Isolated eyelid edema in Melkersson-Rosenthal syndrome: a case series.

PURPOSE: To report the clinicopathologic features of a series of patients with Melkersson-Rosenthal syndrome (MRS). METHODS: Patients in this clinicopathological case series were identified through retrospective review. Five Caucasian patients (2 women and 3 men; age range, 46 to 73 years) with isolated eyelid swelling presented over an 11-month period to two Canadian tertiary Oculoplastic referral centres. All underwent clinical examination and had tissue biopsies. RESULTS: The patients presented with non-pitting, non-pruritic, painless and recurrent eyelid swelling. None had evidence of cranial nerve palsy or fissured tongue. The duration of symptoms before referral was between 1-5 years. Histopathological examination showed granulomatous inflammation in four patients. Granulomatous folliculitis was a novel finding in three patients. CONCLUSIONS: Monosymptomatic patients with MRS require a high index of suspicion. Histopathological confirmation is vital for the diagnosis.

Osteoblastoma of the clavicle.

A 14-year-old female with a 1-year history of a painful clavicle mass underwent resection and reconstruction with an intercalated autograft and rigid plate fixation. The histopathology was diagnostic for osteoblastoma. Although a clavicle mass is a common entity following clavicle trauma, less common disorders such as bone tumors need to be considered in the differential diagnosis. Osteoblastoma is an uncommon, benign bone tumor representing 1% of all primary bone tumors. An extensive review of the literature reveals only one reported case of clavicular osteoblastoma.

Erdheim-Chester disease: two cases of orbital involvement.

Erdheim-Chester disease (ECD) is an increasingly recognised form of fibro-inflammatory process characterised by xanthomatous histiocytes containing large amounts of ingested lipid, plasma cells, macrophages and Touton-type giant cells. Ophthalmic involvement in ECD has been reported in only 22 cases. We describe two patients, one presenting with diabetes insipidus and subsequently developing orbital pseudotumours and retroperitoneal fibrosis, the other presenting with exophthalmos and diplopia. The first patient was treated with cladribine and subsequently developed sudden onset of bilateral blindness while the second required radiation therapy for the retro-orbital process and developed radiation retinopathy. These cases typify the variable presentation and course in patients with ECD.

Iris lymphoma in a pediatric cardiac transplant recipient: clinicopathologic findings.

PURPOSE: To report the unusual presentation of a confirmed iris lymphoma in the setting of posttransplant lymphoproliferative disorder. DESIGN: Case report with clinicopathologic findings. METHODS: The medical record was reviewed for clinical manifestations, course of disease, and pathologic and radiologic findings. RESULTS: A 4-year-old girl who had undergone a cardiac transplant at 3 months of age was found to have unilateral iris lesions associated with granulomatous uveitis. Her condition was unresponsive to local and systemic steroids, so she underwent an iris biopsy that was found to contain a B cell-derived monoclonal lymphoproliferation. Treatment with systemic chemotherapy including cyclophosphamide and prednisone resulted in a transient, partial response. Localized radiotherapy induced total regression of the iris lesions. She continues to be observed and has been free of recurrence or metastasis for the past 12 months. CONCLUSIONS: Because of the increased use of organ transplantation, eye care specialists should consider the diagnosis of intraocular lymphoma when confronted with uveitis-like syndrome unresponsive to topical steroid therapy. Although there are numerous treatment options for intraocular lymphoma, radiation therapy was found to be effective in the treatment of this case.

Expression of vascular endothelial growth factor in uveal melanoma and its correlation with metastasis.

