A pilot study of the use of objective structural clinical examinations for the assessment of ophthalmology education.

PURPOSE: A pilot study was carried out to evaluate the practicality, reliability, and validity of an objective structured clinical examination (OSCE) for assessing the clinical skills and abilities of specialists in ophthalmology. METHODS: Ten unfolded OSCE style, criterion referenced questions were asked to nine candidates to assess their clinical skills and abilities, as opposed to subject knowledge. Candidate and assessor reactions to the examination process were monitored and analyzed using participant observation and questionnaires administered immediately after the event. Relevant statistical techniques were applied to the results. RESULTS: A total of 89% of candidates passed the examination, with the pass boundary set at 70%. Candidates revealed themselves more successful in meeting clinical skill criteria (mean 77%) than clinical ability criteria (mean 72%). Candidates, assessors, and observers all expressed the view that the OSCE pilot had been a successful way of assessing clinical skills and abilities. CONCLUSIONS: OSCE style assessment is an effective and efficient means of assessing skills and abilities in clinical ophthalmology education.

Papillary adenocarcinoma of the ciliary body simulating retinoblastoma.

PURPOSE: To report a 2 1/2-year-old boy who had papillary adenocarcinoma of the ciliary body that simulated retinoblastoma. METHOD: Initially treated for congenital glaucoma, the patient was referred with a white mass involving the iris, chamber angle, and ciliary body. RESULTS: Enucleation of the right eye, which was initially diagnosed as retinoblastoma, showed a papillary adenocarcinoma of the ciliary body epithelium involving the posterior chamber, iris, anterior chamber, and trabeculum. CONCLUSION: Adenocarcinoma of the ciliary body must be included in the differential diagnosis of tumors originating from the ciliary body in young children.

Septo-optic dysplasia associated with bilateral complex microphthalmos.

An 8-month-old girl was examined because of corneal clouding and microphthalmos. The fundi of both eyes could not be visualized because of corneal clouding. Orbital and cranial computerized tomographic scanning and magnetic resonance imaging demonstrated bilateral microphthalmos and presumed retinal dysplasia, hypoplasia of the optic nerves and chiasm, agenesis of the septum pellucidum, thinning of corpus callosum, and a normal pituitary infundibulum. Cerebral cortex and white matter were unremarkable. Other ocular malformations were anterior segment dysgenesis in the right eye and congenital cataract or lens abnormality in the left eye. Endocrine studies revealed normal serum hormone levels. There were no colobomatous lesions and systemic anomalies suggestive of a coloboma syndrome. This case represents the rare association of septo-optic dysplasia with complex microphthalmos.

Focal dermal hypoplasia (Goltz's syndrome).

A 17-year-old female with Goltz's syndrome was examined because of visual acuity loss in her right eye. Ocular examination revealed microcornea, iris, choroid and optic disc coloboma in the right eye. There were several erthematous and hyperpigmented areas on the body. Magnetic resonance (MR) imaging of the orbits and brain demonstrated right optic nerve hypoplasia and diffuse cortical and cerebellar atrophy. Skeletal manifestations were short stature, scoliosis, syndactyly, clinodactyly, and osteopathia striata. Dental defects included hypodontia, developmental defects, and malocclusion. There were multiple papillomatous lesions on the lids and perioral skin and the nose was asymmetric. Her mental development was apparently normal. She had left bifid ureter and renal pelvis, scant hair on the pubic and genital region, and poor breast development. Histopathologic examination of the biopsy taken from a characteristic skin lesion revealed attenuated epidermis, hypoplastic dermis, and subcutaneous fat close to epidermis. Immunofluorescence staining was negative for IgG, IgM, IgA, C3, C4, fibrin, and albumin. Ultrastructural examination showed that no viral particles were present. Prometaphase chromosome analysis revealed a normal 46, XX female karyotype. Cortical and cerebellar atrophy can occur in a patient with Goltz's syndrome.

Retinoblastoma in Turkey: diagnosis and clinical characteristics.

