Search

Histopathologic evaluation, performed in 13 patients with cutaneous horn of the eyelid, revealed malignancy at the base in 23% of patients, including squamous cell carcinoma and sebaceous carcinoma.

Cutaneous horns uncommonly involve the periocular region. Involvement of the ocular surface is particularly rare. The authors present a patient who underwent a perinatal buccal mucosal graft for corneal perforation due to congenital corneal ectasia, most likely resulting from Peters anomaly. She developed a giant ocular horn 10 years later.A giant ocular horn developed as a late complication of a buccal mucosal graft placed for corneal perforation due to congenital corneal ectasia.

To describe the clinical, imaging, and pathology features of oncocytic lesions in the ocular adnexa.

To report four pediatric cases of a bone-destructive orbital tumor that proved to be a benign solitary intraosseous myofibroma.

An 80-year-old woman with a prior diagnosis of age-related macular degeneration in her left eye had rapidly progressive visual loss in her right eye. Orbital MRI revealed a mass involving the left optic nerve and chiasm, interpreted by a radiologist as optic nerve sheath meningioma. Further review of the MRI revealed a mass inside the left eye, characteristic of choroidal melanoma, with apparent extension through the optic nerve to the chiasm and adjacent tissues. Orbital biopsy revealed mixed cell–type melanoma. This case demonstrates that unsuspected choroidal melanoma can invade the optic nerve and chiasm, causing contralateral visual loss. Contralateral blindness as the initial complaint from an unsuspected choroidal melanoma is extremely unusual if not unique.

To report five patients with intraocular invasion of conjunctival squamous cell carcinoma and to make recommendations regarding clinical recognition and treatment of this condition.

The use of Gore-Tex (W. L. Gore & Associates, Flagstaff, AZ, U.S.A.) spheres as orbital implants is investigated. The left eyes of six New Zealand white rabbits were enucleated and spherical implants made of modified Gore-Tex were implanted. After 6 weeks of follow-up, the implants were harvested. No rabbit developed a postoperative infection and no cases of exposure or extrusion were noted. Histopathologic study revealed varying degrees of acute and chronic inflammation surrounding each implant. There was also evidence of inflammatory infiltration and fibrovascular ingrowth into each implant to a maximum distance of 500 μm. This preliminary study demonstrates that the Gore-Tex implant is well tolerated in vivo, allows cellular ingrowth, and may have a role as a permanent implant.

The dermoid cyst is one of the most common space-occupying orbital lesions, but there has been no large series that details the clinical, pathologic, and therapeutic aspects of this condition. Furthermore, despite the various presentations and types of dermoid cysts, there is no clinically applicable classification of these lesions. We performed a retrospective histopathologic study of 197 consecutive orbital and periorbital dermoid cysts excised at Wills Eye Hospital during a 32-year period, correlated the histopathologic findings with the recorded clinical and radiographic observations, and devised a clinically applicable classification of orbital dermoid cysts. The mean age at excision was 17 years (range, 5 months to 85 years), and the most frequent clinical finding was a painless subcutaneous mass, present in 89 of cases. In 72 of patients, the mass was located in the superotemporal orbital region and produced no abnormalities of vision or intraocular pressure. Computed tomography and magnetic resonance imaging, performed mainly in the more recent cases, disclosed a characteristic round to ovoid cystic lesion with a well-defined lining. Histopathologically, 165 of the cysts (84) were lined by keratinizing, stratified squamous epithelium. Ten (5) were lined by nonkeratinizing epithelium resembling conjunctival epithelium. Hair shafts were present in the wall or lumen of the cyst in 195 (99) of specimens, sebaceous glands in 148 (75), and sweat glands in 39 (20); inflammation was present in 75 cases (38). Based on these personal observations and a review of the literature, a classification of orbital dermoid cysts is proposed that we believe will provide guidance for the ophthalmologist in the management of these lesions.

A 29-year-old woman presented with a 1-year history of blurred vision in her left eye while chewing. Examination revealed a rubbery mass with ill-defined borders in the superotemporal brow region. Imaging studies demonstrated a cystic lesion extending from the temporal fossa into the orbit through a bony defect in the lateral orbital wall, assuming a dumbbell configuration. Histopathology of the excised specimen confirmed the clinical diagnosis of a dermoid cyst of cutaneous origin. We believe that the patient's visual disturbance was caused by transient displacement of orbital contents by the dermoid cyst as it was compressed into the orbit during mastication. Dermoid cysts are frequently associated with bony defects, which may require preoperative imaging and may produce unexpected symptoms.

