RETINOBLASTOMA is the most common malignant intraocular tumor in children; it is also one of the most highly curable pediatric solid tumors if detected early. The conventional treatment of retinoblastoma is primary enucleation. Recent research reported a trend toward decreasing frequency of enucleation in the management of retinoblastoma. The trend toward globe-sparing interventions has been largely attributed to earlier diagnosis and recent success with conservative globe-sparing treatment options. Currently, globe-sparing interventions include first-line chemotherapy or chemoreduction, subconjunctival chemotherapy, systemic chemotherapy for metastasis, transpupillary thermotherapy (TIT), chemothermotherapy (CTT), laser photocoagulation, cryotherapy, brachytherapy, and external beam radiotherapy (EBRT). Expanded clinical options currently available have markedly decreased the overall enucleation rate for retinoblastoma CLINICAL SCENARIO: A 10-month old boy is brought to an ophthalmologist because of cats eye reflex in the left eye. The patient had undergone enucleation of his right eye for glaucomatous stage retinoblastoma 6 months earlier. Examination revealed the presence of a solitary retinal mass of about 12 mm in diameter, located nasal to the disc. There was no evidence of vitreous seeding Realizing that this was the only eye of the patient, the ophthalmologist wants to do everything humanly possible to preserve it. He has heard about chemothermotherapy (CTT) but is not sure if this was the best alternative he can offer CLINICAL QUESTION: Among patients with retinoblastoma, is chemoreduction combined with adjuvant treatment effective in preserving the globe and vision? (Author)
EYE ENUCLEATION

Objectives: To evaluate the prevalence, etiologies, demographics, and clinical presentation of enucleated pseudoretinoblastoma. Methods: This retrospective study reviewed ocular pathology records of enucleated globes with clinically diagnosed or suspected retinoblastoma submitted to a public university ocular pathology laboratory from 2013 to 2018. Hematoxylin-eosin-stained sections of pseudoretinoblastoma cases were reevaluated, and additional clinical data were taken from hospital charts. Results: Of the 211 enucleated eyes with clinically diagnosed or suspected retinoblastoma, 202 (95.7%) had histologically confirmed retinoblastoma, while 9 (4.3%) had pseudoretinoblastoma. The most common ocular conditions mimicking retinoblastoma were retinal dysplasia (2 eyes) and persistent fetal vasculature (2 eyes). The pseudoretinoblastoma group consisted of 4 females and 5 males, and enucleated were 6 right eyes and 3 left eyes. The mean age at the time of enucleation was 3.65 years, and the mean symptom duration was 17.36 months. Leukocoria, which was noted in 4 patients, was the most frequent initial symptom. No significant difference between the pseudoretinoblastoma group and the retinoblastoma group were found in terms of sex, laterality of the enucleated eye, age at the time of enucleation, and symptom duration. Conclusion In this retrospective review, the prevalence of pseudoretinoblastoma in enucleated globes clinically suspected or diagnosed with retinoblastoma was 4.3%. Persistent fetal vasculature and retinal dysplasia were the most common pseudoretinoblastomas. Clinicians should perform a thorough clinical evaluation and judiciously utilize the available diagnostic means to differentiate retinoblastoma from pseudoretinoblastoma.
eye enucleation ; retinal dysplasia ; retinoblastoma

Eye Enucleation ; Humans ; Infant ; Retinal Neoplasms ; surgery ; Retinoblastoma ; surgery ; Vitrectomy

Self-enucleation represents an extreme but fortunately rare form of deliberate self-harm. Case reports of patients who self-enucleate reveal some common features. A case of auto-enucleation in a young schizophrenic patient and a short discussion on deliberate self-harm are presented.
Adult ; Eye Enucleation ; Female ; Hallucinations ; Humans ; Schizophrenia ; complications ; Schizophrenic Psychology ; Self-Injurious Behavior