Retinoblastoma (RB) is the most frequent malignant intraocular tumor in childhood. Six hundred and thirty-six cases with 831 RB-affected eyes were diagnosed and treated in our specialist center between 1963-1994. The diagnosis was made by histopathologic examination in 617 cases and clinically in 19 cases. Four hundred and forty-one (69.3%) cases were unilateral and 195 (30.7%) were bilateral. Two hundred and sixty-eight (42.1%) were females and 368 (57.9%) were males. The youngest patient was 20 days old and the oldest was 16 years old at the time of diagnosis (mean: 2.2 years). In thirty-four (5.3%) cases, a family history of RB was present. Ten of these cases were unilateral and 24 were bilateral. The most frequent presenting signs were leukocoria (394 cases, 61.9%), buphthalmos (92 cases, 14.5%), and strabismus (68 cases, 10.7%). The referring initial diagnoses were correct in 519 (81.6%) cases and false-negative in 117 (18.4%) cases. The most frequent initial false-negative diagnoses of the referring physicians were buphthalmos (43 cases, 6.8%), endophthalmitis (37 cases, 5.8%), and retinal detachment (12 cases, 1.9%). Apart from these 636 cases, there were 29 false-positive RB diagnoses during the same study period for which enucleation was performed. False-positive diagnoses included endophthalmitis (9 cases), retinal dysplasia (6 cases), retinal detachment (5 cases), vitreous hemorrhage (4 cases), Coats' disease (4 cases), and toxocariasis (one case). Ancillary testing for metastasis was carried out in all cases with newly diagnosed retinoblastoma. Five hundred and ninety-eight (72%) eyes had intraocular disease and 233 (28%) had extraocular spread. Of these 233 RBS, 58 had systemic disease. Fifty-two out of 58 tumors showing systemic involvement had either optic nerve or extrascleral extension at the histopathologic examination of enucleation material. The remaining six eyes had intraocular Class IV-V RB.

Colour Doppler imaging of the orbital vasculature in Graves' disease with computed tomographic correlation.

AIMS: To evaluate alterations in orbital blood flow parameters and their correlations with extraocular muscle enlargement, proptosis, and intraocular pressure in patients with Graves' disease. METHODS: In this multicentre study blood flow parameters in the ophthalmic artery, superior ophthalmic vein, central retinal artery and vein were determined by colour Doppler imaging in 111 patients with Graves' disease in two groups (A and B) and 46 normal control subjects. Group A consisted of 42 patients with Graves' disease without ophthalmopathy; group B of 69 patients with Graves' disease with ophthalmopathy as detected by orbital computed tomographic scanning. RESULTS: Peak systolic and end diastolic velocities in the ophthalmic artery, peak systolic velocity in the central retinal artery, and maximal and minimal velocities in the central retinal vein in patients in group B were statistically significantly higher than those in group A and the normal controls, whereas maximal and minimal velocities in the superior ophthalmic vein in patients in group B were statistically significantly lower than those in group A and the control subjects. Peak systolic and end diastolic velocities in the ophthalmic artery, peak systolic velocity in the central retinal artery, and maximal and minimal velocities in the central retinal vein also correlated with the sum of all extraocular muscle diameters in group B (r > or =0.31, p< or =0.021). Blood flow parameters had no consistent correlation with proptosis or intraocular pressure (p>0.05). No statistically significant difference was found in resistivity indices between the groups (p>0.05). Reversed blood flow was noted in nine (13%) superior ophthalmic veins in group B. CONCLUSION: Orbital blood flow velocities are altered in patients with Graves' ophthalmopathy and may be detected by colour Doppler imaging. Some of these changes also correlate with the enlargement of extraocular muscles. The increased blood flow velocities in arteries may be secondary to orbital inflammation.

Malignant melanoma of the optic nerve head in a case of oculodermal melanocytosis.

Malignant melanoma of the uveal tract, orbit, and brain have been reported to occur in patients with oculodermal melanocytosis. A 60-year-old Caucasian man with oculodermal melanocytosis developed a malignant melanoma of the optic nerve head in the left eye. This case is the first reported example of a malignant melanoma developing in the optic nerve associated with oculodermal melanocytosis. After presentation the patient refused surgery for 19 months and the progression of the tumour necessitated an exenteration of the orbit.

