İrem Koç

Intravitreal chemotherapy in the management of vitreous disease in retinoblastoma

Purpose To evaluate the therapeutic outcome of intravitreal melphalan injection in the management of vitreous disease in patients with retinoblastoma. We particularly aimed to assess whether higher melphalan dose with lower number of injections was more effective and associated with fewer side effects. Methods This retrospective, interventional, noncomparative, and nonrandomized study included 39 eyes of 37 patients. Vitreous seeds were classified as dust, sphere, and cloud types. Intravitreal injections were performed through pars plana free of any visible tumor using 30-G needle. Response of the seeds (disappearance, conversion into inactive debris, or progression) and enucleation rate were determined as outcome measures. Results All patients previously received systemic or intra-arterial chemotherapy. Vitreous seeding was primary in 54% of eyes and secondary in 46% of eyes. Vitreous seeds were classified as dust in 9 (23.1%) eyes, sphere in 24 (61.5%) eyes, and cloud in 6 (15.4%) eyes. Melphalan dose varied between 20 and 40 µg and 20 (51.3%) eyes received >30 µg. The total number of injections was 70 (range 1-5, mean 1.8 per eye). Various types of regression were obtained in 27 (69.2%) eyes. Sphere-type seeds were the most responsive to melphalan. Nonresponse and disease progression were noted in 12 (30.8%) eyes. After a mean follow-up of 11.8 months, 17 (44%) eyes were enucleated. Vitreous hemorrhage (18%) and retinal pigment epithelial alterations (8%) were the most common side effects. Conclusions Intravitreal melphalan at 30-40 µg in 1 or 2 injections proved effective in 69.2% of eyes with vitreous disease.

The frequency and causes of abnormal head position based on an ophthalmology clinic’s findings: is it overlooked?

Purpose To determine the frequency of abnormal head position (AHP) and identify the underlying causes in patients who presented to an ophthalmology clinic due to any ophthalmologic complaint. Methods The medical records of patients who presented with any ophthalmologic symptoms during a 6-month period were prospectively evaluated. In all, 2,710 patients (1,492 female and 1,218 male) aged 6 months-91 years were included in the study. Each patient underwent complete ophthalmologic evaluation. Results Among the 2,710 patients, 30 (1.1%) (7 female and 23 male) with a mean age of 14.62 ± 17.45 years (range 6 months-60 years) had AHP. In total, 24 (80%) of the patients with AHP were aged ≤16 years. The initial complaint in the patients with AHP was ocular misalignment in 18 (60%) patients, AHP in 4 (13.3%), abnormal ocular movements in 4 (13.3%), double vision in 3 (10%), and droopy eyelid in 1 (3.3%). Comitant strabismus, nystagmus, and Duane syndrome were the most common causes of AHP. Other diagnoses included fourth nerve palsy, sixth nerve palsy, Brown syndrome, congenital muscular torticollis, ptosis, and blowout orbital fracture. Conclusions The leading underlying causes of AHP in patients who presented to an ophthalmology clinic were ocular and treatable. Of note, in only a minority of these patients AHP was the initial presenting complaint. Clinicians must be aware that observation of any head position that is not normal should prompt additional investigation, as the underlying pathology can cause treatable morbidity or in rare instances mortality, such as in cases of acute cranial nerve palsy.

Orbital exenteration: Institutional review of evolving trends in indications and rehabilitation techniques

ABSTRACT Purpose: To determine the changes in indications for orbital exenteration over 20 years and to assess its impact on patient survival. Evolving techniques of rehabilitation of the orbit in our institution were also evaluated. Methods: This was a retrospective review of hospital records of patients who underwent orbital exenteration from 1995 to 2015 in a tertiary care center. Data extracted included primary location of the tumor, preoperative treatments, interval between initial diagnosis and exenteration, status of surgical margins, presence of metastatic disease, and postoperative survival. The types of prosthesis utilized over the years were also reviewed. Cox regression analysis was performed for categorical variables. Kaplan–Meier analysis was used to estimate post-exenteration survival. Results: Over a 20-year period, orbital exenteration was performed on 100 orbits of 100 patients. The mean age was 39.4 years (range: 2 months to 90 years). The most common indications among 98 malignant causes were retinoblastoma, squamous cell carcinoma, basal cell carcinoma, extraocular extension of uveal melanoma, and conjunctival melanoma. Postoperative survival was significantly related to age and tumor location but independent from gender, surgical margin, histopathological diagnosis, previous treatment modality, and preoperative interval. In the whole cohort, 1-year and 5-year survival rates were 97% and 84%, respectively. Conclusions: Exenteration appears to be life-saving in children with orbital extension of retinoblastoma. While patients exenterated for malignant eyelid tumors have the best chance of survival, those with orbital extension of uveal melanoma and adenoid cystic carcinoma of the lacrimal gland have the worst prognosis.