AIMS: To evaluate the expression of vascular endothelial growth factor (VEGF) in uveal melanomas and correlate its presence with tumour characteristics and systemic metastasis. METHODS: 47 cases of ciliochoroidal melanoma enucleated between 1983 and 1993 were retrieved from the pathology archives at the University of Western Ontario. Paraffin sections stained with haematoxylin and eosin, periodic acid Schiff, and periodic acid Schiff without haematoxylin after bleaching of melanin were examined. The expression of VEGF protein was examined by an immunoalkaline phosphatase method following antigen retrieval, using an antibody to VEGF and vector red as the chromogen. The intensity of VEGF immunoreactivity was graded on a scale of 0-7 and correlated with tumour cell type, tumour size, presence or absence of necrosis, pigmentation, mitotic activity, microvascular density, and microvascular pattern. RESULTS: VEGF immunoreactivity was present in 44/47 tumours (94%): the intensity was graded as very weak (1-2) in 29/47 (62%) and as weak or greater in 15/47 (32%). VEGF was also found in the ciliary epithelium, smooth muscle of the ciliary body and iris, retinal ganglion cells, inner photoreceptor segments, and the retinal pigment epithelium. Follow up data were available in 43/47 patients (91.5%), with a median follow up time of 10 years. 16/43 (37%) patients developed metastases. VEGF expression in melanoma was linked to the presence of tumour necrosis and the degree of pigmentation but no statistically significant relation with microvascular pattern, tumour size, or microvascular density was found. There was no statistically significant correlation between VEGF expression and metastasis. CONCLUSIONS: Most ciliochoroidal melanomas express VEGF and expression is correlated with the presence of necrosis but not with the occurrence of systemic metastasis or tumour angiogenesis.

Cyanoacrylate tissue adhesive in blepharoplasty.

This article reports the results of an animal study designed to evaluate the usefulness of butyl 2-cyanoacrylate (Histoacryl) in blepharoplasty skin closure. Bilateral upper and lower lid blepharoplasty skin incisions were made on seven pigs. We compared the wounds closed with 6.0 nylon to those closed with Histoacryl at 1, 2, 4, and 9 weeks postoperatively. The tensile strength of the "glued" incisions was significantly greater than that of the sutured incisions only at the 9-week postoperative interval (p < 0.05). There was no significant difference in bond strength, however, between the two methods at 1, 2 and 4 weeks postoperatively. Histopathologic analyses of sutured and glued wounds were compared at the same designated postoperative periods. No deleterious effects were found in the overall healing of the cutaneous wounds closed with Histoacryl.

Focal posttraumatic choroidal granulomatous inflammation.

PURPOSE: This study was performed to describe the clinicopathologic features of a group of patients with posttraumatic unilateral focal choroidal granulomatous inflammation. METHODS: Enucleated eyes with focal choroidal granulomatous inflammation without clinical signs of contralateral ocular inflammation were identified. Clinical and pathologic features were recorded. RESULTS: Six enucleated eyes that had been injured by projectiles had focal uveal granulomatous inflammation of the posterior choroid. Four eyes had uvea attached to or incarcerated into the wound. Two eyes had foreign material associated with the granulomatous inflammation, and two eyes had a disrupted lens with lens-induced inflammation. Two eyes exhibited the focal granulomatous inflammation at the site of a choroidal rupture. None of the six enucleated eyes contained Dalen-Fuchs' nodules. CONCLUSIONS: Focal choroidal granulomatous inflammation may occur as a result of penetrating ocular trauma. The origin of this condition is unknown, although it likely represents a reaction to a foreign body.

Intralenticular foreign bodies: case reports and surgical review.

Management of intralenticular foreign bodies (ILFBs) is controversial. Correlation between visual outcome, foreign body characteristics, the need for surgical intervention, and the choice of surgical technique are not well established. We report five cases of traumatic ILFBs all managed surgically. Three ILFBs were metal, one was presumably also metal, and one was wood. We chose to proceed with foreign body removal and simultaneous cataract extraction with intraocular lens implantation as a single-staged procedure in four cases. In one case of planned extracapsular cataract extraction, a foreign body was identified only after the expressed nucleus was sent for pathological examination. Visual outcomes were 20/30 or greater in all cases at 5 to 18 month follow up.

Depot drug delivery system for 5-fluorouracil after filtration surgery in the rabbit.

We investigated the potential value of 50:50 poly (DL glycolic acid-lactic acid) (PGLA) copolymer as a degradable depot delivery system for 5-fluorouracil (5-FU) after filtration surgery. Analysis of retrieved discs after implantation in 22 eyes of 22 pigmented rabbits showed a dual drug release profile and polymer mass loss characteristics. In a second group of pigmented rabbits implantation of PGLA discs impregnated with 5-FU (22 eyes) significantly lengthened the survival time of filtration fistulae compared with discs without 5-FU (18 eyes) or no disc (10 eyes) (p < 0.0001). Use of PGLA copolymer impregnated with 5-FU could prove valuable for patients undergoing glaucoma filtering surgery.