Quantitative CT of the orbit in Graves' disease.

In order to determine the value of quantitative CT of the orbit in patients with Graves' disease, we clinically examined 174 orbits of 87 patients with Graves' disease and evaluated them by CT in respect to the density and size of the extraocular muscles, the globe position and the width of the optic nerve-sheath complex. We also determined the normal ranges for density of extraocular muscles in 200 normal orbits of 100 patients for comparison. Normal ranges for the density of extraocular muscles were (mean +/- 2 SD) medial rectus, 28-63 HU; lateral rectus, 24-78 HU; inferior rectus, 20-64 HU; superior muscle group, 28-62 HU. 51 of 77 (66%) patients with Graves' disease had extraocular muscle density changes. Some extraocular muscles showed fatty infiltration on CT. 50 of 87 (57%) patients had at least one enlarged extraocular muscle, 47 (54%) patients had exophthalmos and 59 (68%) patients had either exophthalmos and/or extraocular muscle enlargement. A diagnosis of Graves' ophthalmopathy was made in 69 of 87 (79%) patients using CT and in 50 (57%) patients by clinical examination. We conclude that quantitative CT imaging of the orbit with evaluation of the size and density values of extraocular muscles and the globe position may be very helpful in detecting ophthalmopathy in patients with Graves' disease.

Causes of enucleation: a clinicopathological study.

BACKGROUND: Enucleation is an approach used for unresponsive end-stage ocular disease often resulting in blind, painful or cosmetically unacceptable eyes. METHODS: We reviewed the clinicopathological data on 3506 enucleations performed over a 50-year period, 1945-1995. Histopathological data were divided into eight groups according to the causes leading to enucleation: trauma, phthisis, corneal disease, inflammation, vitreoretinal disease, glaucoma, tumors and infections. RESULTS: The study considered 3506 enucleated eyes of 3482 patients, 2467 (70.8%) males and 1011 (29.1%) females (4 sex unspecified). The z-test showed there were significantly more enucleations in males for phthisis (p < 5.05), infections (p < 0.01), trauma (p < 0.01) and inflammation (p < 0.01) and more enucleations for tumors in females (p < 0.01). There were no differences between males and females with regard to enucleations for glaucoma, vitreoretinal and corneal diseases (p > 0.05). The 0-9 years age group was most frequently affected, accounting for 29.7% of the cases. Patients aged less than 30 years constituted 53.6% of all enucleations. The primary or underlying causes leading to enucleation were tumors (1185 eyes, 33.8%), phthisis (587 eyes, 16.7), glaucoma (561 eyes, 16.0%), vitreoretinal diseases (320 eyes, 9.1%), infections (259 eyes, 7.4%), corneal disease (229 eyes, 6.5%), trauma (209 eyes, 6.0%) and inflammation (156 eyes, 4.4%). Time trends in enucleating eyes with different causes showed the number of enucleations for phthisis, infections, corneal diseases, trauma and inflammations had dropped during the ten-year period 1986-1995 compared to 1976-1985 (z-test, p < 0.01). There were no real changes in enucleations for glaucoma and vitreoretinal diseases and there was an increase in the number of enucleations for tumors (p < 0.01). CONCLUSIONS: Improved diagnostic and therapeutic methods, widespread use of photocoagulation in vascular disorders and vitreoretinal surgery in traumas, effective antimicrobial treatment, increasing use of corticosteroids and immunosuppressants, have contributed to the decreasing frequency of enucleation. Tumor patients generally presented late with advanced tumors totally filling the eye, not salvageable by other non-invasive treatment methods. Prompt diagnosis of intraocular malignant tumors (retinoblastoma and malignant melanoma) may reduce the need for enucleation.

Comparative efficacy of beta-irradiation and mitomycin-C in primary and recurrent pterygium.

