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Biomedical subjects

H Kiratli

Publications and source records attributed to H Kiratli.

At least 55 records · Page 3Linked to original sources

Congenital orbital teratoma: a clinicopathologic case report.

A 5-day-old infant boy was noted to have severe left proptosis at birth. The left eye protruded superotemporally through the palpebral fissure and had exposure keratopathy. There was frank left afferent pupillary defect. Computerized tomography (CT) showed a left orbital soft tissue mass with foci of calcification. Magnetic resonance imaging (MRI) studies revealed a left orbital mass with solid and cystic portions without intracranial extension. As the eye was considered to be nonsalvagable, a lid-sparing type, modified exenteration was performed. Histopathologic examination demonstrated various mature tissues of all three embryonic germinal cell lines. This case represents one of the rare examples of true congenital orbital teratoma, which is an uncommon cause of hideous proptosis in the neonate. MRI may prove useful in differentiating this tumor from more common conditions.

Diagnosis, Differential↗

Metastatic tumours to the conjunctiva: report of 10 cases.

AIMS/BACKGROUND: Ten patients with metastatic tumours to the conjunctiva and the clinical aspects of this rare form of ocular metastasis are described in this study. METHOD: All patients with ocular and adnexal metastatic tumours referred to an ocular oncology service were reviewed and those having conjunctival metastases were studied for the site of their primary tumour, clinical features, and treatment of the conjunctival tumour, associated ocular and systemic findings, and the patients' outcome. RESULTS: The primary malignancy was carcinoma of the breast in four, lung cancer in two, laryngeal carcinoma in one, cutaneous melanoma in two, and unknown in one patient. The conjunctival metastases appeared after the primary tumour over a mean period of 44 (8-130) months. They were solitary in eight cases, located in bulbar conjunctiva in six, palpebral conjunctiva in two, and in limbus and forniceal conjunctiva in one patient each. The tumour was yellow in colour in seven patients, red in two, and brown in one. Eight patients also had metastases to other ocular structures. Seven patients received external beam radiotherapy to the affected eye, two were managed by excisional biopsy, and one with chemotherapy. The mean survival after the diagnosis of conjunctival metastasis was nine (range 2-26) months. CONCLUSION: Metastatic tumours to the conjunctiva appear at an advanced stage of the systemic disease when there are other ocular and organ metastases. The presence of a conjunctival mass in a patient with a prior systemic cancer should alert the ophthalmologist to the possibility of a conjunctival metastasis and evaluation should be pursued.

Adult↗

Metastatic tumors to the iris in 40 patients.

PURPOSE: Metastatic cancer to the iris is rare, and the best approaches to clinical recognition and treatment of this neoplasm are not widely known. We reviewed a series of patients with metastatic cancer to the iris and elucidated the clinical variations, diagnostic approaches, and treatment of this neoplasm. METHODS: The files of the Wills Eye Hospital Ocular Oncology Service were surveyed, and cases of metastatic cancer to the iris were extracted for further analysis. General data, systemic data, tumor characteristics, diagnostic methods used, treatment, and prognosis were tabulated. RESULTS: Of 512 patients with uveal metastasis, the iris was involved in 40 (7.8%). The metastasis originated from breast carcinoma in 16 cases, lung carcinoma in 11, carcinoid tumor in three, melanoma in three, colonic carcinoma in two, and esophageal, laryngeal, prostate, and kidney carcinoma in one case each. In one case the primary tumor was never identified. There was no history of primary cancer in 13 (32%) of 40 patients. All iris metastases were unilateral, and secondary glaucoma was present in 15 (38%). Fine-needle aspiration biopsy of the iris lesion helped to establish the diagnosis in 11 cases. Treatment with chemotherapy, radiotherapy, or both, achieved local tumor control in all patients. The overall mean survival was 20 months and the median survival was 13 months after diagnosis of the iris metastasis. CONCLUSIONS: Iris metastasis can show a spectrum of clinical variations, but its features are generally distinctive enough to differentiate it from other intraocular neoplasms and inflammations. Treatment consists of chemotherapy, which was sometimes combined with ocular irradiation. The visual prognosis was good but the systemic prognosis was generally poor.

