İlhan Günalp

Vascular tumors of the orbit

Eighty-five vascular lesions of the orbit examined and treated between 1963–1993 were reviewed retrospectively to reveal the types of vascular tumors, age and sex distribution, clinical characteristics, treatment options and prognosis. Capillary hemangioma was the most frequent orbital vascular tumor accounting for 37 of 85 cases making up 43.5% of the entire orbital masses. Cavernous hemangioma accounted for 35 cases (41.2%), varices for 3 cases (3.5%), anteriovenous malformations for 3 cases (3.5%), angiosarcomas for 3 cases (3.5%), lymphangiomas for 3 cases (3.5%) and hemangiopericytoma for one case (1.2%). Of the 85 vascular tumor cases 43 were encountered in children aged 18 years or less. Capillary hemangioma was the most frequent pediatric orbital vascular lesion, making up 86% of the vascular tumors in this age group. Capillary hemangiomas have been treated with observation and intralesional corticosteroid injections. Cavernous hemangiomas, vascular malformations, lymphangiomas and two cases of angiosarcomas received orbitotomy. One case each of angiosarcoma and hemangiopericytoma underwent exenteration because of the advanced stage of the lesion. There was one tumor-related death who was a 9-year-old boy diagnosed with angiosarcoma. Vascular tumors with the exception of angiosarcomas and hemangiopericytoma behaved as benign lesions.

Orbital exenteration: a review of 429 cases.

The authors reviewed their experience with 429 cases of orbital exenteration between 1963 and 1993. Apart from the 22 cases operated in concert with other physicians, 407 cases were operated by an ophthalmologist and spontaneous granulation technique was used. Total or eyelid-sacrificing exenteration was carried out for lesions involving the eyelids and for recurrent/infiltrative malignant tumors. Two hundred and thirty-seven cases received total exenteration. The remaining 192 cases were treated with eyelid-sparing or subtotal exenteration. In each case, full exenteration was performed with the removal of the periosteum. Secondary tumors were the most frequent indication for exenterations accounting for 349 (81.3%) cases. Among the secondary tumors, there were 173 eyelid, 104 intraocular, 56 conjunctival tumors, 15 nasopharynx and one maxillary sinus carcinomas. The second leading indication was primary orbital tumors (50 cases, 11.7%). Of the primary orbital tumors, 35 were rhabdomyosarcomas, nine were neurogenic tumors, two were fibrocytic tumors, two were vascular tumors, one was teratoma and one was primary melanocytic tumor. The remaining cases included 16 lacrimal fossa tumors (3.7%), 10 lymphomas (2.3%) and four (1.0%) inflammatory pseudotumors. Squamous cell carcinoma was the single most frequent indication for which exenteration was carried out, accounting for 30.3% of the cases. Spontaneous granulation proved to be a simple technique and produces cosmetically better results for patients not wearing prosthesis.

Cystic lesions of the orbit

Background/aims: Cystic lesions of the orbit constitute a group of lesions with diverse clinical findings, histopathologic features and pathogenesis. Methods: We reviewed the histopathologic and clinical records on 128 orbital cystic lesions diagnosed during a 32-year period from 1963 to 1995. Results: Of the 128 orbital cystic lesions, dermoid cysts were the most frequent (38 cases, 29.7%). The other lesions, in decreasing order of frequency, were hydatid cysts (33 cases, 25.8%), mucoceles (31 cases, 24.2%), pyoceles (10 cases, 7.8%), meningoencephaloceles (9 cases, 7.0%), epidermal inclusion cysts (4 cases, 3.1%), hematoceles (2 cases, 1.6%) and teratoma (one case, 0.8%). Conclusions: Our review showed that 64.1% of patients with cystic lesions were aged 18 years or less. With the exception of mucopyoceles, all the other cystic lesions were more frequent in pediatric patients. Cystic lesions behaved clinically as benign lesions. At a mean follow-up of 6.7 years we did not observe development of malignancy in any of the cystic tumor cases though variable degrees of vision loss developed due to factors such as optic nerve compression, ocular damage and amblyopia.