PURPOSE: To determine the efficacy and safety of mitomycin-C as adjunctive treatment and to compare this drug with beta irradiation with strontium-90 after surgical excision of primary and recurrent pterygia. MATERIALS AND METHODS: The study group consisted of 193 patients with primary and recurrent pterygia who underwent surgical excision with the bare sclera technique. They were divided into two groups according to the type of adjunctive treatment. In group I, 130 patients (141 eyes, 67.8%) were treated with beta irradiation with Sr-90 doses of 1000-7000 cGy. In group II 63 patients (67 eyes, 32.2%) received topical mitomycin-C at a concentration of 0.02% four times daily for one week postoperatively. Recurrence rates, complications and efficacy of these treatments were compared with the chi-square of Fisher's exact test. RESULTS: The recurrence rates were 6.4% in group I after a mean postoperative follow-up of 89 months and 17.9% in group II after a mean follow-up of 14.9 months. Recurrence, rates and complications were higher in group II and the difference was significant (p<0.05, p<0.001). Life-table analysis showed a success rate of 93.6% for Sr-90 and 81.9% for the mitomycin-C, the difference being significant (p<0.005). CONCLUSIONS: Beta irradiation with Sr-90 after surgical excision was more effective than topical mitomycin-C in patients with primary and recurrent pterygium in terms of recurrence rates, and safer in terms of complications.

Superior oblique muscle palsy in a patient with orbital dermoid cyst.

We describe the clinical and radiologic findings and surgical outcome of an orbital dermoid cyst causing a superior oblique muscle palsy in a child. Superior oblique muscle palsy in childhood is most often congenital. Less common causes are trauma, vascular lesions, neoplasms, and infections.(1,2) The most common orbital lesions in children are dermoid and epidermoid cysts.(3-5) A dermoid cyst at the region of trochlea is suspected as the cause of superior oblique muscle palsy in our case. This unusual presentation of a dermoid cyst has not been reported previously.

Metastatic orbital tumors.

A review was made of the clinical data of 28 patients with metastatic orbital disease diagnosed in our clinic between 1972 and 1993. Fifteen were men and 13 were women. The average age at diagnosis was 58.7 years. The right orbit was involved in 12 and the left orbit in 14 cases. Two cases had bilateral orbital involvement. Breast carcinoma was the most frequent tumor (8 of 28, 28.6%) followed by neuroblastoma (7 of 28, 25.0%), lung carcinoma (6 of 28, 21.4%), prostate carcinoma (3 of 28, 10.7%), gastrointestinal carcinoma (2 of 28, 7.1%), renal cell carcinoma and thyroid carcinoma (1 of 28, 3.6% each). Proptosis (67.9%), motility disturbance (57.1%) and mass (50.0%) were the three most common presenting signs. Enophthalmos was noted in two cases with breast carcinoma. Nine cases presented with ophthalmic signs and metastatic tumor was recognized later. In the remaining 19 cases, the diagnosis of the primary tumor preceded the onset of orbital metastasis. The time interval between the detection of the primary malignancy and metastatic orbital tumor was shortest for lung carcinoma (mean: 2 months) and longest for breast carcinoma (mean: 34 months). Radiotherapy and chemotherapy were applied in 12 cases. Improvement in orbital signs and visual acuity was noted in 5 cases. Radiotherapy, chemotherapy and hormonal therapy were used in 8 patients and improvement in orbital signs was noted in 4 of these patients. Four of 28 patients (2 with breast carcinomas, one with prostate carcinoma and one with thyroid carcinoma) survived longer than 5 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Malignant melanomas of the iris.

The recognition of iris melanoma is important because a number of benign lesions clinically resemble these tumors. In this article, the epidemiological, clinical and histopathological features, treatment modalities, and prognosis of 41 iris melanoma patients, seen between 1964 and 1996 were evaluated. Of the patients, 20 were men and 21 women. Their mean age was 44.6 years. After determining the size, localization, and extension of the tumor, the management of choice was observation in 9, sector iridectomy in 15, iridocyclectomy in 6 and enucleation in 11 of the patients. During the follow-up, enucleation was also required in 6 and iridocyclectomy in 1 of the 7 patients who were in the observation or sector iridectomy group initially. Histopathologic examination revealed spindle cell in 27, mixed cell in 6 and epithelioid cell type melanomas in 2 of the 35 cases who underwent iridectomy, iridocyclectomy, and/or enucleation. The mean follow-up was 3.2 years and the mortality rate was found to be 2.4% during this period. One patient who died of metastases had epithelioid cell type melanoma.