Adult↗

Localized transscleral staphylococcal abscess simulating a neoplasm.

A 47-year-old woman with a history of breast cancer developed a focal epibulbar mass in the right eye. The initial diagnosis was nodular episcleritis, but the lesion failed to respond to local and systemic corticosteroids. The rule out an epibulbar metastatic carcinoma an excisional biopsy was performed, which demonstrate a localized staphylococcal abscess that had locally eroded the sclera and exposed the underlying uveal tract. Covering the defect with a scleral patch graft and promptly instituting oral amoxicillin/clavulanic acid and topical cefazolin treatment resulted in an uncomplicated recovery. Infectious scleral abscess in an uncommon entity and the differential diagnosis includes nodular episcleritis and epibulbar metastatic tumor.

Abscess↗

Risk factors for growth and metastasis of small choroidal melanocytic lesions.

PURPOSE: To investigate the clinical features that predict growth and metastasis of an unselected group of small melanocytic choroidal tumors. METHODS: A retrospective review was performed on 1329 patients with small melanocytic choroidal tumors measuring 3 mm in thickness or less. Clinical parameters of the patient and tumor were extracted and analyzed for their relationship to eventual tumor growth and metastasis using a Cox proportional hazards regression model. RESULTS: Tumor growth was documented in 18% of patients. The factors predictive of tumor growth (multivariate analysis) included greater tumor thickness, posterior tumor margin touching optic disc, symptoms of flashes, floaters, and blurred vision, orange pigment on the tumor surface, and the presence of subretinal fluid. The relative risk (rr) was greatest for initial tumor thickness > 2.0 mm (rr 5.2) and posterior margin touching the optic disc (rr 2.6). After adjusting for significant tumor variables, the effect of interventional tumor treatment showed a decreasing risk for tumor growth as compared to continued observation without treatment. Of 1329 patients, 35 (3%) developed metastases. The factors predictive of metastases (multivariate analysis) included: posterior tumor margin touching the optic disc, documented growth, and greater tumor thickness. The relative risk for metastases was greatest for tumor thickness 1.1-3.0 mm (rr 8.8) and growth (rr 3.2). CONCLUSION: Of small choroidal melanocytic tumors measuring 3 mm or less in thickness at the time of initial examination, 18% demonstrate growth and 3% metastasize during the period of followup. Based on this analysis, the clinical features of these tumors can be used to estimate the risk for tumor growth and metastases and assist the clinician with patient management.

Adolescent↗

Risk factors for growth and metastasis of small choroidal melanocytic lesions.

BACKGROUND: The management of small melanocytic choroidal tumors is controversial. An important reason for this controversy is that the natural course and metastatic potential of these lesions are not defined clearly. Prior studies that have attempted to elucidate the natural course of these lesions have focused on selected small groups of patients with presumed small choroidal melanomas. There are no large studies investigating the growth potential and metastatic potential for the spectrum of small melanocytic choroidal tumors when considered as an unselected whole group. In addition, the clinical features of these tumors predictive of metastases have not yet been identified. METHODS: A retrospective review was performed on 1329 patients with small melanocytic choroidal tumors measuring 3 mm or less in thickness. Clinical parameters of the patient and tumor were obtained and analyzed for their relation to eventual tumor growth and metastasis using a Cox proportional hazards regression model. RESULTS: Tumor growth was documented in 18% of patients. The factors predictive of tumor growth (multivariate analysis) included greater tumor thickness (P = 0.0001), posterior tumor margin touching optic disc (P = 0.0001), symptoms of flashes, floaters (P = 0.002), and blurred vision (P = 0.003) relative to no symptoms, orange pigment on the tumor surface (P = 0.004), and the presence of subretinal fluid (P = 0.05). The relative risk (RR) was greatest for initial tumor thickness 2.1 to 3.0 mm (RR = 5.2) and tumor thickness 1.1 to 2.0 mm (RR = 4.3) relative to tumors 1 mm or less in thickness, as well as posterior margin touching the optic disc (RR = 2.6). After adjusting for significant tumor variables, the effect of interventional tumor treatment showed a decreasing risk for tumor growth compared with continued observation without treatment. Of 1329 patients, metastases developed in 35 (3%). The factors predictive of metastases (multivariate analysis) included posterior tumor margin touching the optic disc (P = 0.003), documented growth (P = 0.003), and greater tumor thickness (P = 0.004). The relative risk for metastases was greatest for tumor thickness 1.1 to 3.0 mm (RR = 8.8) and growth (RR = 3.2). CONCLUSION: Of small choroidal melanocytic tumors measuring 3 mm or less in thickness at the time of initial examination, 18% demonstrated growth and 3% metastasized during the period of follow-up. Based on this analysis, the clinical features of these tumors can be used to estimate the risk for tumor growth and metastases and assist the clinician with patient management.

