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Enucleation.

The three most common indications for enucleation are intraocular malignancy, trauma, and a blind, painful eye. Recommending enucleation is one of the most difficult therapeutic decisions in ophthalmology. In some cases of malignancy, cryotherapy, laser photocoagulation, diathermy, chemotherapy, and radiation therapy may be viable alternatives to surgery. When surgery is chosen, evisceration or exenteration may be alternatives to enucleation. Once the decision is made to perform enucleation or evisceration, the surgeon must choose from several types of implants and wrapping materials. These devices can be synthetic, autologous, or eye-banked tissues. With certain implants, the surgeon must decide when and if to drill for subsequent peg placement. In this review, the authors discuss choices, techniques, complications, and patient consent and follow-up before, during, and after enucleation. Controversies and results of the Controlled Ocular Melanoma Study are summarized.

Eye Enucleation↗
PubMed2000

Comparison of artificial eye amplitudes with acrylic and hydroxyapatite spherical enucleation implants.

PURPOSE: To compare artificial eye amplitudes in patients who randomly received either a hydroxyapatite or an acrylic, scleral-covered spherical implant after enucleation. DESIGN: Randomized, controlled trial. PARTICIPANTS: Thirty-four consecutive patients who underwent enucleation because of an intraocular melanoma and 21 healthy control participants from the hospital staff. METHODS: Eligible patients randomly received a hydroxyapatite or an acrylic, scleral-covered spherical orbital implant. Fourteen patients were fitted with a hydroxyapatite implant, and 16 were fitted with an acrylic implant. We measured horizontal and vertical saccadic amplitudes of both the artificial eye and the healthy eye. Measurements were performed with the magnetic search coils technique. Saccadic amplitudes of the artificial eye were compared with the healthy eye of the patient. The amplitudes of the healthy eyes were compared with saccadic amplitudes of control participants. The interval from surgery to measurements was at least 3 months in all patients. Saccadic gain (artificial eye and eye amplitude divided by target amplitude) and saccadic symmetry (artificial eye amplitude divided by healthy eye amplitude) were calculated. MAIN OUTCOME MEASURES: Saccadic gain and saccadic symmetry. RESULTS: The gain in the healthy eyes of the patients was comparable with the gain of the control eyes. Saccadic symmetry was 1.0 in control participants. In patients, it was 0.334 in horizontal saccades and 0.577 in vertical saccades. However, saccadic symmetry did not differ significantly between the acrylic group and the hydroxyapatite group (P: > 0.1 for any saccadic direction). Equivalence was detectable with a power more than 90% for horizontal saccades and more than 80% for vertical saccades. Curvilinearity was rejected for both patient groups and for all saccadic directions (P: > 0.5). CONCLUSIONS: When no motility peg is placed, acrylic and hydroxyapatite spherical implants yield comparable saccadic amplitudes of the artificial eye. Artificial eye amplitudes were markedly more restricted horizontally than vertically. In all saccadic directions, the relation between target amplitude and artificial eye amplitude was linear.

Acrylic Resins↗
PubMed1979

Severe enophthalmos following irradiation of the anophthalmic socket: surgical approaches.

The combination of enucleation plus irradiation often leads to a significant orbital volume loss and severe enophthalmos. Surgery for enophthalmos correction involves the replacement of intraorbital volume with autogenous or alloplastic material, either subperiorbitally, or in the muscle cone. Subperiorbital implants technically are easiest to perform, but implant absorption, migration, and other complications can be cosmetically unacceptable. Alternatively, approaching the muscle cone through a lateral subperiorbital dissection avoids trauma to the irradiated conjunctiva; allows easy access to the muscle cone for implantation of silicone spheres glass beads, or RTV silicone; and provides an excellent cosmetic result.

Adolescent↗
PubMed1979

Surgical approaches to the orbit.

