Anterior chemodenervation of levator palpebrae superioris with botulinum toxin type-A (Botox) to induce temporary ptosis for corneal protection.

AIM: To evaluate the effectiveness of anterior chemodenervation of levator palpebrae superioris with Botulinum toxin type A (Botox) to induce temporary ptosis for corneal protection, and assess the incidence of superior rectus underaction. METHODS: Prospective interventional case series. Patients with ocular surface pathology requiring temporary tarsorrhaphy underwent transcutaneous anterior chemodenervation of levator palpebrae superioris with Botox. The onset and duration of ptosis, corneal healing, and superior rectus underaction was evaluated. RESULTS: Ten eyes of 10 patients underwent transcutaneous anterior chemodenervation of levator muscle. Five patients had Bells palsy with exposure keratopathy, four patients had persistent epithelial defect, and one had neurotrophic ulcer. The median age at presentation was 30 years. Median dose of Botulinum toxin injection was 12.5 U (range 10-15 U). The mean palpebral fissure height of 9 mm (SD+/-2.1 mm) before injection, reduced to 2.8 mm (SD+/-1.9 mm) at 1-week post-injection. More than 50% reduction in palpebral fissure height was seen in nine out of 10 eyes (90%, 95% CI 71.4-100%) at 1 week, seven of nine eyes (77.8%, 95% CI 50.6-100%) at 2 weeks, and two of nine eyes (22.2%, 95% CI 0-49.4%) at 4 weeks, and returned to pretreatment level after mean duration of 9.2 weeks (range 5-16 weeks). Superior rectus underaction was not noted in any of the patient (95% CI 0-30%). Corneal pathology improved in all cases. CONCLUSION: Anterior chemodenervation of levator palpebrae superioris with Botulinum toxin type A (Botox) induces significant temporary ptosis and aids in corneal healing. Anterior placement of the toxin injection may avoid superior rectus underaction.

Primary orbital aspergilloma of the exenterated orbit in an immunocompromized patient.

Aspergilloma is a fungal ball that usually forms in a preformed stationary cavity, mostly in lung and paranasal sinuses. We report a rare case of primary orbital Aspergilloma following exenteration for an invasive ocular surface squamous neoplasia, clinically mimicking a recurrence of the tumor. The fungal ball showed the presence of conidiophores with a globular head and a complete row of uni and biserrate phialides, suggestive of Aspergillus flavus species. The exposure to air in the orbit, possibly promoted the formation of conidiophores, which are normally seen when the organism is located in air cavities.

Botulinum toxin in ophthalmic plastic surgery.

Botulinum toxin chemodenervation has evolved greatly over the past 30 years since its introduction in the 1970s for the management of strabismus. Among ophthalmic plastic surgeons, botulinum toxins are often used as the first line treatment for facial dystonias. These toxins are also efficacious for the temporary management of various other conditions including keratopathies (through so called chemo-tarsorraphy), upper eyelid retraction, orbicularis overaction-induced lower eyelid entropion, gustatory epiphora, Frey's syndrome, and dynamic facial rhytids such as lateral canthal wrinkles (crow's feet), glabellar creases and horizontal forehead lines. This article describes the pharmacology, reconstitution techniques and common current applications of botulinum toxins in ophthalmic plastic surgery.

Circumscribed choroidal hemangioma: clinical manifestations and factors predictive of visual outcome in 200 consecutive cases.