Magnetic resonance imaging of unilateral lacrimal gland lesions

PurposeTo report the findings on magnetic resonance imaging (MRI) of various benign and malignant unilateral lacrimal gland lesions.MethodsThis is a retrospective noncomparative interventional case series. Thirty-one consecutive patients with a unilateral lacrimal gland lesion were analyzed. The preoperative MRI findings were correlated with the pathology results. The main outcome measures were anatomic extent, configuration, margins, angulation, internal features on T1- and T2-weighted images (with respect to extraocular muscles and cerebral gray matter), contrast enhancement of the lesion and adjacent bone change on MRI.ResultsOf the 31 patients, 21 had chronic dacryoadenitis, 3 had lymphoid tumors, and 7 had epithelial tumors including pleomorphic adenoma (3), adenoid cystic carcinoma (3), and pleomorphic adenocarcinoma (1). The results of the patients with chronic dacryoadenitis demonstrated involvement of the orbital lobe alone in 13 patients (62%), involvement of both orbital and palpebral lobes in 8 (38%), a molded configuration with ill-defined margins, sharp angles in 13 (62%), round angles in 8 (38%), lack of bone change, an isointense internal signal on T1-weighted images, a hypointense signal on T2-weighted images, and moderate contrast enhancement. The patients with lymphoid tumors demonstrated involvement of the orbital lobe, a molded configuration with ill-defined margins and sharp angles, lack of bone change, an isointense internal signal on T1-weighted images, an isointense signal on T2-weighted images, and moderate contrast enhancement. Those with epithelial tumors showed involvement of the orbital lobe, a well-circumscribed oval configuration, and round angles. Pleomorphic adenoma demonstrated smooth margins, bone expansion in two patients , and no bone change in one. Adenoid cystic carcinoma and pleomorphic adenocarcinoma showed irregular margins and bone destruction. All epithelial tumors demonstrated an isointense internal signal on T1-weighted images, a hyperintense signal on T2-weighted images, and moderate contrast enhancement.ConclusionsIt is difficult to uniformly correlate the MRI features and histopathologic findings in lacrimal gland lesions. However, MRI seems to be useful in determining the etiology of a unilateral lacrimal gland lesion. Internal tissue features on T1- and T2-weighted images of MRI are most helpful in categorizing these lesions. Although the number of patients is small, our findings suggest that there are differences in orbital MRI findings of inflammatory lesions and lymphoid tumors as compared to benign and malignant epithelial tumors in the lacrimal gland fossa.

Pediatric orbital tumors in Turkey

Summary A retrospective review of 376 pediatric orbital tumor cases seen at the Ankara University Eye Clinic between 1963 and 1993 was undertaken. The diagnosis was made histologically in every case. Secondary tumors accounted for 127 cases (33.8%), cystic lesions for 82 cases (21.8%), rhabdomyosarcomas for 66 cases (17.6%), neurogenic tumors for 23 cases (6.1%), vascular lesions for 21 cases (5.6%), inflammatory lesions for 21 cases (5.6%), lymphoma and leukemias for 18 cases (4.8%), other mesenchymal tumors for 11 cases (2.9%), metastatic tumors for 5 cases (1.3%), traumatic foreign bodies for 2 cases (0.5%), and lacrimal fossa lesions for 1 case (0.3%). The most common benign orbital tumors were the cystic lesions. The most common primary malignant tumor was rhabdomyosarcoma. Overall, the most frequent orbital lesion was the secondary orbital invasion of retinoblastoma.