After the appropriate workup, the size, location, and consistency of an orbital mass will dictate the surgical approach chosen. Masses in the muscle cone area or in the temporal portion of the orbit are most easily approached by the lateral orbitotomy. For lacrimal gland tumors a superior extension of the lateral orbitotomy will expose the lacrimal fossa area. The superior medial subperiosteal approach is indicated for masses in the superior medial orbit. For the more difficult area between the medial rectus and optic nerve, the combination lateral-medial orbitotomy through the medial fornix will expose the medial retrobulbar area. To reduce postoperative morbidity, emphasis is placed on adequate exposure, careful manipulation of tissues, and meticulous hemostasis.

Cranial Nerve Neoplasms↗
PubMed1980

Idiopathic acquired melanosis.

Acquired melanosis is the clinical term used to describe a slowly enlarging, diffusely pigmented lesion of the conjunctiva which may also involve the lids, caruncle or cornea. The controversial histologic classifications are reviewed and suggestions made regarding useful and accurate terminology. In acquired melanosis, development of malignant melanoma may occur. Previous work indicates that the prognosis of the resulting melanoma may be assessed by histopathologic staging as is done in cutaneous melanoma. The suggested management of acquired melanosis is close observation with excisional biopsies of areas clinically suggestive of invasive melanomas. Examination of the entire biopsy by serial or staged sections is essential for accurate histopathologic diagnosis. Invasive melanomas have been conventionally treated by local excision or exenteration. Radiation therapy may be a beneficial adjunct.

Adult↗
PubMed1984

von Recklinghausen neurofibromatosis. II. Incidence of optic gliomata.

The association of optic glioma with von Recklinghausen neurofibromatosis (NF) is well established. However, the incidence of these tumors in a large population of NF patients, prospectively evaluated with modern radiologic techniques, has not been established. We investigated the ophthalmic and intracranial features of NF in 217 patients aged 4 weeks to 69 years, in whom the diagnosis was based on stringent criteria. Tumors at various locations along the anterior visual pathway occurred in 15% of patients and were occasionally bilateral. The mean age of patients with chiasmal tumors was approximately 15 years less than patients with tumors of the optic nerve only. Two-thirds (67%) of all tumors were neither suspected historically nor detected by ophthalmologic examination. Neither the ophthalmoscopic absence of optic atrophy nor the normal results of roentgenograms of the optic foramina were reliable predictors of tumors detected by CT scan. The presence of optic glioma is not correlated to other ocular, skeletal, neurologic, or anamnestic risk factors.

Adolescent↗
PubMed1984

von Hippel-Lindau disease: a familial, often lethal, multi-system phakomatosis.

A review of 36 cases of von Hippel-Lindau disease underscores the important role of the ophthalmologist in evaluating this phakomatosis. Retinal angiomatosis occurred in almost two-thirds of those affected, was usually diagnosed before other target organs, and frequently led to decreased visual acuity. Common non-ophthalmic manifestations included cerebellar (69%), medullary (11%) and spinal (8%) hemangioblastoma, renal cell carcinoma (22%), and polycythemia (17%). Pheochromocytoma was a les common association. The most common causes of death were cerebellar hemangioblastoma and renal cell carcinoma. For the surveillance of suspected von Hippel-Lindau victims, a detailed family history and retinal examination is recommended. Essential laboratory tests include a complete blood count and computed tomography of the head, upper cervical region, and abdomen. Periodic pre-symptomatic screening is imperative for follow-up investigation of affected individuals to determine possible additional occult manifestations and of non-affected family members at risk to von Hippel-Lindau disease.

Adenocarcinoma↗
PubMed1985

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.

Biopsy, Needle↗
PubMed1986

Retinoblastoma. A clinical, immunohistochemical, and electron microscopic case report.