Retinoblastoma in Turkey--treatment and prognosis.

The treatment modalities and prognosis of 636 retinoblastoma (RB) cases diagnosed and treated in our specialist center between 1963 and 1994 were evaluated. Patient age ranged from 20 days to 16 years, the mean age being 2.2 years (26.4 months). Of the 636 cases, 441 were unilateral and 195 were bilateral. Enucleation was the most frequent treatment employed in unilateral RB patients (412 cases). Follow-up treatment included exenteration (48 cases), radiotherapy (154 cases) and chemotherapy (108 cases) for cases with optic nerve invasion and/or orbital recurrence following enucleation. Seventeen cases displayed massive proptosis, ocular damage and blindness at initial presentation and underwent exenteration as the initial treatment. Two cases were subjected to external beam radiotherapy without invasive surgical procedures. Ten cases regressed spontaneously without treatment. For bilateral cases, the most frequent treatment used was enucleation for one eye and radiotherapy for the other (132 cases). Adjuvant treatment included exenteration (9 cases) and chemotherapy (50 cases) depending on orbital recurrence and/or systemic metastasis. Spontaneous bilateral regression was noted in one case. Six cases underwent bilateral external beam radiotherapy without surgery. One eye of the remaining 56 bilateral cases underwent enucleation. The treatment for the contralateral eyes included cryotherapy in 14 cases, enucleation in 11 cases, Cobalt plaque (Co plaque) therapy in 10 cases, photocoagulation in 6 cases and exenteration in one case. No treatment was undertaken in the contralateral eyes of 14 cases. Secondary treatment modalities employed in these 56 bilateral cases were radiotherapy (11 cases), chemotherapy (8 cases), Co plaque (8 cases) and exenteration (5 cases). Treatment complications were detected in 25 cases followed for at least 18 months. Eighteen cases had radiation cataracts and 6 of these 18 patients underwent intraocular lens implantation. Post-radiation orbital malignancy (osteosarcoma) was noted in two cases aged 14 and 15 years. Phthisis bulbi was observed in three cases and radiation keratitis in two cases. The overall survival rate was 82.2% after a mean follow-up of 5 years. The survival rate of unilateral cases was 82.8% and that of bilateral cases was 81.1% at 5 years.

Orbital rhabdomyosarcoma: a twenty-year survey in Turkey.

Orbital rhabdomyosarcoma is the most frequently encountered primary malignant orbital tumor in children. Between 1970 and 1991, 68 primary orbital rhabdomyosarcoma cases were diagnosed and treated in our clinic. Mean age at the time of diagnosis was 8.8 years. Seventy-two percent of the cases were of the embryonal type while 14% had alveolar and 14% had botryoid tumors. Following tissue diagnosis by anterior orbitotomy, radiotherapy and multimodal chemotherapy were instituted promptly. Subtotal exenteration was used in massive orbital involvement and total exenteration in recurrences. Using the Kaplan-Meier method of analysis, the survival rate was 84% at three years and 80% at five years. While morbidity due to the disease itself and treatment complications may still be a problem, the survival rates achieved in the long term are promising.

Amiodarone-related optic neuropathy.

BACKGROUND: To evaluate a case of atypical optic neuropathy that presented with blurred vision following the use of an antiarrythmic agent. CASE: Record of the patient was reviewed to determine the etiology of his optic neuropathy. OBSERVATIONS: Ophthalmological examination revealed unilateral optic disc edema with relatively well-preserved visual acuity. In routine tests, results of complete blood count, erythrocyte sedimentation rate, liver and kidney function tests, chest x-ray, Goldmann visual field examination, and brain computed tomography scan were normal. Orbital ultrasonography revealed optic disc edema with prominent optic nerve head and without orbital pathology. CONCLUSIONS: Systemic history and drug intake should be investigated in every patient with optic disc edema. Discontinuation of the medication can prevent further optic nerve damage or involvement of the other eye.