Secondary orbital tumors

Summary The authors reviewed their experience with 524 secondary orbital tumor cases diagnosed histopathologically during 1963–1993. Secondary tumors constituted 48.0% of all orbital biopsies during the same period. There were 186 (35.5%) cases with eyelid tumors, 146 (27.9%) cases with intraocular tumors, 137 (26.1%) cases with conjunctival tumors, 26 (5.0%) cases with nasopharyngeal tumors, 22 (4.2%) cases with sinus carcinomas, 3 (0.6%) cases with intracranial meningiomas, 3 (0.6%) cases with esthesioneuroblastomas, and 1 (0.2%) case with chordoma. The three most frequent tumors making secondary orbital invasion were basal cell eyelid carcinoma (129 cases; 24.6%), squamous cell conjunctival carcinoma (125 cases; 23.8%), and retinoblastoma (123 cases; 23.5%). Squamous cell carcinoma, from various sites of origin, was the most frequent histopathologic tumor variant, accounting for 215 (41.0%) cases. Exenteration was performed on 334 (63.7%) of 524 cases with secondary orbital invasion. The ignorance of many lid and conjunctival lesions until the advanced stages, which could otherwise be easily treatable, caused blindness and life-threatening complications under our conditions. Orbital invasion from ocular retinoblastoma and uveal malignant melanoma led to a markedly poor prognosis, again related to the late presentation of many patients. Lack of health consciousness, inadequate primary surgery, and/or insufficient follow-up were the main reasons for secondary orbital invasion in many tumor cases.

Biopsy-proven orbital lesions in Turkey:A survey of 1092 cases over 30 years

1092 biopsy proven orbital lesions were seen during 1963-1993. Secondary tumors were the most frequent type of lesion, accounting for 533 cases (48.9%). Cystic lesions accounted for 128 cases (11.8%), inflammatory masses (pseudotumors) for 109 cases (10%), muscle-tissue tumors for 72 cases (6.6%), vascular lesions for 63 cases (5.8%), lymphoma and leukemias for 55 cases (5%), gliomas and meningiomas for 37 cases (3.4%), other mesenchymal tumors for 32 cases (2.9%), peripheral nerve tumors for 16 cases (1.5%), metastatic tumors for nine cases (0.8%), other lesions for four cases (0.4%) and primary orbital melanoma for three cases (0.3%). The most frequent primary benign orbital tumor in adults is cavernous hemangioma and the most common primary malignant orbital tumor in adulthood are the various lymphoid tumors. The most frequent primary benign orbital lesion in children is dermoid cysts and the most common primary malignant lesion is rhabdomyosarcoma. Overall, the commonest primary benign and malignant l...

The Use of Ultrasound Biomicroscopy in the Evaluation of Anterior Segment Tumors and Simulating Conditions

Purpose: To report the ultrasound biomicroscopy (UBM) findings of anterior segment tumors and simulating conditions. Methods: Thirty-five patients underwent UBM. Of those, 16 had histopathologically or cytopathologically diagnosed tumors, and 19 had clinically diagnosed lesions. Results: The study material comprised 13 iris pigment epithelial (IPE) cysts, 7 ciliary body melanomas, 4 iris melanomas, 4 iris nevi, 3 intraocular invasions of conjunctival squamous cell carcinoma, 2 ring melanomas of the anterior chamber angle, 1 medulloepithelioma and 1 pars plana cyst. On UBM, all IPE cysts presented as cystic lesions with a thin cyst wall and no solid components. All ciliary body melanomas showed low to medium reflectivity, with cavitation in one case and extraocular extension in another. Iris melanomas presented as anterior (stromal) iris lesions with medium to high internal reflectivity. There was irregularity and convex bowing of the posterior iris plane in iris melanomas, a feature not seen in iris nevi. Intraocular invasion of conjunctival squamous cell carcinoma was evidenced as areas of medium to high reflectivity in the ciliary body and iris, loss of the acute angle shape and highly reflective spots in the anterior chamber. Conclusions: UBM was particularly useful in the diagnosis of IPE cysts, in the visualization of small ciliary body melanomas, in the differentiation of iris melanomas from iris nevi and in the demonstration of intraocular invasion from conjunctival squamous cell carcinoma.

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.

Focal dermal hypoplasia (Goltz's syndrome)

A 17-year-old female with Goltzs 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 Goltzs syndrome.