PURPOSE: To review the clinical features and management of circumscribed choroidal hemangioma and determine factors predictive of poor visual outcome. DESIGN: Retrospective consecutive noncomparative interventional case series. PARTICIPANTS: Two hundred consecutive patients with circumscribed choroidal hemangioma. MAIN OUTCOME MEASURES: The main outcome measures were analyzed in 155 patients with follow-up of at least 3 months and included complete resolution of subretinal fluid, worsening of visual acuity (more than 2 Snellen lines), and poor final visual acuity (20/200 or worse). RESULTS: The patients were seen at a mean age of 45 years with symptoms of decreased visual acuity (81%), visual field defect (7%), metamorphopsia (3%), floaters (2%), progressive hypermetropia (1%), photopsia (1%), pain (1%), and no symptoms (6%). The referring diagnoses were choroidal hemangioma (29%), choroidal melanoma (29%), choroidal metastasis (9%), retinal detachment (6%), central serous chorioretinopathy (5%), and others. The tumor had a median base of 6.0 mm and median thickness of 3.0 mm. Secondary retinal detachment in the foveal region was present in 81% of the patients. Initial treatment included observation (51%), laser photocoagulation (44%), plaque radiotherapy (4%), external beam radiotherapy (1%), surgical repair of retinal detachment (1%), and enucleation for painful neovascular glaucoma (1%). Kaplan-Meier estimates revealed complete resolution of subretinal fluid in 60% patients at 5 years and 76% patients at 10 years follow-up. By multivariable analysis, clinical factors predictive of complete resolution of subretinal fluid included shorter duration of symptoms (P = 0.03) and inferior quadrant location of tumor (P = 0.001). At initial presentation, 82 of 155 (53%) patients had poor visual acuity (20/200 or worse), and 73 of 155 (47%) patients had good to moderate visual acuity (20/100 or better). Of those 82 patients with poor initial vision, poor final vision was found in 54% at 5 years and 80% at 10 years. Of the 73 patients with good to moderate initial vision, poor final vision was found in 12% at 5 years and 43% at 10 years. By multivariable analysis, clinical factors predictive of poor final visual acuity included poor initial visual acuity (P < 0.001), failure of previous laser photocoagulation before referral (P = 0.01), and tumor management with observation after referral (P = 0.02). Worsening of visual acuity (by more than 2 Snellen lines) was observed in 8% at 5 years and 28% at 10 years of those 82 patients who were initially seen with poor vision. Worsening of visual acuity was found in 10% at 5 years and 30% at 10 years of those 73 patients who initially were seen with good to moderate vision. CONCLUSIONS: Circumscribed choroidal hemangioma is a rare intraocular tumor. In 38% of cases, this tumor is initially misinterpreted before referral as choroidal melanoma or metastasis. Visual acuity is poor in more than 60% of patients at 10 years, despite successful control of associated subretinal fluid in 76% patients.

Clinical spectrum of primary ophthalmic rhabdomyosarcoma.

PURPOSE: To review the clinical presentation, histopathologic analysis, management, and ocular and systemic outcome of rhabdomyosarcoma affecting the ocular region. DESIGN: Retrospective, noncomparative, consecutive, interventional case series. PARTICIPANTS: Thirty-three consecutive patients with primary ophthalmic involvement of rhabdomyosarcoma from a single tertiary care center specializing in ocular oncology. MAIN OUTCOME MEASURES: Final visual acuity, treatment complications, local recurrence, and distant metastasis. RESULTS: The mean age at presentation was 10 years (median, 7 years; range, 1 month-68 years). At presentation, 8 patients (24%) were older than age 10 years and 4 patients (12%) were older than 20 years. The rhabdomyosarcoma was primarily located in the orbit in 25 cases (76%), conjunctiva in 4 cases (12%), eyelid in 1 case (3%), and uveal tract in 3 cases (9%). Symptoms or signs related to the tumor were present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 patients (21%), and blepharoptosis in 6 patients (18%). The initial diagnosis before referral to us included rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 cases (15%), orbital or preseptal cellulitis in 5 cases (15%), idiopathic orbital inflammatory pseudotumor in 4 cases (12%), and others. Using the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols, the tumor was classified as group I in 4 cases (12%), group II in 12 cases (36%), group III in 16 cases (48%), and group IV in 1 case (3%). Treatment included surgical debulking and various regimens of chemotherapy and radiotherapy for the periocular tumors and enucleation for the three intraocular tumors. Local tumor recurrence was detected in 6 patients (18%). Orbital exenteration was necessary for tumor recurrence in 2 cases (6%). Long-term visual outcome of the 28 patients who maintained their globe was 20/20 to 20/40 in 11 patients (39%), 20/50 to 20/100 in 5 patients (18%), and 20/200 to no light perception in 12 patients (43%). Regional lymph node metastasis was detected in 2 patients (6%), one at initial visit and one after therapy. Distant metastasis occurred in 2 patients (6%), one detected at initial visit and one after therapy. With mean follow-up of 8.3 years, tumor-related death occurred in 1 patient (3%). CONCLUSIONS: Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. After treatment, local tumor recurrence occurs in 18%, metastasis in 6%, and death in 3%.

