Hassan A. Aziz

Supraselective injection of intraarterial melphalan as the primary treatment for late presentation unilateral multifocal stage VB retinoblastoma

Purpose: The purpose of this study was to report a case of a 7-year-old girl with unilateral, multifocal Reese Ellsworth Stage Vb retinoblastoma who was successfully treated using intraarterial chemotherapy infusion as the primary therapy. Methods: This is an interventional case report. A 7-year-old girl presented with advanced unilateral retinoblastoma. The patient received intraarterial melphalan infusion therapy as the primary treatment. Results: Complete tumor resolution was seen at 1 month after intraarterial melphalan infusion. Conclusion: This case of advanced retinoblastoma in a 7-year-old girl was successfully treated with intraarterial melphalan infusion alone. Treatment resulted in complete resolution of the tumor 1 month after treatment. In comparison with systemic chemotherapy, intraarterial melphalan infusion therapy may be a less toxic and more effective primary treatment option in the future management of advanced retinoblastoma.

Retinoblastoma treatment burden and economic cost: impact of age at diagnosis and selection of primary therapy.

Purpose To follow the treatment history of patients with retinoblastoma to identify the trends in the number of hospital visits over time and the direct cost of medical care as determined by age at diagnosis and selected primary treatment modality. Design An Institutional Review Board (IRB) approved consecutive retrospective case series. Materials and methods Records from the Bascom Palmer Eye Institute were reviewed to identify 115 eligible patients (176 eyes) with retinoblastoma who underwent treatment at the Ocular Oncology Service between 1995 and 2010 and were available for extended follow-up evaluation. Results Bilateral disease was present in 53% (N = 61) of all patients, and 79% (N = 90) of patients were diagnosed in the first six months of life. Chemotherapy was used to treat 75% (N = 86) of all patients and 95% (N = 36) of patients diagnosed in the first six months of life. 100% (N = 4) of patients presenting between the age of five and nine were enucleated. Per episode of care, the lowest-cost treatment strategy was enucleation, followed by focal laser therapy, systemic chemotherapy with planned enucleation, systemic chemotherapy, and lastly, intra-arterial melphalan chemotherapy. Conclusion Age at diagnosis is directly associated with the type of treatment chosen for retinoblastoma. The burden of retinoblastoma treatment on children and families is significant. The direct medical cost of intra-arterial chemotherapy per episode of care is comparable to systemic chemotherapy, but current strategies utilizing multiple planned episodes of intra-arterial chemotherapy are significantly more costly and may be associated with less systemic side effects and similar favorable outcomes. At the Bascom Palmer Eye Institute, intra-arterial chemotherapy has quickly become the treatment of choice for globe conserving therapy of retinoblastoma.

Acute macular neuroretinopathy: a case report and review of the literature, 2002-2012.

Acute macular neuroretinopathy (AMNR) is a rare condition that primarily affects young women in their reproductive years. Many of the affected young women are on oral contraceptives. Patients report a sudden decrease in visual acuity, usually a few days after the onset of a febrile illness, with paracentral scotomas either unilaterally or bilaterally. Although AMNR was initially thought to be an inner retinal pathology, with the publication of 84 case reports in the English medical literature and the advent of new imaging modalities, it is now thought to be a disease of the outer retina. In 2003, Turbeville et al published a review of more than 41 AMNR cases reported from 1975 to March 2002, which summarized the available literature and suggested potential fields of research to be explored. This article summarizes the 43 case reports that were published in the English literature from April 2002 to October 2012 and also presents a unique case of AMNR.

Histopathology of the Ex-PRESS Shunt.

The authors report the histopathologic features of a human enucleated eye with an Ex-PRESS shunt (Optonol, Ltd., Neve Ilan, Israel). An 86-year-old man with a blind painful eye underwent enucleation. He had a history of glaucoma with an Ex-PRESS shunt implanted. Histopathologic evaluation of the specimen showed a thin layer of fibrotic tissue surrounding the implant. In this case, the Ex-PRESS shunt was relatively well tolerated in the human eye.

Retinoblastoma: evidence for stage-based chemotherapy.