Adolescent↗

Cerebellar ataxia associated with hypogonadotropic hypogonadism and chorioretinopathy: a poorly recognized association.

We report a male with cerebellar ataxia, hypogonadism and chorioretinopathy. The age of onset was 12. The parents were first cousins. Endocrinologic studies demonstrated hypogonadotropic hypogonadism due to pituitary dysfunction. The ocular disorder involved the choriocapillaris and the retina. The association may represent a separate syndrome, seldom recognized in the past.

Adult↗

Poland-Moebius syndrome: a case report.

BACKGROUND: The primary site of pathology in Moebius syndrome is still unknown, although several studies have variably localized the lesion in the extraocular muscles, cranial nerves, or central nervous system. CASE: A 24-year-old man with Poland-Moebius syndrome and acquired progressive bilateral paralytic lower eyelid ectropion is described. OBSERVATIONS: In this patient, magnetic resonance imaging studies revealed a barely detectable pontine hypoplasia and normal recti muscles. Nerve conduction studies of the facial nerves showed a severe demyelinating or dysmyelinating type of neuropathy. Bilateral lower eyelid ectropium of the patient was successfully corrected by canthal tightening procedures. CONCLUSION: Contrary to many reported cases, this patient serves as a rare example of a progressive type of Poland-Moebius syndrome presumably resulting from a combination of a brainstem abnormality and a peripheral neural degenerative process.

Adult↗

Scintigraphic evaluation of tumors metastatic to the choroid using technetium-99m(V)-dimercaptosuccinic acid.

Technetium-99m(V)-dimercaptosuccinic acid scintigraphy was used to evaluate three patients with intraocular tumors who had metastatic breast, lung, and rectal carcinomas, respectively. At the time of initial examination, two patients had no known systemic cancer, but the scintigraphy results in one patient revealed the primary site and were highly suggestive of disseminated carcinomatosis in the other patient. In the third patient, scintigraphy was successful to confirm very small bilateral intraocular tumors and also other systemic lesions. Technetium-99m(V)-dimercaptosuccinic acid scintigraphy can be reliably employed in a very select group of patients with intraocular tumors where metastatic carcinoma is a serious diagnostic possibility against a primary intraocular malignancy. This safe and promising tumor-imaging agent has the ability to demonstrate the ocular lesions and other systemic foci simultaneously, information that would prove to be crucial in both the diagnosis and the management of the patient.

Adenocarcinoma↗

The phenotype of arg555trp mutation in a large Turkish family with corneal granular dystrophy.

PURPOSE: A large Turkish family with 52 members, 26 of whom had Groenouw type 1 corneal granular dystrophy was evaluated by genetic linkage studies and mutation analyses. Phenotype-genotype correlations were also assessed. METHODS: DNA from peripheral blood lymphocytes of 22 family members was used in establishing linkage to chromosome 5q31. Single-strand conformation polymorphism analysis was done to detect mutations in exons 4 and 12 of the human transforming growth factor beta-induced gene located on chromosome 5q31. Automated sequencing was performed on exon 12 of an affected patient. RESULTS: Patients yonger than 15 years of age had typical linear, granular opacities whereas adults had coarser, deeper granular stromal deposits. These changes were not associated with recurrent erosions or significant visual disabilities. The family was linked to chromosome 5q31 and a DNA shift was observed on exon 12 of affected patients. CGG to TGG transition producing R555W mutation was found. CONCLUSIONS: Segregation of Arg555Trp has been described as causing Groenouw type I corneal dystrophy of variable severity in patients of various ethnic backgrounds. In this large Turkish pedigree, the Arg555Trp mutation was associated with a mild phenotype that became clinically evident at five years of age but which remained asymptomatic in terms of corneal erosions.

