Barrett G. Haik

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.

Treatment of Intraocular Retinoblastoma With Vincristine and Carboplatin

PURPOSE To evaluate the efficacy of chemoreduction using vincristine and carboplatin in preventing or delaying external-beam radiotherapy (EBRT) or enucleation in patients with intraocular retinoblastoma. PATIENTS AND METHODS Twenty-five patients (43 eyes) with newly diagnosed intraocular retinoblastoma received primary treatment with eight courses of vincristine and carboplatin. Focal treatments were delayed until documentation of disease progression. Outcome measures for each eye were length of time to disease progression, avoidance or delay of EBRT, and globe survival. Event-free survival was defined as the length of time to EBRT or enucleation. RESULTS Disease in all eyes responded to chemotherapy and progressed in only two patients before completion of the eight courses of therapy. Disease in all but four eyes progressed and required focal treatments. Event-free survival estimates at 2 years were 59.2% +/- 12.0% for Reese-Ellsworth group I, II, and III eyes and 26.3% +/- 9.2% for group IV and V eyes. Nineteen eyes (44.2%) required EBRT and 13 eyes (30.2%) were enucleated. The ocular salvage rate was 83.3% for Reese-Ellsworth group I to III eyes and 52.6% for group IV and V eyes. For those patients receiving EBRT, the median time from enrollment to EBRT was 9.5 months (median age at EBRT, 21 months). CONCLUSION In combination with appropriate early intensive focal treatments, chemoreduction with vincristine and carboplatin, without etoposide, may be an alternative treatment for patients with early-stage intraocular retinoblastoma, although additional studies are needed. Patients with advanced intraocular disease require more aggressive treatments.

Retinoblastoma: One World, One Vision

Retinoblastoma is curable when diagnosed early and treated appropriately; however, the prognosis is dismal when the basic elements of diagnosis and treatment are lacking. In developing countries, poor education, lower socioeconomic conditions, and inefficient health care systems result in delayed diagnosis and suboptimal care. Furthermore, the complexity of multidisciplinary care required is seldom possible. Whereas ocular salvage is a priority in the Western world, death from retinoblastoma is still a major problem in developing countries. To bring the 2 ends of this spectrum together and provide a forum for discussion, the “One World, One Vision” symposium was organized, at which clinicians and researchers from various cultural, geographic, and socioeconomic backgrounds converged to discuss their experiences. Strategies for early diagnosis in developing countries were discussed. Elements of the development of retinoblastoma centers in developing countries were discussed, and examples of successful programs were highlighted. An important component in this process is twinning between centers in developing countries and mentor institutions in high-income countries. Global initiatives by nongovernmental organizations such as the International Network for Cancer Treatment and Research, Orbis International, and the International Agency for Prevention of Blindness were presented. Treatment of retinoblastoma in developing countries remains a challenge; however, it is possible to coordinate efforts at multiple levels, including public administrations and nonprofit organizations, to improve the diagnosis and treatment of retinoblastoma and to improve the outcome for these children.

Multiagent chemotherapy as neoadjuvant treatment for multifocal intraocular retinoblastoma.

PURPOSE To evaluate the efficacy of multiagent chemotherapy in the neoadjuvant treatment of retinoblastoma. DESIGN Noncomparative, prospective case series. PARTICIPANTS Twenty consecutive patients with multifocal intraocular retinoblastoma (4 unilateral, 16 bilateral [36 eyes]). INTERVENTION Eight cycles of chemotherapy with carboplatin and vincristine were administered at 3-week intervals over a 6-month period. Supplemental therapy was withheld until disease progression was documented. MAIN OUTCOME MEASURES Disease progression (defined as tumor growth, vitreous or subretinal seed progression, and new tumor formation), delay of external beam radiotherapy, and ocular survival. RESULTS Thirty-six eyes were treated. Eighteen eyes had Reese-Ellsworth group I-III tumors, and 16 eyes had Reese-Ellsworth group IV-V tumors at diagnosis. Two patients, who had unilateral disease at diagnosis, subsequently had tumors develop in the contralateral eye. Nineteen of 20 patients (95%) completed eight cycles of chemotherapy without disease progression. Three eyes of three different patients were successfully treated with chemotherapy alone. Thirty-three of 36 eyes (92%) progressed after completion of chemotherapy: 15 of the 18 eyes (83.3%) with Reese-Ellsworth group I-III and 16 of 16 eyes (100%) with Reese-Ellsworth group IV-V tumors. Seventeen eyes (52%) had growth of a tumor, whereas 14 eyes (42%) had progressive vitreous seeding, and 2 eyes (6%) had new tumors develop. Fifteen eyes (42%) required external beam radiotherapy. Twenty-nine of 36 (80.5%) eyes were salvaged. The median follow-up after chemotherapy was 19 months (range, 3-42 months). CONCLUSIONS Multiagent chemotherapy alone does not ensure a cure for multifocal intraocular retinoblastoma. Supplemental focal therapy is needed to control disease progression.

Cobalt plaque versus enucleation for uveal melanoma: comparison of survival rates.

Two hundred and twenty-three patients treated by cobalt plaque for uveal melanoma were compared with 416 patients treated by enucleation for uveal melanoma in terms of patient survival. The median follow-up time for the patients treated by cobalt plaque was 4.3 years. Kaplan-Meier survival curves were calculated up to five years following treatment based on time to tumour-related deaths. Coxs proportional hazards multivariate analysis was performed to determine which variables were related to melanoma-related deaths while controlling for age, size, and location of the tumours. Statistically significant predictive factors were location of tumour and largest tumour dimension. There was not a statistically significant difference in survival between patients treated by cobalt plaque and those treated by enucleation.