Anterior segment ischemia following pterygium surgery.

A 60-year-old woman developed anterior segment ischemia 1 week after an uneventful pterygium excision in the left eye. Corrected visual acuity fell from 20/20 to 20/200. Ophthalmic examination found keratic precipitates on the corneal endothelium, ++ cells in the aqueous humor, and necrosis of the lower half of the iris with posterior synechiae resulting in a fixed and distorted pupil. On therapy of topical corticosteroid eyedrops hourly and atropine eyedrops three times a day, the iridocyclitis resolved in 2 weeks. At the final examination 3 months after the surgery, the corrected visual acuity was 20/20. The atrophy of the lower half of the iris and posterior synechiae in the lower half of the pupil, giving the fixed and distorted pupil, were noted as sequelae. Iris fluorescein angiography revealed filling defects in the lower half of the iris but no leakage from iris vessels. Systemic evaluation was unremarkable except for mild hypertension. Doppler studies of carotid, ophthalmic, and central retinal arteries were normal. Mitomycin C, beta-irradiation and rectus muscle fixation sutures were not used. Only conjunctival dissection or episcleral cauterization were seen as possible causes of interference with the anterior segment blood supply. We believe this is the first report of anterior segment ischemia following pterygium surgery.

Neurogenic tumors of the orbit.

Fifty-three patients with neurogenic orbital tumors were diagnosed and treated at our university hospital during the past three decades. There were 16 patients with juvenile pilocytic astrocytomas, 21 patients with meningiomas and 16 with peripheral nerve tumors. Of the 16 peripheral nerve tumor patients, 8 had schwannoma, 4 had neurofibroma, 2 had malignant schwannoma, one had paraganglioma and one had amputation neuroma. Optic nerve tumors were treated via a transfrontal craniotomy if there was no chiasmal involvement. Cases with chiasmal involvement, incomplete resections and recurrences were treated with radiotherapy (5000 cGy). Far advanced cases with progressive proptosis and ocular damage underwent exenteration. Peripheral nerve tumors were treated by local resection. Exenteration was applied when there was an advanced or malignant lesion. Four of the 16 glioma patients and 5 of the 21 meningioma patients died during follow-up. The prognosis for peripheral nerve tumors was generally good, except for malignant schwannomas. Two patients with this malignant tumor died within 2 years after surgery.

Chronic dacryoadenitis misdiagnosed as eyelid edema and allergic conjunctivitis.

PURPOSE: To report the case of a 53-year-old woman with a 2-year history of episodic upper eyelid swelling and nonspecific complaints, who was diagnosed as having allergic conjunctivitis. METHODS: A complete ocular examination, orbital computerized tomographic (CT) scans followed by complete physical and systemic examinations. RESULTS: The results of physical and systemic examinations were unremarkable for systemic lymphoma and a primary focus of cancer. The results of the ocular examination were normal. CT scans demonstrated well-defined lesions bilaterally with a homogeneous internal structure in the lacrimal gland fossa, which suggested a diagnosis of chronic dacryoadenitis. The differential diagnosis included lymphoma and orbital metastases. The patient refused a biopsy and was started on a tapering dose of 60 mg oral prednisolone daily. The follow-up CT scans 1 month after cessation of 6-week oral corticosteroid treatment showed near complete resolution of the orbital lesions. CONCLUSION: This case demonstrates that orbital inflammation can be misdiagnosed as refractory allergic conjunctivitis.

A linkage analysis in two families with bilateral retinoblastoma.

Using polymerase chain reaction (PCR) amplification from blood samples, Xbal and BamHI polymorphisms were analyzed in two families with bilateral retinoblastoma. In one of the families it was predicted using the BamHI polymorphism that the 200 bp allele co-segregates with the disease. This family was uninformative for Xbal polymorphism. The second family was uninformative for both Xbal and BamHI polymorphism.