Intraocular surgery after treatment of retinoblastoma.

OBJECTIVES: To analyze the results of intraocular surgery in patients treated for retinoblastoma and to assess the ocular and systemic outcomes. DESIGN: Retrospective noncomparative case series. PATIENTS: Forty-five consecutive patients who underwent an introcular surgery after treatment for retinoblastoma. MAIN OUTCOME MEASURES: (1) Recurrence of retinoblastoma, (2) need for enucleation, and (3) systemic metastasis. Overall outcome was defined as favorable in the absence of any of these measures and unfavorable in the presence of 1 or more. RESULTS: Thirty-four patients (76%) underwent a single procedure of cataract surgery, a scleral buckling procedure, or pars plana vitrectomy and 11 (24%) underwent a combination of 2 or more surgical procedures. In all, 16 patients (36%) achieved final visual acuity better than 20/200. Unfavorable outcomes included recurrence of retinoblastoma in 14 patients (31%), enucleation in 16 (36%), and systemic metastasis in 3 (7%). Five patients (20%) who underwent cataract surgery, 5 (63%) who underwent a scleral buckling procedure, and 9 (75%) who underwent pars plana vitrectomy manifested an unfavorable outcome. The median interval between completion of treatment for retinoblastoma and intraocular surgery was 26 months in patients with a favorable outcome vs 6 months in those with an unfavorable outcome. CONCLUSIONS: Intraocular surgery after treatment for retinoblastoma may be justified in certain exceptional clinical situations. Cataract surgery is safe and effective in most cases. However, the need for a scleral buckling procedure and pars plana vitrectomy may be associated with a higher risk for recurrence of retinoblastoma, enucleation, and systemic metastasis, and a cautious approach is warranted.

Experience with eyelid-sparing orbital exenteration: the 2000 Tullos O. Coston Lecture.

PURPOSE: To provide data on types of lesions that require orbital exenteration and discuss our experience with exenteration, emphasizing the eyelid-sparing technique. METHODS: The records were reviewed on all patients who underwent orbital exenteration by the senior author and his colleagues. The underlying disease, method of exenteration, healing times, complications, cosmetic outcome, local tumor control, and patient survival were analyzed. RESULTS: Of 56 orbital exenterations, 47 were done by eyelid-sparing technique and 9 by eyelid-sacrificing technique. The 9 tumors of eyelid origin that required exenteration included basal cell carcinoma (4), sebaceous gland carcinoma (3), squamous cell carcinoma (1), and melanoma (1). Four of the eyelid lesions were managed by the eyelid-sparing technique and 5 by the eyelid-sacrificing method. The 24 tumors of conjunctival origin included melanoma (20) and squamous cell carcinoma (4). The eyelid-sparing method was used in 22 of the 24 cases. The 10 primary orbital lesions included melanoma (3), malignant lacrimal gland tumor (3), a rhabdoid tumor, meningioma, Wegener's granulomatosis, and aspergillosis. The eyelid-sparing method was used in 8 of the 10 cases. Among intraocular tumors were uveal melanomas (10) and retinoblastoma (1). The eyelid sparing method was used in all 11. With the eyelid-sparing technique, the mean time from exenteration to wound healing and readiness for a prosthesis was 4 weeks (median, 2 weeks; range, 2 to 24 weeks). With the eyelid-sacrificing technique, the mean time was 68 weeks (median, 34 weeks; range, 2 to 340 weeks). Complications were few and cosmetic results were satisfactory. Local tumor control with exenteration was achieved in 53 of 56 cases, but metastasis occurred in 11 of 20 cases of conjunctival melanoma (55%) and in 6 of 10 patients with uveal melanoma (60%). Metastasis has developed in none of the 3 patients with malignant epithelial tumors of lacrimal gland origin. CONCLUSIONS: Orbital exenteration is an appropriate procedure for orbital involvement by unresectable malignant tumors of the eyelids, conjunctiva, intraocular structures, and orbit. In most of these cases, including about half of tumors originating in the eyelids, an eyelid-sparing exenteration can be used to achieve more rapid healing.

