Orbital solitary fibrous tumor: radiographic and histopathologic correlations.

OBJECTIVE: To correlate the clinicopathologic and radiographic features characteristic of orbital solitary fibrous tumor (SFT). METHODS: The diagnostic features and clinical outcome of seven adults with orbital SFT are retrospectively outlined. Orbital imaging was performed by ultrasonography, computed tomography, or magnetic resonance imaging. Some cases were imaged by multiple modalities. Histopathologic examination of each tumor specimen included standard light and immunohistochemical stains. RESULTS: Heterogeneous internal composition was better appreciated on magnetic resonance imaging than on computed tomography. All cases undergoing magnetic resonance imaging showed T1 isointensity and T2 hypointensity relative to gray matter. Strong, generalized immunohistochemical reactivity to vimentin and CD34 validated the diagnosis of SFT and differentiated the specimens from other spindle cell neoplasms. After complete tumor resection, our patients remain tumor free with postoperative intervals of 15 to 45 months. CONCLUSIONS: Solitary fibrous tumor has now been reported in 26 orbits. No physical finding is pathognomonic, but several imaging traits are highly characteristic. Intralesional image heterogeneity and a predominantly low T2 signal intensity are distinctive of SFT. Complete tumor resection and immunohistologic specimen evaluation are emphasized. Clinicians should consider the diagnosis of SFT when confronted with an adult patient having an orbital soft tissue mass demonstrating the distinctive magnetic resonance imaging findings.

Primary signet ring cell carcinoma of the eyelid: report of a case demonstrating further analogy to lobular carcinoma of the breast with a literature review.

Primary signet ring cell carcinoma of the eyelid is a rare neoplasm with less than ten cases described. This report details another case, which shows further parallels to lobular carcinoma of the breast, and reviews the literature on this subject. A 73-year-old white female presented with diffuse induration of her left eyelids. Histopathology revealed a delicate infiltrate of epithelial cells scattered throughout the lid stroma in a non-destructive pattern. The cells were relatively monomorphous and showed only mild atypia and rare mitotic figures. Many had slightly granular amphophilic cytoplasms, others showed distinct signet ring cell morphology, and all were strongly positive for epithelial mucin. Immunomicroscopy showed strong reactivity for estrogen receptor (ER), progesterone receptor (PR) and gross cystic disease fluid protein-15 (GCDFP-15), and was negative for Her-2/neu (erb-2) and cytokeratin 20. An extensive workup for other primary sites was negative. Orbital exenteration showed extensive involvement of both lids and soft tissue, including diffuse muscle and lacrimal gland infiltration. In the breast, signet ring cell carcinoma is considered a variant of lobular carcinoma. The delicate infiltrating pattern in our case and the ER+, PR+, GCDFP-15+, Her-2/neu-phenotype further strengthen this analogy. Together, these data also support apocrine differentiation of primary eyelid signet ring cell carcinoma.

5-Fluorouracil for the treatment of intraepithelial neoplasia of the conjunctiva and cornea.

OBJECTIVE: To evaluate the efficacy of pulse dosing of topical 5-fluorouracil (5-FU) in the treatment of conjunctival and corneal intraepithelial neoplasia. DESIGN: Prospective, noncomparative case series. PARTICIPANTS: Seven patients with histologic evidence of intraepithelial neoplasia were identified by conjunctival biopsy or tumor excision. METHODS: Seven patients with a minimum of 7 months of follow-up were treated with pulsed dosing of 1% 5-FU. Topical 1% 5-FU was administered four times daily for 2 to 4 days for each cycle. The number of initial treatment cycles was two to six, with the time between cycles being 30 to 45 days. MAIN OUTCOME MEASURES: The presence or absence of clinically evident intraepithelial neoplasia was evaluated after each treatment interval. Patients were also monitored for adverse reactions to the use of topical 5-FU. RESULTS: Four patients remain disease free with a mean follow-up of 18.5 months (range, 7-36 months) with no additional treatment after the initial treatment cycles (mean, 3.75 cycles; range, 2-5 cycles). Three patients had recurrence of disease after the initial treatment cycles. Two patients were treated with additional cycles for recurrent disease (six cycles in one patient and five cycles in the other patient) and are free of disease at 20 and 21 months after treatment, respectively. One patient had persistent disease despite treatment with topical 5-FU and was treated with topical mitomycin C with resolution of the disease without recurrence for 16.5 months. No adverse reactions to pulse dose treatment with topical 5-FU were noted. CONCLUSIONS: Pulsed dosing with 1% topical 5-FU for the treatment of conjunctival and corneal intraepithelial neoplasia, alone or as an adjunct to excision of bulky disease, is a well-tolerated and effective method of treatment.

Abnormalities of eyelid position and function.