A 4-year, 9-month-old boy had a history of leukocoria of the right eye for approximately six months prior to admission. The other eye was normal. There was no family history of retinoblastoma. Funduscopy disclosed a large white mass extending from the nasal pars plana to the mid-pupillary zone and the posterior pole with a near total retinal detachment in the superior temporal quadrant. A B-scan ultrasound showed an echo dense area of the anterior portion of the mass. A CT scan showed intraocular tissue densities with no evidence of optic nerve involvement or extraocular extension. Immunohistochemistry of a fresh frozen portion of tumor revealed reactivity with antibodies directed against interphotoreceptor retinoid-binding-protein (IRBP), neuron-specific enolase, glial fibrillary acidic protein, S-antigen, focal reactivity with opsin, and scattered cytoplasmic staining for cyclic GMP and cyclic GMP phosphodiesterase. Biochemical analysis of fresh frozen tissue samples confirmed the presence of IRBP. Transmission electron microscopy disclosed occasional Flexner-Wintersteiner rosettes connected by zonula adherens-like junctions. These showed inner segment-like structures containing prominent mitochondria, portions of cilia and fragments of outer segment material. These data, along with the immunocytochemistry indicates a predominant neuronal nature of the tumor cells with significant photoreceptor-like differentiation.

Child, Preschool↗
PubMed1986

Ocular findings in patients with dysplastic nevus syndrome.

Ninety-two patients with biopsy-proven dysplastic nevus syndrome were evaluated ophthalmologically and compared to a non-dysplastic nevus syndrome control population. A statistically significant increase in the percentage of dysplastic nevus syndrome patients versus controls harboring conjunctival nevi (9.78% vs. 1.85%), iris nevi (31.52% vs. 12.96%), and choroidal nevi (18.48% vs. 4.63%) was found. Although not statistically significant, the number of iris nevi encountered per patient was greater for the dysplastic nevus syndrome group. All dysplastic nevus syndrome patients and their families should be evaluated ophthalmologically, with special attention being given to dilated indirect ophthalmoscopy.

Adolescent↗
PubMed1987

Multivariate analysis of risk factors for metastasis in retinoblastoma treated by enucleation.

Retinoblastoma, when it kills by metastasis, almost always does so within 5 years of enucleation. The median time to death was 6.4 months in unilateral cases and 14.2 months in bilateral cases. Multivariate statistical analysis of 361 cases of retinoblastoma indicated that histologic detection of invasion of the tumor into the optic nerve or orbit were the risk factors most highly predictive of death from retinoblastoma. Choroidal invasion was not significantly associated with a fatal outcome. Histologic evidence of a cataract, failure to diagnose retinoblastoma clinically, and bilaterality were also significant risk factors. The authors believe that in at least two of the cases of bilateral retinoblastoma death did not result from metastasis to the brain as was originally diagnosed, but resulted from the development of an independent primary intracranial neuroblastic tumor, a syndrome designated "trilateral retinoblastoma."

Cataract↗
PubMed1987

Retinoblastoma. Immunohistochemistry and cell differentiation.

Tumor from eight enucleated eyes was analyzed by immunohistochemistry, using a panel of specific antibodies including interphotoreceptor retinoid-binding protein (IRBP), S-antigen (S-Ag), opsin, neuron-specific enolase (NSE), glial fibrillary acidic protein (GFAP), laminin, and vimentin. In addition, immunoelectron microscopy and enzyme-linked immunosorbent assay (ELISA) for IRBP were performed. Immunohistochemical staining disclosed the most pronounced labeling of tumor cells with NSE and IRBP antibodies. A correlation was found between the degree of tumor differentiation and amount of IRBP, a protein specifically synthesized by photoreceptor cells. Moderate labeling of the better differentiated tumors was also observed with antibodies against S-Ag and focal labeling in a few tumors with opsin antibodies. Anti-GFAP labeling was limited to a smaller number of reactive glial cells and perivascular glial cells. These data indicate the essential neuronal nature of retinoblastoma tumor cells in situ as well as at least partial photoreceptor-like features, as shown by the presence of recognized photoreceptor cell markers (IRBP, S-Ag, opsin). Tissue culture studies using the human Y-79 retinoblastoma cell line also demonstrate that the tumor cells are primitive multipotential retinoblasts capable of at least partial differentiation along neuronal, glial, or pigment epithelial cell lines.

Antibodies, Anti-Idiotypic↗