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

Objective: This study reported the demographic profile, clinical presentation, treatment, and outcomes of retinoblastoma (RB) patients seen at Jose R. Reyes Memorial Medical Center (JRRMMC) from January 2011 to December 2020. Methods: This was a single-center, retrospective study. Medical records of patients diagnosed with RB at JRRMMC from January 2011 to December 2020 were reviewed. Descriptive statistics were used to summarize the characteristics of the participants. Results: A total of 31 confirmed RB cases were seen, with almost half residing outside Metro Manila (52%). The median age at diagnosis was 2 years, with a slight male predominance (55%). Majority (81%) of patients had unilateral presentation, with leukocoria as the most common sign (19%) prompting consult. Nearly half (43%) of 37 eyes were diagnosed as Group E using the International Classification of Retinoblastoma (ICRB) system. Majority were in the advanced stage; 57% of eyes underwent enucleation and 29, 3 and 3% of patients required additional treatments such as chemotherapy, laser, and radiotherapy, respectively. RB was confirmed in 20 eyes (54%) through histopathology. Survival outcomes showed that 4 patients (13%) were alive, with either completed or ongoing treatment, and 19 (61%) did not complete prescribed management or were lost to follow-up. There were 8 (26%) known deaths. Conclusion Majority of cases were unilateral and at an advanced stage needing enucleation which may indicate low levels of awareness and screening efforts. Survival rates are difficult to ascertain due to patients abandoning treatment that may be attributed to limited social service support. The study reflects the 10-year data prior to the establishment of a collaborative, multispecialty RB team in the institution and exposes various areas that need to be addressed to improve clinical outcomes.
Retinoblastoma ; Enucleation ; Chemotherapy ; Epidemiology

PURPOSE: We present clinical results of the use of the multipurpose conical porous synthetic orbital implant (MCOI) in surgical procedures of evisceration, enucleation, and secondary enucleation in ophthalmology patients. METHODS: A retrospective review was performed of 59 eyes in which conical implants were used, including 36 cases of eviscerations, 11 enucleations, and 9 secondary enucleations. In all of the cases, the follow-up period was greater than six months between 2004 and 2013. The results focus on documenting surgical findings, as well as postoperative complications among patients. RESULTS: Superior sulcus deformities were found in six eyes (10.2% of conical implant patients), and two eyes received additional surgical interventions to correct the deformities (3.4%). Blepharoptosis was found in four eyes (6.8%), two of which received upper eyelid blepharoplasty (3.4%). Fornix shortening was reported in only one eye (1.7%). Forty-one eyes had a satisfactory cosmetic appearance after the final prosthetic fitting of conical implants (69.5%). The most frequent postoperative complication was orbital implant exposure, which seemed to occur when the preoperative status of the conjunctiva, Tenon's capsule, and sclera preservation were poor in the eyes of the patients. CONCLUSIONS: There was a lower incidence of blepharoptosis and fornix shortening with the MCOI in comparison to spherical implants, while the incidence of orbital implant exposure was similar with the MCOI in comparison to other types of orbital implants. In addition, the MCOI may have advantages with respect to postoperative cosmetic outcomes.
Adult ; Eye Diseases/*surgery ; *Eye Enucleation ; *Eye Evisceration ; Female ; Follow-Up Studies ; Humans ; Incidence ; Male ; Middle Aged ; *Orbital Implants ; Postoperative Complications/*epidemiology ; Prosthesis Implantation/*methods ; Republic of Korea/epidemiology ; Retrospective Studies ; Time Factors ; Treatment Outcome

No abstract available.
Animals ; Anti-Bacterial Agents ; Antihypertensive Agents ; Corneal Edema/diagnosis/etiology/therapy ; Corneal Injuries/diagnosis/*etiology/therapy ; Corneal Ulcer/diagnosis/etiology/therapy ; Drug Combinations ; Eye Enucleation ; Eye Pain/etiology ; Glaucoma/diagnosis/etiology/therapy ; Glucocorticoids ; Humans ; Insect Bites and Stings/diagnosis/*etiology/therapy ; Intraocular Pressure ; Male ; Middle Aged ; Mydriatics ; Ophthalmia, Sympathetic/diagnosis/*etiology/therapy ; Visual Acuity ; *Wasps