Ring melanoma of the anterior chamber angle: a report of fourteen cases.

PURPOSE: Ring melanoma of the anterior chamber angle is a rare variant of uveal melanoma that manifests as circumferential, flat tumor growth predominantly confined to the trabecular meshwork and other anterior chamber angle structures. We report the clinical and histopathological features, management, and prognosis of ring melanoma of anterior chamber angle. METHODS: A retrospective review of clinical features, management, histopathologic features, and prognosis was performed. Of 8800 patients with uveal melanoma examined on the Oncology Service at Wills Eye Hospital over a 25-year period, only 14 patients (0.2%; 14 eyes) were classified clinically as ring melanoma of the anterior chamber angle. In no case was there appreciable involvement of the iris or ciliary body on clinical examination. RESULTS: The patients were followed elsewhere for a mean of 8 months before intraocular malignancy was suspected. Upon referral to the Oncology Service, the intraocular pressure was greater than 22 mm Hg in the affected eye in all patients (mean, 36 mm Hg; median, 35 mm Hg; range, 24 to 48 mm Hg), and all patients were using at least two glaucoma medications. The melanoma infiltrated the anterior chamber angle for a mean of 10 clock hours (range, 8 to 12 clock hours). Tumor management consisted of enucleation in 13 cases and plaque radiotherapy in one case. Histopathologic examination revealed epithelioid cell type melanoma in one case, mixed cell type in nine, and spindle cell type in four. Of the 13 patients who underwent enucleation, tumor cells were found within the Schlemm canal in all cases. Five cases displayed invasion of the intrascleral canals, and three showed episcleral invasion. With the exception of two recent cases, 12 patients were followed for a mean of 64 months (median, 47 months; range, 23 to 225 months). Distant metastasis to the liver was detected in three of 12 patients (25%) after a mean follow-up of 78 months (median, 70; range, 37 to 128 months). CONCLUSIONS: Ring melanoma of the trabecular meshwork and angle structures is a rare variant of diffuse uveal melanoma. It often masquerades as unilateral glaucoma and can be difficult to recognize clinically unless careful comparative gonioscopy is performed. Despite the relatively small tumor volume, life prognosis is guarded with distant metastasis in 25% at mean 6 years follow-up.

Sclerochoroidal calcification: clinical manifestations and systemic associations.

