Zeynel A. Karcioglu

Capillary hemangioma (infantile periocular hemangioma)

Capillary hemangiomas are the most common orbital tumors in children. They typically arise early in life, grow rapidly during a proliferative phase and then slowly regress in an involutional phase. The tumors may present as small isolated lesions of minimal clinical significance or as large disfiguring masses that can cause visual impairment and systemic symptomatology. Capillary hemangiomas are managed effectively by establishing a secure diagnosis, outlining the extent of the tumor, and understanding the natural history of the lesion, as well as its response to therapy. The ophthalmic and systemic manifestations of capillary hemangiomas are discussed in detail, as are the histopathology, radiologic findings, differential diagnosis, and therapeutic alternatives.

Risk Factors for Metastasis in Retinoblastoma

Children with retinoblastoma typically survive their cancer due to advances in early diagnosis and treatment. Despite this success, risk factors persist for metastasis that are thought to be related to patient age, sex, laterality, treatment, genetics, histopathology, and extraocular extension. This review has found that invasion of the uvea, orbit, and optic nerve continue to be the most important predictors of metastatic retinoblastoma. Bilaterality and delays in diagnosis are also important factors. We examine molecular and genetic studies that offer the potential of predicting which tumors are likely to metastasize, which will recur within the eye, and which will undergo senescence. In this review, we describe which clinical evaluations, genetic studies, and histopathologic evaluations of retrieved specimens are currently used widely. This review has been performed to help those caring for patients with retinoblastoma and to aid informed consent.

Zinc in the eye.

Zinc has long been recognized as an essential constituent of various tissues. Many clinical conditions and dietary factors reduce the absorption or the biological availability of zinc, and lead to zinc deficiency which produces structural and functional alterations in many organ systems. The highest concentration of this trace element in the human body is measured in the eye, particularly in the pigment-containing components. The deficiency of zinc has a dramatic effect on ocular development especially when it occurs during early prenatal period. Zinc is required for the structure and activity of many ocular metalloenzymes. Although the exact mechanism of its molecular and cellular functions are largely unknown the essentiality of this element in the component of the eye, including the retina, choroid, cornea and lens, is well established; it is also well known that zinc deficiency causes functional impairments in various parts of the eye. Zinc related toxicities have been shown in human and animal eyes.

Tumor seeding in ocular fine needle aspiration biopsy.

The purpose of this study was to investigate the occurrence of tumor cell seeding in needle tracks following fine needle aspiration biopsy of intraocular tumors. Sixteen biopsies were performed in four enucleated globes (3 with retinoblastoma and 1 with choroidal melanoma). The biopsies were done immediately after surgery and the needle tracks from the pars plana region were serially sectioned for histological examination. Clusters of tumor cells were seen within six of the 11 needle tracks that could be histologically identified.

Porous polyethylene orbital implant in patients with retinoblastoma

OBJECTIVE The purpose of the study was to assess integration of porous polyethylene allografts in 37 anophthalmic sockets of retinoblastoma enucleations. DESIGN A clinical review. PARTICIPANTS Thirty-four patients (19 female, 15 male) whose age when diagnosed with retinoblastoma ranged from 1 to 72 months participated. Sixteen patients had unilateral retinoblastoma and 18 had bilateral retinoblastoma. INTERVENTION Enucleation (3 bilateral, 31 unilateral) with implantation of a porous polyethylene (PP) sphere (16-20 mm in diameter) was performed. Of the 37 spheres, 34 were primary implants and 3 were secondary implants. In the eight patients with postimplantation exposure, the PP implant had to be removed. MAIN OUTCOME MEASURES Data on eight sockets with exposed PP implant were measured. RESULTS There were no implant extrusions, but conjunctival dehiscence-exposure occurred in eight patients (21.6%). In 3 of 37 sockets, the volume replacement was not good, and in 5 cases, fornices were too shallow to accommodate a well-fitting prosthesis. In all but one socket, the overall cosmesis was graded subjectively from acceptable (grade 2) to excellent (grade 3). CONCLUSIONS Although the PP implant is a useful and a less-expensive alternative to hydroxyapatite in the porous matrix implant category, if conjunctival exposure takes place, the implant presents a serious management problem because of its nonresponsiveness to medical and surgical treatments.

Penetrating orbital injury with organic foreign bodies

OBJECTIVE The authors reviewed the clinical features, diagnostic workup, and management of patients of penetrating orbital injuries with retained organic foreign bodies. DESIGN Retrospective, noncomparative case series. PARTICIPANTS Nineteen patients (15 males, 4 females) with penetrating orbital injuries due to organic foreign bodies. RESULTS The series included 15 (78.9%) males and 4 (21.1%) females who ranged in age from 6 months to 40 years (mean = 14.6 years); 12 (63.2%) patients were younger than 12 years of age. Twelve (63.2%) right and 7 (36.8%) left orbits were involved. Time between injury and presentation varied from a few hours to 9 months. Most common injury site was the superior orbit in 11 (57.9%) patients leading to abnormal extraocular motility (84.2%), proptosis (68.4%), and upper lid ptosis (47.4%). Associated pathologies also included acute cellulitis in 11, orbitocutaneous fistula in 5, and osteomyelitis in 2 patients. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) identified the foreign bodies in 42% and 57% of the patients, respectively. CONCLUSION Preoperative identification of the foreign material in the orbit was found to be very helpful for patient management but was only possible in approximately 50% of our cases with the use of CT and MRI. The vision in our patients usually improved shortly after treatment; the long-term complications more often included extraocular muscle and eyelid motility problems and periorbital scarring.