Retinoblastoma is a rare disease, but most common tumor which arises in eye. It can affect one or both eyes, and the main pathophysiology is explained by the "Two-hit theory" - the germline mutation of the RB1 gene. Most common clinical symptoms are leuocoria, strabismus, poor visual tracking, glaucoma, and orbital cellulitis. Diagnosis is made by ophthalmologist through fundoscopic examination; Examination under General Anesthesia (EUA) is recommended until the age 3. Orbital CT and MRI can detect the tumor invasion on optic nerve, central nervous system. CSF studies, examination of bone is helpful if the distant metastasis is suspected. Biopsy is rarely done unless in the case of enucleation. Enucleated eye should be explored for the invasion to the optic nerve, choroid, anterior chamber, iris and pupil. Treatment strategies can be different according to the disease status. If the single eye is involved, the treatment goal will be the removal of tumor and prevention of relapse. Local therapies include cryotherapy, laser photocoagulation, thermotherapy can be the choice, and if the tumor is too large for the local therapy, enucleation should be concerned. Nowadays, chemo-reduction combined with local therapy, intra-arterial and intravitreous chemotherapeutic agent injections are studied to avoid enucleation. In bilateral retinoblastoma, multidisciplinary treatments include chemoreduction, external beam radiotherapy, local therapy and other experimental therapies are needed: like intra-arterial injection, intra-vitreal injection, and high-dose chemotherapy with autologous stem cell transplantation. Early detection of retinoblastoma is important to save the vision and eyeball.
Anesthesia, General ; Anterior Chamber ; Biopsy ; Central Nervous System ; Choroid ; Cryotherapy ; Diagnosis ; Drug Therapy ; Eye Enucleation ; Germ-Line Mutation ; Glaucoma ; Hyperthermia, Induced ; Injections, Intra-Arterial ; Intravitreal Injections ; Iris ; Light Coagulation ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Optic Nerve ; Orbit ; Orbital Cellulitis ; Pupil ; Radiotherapy ; Rare Diseases ; Recurrence ; Retinoblastoma ; Stem Cell Transplantation ; Strabismus ; Therapies, Investigational

BACKGROUNDLocal resection is an effective method for treating the uveal melanoma. The aim of this study is to evaluate the survival and clinical outcomes of patients with uveal melanoma treated by local resection or enucleation.

METHODSTotally, 167 consecutive patients with uveal melanoma were recruited for the study, of whom 57 patients were treated with local resection and 110 patients were treated with enucleation. The main outcome was measured by the visual acuity, local recurrence, eye retention, metastases, and melanoma-related mortality.

RESULTSThere were statistically significant differences in the largest basal diameter of the tumor (t = -3.441), the tumor thickness (t = -4.140), the ciliary body infiltration (χ(2) = 8.391), and the duration of follow-up (Z = 3.995) between the two groups (P < 0.05). The univariate survival analysis showed that the method of treatment was not significantly associated with metastases. The Cox proportion hazard analysis showed that the risk factors for metastasis involved the age at the time of diagnosis (RR = 1.752, 95% CI 1.066-2.880, P = 0.027), the largest basal diameter of the tumor (RR = 3.508, 95% CI 1.934-6.336, P = 0.000), and the histological type (RR = 2.444, 95% CI 1.106-5.877, P = 0.046). The 5-year metastases rate was 18.60% for the group with local resection and 27.81% for enucleation (χ(2) = 1.214, P > 0.05); the 5-year melanoma-related mortality was 16.27% for the group with local resection and 25.33% for enucleation (χ(2) = 1.304, P > 0.05). The 5-year local tumor recurrence rate was 29.50% and the 5-year accumulated eye retention rate was 69.00% after local resection. The visual acuity which light perception or better of 60 months after local resection was observed in 25 (92.60%) among persons retaining eye.