BACKGROUND: Sclerochoroidal calcification is an unusual ocular condition that is believed to be idiopathic in most cases. OBJECTIVES: To describe the clinical manifestations of sclerochoroidal calcification and to investigate its possible systemic associations. METHODS: This noncomparative consecutive case series included patients diagnosed as having sclerochoroidal calcification based on clinical characteristics and diagnostic test findings. We analyzed the demographic, systemic, and ocular features of 27 such patients. Systemic evaluation included tests for calcium-phosphorus metabolism in 19 patients and renal tubular hypokalemic metabolic alkalosis syndromes (Bartter or Gitelman syndrome) in 13. RESULTS: All the patients were asymptomatic older (mean age, 70 years) white individuals, incidentally noted as having a choroidal lesion on routine examination. Among 38 eyes, the main referral diagnoses were choroidal metastasis in 10 eyes (26%), choroidal melanoma in 8 (21%), and choroidal nevus in 4 (11%). Sixteen patients (59%) had unilateral clinical findings, while 11 (41%) had bilateral. The Snellen visual acuity was 20/50 or better in 37 eyes (97%). Cogan scleral plaque was visible anterior to the insertion of horizontal rectus muscles in 10 eyes (26%). Among 77 foci, there were a mean of 2 foci of sclerochoroidal calcification in each eye, 41 yellow (53%), 32 yellow-white (42%), 2 white (3%), and 2 orange (3%), measuring a mean 2.6 mm in diameter and 1.1 mm in thickness. The most common locations were postequatorial in 45 (58%), along the temporal vascular arcades in 30 (39%), and in the superotemporal quadrant in 43 (56%). A-scan and B-scan ultrasonography revealed dense echoes compatible with calcium, with orbital shadowing. All the lesions remained stable in size and configuration during a mean follow-up of 38 months. One patient developed a choroidal neovascular membrane over the area of sclerochoroidal calcification. Investigations for abnormal calcium-phosphorus metabolism in 19 patients revealed primary hyperparathyroidism in 1 patient (5%). Clinical and biochemical evaluation of 13 patients demonstrated hypomagnesemia in 6 (46%). Four patients (31%) met the criteria for the diagnosis of Gitelman syndrome. CONCLUSIONS: Sclerochoroidal calcification usually manifests as multiple discrete yellow placoid lesions in the midperipheral fundus of asymptomatic older white individuals. Although most cases may be idiopathic in nature, some patients may have underlying systemic disorders involving abnormal calcium-phosphorus metabolism or renal tubular hypokalemic metabolic alkalosis syndromes. All patients with sclerochoroidal calcification should be tested for these treatable systemic associations.

Primary intraepithelial sebaceous gland carcinoma of the palpebral conjunctiva.

Sebaceous gland carcinoma usually arises from meibomian or Zeis glands deep within the eyelid, but it can rarely arise within the conjunctival epithelium without a deep component. We describe a woman with a history of chronic blepharoconjunctivitis unresponsive to topical medications. Examination disclosed confluent papillary hypertrophy of the upper palpebral conjunctiva and deposits of white flaky material. Tarsoconjunctival punch biopsy revealed intraepithelial sebaceous gland carcinoma. Management consisted of frozen section-controlled complete tumor excision with removal of the entire posterior lamella of the right upper eyelid, cryotherapy to the margins, and reconstruction. Histopathologic analysis confirmed primary sebaceous gland carcinoma localized to the conjunctival epithelium without involvement of underlying meibomian or Zeis glands or the caruncle. Patients with unexplained chronic unilateral blepharoconjunctivitis or papillary hypertrophy of the palpebral conjunctiva should be considered for biopsy to rule out neoplasia, even when there is no sign of an underlying eyelid mass.

Classification and management of Coats disease: the 2000 Proctor Lecture.

