Sections were cut at 5 microm, and the slides were stained with hematoxylin-eosin, periodic acid-Schiff, alcian blue, and colloidal iron methods. RESULTS: We report 3 conjunctival tumors that histopathologically proved to be adenoid cystic carcinomas that had arisen from the accessory lacrimal glands of the conjunctiva (cases 1 and 2) and from ectopic lacrimal gland tissue (case 3). The age of the patients ranged between 53 and 68 years. In 2 of the cases, the tumor involved the tarsal conjunctiva. The third patient had a mass involving the limbal conjunctiva and two thirds of the cornea inferiorly. Histopathologically, acini of accessory lacrimal glands of the conjunctiva were found near the neoplastic lobules in 2 of the tumors. Foci of perineural invasion were observed in 1 of the tumors (case 1). Follow-up examination showed no evidence of recurrence or metastatic disease in cases 1 and 2 (10 and 8 years, respectively). In case 3, the patient was alive and without any evidence of recurrence 1 year after surgical excision of the mass. CONCLUSIONS: Adenoid cystic carcinoma arising from the accessory lacrimal glands of the conjunctiva is a rare occurrence. Only 2 previously reported cases have appeared in the literature, and to our knowledge, there are no reports of this tumor arising from ectopic lacrimal gland tissue in the conjunctiva. Follow-up studies are mandatory to evaluate the biologic behavior of the tumor.

Cutaneous malignant melanoma metastatic to the vitreous.

We describe a patient with dysplastic nevus syndrome and cutaneous melanoma in whom vitreous involvement was the first sign of metastatic disease. Cytologic examination of vitreous aspirate can usually confirm the diagnosis. Enucleation may be a treatment option if the vitreous is the sole metastatic site.

Histological parameters helpful in recognising steroid-treated temporal arteritis: an analysis of 35 cases.

AIM: To establish the histological and immunohistochemical parameters that are helpful in recognising temporal arteritis in patients who have been treated with steroids before biopsy, and to analyse the clinical features and correlate them with the histological findings. METHODS: A retrospective review of charts of 35 patients treated with steroids before obtaining temporal artery biopsy specimens, spanning a 11-year period from 1995 to 2005. The study was conducted at the Ophthalmic Pathology Laboratory, Cullen Eye Institute, Houston, Texas, USA. The clinical features were evaluated and correlated with the histopathological findings. Each case was evaluated with respect to age, sex, race, clinical findings, erythrocyte sedimentation rate, corticosteroid dosage (oral versus intravenous) and the duration of treatment. The time interval between obtaining the biopsy specimen and the onset of steroid treatment was carefully recorded for each patient. In selected cases, histiocytic markers (CD-68 and HAM-56) were used to identify the presence of epithelioid histiocytes, which characterises a granulomatous inflammation. Other immunohistochemical studies (CD3, CD20, CD4, CD8, CD45RO, CD45RA and S-100 protein) were performed in selected cases to characterise the inflammatory cells. RESULTS: The three most reliable histopathological parameters of corticosteroid-treated temporal arteritis are the following: (1) complete or incomplete mantle of lymphocytes and epithelioid histiocytes located between the outer muscular layer and the adventitia; (2) large circumferential defects in the elastic lamina (best seen with the Movat's pentachrome); and (3) absent or few small multinucleated giant cells. In some cases the main artery appears normal, whereas the primary branches show evidence of a healing arteritis. The histological findings vary according to the duration of treatment before obtaining the biopsy specimen. CONCLUSION: Striking histological differences can be recognised objectively between patients with active (untreated) giant cell arteritis and patients who have been treated with corticosteroids. The earliest histopathological changes were detected by the end of the first week after steroid treatment (usually after day 4 to the end of the first week). The histological findings were more difficult to recognise at 2-3 months after steroid treatment. Ophthalmic and general pathologists should be able to recognise this entity on the basis of the histological findings including the special stains and results of immunohistochemical studies (CD-68 and HAM-56).

IgG-kappa immunoglobulin deposits involving the predescemetic region in a patient with multiple myeloma.

PURPOSE: To report the clinico-pathologic features of corneal deposits in a patient with multiple myeloma with surgical intervention and follow-up. DESIGN: Interventional case report. METHODS: We reviewed the patient's chart and the relevant literature on immunoglobulin corneal deposits and its prognosis. RESULTS: A 52-year-old man with a history of multiple myeloma underwent penetrating keratoplasty sequentially for decreased vision in both eyes secondary to abnormal corneal deposits. Pathologic examination of the keratectomy specimens, including immunohistochemistry and transmission electron microscopy, revealed IgG-kappa immunoglobulin deposits in the predescemetic region in both corneas. After keratoplasty, he regained excellent vision in both eyes, which was maintained at the end of 18 months of follow-up in both eyes despite early signs of recurrence in the right eye. His systemic condition was well controlled during the period of follow-up. CONCLUSION: Corneal deposits in multiple myeloma are well described in the literature, but there are few reports regarding the prognosis and visual function after penetrating keratoplasty. Our report shows that when the systemic condition is well controlled, penetrating keratoplasty has an excellent prognosis in these patients.

Primary squamous cell carcinoma arising from an epithelium-lined cyst of the lacrimal gland.