Ocular toxicity of mitomycin C and 5-fluorouracil in the rabbit.

We studied the histotoxic effects of mitomycin C and 5-fluorouracil (5-FU) in the eyes of New Zealand white rabbits using light microscopy. Twenty-four eyes of 24 rabbits received subconjunctival injections of mitomycin C (0.025, 0.05, 0.10, 0.20, 0.40 or 0.80 mg/mL) or 5-FU (10.0 mg/mL), either alone (0.25 mL) or in combination with anterior chamber injections (0.05 mL), once daily for 4 consecutive days. Two eyes of two rabbits received subconjunctival and anterior chamber injections of unpreserved sterile saline. The eyes were examined regularly for external signs of toxicity and were enucleated 4 weeks after the last injection. Mitomycin C produced considerable tissue damage in the anterior segment, the severity being related to both the concentration and the route of administration. Eyes that received both subconjunctival and anterior chamber injections showed more damage than those that received subconjunctival injections alone. At the highest concentration of mitomycin C the cornea was inflamed, with stromal necrosis and marked endothelial loss. Hemorrhagic iris necrosis was also seen. In contrast, the 5-FU-treated eyes showed no microscopic evidence of toxicity. We conclude that there is greater risk of toxic anterior segment effects when mitomycin C is used as adjunctive therapy following filtration or pterygium surgery.

Epithelioid sarcoma of the orbit.

BACKGROUND: Epithelioid sarcoma is a rare and aggressive malignancy commonly occurring in the tendon sheaths of the distal extremities of young adults. This article presents the clinical and pathologic details of two patients with epithelioid sarcoma in the orbit, the first to be reported as occurring in this location. METHODS: The clinical history, results of physical and radiologic examination, surgical procedures, pathologic specimens, and clinical course of each patient were reviewed and recorded. RESULTS: Biopsies were performed of the masses that were found in the superotemporal orbit of both patients. Results of pathologic examination in each patient caused considerable difficulty, and the correct diagnosis was reached only after extensive consultation. The first patient underwent wide soft tissue and bone exenteration and is alive without recurrence at 3 years. The second patient had multiple recurrences and died of recurrent and metastatic disease at 29 months. CONCLUSIONS: Epithelioid sarcoma must be added to the differential diagnosis of orbital sarcomas. The histology is nodular with a pseudogranulomatous appearance. Based on our limited experience, epithelioid sarcoma of the orbit may have a course similar to that which occurs elsewhere in the body, requiring aggressive surgical treatment at the outset.

Fifty:fifty poly (DL glycolic acid-lactic acid) copolymer as a drug delivery system for 5-fluorouracil: a histopathological evaluation.

Fifty:fifty poly (DL glycolic acid-lactic acid) copolymer (PGLA) is a potentially useful depot drug delivery system for 5-fluorouracil (5-FU). The purpose of this study was to evaluate the fibroinflammatory reaction induced by this polymer. Polymer discs without 5-FU were inserted subconjunctivally in one eye of each of two guinea pigs and four pigmented rabbits (control group), and discs containing 20% 5-FU were inserted subconjunctivally in both eyes of nine pigmented rabbits (study group). The tissue reaction to the copolymer did not differ between rabbits and guinea pigs, with a mild mixed inflammatory reaction 1 week after implantation. At 2 weeks a thin fibrous capsule surrounded the discs, with no change in the amount of inflammation. At 4 weeks the disc had disintegrated, but residual polymer was seen within multinucleated giant cells in the episcleral tissue. Granulation tissue and inflammatory responses were mild. Less inflammation and fibrosis occurred in the study eyes, although the pattern of response was similar in the two groups. The inflammatory response to PGLA was markedly less than that to implanted collagen shields, and our findings suggest that PGLA implant is a promising ocular drug delivery system for 5-FU after filtration surgery.