PURPOSE: To review the methods and results of management in a large series of patients with Coats disease, to determine risk factors for poor visual outcome and enucleation, and to propose a practical classification of Coats disease. METHODS: In a retrospective consecutive series in 150 patients, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with management, including observation, laser photocoagulation, cryotherapy, and various techniques of retinal detachment surgery and enucleation. The anatomic outcome, complications of treatment, visual results, and reasons for enucleation were tabulated. Factors predictive of poor visual outcome (20/200 or worse) and enucleation were determined using Cox proportional hazards regression models. Based on these observations, a staging classification of Coats disease, applicable to treatment selection and ocular prognosis, is proposed. RESULTS: In 117 patients (124 eyes) with a mean follow up of 55 months (range, 6 months to 25 years) primary management was observation in 22 eyes (18%), cryotherapy in 52 (42%), laser photocoagulation in 16 (13%), various methods of retinal detachment surgery in 20 (17%), and enucleation in 14 (11%). Anatomic improvement or stability was achieved in 76% of eyes, and final visual acuity was 20/50 or better in 17 eyes (14%), 20/60 to 20/100 in eight (6%), 20/200 to finger counting in 30 (24%), and hand motion to no light perception in 49 (40%) Enucleation was ultimately necessary in 20 eyes (16%). Risk factors predictive of poor visual outcome (20/200 or worse) included postequatorial (P =.01), diffuse (P =.01), or superior (P =.04) location of the telangiectasias and exudation, failed resolution of subretinal fluid after treatment (P =.02), and presence of retinal macrocysts (P =.02). The main risk factors for enucleation were elevated intraocular pressure (greater than 22 mm Hg; P less than or equal to.001) and iris neovascularization (P less than or equal to.001). Coats disease was classified into stage 1, telangiectasia only; stage 2, telangiectasia and exudation (2A, extrafoveal exudation; 2B, foveal exudation) stage 3, exudative retinal detachment (3A, subtotal; 3B, total); stage 4, total detachment and secondary glaucoma; and stage 5, advanced end-stage disease. Poor visual outcome (20/200 or worse) was found in 0% of eyes with stage 1, 53% with stage 2, 74% with stage 3, and 100% of stages 4 and 5 Coats disease. Enucleation was ultimately necessary in 0% of stages 1 and 2, 7% of stage 3, 78% of stage 4, and 0% of stage 5 disease. CONCLUSIONS: Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in 76% of eyes. However, poor visual outcome of 20/200 or worse commonly results. Patients who present with stages 1 to 3 Coats disease have the best visual prognosis, and patients with stages 4 and 5 have a poor visual prognosis.

Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture.

PURPOSE: The purpose of this report is to review the clinical variations and natural course of Coats disease, using strict diagnostic guidelines. METHODS: In a retrospective, consecutive series, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with the clinical features, complications, and diagnostic approaches to Coats disease. RESULTS: In 150 consecutive patients (158 eyes), Coats disease was diagnosed at a median age of 5 years (range, 1 month to 63 years), occurred in 114 males (76%), and was unilateral in 142 patients (95%). There was no predilection for race or laterality. The most common referral diagnoses were Coats disease in 64 cases (41%) and retinoblastoma in 43 (27%). The first symptom or sign was decreased visual acuity in 68 cases (34%), strabismus in 37 (23%), leukocoria in 31 (20%), and 13 patients (8%) were asymptomatic. Visual acuity at presentation was 20/200 to no light perception in 121 eyes (76%). The anterior segment was normal in 142 eyes (90%). The retinal telangiectasia involved the midperipheral or peripheral fundus in 156 of the 158 eyes (99%) and was restricted to the macular area in two eyes (1%); involved mainly the temporal fundus in 66 eyes (42%), inferior fundus in 41 eyes (26%), and more than one sector in 34 eyes (22%). Retinal exudation was present in all 12 clock hours in 86 eyes (55%) and six or more clock hours in 115 eyes (73%). There was a total retinal detachment in 74 eyes (47%) and neovascular glaucoma in 12 (8%). Retinal macrocysts were present in 18 eyes (11%), a vasoproliferative tumor in nine eyes (6%) and retinal neovascularization in four eyes (3%). Fluorescein angiography in 49 of the 158 eyes (37%) disclosed early hyperfluorescence of the telangiectasias and macular edema in 18 of eyes (37%). Ultrasonography typically showed a retinal detachment but no solid mass. CONCLUSIONS: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment. The clinician should follow strict criteria in making the diagnosis, to avoid confusing Coats disease with other forms of exudative retinopathy.