Evaluation of the eyelids is an important part of the neuro-ophthalmic examination. Abnormal eyelid position and function can be caused by disorders involving the third cranial nerve, the oculosympathetic pathway, and the seventh cranial nerve, as well as supranuclear pathways, or as a result of neuromuscular diseases. To avoid unwarranted neurological investigations, it is also important for the clinician to recognize non-neurological eyelid abnormalities (such as ptosis from levator dehiscence or eyelid edema).

Combined intraepithelial squamous neoplasia and atypical fibroxanthoma of the cornea and limbus.

PURPOSE: To investigate a case of an unusual neoplasm of the cornea and limbus. METHODS: A 59-year-old man presented with a highly vascularized, nodular mass involving the left cornea and limbus. An excisional biopsy and, subsequently, a superficial lamellar keratectomy and multiple conjunctival biopsies were performed. At the 6-month follow-up examination, repeat conjunctival biopsies were performed. RESULTS: Histopathologic examination of the corneal specimen showed a high-grade intraepithelial squamous neoplasia (in situ carcinoma) overlying an atypical fibroxanthoma. CONCLUSION: We report the clinical and histologic appearance of a corneal/limbal neoplasm consisting of an intraepithelial squamous neoplasia and an atypical fibroxanthoma.

Use of mitomycin C in repeat dacryocystorhinostomy.

PURPOSE: The usefulness of a single intraoperative application of mitomycin C was investigated in repeat dacryocystorhinostomy for membranous failure. METHODS: Mitomycin C (0.3 mg/ml for 3 minutes) was topically applied to the fistula site in eight patients undergoing repeat dacryocystorhinostomy for membranous failure. RESULTS: All patients remain asymptomatic and anatomic patency confirmed by probe and irrigation with a mean follow-up period of 14.6 months (range, 6-26 months). No postoperative complications associated with the use of mitomycin C were observed. CONCLUSIONS: In patients who do not maintain a patent fistula after dacryocystorhinostomy because of membranous occlusion of the rhinostomy site, the adjunctive use of mitomycin C may increase the success rate of repeat dacryocystorhinostomy.

Primary temporal fossa dermoid cysts. Characterization and surgical management.

OBJECTIVE: To detail the characteristics and management of rarely reported and incompletely described dermoid cysts originating in the temporal fossa. DESIGN: Retrospective case series. PARTICIPANTS: Five patients ranging from 2 to 38 years of age with a mass in the temporal region (posterior to the lateral orbital rim) participated. INTERVENTION: Computed tomography (CT) and excisional biopsy were performed. MAIN OUTCOME MEASURES: Clinical and CT characteristics and surgical outcomes were measured. RESULTS: Computed tomography showed cystic lesions, originating from the region anterior to the confluence of the greater wing of the sphenoid, frontal, and zygomatic bones. Displacement of the anteriormost portion of the temporalis muscle was common. Three cysts were isolated to the temporalis fossa, while two showed more extensive bony erosion and extension into the cranial and orbit cavities. At surgical excision, gross rupture of the cysts was noted in two cases, and two were completely liquefied. Histopathology showed variable inflammation surrounding all of the dermoid cysts. All patients did well after surgery. CONCLUSIONS: Dermoid cysts may infrequently occur "primarily" in the temporal fossa. Bone involvement and anterior temporalis muscle displacement are common. An origin from the area anterior to the confluence of the greater wing of the sphenoid, frontal, and zygomatic bones is seen. A coronal approach facilitates wide exposure and excision. When dural extension is suggested on CT, neurosurgical assistance may be required.

Pneumo-orbital cyst after orbital fracture repair.

PURPOSE: To report a case of a respiratory epithelial-lined, air-filled orbital cyst as a late complication of orbital fracture repair. METHOD: Case report. RESULTS: Recurrent episodes of diplopia and hyperophthalmia developed secondary to pneumatic inflation of a respiratory epithelial-lined orbital cyst 6 months after orbital fracture repair. This cyst remained in communication with an ethmoidal air cell and became inflated during pressurization of the ethmoid sinus. Removal of the cyst and orbital implant was curative. CONCLUSION: Air-filled, respiratory epithelial-lined orbital cyst is a rare cause of episodic diplopia and globe displacement after orbital trauma.

Current concepts in brow lift surgery.

During the past decade, surgeons have increasingly recognized that brow ptosis is an important contributor to dermatochalasis and ptosis of the upper eyelid. Surgical elevation of the ptotic brow improves eyelid esthetics; conversely, failure to appreciate and to alleviate brow abnormalities frequently compromises the results of upper eyelid surgery. Improved understanding of eyebrow anatomy and of the pathophysiology of the aging face, combined with advances in small-incision surgery, have culminated in a unified approach to correction of the ptotic brow. Despite improvements in surgical techniques, the surgical outcome can deviate from the perceived eyelid-brow ideal. Both the surgical management and objectives of eyebrow surgery continue to evolve.