Retinoblastoma is the most common primary intraocular cancer in children. Representing 6.1% of all childhood cancer under the age of 5 years, retinoblastoma primarily affects young children, with approximately 90% of cases diagnosed in patients younger than 3 years of age. In the United States, the age-adjusted incidence rate of retinoblastoma has remained stable for the last 30 years at 11.8 per million children aged 0 to 4 years, corresponding to a cumulative incidence estimate of 62 per million for the first 14 years of life. Being a highly malignant tumor, retinoblastoma results in death in 1 to 2 years if left untreated. It is estimated that 50% of cases of retinoblastoma seen worldwide present with extraocular manifestations, indicative of advanced disease with metastases that contribute to high mortality rates in the developing world—39% in Asia and 70% in Africa. In contrast, disease confined to the globe is more common in the developed countries, where 5-year survival rates are as high as 96% to 100%. Such excellent outcome is attributable to better prognosis associated with the earlier diagnosis, but more importantly, recent advances in the field has made available numerous therapeutic modalities that allows for the development of individualized approaches to treatment – largely dependent on the number, localization, and extent of the tumors, status of the contralateral eye, presence of germline retinoblastoma gene mutations, and risk of systemic involvement. Management of retinoblastoma is complex and involves a balance between preservation of the patient’s life, the eye, and visual potential,

Intraocular infections in the neonatal intensive care unit

Background The purpose of this study was to report on the incidence and treatment outcomes of endogenous endophthalmitis among newborns in the neonatal intensive care unit (NICU) of a single medical center. Methods This was a noncomparative, retrospective case series of endogenous endophthalmitis among infants at the Jackson Memorial Hospital NICU treated between March 1, 2002 and March 1, 2007. Results Of 4323 infants admitted to the NICU, seven eyes of six (0.139%) infants (two males, four females) were diagnosed with endophthalmitis during the study period. Four patients were born prematurely with a mean gestational age of 27.5 weeks and a mean birth weight of 1153 g. Retinopathy of prematurity was reported in two of the six patients. Mean follow-up was 3.5 years. The diagnosis was confirmed by positive cultures or polymerase chain reaction testing at a median age of 34 postnatal days. Positive cultures included Candida albicans (n = 4), Pseudomonas aeruginosa (n = 1), and Herpes simplex type 2 (n = 1). All patients received systemic treatment and five received adjunctive ophthalmic interventions, including intravitreal antibiotics in five eyes of four patients and vitrectomy with pars plana lensectomy in three eyes. One patient underwent primary enucleation and another had delayed evisceration. In the remaining five eyes, there was a normal appearing posterior segment and normal intraocular pressures at last follow-up. Conclusion Endogenous endophthalmitis is a rare complication in infants in the NICU, but may occur in patients with candidemia, bacteremia, retinopathy of prematurity, and low birth weight. Despite early and appropriate treatment, involved eyes may have poor outcomes.

Changing Treatment Patterns of ROP at a Tertiary Medical Center Between 2002 and 2012.

BACKGROUND AND OBJECTIVE To clinically correlate and evaluate the screening and treatment patterns of retinopathy of prematurity by a single physician at a tertiary care center between 2002 and 2012. PATIENTS AND METHODS A group of 10,924 newborns screened for ROP between 2002 and 2012 were retrospectively analyzed in a consecutive case series. Records of the neonatal intensive care unit (NICU) at Jackson Memorial Hospital/Bascom Palmer Eye Institute were reviewed to identify patients clinically diagnosed with retinopathy of prematurity who underwent treatment. RESULTS Laser photocoagulation was used to treat 231 of 10,924 (2.1%). Of these 231 patients, 176 (76.2%) were included in the study (55 infants were excluded because they were referred from outside institutions for advanced ROP). Of the 176 treated patients, 89 (50.6%) were male and 87 (49.4%) were female. The average birth weight was 687.3 g. The mean gestational age was 24.8 weeks. The mean time between birth and treatment was 3 months. Of the 176 patients, 31 required re-treatment with laser photocoagulation. The rates of treatment and retreatment significantly declined with the experience of the treating physician ( P < .01). Retinal detachment occurred in two of 176 patients (1.1%). CONCLUSION Experience is essential in delivering optimal care in a complex disease such as ROP. Between 2002 and 2012, the rate of treatment and retreatment significantly decreased. Possible reasons include improvements in neonatal care, but it is more likely that the rate of treatment and re-treatment is inversely proportional to the ophthalmologists experience with and comfort in managing ROP.