A 66-year-old man presented with a slowly enlarging, nontender left orbital mass of 2 months' duration. CT and MRI showed a left lacrimal gland mass with enhancement and internal irregularity of cystic structures. Histopathologic analysis of the biopsy specimen revealed a squamous cell carcinoma arising from an epithelium-lined cyst. The patient underwent left orbital exenteration followed by radiation treatment. No evidence of tumor recurrence was observed after a follow-up of 30 months. We believe this primary squamous cell carcinoma may have arisen either from preexisting lacrimal duct cyst (dacryops) with areas of squamous metaplasia or, less likely, from a choristomatous epithelium-lined cyst of the lacrimal gland. Although rare, this entity should be included in the differential diagnosis of cystic lesions of the lacrimal gland.

Leiomyoma of the orbit and periocular region: a clinicopathologic study of four cases.

PURPOSE: Leiomyomas are exceedingly rare tumors of the orbit and periorbital region. Our objective is to describe the clinical, histopathologic, and radiologic features and the management of 4 cases of orbital leiomyomas and to summarize the cases from the literature. METHODS: This retrospective review describes 4 patients with orbital leiomyoma diagnosed by clinical and histopathologic studies. Electron microscopy was performed in 2 cases. Immunohistochemical techniques were performed with 5 monoclonal antibodies. Selected papers describing well-documented cases of orbital leiomyoma in the English literature published since 1960 were reviewed. RESULTS: CT and MRI showed well-circumscribed contrast-enhancing mass lesions. Three tumors were completely excised and 1 had subtotal excision. All 4 tumors showed immunoreactivity for actin, desmin, and vimentin. Follow-up examination showed no evidence of recurrence in 3 patients. One patient was lost to follow-up. CONCLUSIONS: Leiomyoma should be considered in the differential diagnosis of a well-circumscribed mass lesion involving the orbit and periorbital region. Immunohistochemistry provides conclusive evidence to confirm the diagnosis.

Sebaceous gland tumors of the eyelids and conjunctiva in the Muir-Torre syndrome: a clinicopathologic study of five cases and literature review.

PURPOSE: To study the sebaceous tumors of eyelid/conjunctiva associated with Muir-Torre syndrome (MTS) and to determine the role of immunohistochemical markers (MSH2, mismatch repair gene) in the diagnosis of patients with MTS. METHODS: A retrospective, noncomparative case series of 5 patients diagnosed with MTS from our laboratory. We also reviewed all previously reported cases of sebaceous eyelid tumors with a visceral malignancy. RESULTS: Four of the 5 patients were men, with a mean age of 55 years (range, 41 to 76 years). Four of the 5 patients had gastrointestinal carcinoma. On histopathological examination, 4 of the 5 tumors were classified as sebaceous adenomas that exhibited a distinct lobular pattern with prominent basaloid cells at the periphery of the lobules. One tumor was classified as a well-differentiated sebaceous gland adenocarcinoma. The diagnosis of MTS in all 5 patients was made after the diagnosis of the eyelid lesions. Immunohistochemical stains showed a lack of MSH2 expression in two tumors, which is highly consistent with MTS. CONCLUSIONS: Muir-Torre syndrome should be considered in patients who develop sebaceous tumors of the ocular adnexa. Immunohistochemistry for MSH2 is a practical initial approach for screening MTS in patients with sebaceous tumors.

Sebaceous gland adenoma of the tarsal conjunctiva in a patient with Muir-Torre syndrome.

PURPOSE: To highlight the recognition and diagnosis of Muir-Torre syndrome (MTS) in patients with sebaceous tumors of the eyelid/conjunctiva and to determine the role of immunohistochemical markers as a screening test in patients with Muir-Torre syndrome. DESIGN: Single interventional case report. METHODS: The clinical and family history was reviewed from the records of a 41-year-old man who had a sebaceous gland adenoma of the tarsal conjunctiva of the left upper eyelid. The lesion was completely excised and submitted for histopathologic examination. Immunohistochemical stains using an antibody to MSH2 were performed on the paraffin-embedded sections of the tumor. RESULTS: Histopathologic examination of the tumor showed a lobular pattern with basaloid cells at the periphery of the lobules with central areas of sebaceous differentiation. Immunohistochemical stains showed a lack of MSH2 expression in the tumor, which is highly consistent with MTS. CONCLUSIONS: Muir-Torre syndrome should be considered in patients initially seen with sebaceous tumors of the eyelid. Immunohistochemistry for MSH2 is a practical initial approach to screen for MTS in patients with sebaceous tumors.

Giant cell reparative granuloma of the orbit associated with cherubism.

PURPOSE: To report a case of cherubism with extensive, bilateral orbital involvement occurring in a 27-year-old woman who had the diagnosis established at the age of 4 years. DESIGN: Single interventional case report. INTERVENTION: Ophthalmologic examination and computed tomography were performed. The patient underwent multiple surgical excisions using a bicoronal and transorbital approach. The excised orbital tissues were studied histopathologically. RESULTS: Computed tomography showed bilateral inferior lateral masses involving the orbital floors and producing marked superior displacement of the orbital contents. The intrinsic expansile bone lesions involved the inferior and lateral orbital walls with apical compression of the optic nerves. Histopathologic examination of the masses revealed scattered giant cells in a fibroblastic stroma containing small vascular channels. The lesion was interpreted as giant cell reparative granuloma. CONCLUSIONS: Giant cell reparative granuloma is an uncommon bone lesion that might involve the orbit. Cherubism should be included in the differential diagnosis of lesions that show the histopathologic features of giant cell reparative granuloma.