Basal cell carcinoma of the eyelid associated with Gorlin-Goltz syndrome.

PURPOSE: To describe the ophthalmic and systemic features in a series of patients initially seen with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Of 105 consecutive patients with eyelid basal cell carcinoma managed at an Ocular Oncology Center between January 1973 and December 1999, four patients with Gorlin-Goltz syndrome were identified. METHODS: The ophthalmic and systemic features, management, and outcome of patients with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome were analyzed. The published literature on Gorlin-Goltz syndrome, specifically related to genetics, systemic features, ophthalmic associations, and prophylactic management strategies, was reviewed. MAIN OUTCOME MEASURES: Response of the eyelid basal cell carcinoma to treatment and the final systemic condition were the main outcome measures. RESULTS: All four patients had a family history of Gorlin-Goltz syndrome. The systemic manifestations included multiple basal cell carcinomas in all the patients, frontal bossing or increased occipitofrontal circumference in three patients, palmar pits in two patients, odontogenic keratocyst in one patient, ectopic calcification in one patient, and bifid rib in one patient. The mean age at the detection of the first basal cell carcinoma was 30 years (range, 16-38 years). All four patients had multiple basal cell carcinomas on the face and elsewhere. The eyelid basal cell carcinoma was advanced with orbital infiltration in three patients, one of whom opted for palliative radiotherapy, whereas the other two underwent orbital exenteration. The fourth patient, who had localized recurrent basal cell carcinoma in the upper eyelid, was treated with excision and eyelid reconstruction. At the final follow-up (mean, 41 months), eyelid basal cell carcinoma was cured in three patients and stable in one patient. No patient had life-threatening sequelae of Gorlin-Goltz syndrome. CONCLUSIONS: Gorlin-Goltz syndrome is a rare autosomal dominant cancer predisposition syndrome that may be associated with eyelid basal cell carcinoma. The associated systemic findings may be a clue to the diagnosis of this condition. It is important to recognize Gorlin-Goltz syndrome when a patient has multiple basal cell carcinomas or when a young patient with eyelid basal cell carcinoma is seen by an ophthalmologist, because lifelong monitoring is essential for patient management.

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.

The association of unilateral congenital glaucoma and congenital lower lid entropion: causal or casual?

This case documents unilateral congenital glaucoma associated with congenital lower lid entropion. A 2-year-old female infant was referred for evaluation and treatment of right-side buphthalmos caused by congenital glaucoma associated with bilateral congenital lower lid entropion that was prominent on the right side and present at birth. Examination disclosed a lower eyelid entropion of the right side that was treated surgically by reinserting the disinserted retractor aponeurosis to anterior inferior tarsal border. After three weeks, the patient was successfully treated with primary combined trabeculotomy-trabeculectomy for congenital glaucoma. The entropion of the left lower lid was asymptomatic and did not require any surgery. Buphthalmos caused by congenital glaucoma may be associated with congenital lower lid entropion and the association may be causal or coincidental.

Extramedullary plasmacytoma confined to the choroid.

PURPOSE: To report a case of an extramedullary plasmacytoma confined to the choroid. METHODS: Interventional case report with cytopathologic correlation. RESULTS: A 76-year-old asymptomatic woman presented with 2 globular orange and amelanotic choroidal lesions in the right eye. Fine needle aspiration biopsy of one of the choroidal lesions followed by immunocytochemical studies revealed monoclonal plasma cells, diagnostic of plasmacytoma. Systemic evaluation was negative, except for increased serum gamma globulin with immunoglobulin kappa spike on electrophoresis. The patient was managed by ocular external beam radiotherapy. At 9 years follow-up, she was free of local disease and never developed multiple myeloma. CONCLUSION: Extramedullary plasmacytoma confined to the choroid is rare. It may present simulating other common amelanotic choroidal lesions. Fine needle aspiration biopsy and proper systemic evaluation are critical in establishing the diagnosis.