Intraoperative OCT of Bilateral Macular Coloboma in a Child With Down Syndrome

A 3-year-old girl with Down syndrome presented with a macular lesion in both eyes. With intraoperative optical coherence tomography confirmation, the patient was diagnosed as having bilateral macular coloboma. These findings were previously reported in two patients with Down syndrome. The documentation of similar findings in three separate patients suggests that macular coloboma may be a rare ophthalmic pathology associated with Down syndrome. Moreover, optical coherence tomography imaging may be a useful adjunct in diagnosing macular coloboma in the pediatric population.

Clinical and Echographic Features of Retinochoroidal and Optic Nerve Colobomas

PURPOSE We reported the clinical and echographic features of colobomas, prevalence of retinal detachment, and associated visual acuity in these patients. METHODS The study is a nonrandomized consecutive case series of 140 colobomatous eyes in 98 patients (age range, 0-83 years). Coloboma depth, width, volume, and relative coloboma excavation (coloboma depth/axial length) were measured using standardized echographic images. The presence of structural and other ocular abnormalities was noted. The clinical and echographic findings present were correlated with visual acuity of the patient. In addition, these features were correlated with the presence or absence of retinal detachment. RESULTS Increased relative coloboma excavation was significantly associated with an increased risk of retinal detachment. A relative coloboma excavation (ratio of coloboma depth to axial length) greater than 0.15 was associated with an increased risk of retinal detachment (52%), compared to those with a relative coloboma excavation less than 0.15 (23%, P = 0.014). The presence of any structural abnormality and the presence of a retrobulbar cyst were associated with increased risk of retinal detachment and severe visual impairment (worse than 20/200). Increased coloboma depth, width, volume, and relative coloboma excavation were not associated with increased risk of severe visual impairment. CONCLUSIONS Clinical and echographic features of colobomas may be used in predicting the risk of retinal detachment. Measuring relative coloboma excavation upon presentation may alter follow-up and assist in the diagnosis of retinal detachment.

RETINAL DETACHMENT SURGERY IN A PEDIATRIC POPULATION: Visual and Anatomic Outcomes

Purpose: Pediatric retinal detachments (RDs) are unique in etiology, anatomy, and prognosis compared with the adult population. The mechanisms of pediatric RD include tractional (TRD), rhegmatogenous retinal detachment, traumatic, and other types, such as exudative or hemorrhagic. This study examined visual and anatomical outcomes of pediatric RD undergoing surgical repair at a single university referral center. Methods: A retrospective consecutive case series of patients clinically diagnosed and undergoing surgery for RD between birth and 15 years of age from 2002 to 2013 at a single academic institution. Results: A total of 206 patients (231 eyes) were included in this study, of which 25 (12%) had bilateral RD. Of those patients, 67 (29%) had TRD (retinopathy of prematurity, persistent fetal vasculature, or familial exudative vitreoretinopathy), 51 (22%) had rhegmatogenous retinal detachment (myopia, X-linked retinoschisis, or Stickler syndrome), 60 (26%) had traumatic RD, and 53 (23%) were due to other types of RD, such as Coats disease or coloboma. Presenting best-corrected visual acuity better than 20/200 correlated with better final best-corrected visual acuity (P < 0.0001). Anatomical success was strongly correlated with visual acuity outcome (P < 0.00001) and was significantly more likely in rhegmatogenous retinal detachment versus TRD (78% vs. 39%, P < 0.05). The rates of obtaining a final best-corrected visual acuity > 20/200 were poorer in TRD (10%) compared with rhegmatogenous retinal detachment (39%, P < 0.01) or traumatic RD (28%, P < 0.05). Conclusion: Visual and anatomical outcomes varied among categories of RD. Rhegmatogenous retinal detachments were associated with the best outcomes (anatomical success and globe conservation), whereas TRDs generally had poorer visual and anatomical outcomes.