Purpose: To combine the advantage of porosity and biocompatibility of Biphasic Tricalcium Phosphate implant with Muscle Resection Methodology: Non-randomized, uncontrolled clinical case series Results: No migration or extrusion was observed. With improved muscle resection, socket and prosthesis mobility were noted. (Author)
Human ; Young Adult ; Adolescent ; EYE ENUCLEATION ; PROSTHESIS ; IMPLANTS ; ORBITAL IMPLANTS ; ARTIFICIAL IMPLANTS

PURPOSE: Retinoblastoma (RB) is the most common primary malignant intraocular tumor in children. Although systemic chemotherapy has been the primary treatment, intra-arterial chemotherapy (IAC) represents a new treatment option. Here, we performed alternate systemic chemotherapy and IAC and retrospectively reviewed the efficacy and safety of this approach. METHODS: Patients diagnosed with intraocular RB between January 2000 and December 2011 at Severance Children's Hospital, Yonsei University, were reviewed. Before February 2010, the primary treatment for RB was chemotherapy (non-IAC/CTX). Since February 2010, the primary treatment for RB has been IAC (IAC/CTX). External beam radiotherapy or high-dose chemotherapy were used as "last resort" treatments just prior to enucleation at the time of progression or recurrence during primary treatment. Enucleation-free survival (EFS) and progression-free survival were assessed. RESULTS: We examined 19 patients (median age, 11.9 months; range, 1.4 to 75.6 months) with a sum of 25 eyes, of which, 60.0% were at advanced Reese Ellsworth (RE) stages. The enucleation rate was 33.3% at early RE stages and 81.8% at advanced RE stages (P=0.028). At 36 months, EFS was significantly higher in the IAC/CTX group than in the non-IAC/CTX group (100% vs. 40.0%, P=0.016). All 5 patients treated with IAC achieved eye preservation, although most patients were at advanced RE stages (IV-V). CONCLUSION: Despite the limitation of a small sample size, our work shows that an alternative combined approach using IAC and CTX may be safe and effective for eye preservation in advanced RB.
Child ; Disease-Free Survival ; Drug Therapy, Combination ; Eye ; Eye Enucleation ; Humans ; Infusions, Intra-Arterial ; Recurrence ; Retinoblastoma ; Retrospective Studies ; Sample Size

OBJECTIVETo evaluate the therapeutic effects and complications of hydroxyapatite (HA) orbital implantation on patients after trauma-related surgeries.

METHODSRetrospective analysis was made from 211 cases (211 eyes) who underwent HA orbital implant placement after trauma-related enucleation or evisceration, including 68 cases of evisceration and primary HA implant placement, 77 cases of enucleation and HA implant placement wrapped with multi-windowed sclera, 66 cases of enucleation and HA implant placement free of wrapping. All the cases were followed up for 1-5 years to observe the therapeutic effects and major complications.

RESULTSFive of 211 cases had wound dehiscence. Ten cases had HA implants exposure, including 1 case suffering severe orbital infection and requiring HA implant removal. The implants exposure incidences by the three surgical methods were from 1.30% to 10.06% and averaged 4.74%. Significant difference was found in late exposure incidence and total incidence from the three methods (chi(2)=13.372, P < 0.01 and chi(2)=7.540, P < 0.05). Two cases had shrinkage of the lower fornix. Enophthalmos occurred in 1 case treated by method 1 and was corrected by implanting porous polyethylene (Medpor) plate into the bottom of orbit. In 210 cases, the artificial eye moved well and the cosmetic results were satisfactory.

CONCLUSIONSDifferent surgical methods have their own merit and disadvantage. Enucleation and placement of HA implant wrapped with multi-windowed sclera has corroborated fewer complications than others.