Granulomatous blepharitis as a sign of Melkersson-Rosenthal syndrome.

OBJECTIVE: The objective of our study was to describe the ophthalmic features and histologic eyelid findings of Melkersson-Rosenthal syndrome. DESIGN: Three patients with eyelid edema underwent eyelid biopsy to establish the diagnosis of Melkersson-Rosenthal syndrome. RESULTS: Of the three patients, only one patient manifest the classic triad of facial edema, facial paralysis, and furrowed tongue. Histoloppgically, the eyelid skin in all patients showed characteristic perilymphatic granulomas with marked dermal edema. CONCLUSIONS: The histopathologic features assist in establishing the diagnosis of Melkersson-Rosenthal syndrome in oligo- and monosymptomatic patients. Melkersson-Rosenthal syndrome should be considered in patients presenting with eyelid edema of unknown etiology and biopsy performed.

Doppler-assisted vascular pedicle flaps in eyelid and periorbital reconstruction.

The use of a transcutaneous ultrasonic Doppler flow detector to identify the supratrochlear and superficial temporal arteries permits the design of narrow-based, thin-tipped forehead flaps for use in medial canthal and eyelid reconstruction. In the 13 cases described, the axial, vascular supply of a proposed myocutaneous forehead flap was determined with a transcutaneous ultrasonic Doppler flow detector permitting narrow-based pedicle widths of 0.8 to 1.2 cm. The design of the distal portion of the flap was determined by the primary defect. The width of the flap varied from 1.5 to 4.0 cm, with the flap's axial length limited only by the hairline. This use of the ultrasonic Doppler flow detector, permitting narrow-based, thin-tipped vascular pedicle flaps, has assisted in refining the concept of forehead flaps and has made these flaps an acceptable primary reconstructive technique in the periorbital region.

Current concepts in lacrimal drainage surgery.

Dacryocystorhinostomy remains the mainstay of treatment of acquired primary and secondary obstructions of the lacrimal drainage system. The advent of the endoscope has prompted renewed interest in the intranasal or endonasal approach to fistulae formation. As results of the intranasal approach are reported, they are compared with the time-tested and effective external approach, bringing controversy to the once staid subject of lacrimal drainage surgery. Debate continues regarding the optimal timing of intervention in the treatment of congenital nasolacrimal duct obstruction. Clinical outcome analysis suggests that early office-based lacrimal probing is more cost-effective than a hospital-based procedure. Others warn that when other factors are included in the decision process, the recommended course of intervention may be different. If dacryocystorhinostomy is required in a child with congenital nasolacrimal duct obstruction, the results by an experienced surgeon are comparable with those in an adult operated on for primary acquired lacrimal obstruction.

Topical 5-fluorouracil in treating epithelial neoplasia of the conjunctiva and cornea.

PURPOSES: To evaluate the efficacy of topical 5-fluorouracil (5-FU) in treating conjunctival and corneal epithelial neoplasia. METHODS: Three patients underwent surgical excision of bulky disease followed by topical 1% 5-FU in artificial tear base for 2 to 3 weeks or until epithelial separation occurred. An additional three patients underwent treatment with topical 1% 5-FU alone. RESULTS: Minimum follow-up was 6 months. In one patient with conjunctival in situ carcinoma and no detectable normal conjunctiva, who had normal findings on conjunctival histologic examination after application of topical 5-FU, a focus of invasive disease requiring orbital exenteration. One patient had a favorable response to 5-FU therapy but required a repeat excision for control of bulky disease. Four patients have remained disease-free for 10, 13, 18, and 30 months after topical 5-FU therapy. CONCLUSION: With its potential selective toxicity on dysplastic epithelium, topical 5-FU shows promise as an adjunctive treatment for managing conjunctival and corneal epithelial neoplasia.

Orbital cellulitis, subperiosteal abscess, sinusitis, and septicemia caused b Arcanobacterium haemolyticum.

PURPOSE/METHODS: A 16-year-old boy had orbital cellulitis, subperiosteal abscess, sinusitis, and septicemia. Arcanobacterium haemolyticum was identified as the causative organism. RESULTS/CONCLUSIONS: This organism is a cause of orbital cellulitis and may require aggresive therapy in order to achieve a therapeutic response.

Graves' ophthalmopathy.

Graves' ophthalmopathy is an organ-specific autoimmune process strongly linked to Graves' hyperthyroidism. Although the hyperthyroidism can be successfully treated, it is often the ophthalmopathy that produces the greatest long-term disability for patients suffering from this disease. Eyelid retraction, proptosis, periorbital edema, chemosis, and disturbances of ocular motility have both cosmetic and functional consequences. In some cases, the disease may progress to visual loss by exposure keratopathy or compressive optic neuropathy. This article discusses the manifestations of Graves' ophthalmopathy that require urgent attention and the relationship of the activity of Graves' ophthalmopathy to thyroid function status.