Macular Corneal Dystrophy in Saudi Arabia: A Study of 56 Cases and Recognition of a New Immunophenotype

PURPOSE To determine the immunophenotype or immunophenotypes of macular corneal dystrophy in Saudi Arabia. METHODS We studied 56 cases of macular corneal dystrophy. Tissue from 60 corneal transplant buttons was stained by the avidin-biotin complex method using an anti-keratan sulfate monoclonal antibody. The serum antigenic keratan sulfate was measured in 23 of the 56 patients, four unaffected relatives, and 13 individuals with chronic actinic keratopathy. Serum and corneal tissue were studied in 17 of the 50 affected individuals with corneal transplant material. RESULTS Thirty-five corneas (58.3%) of 29 of 50 patients did not react with anti-keratan sulfate monoclonal antibody. The stroma and abnormal intracellular and extracellular corneal accumulations reacted with anti-keratan sulfate monoclonal antibody in seven corneas (11.7%). The stroma in the other 18 corneas (30.0%) from 15 patients did not react with the anti-keratan sulfate monoclonal antibody, but corneal fibroblasts did. Twenty-one of the 23 patients with macular corneal dystrophy had no detectable serum antigenic keratan sulfate (< 9 ng/ml); two had values of 12 and 51 ng/ml, respectively, and their corneal stroma and abnormal accumulations reacted with anti-keratan sulfate monoclonal antibody. CONCLUSIONS We detected macular corneal dystrophy type IA, a new immunophenotype characterized by the lack of detectable antigenic keratan sulfate in the serum (< 9 ng/ml), and a corneal stroma that did not react with the keratan sulfate monoclonal antibody but in which corneal fibroblasts did react with keratan sulfate monoclonal antibody (in 15 of 50 patients).

Secondary Cataracts in Infants After Lensectomies

Three infants, one who was two weeks old, and two who were two months old, underwent lensectomy and vitrectomy in a total of four eyes for congenital cataracts by means of an automated suction cutter. Two patients with unilateral opacities underwent combined cataract extraction and epikeratophakia, and one with bilateral congenital cataracts underwent cataract extractions and was fit with extended wear contact lenses. In all cases, the surgery involved at least a 5-mm posterior capsulotomy with a shallow anterior vitrectomy, and was uneventful. Three of the four eyes developed new opacities that required surgical removal three to five months after the original surgery. Cytological evaluation of the specimen obtained from one patient showed this material to be lens epithelium. In all three cases, the material grew without the support of the posterior capsule; in one patient the material appeared to have seeded onto the iris. This previously unreported complication in infants with congenital cataracts who have undergone posterior capsulotomy and anterior vitrectomy emphasizes the need for frequent retinoscopies on such patients. The absence of the posterior capsule does not guarantee that these children will not develop secondary growth of lens epithelium which may obstruct the visual axis.

Workup for Metastatic Retinoblastoma: A Review of 261 Patients

PURPOSE The means available to screen for retinoblastoma metastases, including bone marrow aspiration, lumbar puncture, and radionuclide scans, offer variable usefulness at different stages of the disease. In this study, the authors attempted to assess the value of these tests as part of the initial workup for metastases. METHODS Medical files of 261 patients with retinoblastoma were reviewed, and the results of bone marrow, lumbar puncture, and bone and liver radionuclide scans were correlated with the laterality, clinical staging, and histopathologic findings. The presence or absence of tumor in the choroid and the optic nerve also were correlated with the results of diagnostic tests. RESULTS Of 261 patients with retinoblastoma, 147 (56.3%) and 114 (43.7%) had unilateral and bilateral disease, respectively; 11.6% of unilateral cases and 14.9% of bilateral cases had distant metastasis (P = 0.2). Bone marrow aspirations were performed on 101 patients (38.7%), and 10 (9.9%), of these specimens showed the presence of retinoblastoma cells in the aspirate. Ninety-four (36%) of the patients were examined with spinal tap cytology, and the results of four (4.3%) of these were positive for tumor cells. Radionuclide bone and liver scans were performed on 49 (18.8%) and 48 (18.4%) patients, respectively; 5 (10.2%) had abnormal bone scans and 3 (6.2%) had abnormal liver scans. The average age in the metastatic group was 3.1 years, whereas the average age in the nonmetastatic group was 2.3 years. All of our abnormal test results were found in patients with stages III and IV disease, with the exception of one abnormal lumbar puncture in a patient with stage II disease. The correlation of diagnostic tests with the histopathologic findings showed that with no choroidal involvement, no positive diagnostic tests were encountered. Correlation between positive diagnostic test results and the level of optic nerve involvement failed to indicate any trends. CONCLUSION Our study indicated a good interrelation between the positivity of diagnostic tests (bone marrow and bone scan) and higher stages of the disease (stages III and IV) and choroidal involvement in enucleated eyes, with statistically significant correlations; correlations with lumbar puncture and liver scan were not significant.

Diagnosis and Management of Iris Juvenile Xanthogranuloma

BACKGROUND Juvenile xanthogranuloma is a benign, self-limiting cutaneous disorder most commonly encountered during infancy. Approximately 10% of cases may develop ocular or adnexal involvement, most commonly in the iris. METHODS We review clinical and morphological features of four cases of iris juvenile xanthogranuloma that reflect the diagnostic and therapeutic spectrum. RESULTS Tissue diagnosis was confirmed in all cases; in one case, the disease was diagnosed with a skin biopsy and treated with local and systemic steroids, and its persistence in the iris was confirmed with a second tissue specimen obtained five months after systemic steroid treatment. CONCLUSION The diagnosis and treatment of juvenile xanthogranuloma may be straightforward, particularly in cases when the ocular lesion receives early attention and responds well to topical steroids, and when there is no hyphema. However, in other instances, this entity may be difficult to manage and may necessitate iris biopsy for diagnosis and radiation therapy for treatment.