Adult ; Durapatite ; Eye Enucleation ; methods ; Eye Evisceration ; methods ; Eye Injuries, Penetrating ; surgery ; Female ; Humans ; Male ; Middle Aged ; Ophthalmologic Surgical Procedures ; methods ; Orbital Implants ; Prosthesis Implantation ; methods ; Retrospective Studies

Schwannomas rarely present as intraocular tumors and are often misdiagnosed as malignant melanoma. We describe a choroidal schwannoma confirmed by sclerouvectomy. A 30-year-old woman presented with a large nonpigmented intraocular mass of the choroid in the right eye and underwent surgical excision by sclerouvectomy. Histologically, the tumor was composed of a mixture of cellular solid components (Antoni A) and loose myxoid components (Antoni B). The tumor was eventually diagnosed as a schwannoma. Currently available ancillary studies are still of little value in definitively differentiating schwannomas from other choroidal tumors. In the case of atypical findings for a malignant melanoma, a benign neoplasm should be included in the differential diagnosis. This patient avoided enucleation by first having the mass excised. We are unaware of previous reports in which a choroidal schwannoma was diagnosed by surgical excision.
Adult ; Choroid/*pathology ; Choroid Neoplasms/*diagnosis/surgery ; Diagnosis, Differential ; Eye Enucleation/*methods ; Female ; Humans ; Magnetic Resonance Imaging ; Neurilemmoma/*diagnosis/surgery

PURPOSE: Radiotherapy is effective in local treatment for retinoblastoma. However, asymmetric facial hypoplasia after radiation is a serious late effect. This study was performed to investigate the effects of enucleation and chemotherapy with or without radiotherapy in advanced intraocular and intraorbital retinoblastoma. METHODS: Between 1985 October and 2006 December, the records of thirty five patients who were diagnosed as retinoblastoma at Yeungnam University Hospital were reviewed. Advanced intraocular and intraorbital retinoblastoma patients classified as Reese-Ellsworth group III, IV, and V and Grabowski- Abramson class II were selected for the study. RESULTS: Eighteen patients were enrolled in this study. All patients were enucleated and had received chemotherapy. Nine patients received radiotherapy and nine patients didn't receive radiotherapy. Tumor cells were found on resection margin of optic nerve in five of nine patients who received radiotherapy, but none of nine who didn't receive radiotherapy. Chemotherapy included vincristine, adriamycin, cyclophosphamide, VM-26, cisplatin before 2001, and vincristine, etoposide, and carboplatin after 2001. There were no recurrences or metastases in nine patients who didn't receive radiotherapy. But two of nine patients who received radiotherapy had metastases to brain. However, all survivors who received radiotherapy had significant facial asymmetry. CONCLUSION: In advanced intraocular and intraorbital retinoblastoma without tumor cell on resection margin of optic nerve, enucleation and chemotherapy without local radiotherapy appears to be safe for long-term survival. However, in those with tumor cells on resection margin of optic nerve, enucleation and chemotherapy with local radiotherapy seems to be necessary to improve survival.
Brain ; Carboplatin ; Cisplatin ; Cyclophosphamide ; Doxorubicin ; Etoposide ; Eye Enucleation ; Facial Asymmetry ; Humans ; Neoplasm Metastasis ; Optic Nerve ; Recurrence ; Retinoblastoma ; Survivors ; Teniposide ; Vincristine

Calcium-binding proteins play an important role in the protection, differentiation, and reorganization of the central nervous system. The effects of neonatal retinal deafferentiation on calretinin, and tracing of retinotectal pathway were examined immunohistochemically in the superficial layer of the rat superior colliculus. Tracing with cholera toxin was revealed on the superior colliculus contralateral to the ocular injection. On the contralateral side of superior colliculus, the calretinin-immunoreactive (IR) cells were dramatically increased, calretinin-IR fibers were markedly decreased in the superficial layer. These results show that retinal deafferentation results in an increase of calretinin-immunoreactive cells within the superficial layers of the superior colliculus, which suggest reorganization of neurons in superior colliculus.
Animals ; Calbindin 2 ; Calcium-Binding Proteins ; Central Nervous System ; Cholera Toxin ; Eye Enucleation* ; Neurons ; Rats* ; Retinaldehyde ; Superior Colliculi