A total of 24 patients (12 men and 12 women) with squamous cell carcinoma of the eyelid were identified from pathology records at Wills Eye Hospital from 1978 through 1987. Squamous cell carcinoma accounted for 24 of 648 (3.7) malignant eyelid lesions submitted during the 10-year study period. The median age of patients at the time of diagnosis was 72 years (range, 55 to 96 years). Initial therapy of all tumors involved surgical excision. Four patients developed clinical recurrence of squamous cell carcinoma in a median of 7.5 months after surgery. There were no tumor recurrences when frozen section techniques were applied during the initial surgical excision. The data suggest that the risk of persistence or recurrence of tumor was increased for patients who delayed seeking medical care after the lesion was first noticed and when frozen section control was not used during surgery. None of the patients were known to have developed metas-tases, and there were no tumor-related deaths.

A 35-year-old woman developed painful proptosis of the left eye over a period of 3 weeks. Orbital computed tomography and magnetic resonance imaging revealed a well-circumscribed superior orbital tumor with variable density. The mass was removed entirely by way of a superior orbitotomy. Histopathologically, it was found to be a neurofibroma. The patient had no clinical findings of neurofibromatosis. The presence of extensive myxomatous degeneration and the dense collection of collagen bundles in the tumor are correlated with the unusual computed tomography and magnetic resonance imaging features.

Temporal artery biopsy (TAB) is considered the gold standard in the diagnosis of suspected giant cell arteritis. The most commonly accepted length for an adequate postfixation TAB specimen is 20 mm. There is a reported 2.4-mm mean shrinkage with the fixation process, but to date, there is no data correlating shrinkage after specimen fixation with the biopsy results.

To describe the demographics, characteristics, and treatment of giant fornix syndrome, a rare cause of chronic purulent conjunctivitis in the elderly.

A healthy, 17-year-old boy presented with a 2-month history of an epibulbar mass on the left eye. Slit lamp examination showed an amelanotic, translucent, round, well-circumscribed, superonasal subconjunctival nodule. On ultrasound biomicroscopy, the tumor was acoustically hollow. The lesion was excised intact, and cryotherapy was applied to the surrounding conjunctiva. Histopathologic examination revealed a tumor composed of spindle cells with slender cytoplasmic processes and bland cigar-shaped nuclei, consistent with schwannoma. Immunohistochemistry showed positivity for S100 protein. The patient had no signs of neurofibromatosis. There was no tumor recurrence after 18 months. This case shows that conjunctival schwannoma can present as a translucent, well-circumscribed subconjunctival mass.

A 16-year-old girl presented with diplopia and gradual-onset, painless proptosis of the left eye. Orbital CT showed a well-circumscribed, enhancing, extraconal mass in the superior orbit, and the surgical excision was performed. Histopathology was interpreted as capillary hemangioma. Five years later, her symptoms recurred, and she was referred to the Oncology Service, Wills Eye Institute. Repeat orbital MRI showed a well-defined, extraconal mass with loculated areas of enhancement in the left orbit superonasally. Complete surgical excision was performed. Histopathologic examination showed benign, patternless spindle-cell proliferation with prominent intrinsic vascularity and multinucleated giant cells, consistent with giant cell angiofibroma, a variant of solitary fibrous tumor. There was intense immunoreactivity for CD34. After 20 months follow-up, there was no recurrence or development of metastasis. Giant cell angiofibroma, a variant of solitary fibrous tumor, is a rare orbital tumor that presents as a well-circumscribed, enhancing mass and can be found in children.

A dermoid cyst of conjunctival origin (“conjunctivoid”) is a rare variant that has conjunctival nonkeratinizing squamous epithelium and goblet cells, instead of keratinizing squamous epithelium of epidermal origin with epidermal appendages. We report a case of a 65-year-old man with a chronic orbital mass, a conjunctival dermoid that was found to have the unusual histopathologic finding of oncocytic differentiation. This is an infrequent benign chronic change in glandular cells characterized by the accumulation of vast numbers of intracellular mitochondria. Occasionally, oncocytic cellular change occurs in malignant tumors, and so complete excision of such lesions is recommended.

Rhabdomyosarcoma is the most common pediatric primary neoplasm in the orbit, often presenting with rapid proptosis and orbital symptoms. We describe a 15-year-old girl who presented with an acute mass in her medial rectus muscle that was subsequently diagnosed as widely disseminated alveolar rhabdomyosarcoma. To our knowledge, this represents the first reported case in which an enlarged extraocular muscle was the initial manifestation of disseminated alveolar rhabdomyosarcoma.