Carboplatin-Associated Ototoxicity in Children With Retinoblastoma

PURPOSE Carboplatin-induced ototoxicity remains poorly defined but is of potential great consequence in children with retinoblastoma. We retrospectively assessed the incidence of ototoxicity and its risk factors in children with retinoblastoma who were treated with carboplatin. PATIENTS AND METHODS We reviewed the audiologic test results of 60 patients with retinoblastoma who received front-line treatment with systemic carboplatin and vincristine according to the St Jude RET-3 protocol (n = 23) or best clinical management (n = 37). Ototoxicity was evaluated by three different grading systems. RESULTS Twelve patients (20%) developed ototoxicity at some time after treatment initiation; however, ototoxicity resolved in two patients, and thus,10 patients (17%) had sustained hearing loss as documented at their most recent audiologic evaluation. Nine of these 10 patients had grade 3 or 4 ototoxicity, and nine patients were less than 6 months of age at the start of chemotherapy. Age at the start of chemotherapy was the only risk factor identified as a significant predictor of sustained hearing loss. Younger age was associated with an increased incidence of hearing loss. The different ototoxicity grading systems showed good overall agreement in the identification of patients with ototoxicity. Agreement was greatest between the Brock and Childrens Cancer Group systems. CONCLUSION We found that young patients with retinoblastoma who were treated with systemic carboplatin had a higher incidence of ototoxicity than previously reported. Younger patients (< 6 months of age at the start of treatment) were more likely to have ototoxicity than were older patients. Children treated with carboplatin should routinely undergo thorough, long-term audiologic monitoring.

Treatment of metastatic retinoblastoma.

PURPOSE The risk for death in patients with retinoblastoma is increased in those who present with metastatic disease, and the role of intensive chemotherapy and autologous hematopoietic stem cell rescue in these patients remains unclear. DESIGN Nonrandomized interventional case series. PARTICIPANTS Four consecutive patients with metastatic retinoblastoma. METHODS We treated four patients with retinoblastoma metastatic to the bone and bone marrow with intensive chemotherapy, consolidation with megatherapy, and autologous hematopoietic stem cell rescue. Chemotherapy included courses of carboplatin and etoposide alternating with cyclophosphamide, etoposide, and either carboplatin or cisplatin. Radiation therapy was delivered to areas of bone metastases. MAIN OUTCOME MEASURES Patient survival. RESULTS All patients completed and responded to the scheduled therapy; complete response of the bone marrow disease was documented after two courses of chemotherapy in all cases. Two patients are long-term survivors. CONCLUSIONS The treatment described has been successful in obtaining disease-free survival in patients with metastatic retinoblastoma.

Retained Intraorbital Metallic Foreign Bodies

Purpose: To document the biological tolerance of retained metallic orbital foreign bodies managed with conservative treatment. Methods: A retrospective chart review of 43 patients treated between 1987 and 1993 with retained intraorbital metallic foreign bodies was performed. Age, sex, injury, imaging studies obtained, location of the foreign body, initial and final visual acuity, length of follow-up, treatment, and related adverse reactions and/or complications were recorded. Results: Fifty cases involving 34 male and 9 female patients ranging in age from 2 through 63 years (median, 25 years) were included. Seventeen cases were the result of gunshot wounds, 17 were from BB gun injuries, and 16 cases were from shotgun injuries. Forty-two patients had CT scans to assess the injury and one patient had plain radiographs. In 37 cases, the metallic foreign bodies were located posterior to the globe. There were 19 ocular penetrating injuries, 11 ocular contusion injuries (sclopetaria, commotio retinae, vitreous hemorrhages), and 20 injuries with no ocular involvement. Initial visual acuity ranged from 20/20 to no light perception, as did final visual acuity. The metallic foreign bodies were retained from 6 months to 68 years (median, 2 years). There were 19 ocular penetrating injuries, including 12 enucleations and 7 primary repairs. When contusion injuries were sustained, treatment consisted of vitrectomy for the removal of persistent hemorrhages (3 cases), exploration of the globe (1 case), and nonsurgical treatment (7 cases). Among the 20 cases with no ocular involvement, only 2 patients had secondary complications that required surgical intervention: One had mechanical strabismus, the other a sterile abscess. Visual acuity remained stable or improved in all cases. There were no late complications from the retained foreign bodies in 36 (95%) of 38 cases when the eye remained intact. Conclusions: Retained intraorbital metallic foreign bodies are well-tolerated and typically have minimal adverse visual prognosis. They should be managed conservatively in the absence of specific indications for removal.

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.

Succinylcholine Increases Intraocular Pressure in the Human Eye with the Extraocular Muscles Detached

BackgroundThe increase in intraocular pressure in the human eye that is associated with the use of intravenous succinylcholine has long been ascribed to contraction of the extraocular muscles leading to compression of the globe. This created concern that such contraction would extrude global contents in the patient with an open globe, and led clinicians to avoid the use of succinylcholine in these patients. MethodsThe authors studied 15 patients undergoing elective enucleation, and compared the intraocular pressure change after the administration of succinylcholine in the diseased eye after all the extraocular muscles had been detached to that of the normal eye that had the extraocular muscles attached. ResultsThe authors found no difference in baseline intraocular pressure (mmHg) between eyes (15.1 vs. 16.1) or at peak intraocular pressure (25.2 vs. 24.7), but did observe a significant difference in pressure in both eyes when baseline was compared with peak pressure. ConclusionsThe authors concluded that extraocular muscle contraction does not contribute to the increase in intraocular pressure after succinylcholine.