CONCLUSIONSThe survival outcomes of the patients with local resection were not worse than that of the patients with enucleation, and local resection could make the patient retain eye and partial visual functions. Hence, local resection may be an effective method for patients with uveal melanoma eligible for operation.

Adult ; Eye Enucleation ; methods ; Female ; Humans ; Male ; Melanoma ; surgery ; Treatment Outcome ; Uveal Neoplasms ; surgery ; Visual Acuity ; physiology

PURPOSE: To investigate the analgesic effect and incidence of postoperative nausea and vomiting (PONV) between the opioid fentanyl and the non-steroidal anti-inflammatory drug ketorolac in patients who underwent eye amputation surgery. METHODS: Retrospective observational case series. Eighty-two patients underwent evisceration or enucleation surgery by one surgeon over a 2-year period. Fentanyl by intravenous patient-controlled analgesia (IV-PCA) at 20 microg/kg with 12 mg/kg ondansetron or intravenous ketorolac at 2 mg/kg/day was administered to patients at postoperative days 0, 1, and 2. The pain score was measured using an 11-point visual analog scale (VAS). The incidence of severe nausea requiring anti-emetics and the incidence of vomiting were reviewed. RESULTS: The mean postoperative VAS in the fentanyl group was significantly lower than that in the ketorolac group on the day of operation for both types of surgery (p = 0.001 and p = 0.004, respectively). At postoperative days 1 and 2, the mean VAS was not different between the two groups for either surgical type (p > 0.05 for both days). The mean VAS was significantly higher in eviscerated patients than in enucleated patients at postoperative days 0 and 1 in the fentanyl group (p = 0.023 and p = 0.016, respectively). However, this was not observed in the ketorolac group. The incidence of PONV was higher in the fentanyl group than in the ketorolac group, although this was not statistically significant for either surgical type (p > 0.05 for both groups). CONCLUSIONS: Fentanyl was more effective as an analgesic than was ketorolac on the day of operation for both surgical types. There was no difference between the two analgesics on postoperative day 1. The analgesic effect of fentanyl in enucleated patients was significantly higher than in eviscerated patients at postoperative days 0 and 1. The use of fentanyl by IV-PCA was associated with greater PONV despite co-administration with anti-emetics, although this finding was not significant.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Analgesics, Opioid/therapeutic use ; Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; *Eye Enucleation ; *Eye Evisceration ; Female ; Fentanyl/*therapeutic use ; Humans ; Ketorolac/*therapeutic use ; Male ; Middle Aged ; Pain, Postoperative/*drug therapy ; Retrospective Studies ; Treatment Outcome ; Young Adult

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

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

A 68-year-old woman presented with pain in her left eye. Necrosis with calcium plaques was observed on the medial part of the sclera. Aspergillus fumigatus was isolated from the culture of the necrotic area. On systemic work-up including serum and urine electrophoresis studies, the serum monoclonal protein of immunoglobulin G was detected. The patient was diagnosed with monoclonal gammopathy of undetermined significance and fungal scleritis. Despite intensive treatment with topical and oral antifungal agents, scleral inflammation and ulceration progressed, and scleral perforation and endophthalmitis developed. Debridement, antifungal irrigation, and tectonic scleral grafting were performed. The patient underwent a combined pars plana vitrectomy with an intravitreal injection of an antifungal agent. However, scleral and intraocular inflammation progressed, and the eye was enucleated. Aspergillus fumigatus was isolated from the cultures of the eviscerated materials. Giemsa staining of the excised sclera showed numerous fungal hyphae.
Aged ; Amphotericin B/administration & dosage ; Antifungal Agents/administration & dosage ; *Aspergillosis/therapy ; *Aspergillus fumigatus ; Disease Progression ; Eye Enucleation ; Female ; Humans ; Injections, Intraocular ; Paraproteinemias/*complications ; Sclera/pathology/ultrasonography ; Scleritis/*complications/diagnosis/*microbiology/physiopathology ; Vitrectomy