We report the rare but serious association between sebaceous carcinoma of eyelid and Muir-Torre syndrome in two cases, a 79-year-old woman and 54-year-old man. The presenting sign was a yellow-pink nodule with telangiectatic vessels on the left lower eyelid in one patient and a yellowish-red, pedunculated lesion with intrinsic vascularization on the right superior tarsal conjunctiva in the other. A history of multiple primary colon cancers was present in both patients, associated transitional cell carcinoma of bladder and renal cancer in one patient and prostate cancer in the other. Both patients underwent excisional biopsy and cryotherapy of the eyelid tumors. Histopathology revealed sebaceous carcinoma of eyelid and conjunctiva. Immunohistochemical staining for MSH2 DNA mismatch repair gene performed in one patient showed lack of expression. Muir-Torre syndrome was diagnosed on the basis of sebaceous carcinoma, associated with multiple visceral malignancies in both patients. We conclude that patients with sebaceous carcinoma should have evaluation for visceral malignancies, mainly colon cancer.

An 82-year-old man with hypothyroidism had vertical diplopia and swelling around his left eye. Visual acuity was 20/20 OD and 20/50 OS. There was moderate blepharoptosis and edema of the left eyelids and superior scleral show of the right eye. The left eye showed 4 mm of proptosis, motility restriction, afferent pupillary defect, and normal optic disc. Orbital MRI revealed enlargement of the left superior and medial rectus muscles without tendinous involvement. These findings were initially suggestive of thyroid orbitopathy. Thyroid function tests were normal. Coronal MRI showed additional superior oblique enlargement and involvement of the levator superioris palpebrae muscle, which are both suggestive of a non-thyroid pathology. Muscle biopsy revealed large B-cell lymphoma. The patient was treated with chemotherapy, immunotherapy, and radiotherapy, with complete tumor control. Orbital lymphoma can simulate thyroid orbitopathy, even in patients with classic “thyroid-like” symptoms and imaging.

A 56-year-old woman with left periocular pain was discovered on CT to have a partially calcified, circumscribed cystic lesion in the lacrimal gland fossa, suggestive of a malignant lacrimal gland tumor. Adjacent bone fossa formation without erosion was noted. Histopathologic examination of the excised mass disclosed a cyst with chronic granulomatous inflammation and calcification of the cyst wall. The combination of chronic pain, cystic changes, and calcification in a lacrimal gland fossa mass in an adult should suggest a malignant neoplasm, but a long-standing orbital cyst with calcification of its rim can produce the same features.

We report a 59-year-old man who had a slowly enlarging mass of the central upper eyelid that proved histopathologically to be a benign mixed tumor (pleomorphic adenoma) arising from an accessory lacrimal gland of Wolfring. The tumor was totally separate from the main lacrimal gland, and its deep location in the lid excluded origin from dermal sweat glands. Benign mixed tumors of the accessory lacrimal glands are exceedingly rare tumors of the ocular adnexa.

Although Ascaris lumbricoidesinfestation is extremely prevalent in some populations, the nematode is an extremely rare cause of nasolacrimal duct obstruction. Obstruction of the nasolacrimal duct in a 4-year-old Brazilian girl by a young adult A. lumbricoidesis reported. In contrast to previously reported cases, the nematode involved the inferior, rather than superior, canaliculus and punctum.

A 62-year-old woman with a history of dry eyes was found to have unilateral pedunculated, nonpigmented palpebral conjunctival lesions. Excisional biopsy was performed, and the lesions were studied histopathologically. Microscopic examination of the lesions demonstrated exuberant granulation tissue with a granulomatous foreign body giant cell reaction surrounding pigmented and partially birefringent foreign material. Histopathologic examination of the patient’s cosmetics revealed that the pigmented foreign bodies seen on the biopsy specimens were compatible with her mascara andor eyeliner. While cosmetics have been reported to have ophthalmic sequelae, palpebral lesions such as these have not been reported to the authors’ knowledge.

A 39-year-old woman presented with a vermiform superior conjunctival mass that progressively enlarged during 4 years of follow-up. Although magnetic resonance imaging suggested that the lesion was located in the superior forniceal conjunctiva, surgical exploration disclosed a vascular anomaly (varix) that extended deep into the orbit. The clinical features and management of orbital varices are discussed.