Adolescent↗

Ipsilateral orbital cavernous hemangioma and choroidal hemangioma.

PURPOSE: A unique case of simultaneous ipsilateral choroidal and orbital hemangiomas is described. METHODS: A 71-year-old man was found to have a left orbital intraconal mass while being worked up for intractable headache. He also had a left juxtapapillary choroidal hemangioma which did not cause any leakage to macular area. RESULTS: The left intraconal tumor was removed by medial transconjunctival orbitotomy. Histopathological examination revealed cavernous hemangioma. The ipsilateral choroidal hemangioma was managed by observation only. During 15 months of follow-up, there has been no growth or accumulation of subretinal fluid. CONCLUSIONS: The occurrence of orbital cavernous and choroidal hemangiomas on the same side suggests common pathogenic derangement acting on two different sites.

Aged↗

Results of trabeculotomy and guarded filtration procedure for glaucoma associated with Sturge-Weber syndrome.

PURPOSE: To assess the efficacy of trabeculotomy and guarded filtration procedure in the management of glaucoma associated with the Sturge-Weber syndrome. METHODS: Six patients (seven eyes) with Sturge-Weber syndrome who underwent glaucoma surgery were retrospectively reviewed. One patient had bilateral glaucoma. Age at the time of operation ranged between 23 days and 9 years. The mean preoperative intraocular pressure was 30.6 mmHg (range 25-38 mmHg). Trabeculotomy and guarded filtration procedure were performed in three eyes each and both techniques were used in one eye. RESULTS: The mean follow-up was 6.3 (range 2-11) years. A single procedure lowered the intraocular pressure and arrested the progression of the disease in five eyes. Two patients required topical antiglaucoma medication. No patient needed reoperation. Two eyes suffered intra- and post-operative temporary choroidal effusions. CONCLUSIONS: Trabeculotomy in infancy and guarded filtration procedure in older children effectively stabilized the eyes and prevented further glaucomatous damage in our patients with Sturge-Weber syndrome.

Child↗

Treatment of retinoblastoma with indirect ophthalmoscope laser photocoagulation.

INTRODUCTION: The indirect ophthalmoscope laser photocoagulation delivery system is relatively new and is potentially applicable for treating selected small retinoblastomas. There has been very little reported on the results achieved with this laser delivery system in the treatment of retinoblastoma. METHODS: We reviewed all patients with retinoblastoma managed by indirect ophthalmoscope laser photocoagulation on the Ocular Oncology Service between July 1, 1991 and July 1, 1994. The individual tumor size, location, and proximity to the optic disc and foveola, as well as the laser power, duration, and number of sessions ,were recorded. We assessed the tumor response to treatment and the visual outcome of the patients. RESULTS: There were 30 retinoblastomas in 20 eyes of 18 patients managed with indirect ophthalmoscope laser photocoagulation over the 3-year period. The mean tumor base was 2.1 mm (range: 1 mm to 8 mm); the mean tumor thickness, 1.2 mm (range: 0 mm to 3 mm). The tumor margin was a mean of 6.5 mm (range: 0 mm to 19 mm) from the foveola, and 7.7 mm (range: 0 mm to 22 mm) to the optic disc. In general, it required a power of approximately 350 mW and a continuous duration lasting 1 to 4 seconds to obtain satisfactory obliteration of feeding blood vessels. The tumor responded in one to three sessions (mean: 1.9 sessions per tumor). Complete regression occurred in 21 tumors (70%) and local recurrence in nine (30%). The recurrent tumors were successfully treated with plaque radiotherapy in seven cases and cryotherapy in two cases. The central vision was minimally distorted due to foveal traction from the laser treatment in three cases. CONCLUSION: Indirect ophthalmoscope laser photocoagulation is an effective conservative method to manage selected small retinoblastomas. Tumors treated by this technique should be followed closely due to the moderate risk for local recurrence.

Child, Preschool↗