Ocular Causes of Abnormal Head Position: Strabismus Clinic Data

Objectives: To determine the most common ocular causes and types of abnormal head position (AHP) and describe their clinical features. Materials and Methods: Patients with AHP who had been followed in the strabismus unit were retrospectively reviewed. Demographic features and orthoptic characteristics were recorded. Results: A total of 163 patients including 61 women (37.4%) and 102 men (62.6%), with a mean age of 19.9±18.3 were recruited. The most common causes of AHP were determined as fourth cranial nerve palsy (33.7%), Duane retraction syndrome (21.5%), sixth cranial nerve palsy (11%), nystagmus blockage syndrome (9.8%) and Brown syndrome (6.7%). Other less frequent causes were A-V pattern strabismus, comitant strabismus, thyroid orbitopathy and third cranial nerve palsy. The most common types of AHP were head tilt (45.4%) and face turn (36.8%). Out of 142 patients whose visual acuity could be evaluated, 28.2% had amblyopia. The frequency of amblyopia varied depending on the diagnosis (p<0.001), while there was no relation between amblyopia and different types of AHP (p=0.497). Stereopsis and fusion could be tested in 128 patients and 43.8% of them had stereopsis and fusion. The presence of stereopsis and fusion was found to be related with the diagnosis (p=0.001), whereas it was not related with the types of AHP (p=0.580). The presence of amblyopia was not significantly associated with fusion (p=1.000) or stereopsis (p=0.602). Conclusion: There are many ocular pathologies that cause AHP. Patients with similar diagnoses may have different types of AHP. Patients may have amblyopia and impaired binocularity despite AHP. Therefore, all patients with AHP should be examined in detail and these points should be considered in the treatment plan.

INTRAVITREAL DEXAMETHASONE (OZURDEX) IMPLANT FOR RADIATION MACULOPATHY SECONDARY TO STEREOTACTIC RADIOTHERAPY FOR POSTERIOR UVEAL MELANOMA

PURPOSE To evaluate the efficacy of 0.7 mg intravitreal dexamethasone implant in the treatment of radiation maculopathy after stereotactic radiotherapy for posterior uveal melanoma. METHODS Retrospective chart review of seven eyes of seven consecutive patients was performed. Extracted data included age, sex, initial and follow-up visual acuities and central macular thickness values, intraocular pressure, follow-up time, number of implants, and time elapsed from radiotherapy to implantation. Main outcome measures were visual acuity and central macular thickness. Glaucoma, cataract formation, or systemic side effects, if any, were recorded. RESULTS Female to male ratio was 4:3. Mean age was 49.9 ± 17.0 (range: 27-73). Initial mean visual acuity was 20.4 ± 12.5 Early Treatment Diabetic Retinopathy Study letters and initial central macular thickness measured 514.1 ± 135.1 μm on spectral domain optical coherence tomography. All patients except one showed improvement in visual acuity and a mean improvement of 7.4 ± 6.2 letters was observed in the whole group (range: 0-16). The mean reduction in central macular thickness was 226.7 ± 157.0 μm after a mean 9.1 ± 3.4 months of follow-up. On average, implantation of intravitreal dexamethasone was performed 35.2 ± 16.5 months after radiotherapy. Four patients were treatment naive and three had previous intravitreal bevacizumab injections with limited response. Ozurdex reimplantations were performed in four patients and the mean number of injections was 1.7 ± 0.8. Mean time to reimplantation was 5.0 ± 2.12 months. Only one patient developed posterior subcapsular cataract and all patients had intraocular pressures within normal limits. No systemic side effects were observed. CONCLUSION In our experience, intravitreal implantation of 0.7 mg dexamethasone is an anatomically, and to a lesser extent functionally effective procedure for radiation maculopathy after stereotactic radiotherapy for posterior uveal melanoma.

Orbital Extension of an Unsuspected Choroidal Melanoma Presumably through an Aqueous Tube Shunt.

Aims: To report a patient with unrecognized small macular melanoma, who gradually developed neovascular glaucoma that was treated with an Ahmed valve. We presume that tumor cells gained access to the orbit through this aqueous drainage device. Methods: The medical records pertaining to 5 different centers, the results of imaging studies, the clinical course of the patient and histopathological findings were retrospectively reviewed. Results: A 61-year-old man presented with a blind and painful proptotic right eye. Retinal hemorrhage had been suspected 3 years earlier and followed accordingly thereafter. Yearly performed MRI studies demonstrated the steady growth of the hemorrhagic lesion/tumor. Meanwhile, he developed neovascular glaucoma first treated unsuccessfully with cyclocryotherapy then with implantation of an Ahmed valve. Right proptosis became evident within a year, and a final MRI study revealed a large retrobulbar mass. Orbital exenteration was performed and histopathological examination showed intraocular and orbital epithelioid cell melanoma. Tumor cells were also found within the reservoir of the Ahmed valve. The patient died of widespread metastases 1 year later. Conclusions: Ahmed valve implantation in an eye with unsuspected macular choroidal melanoma may ultimately be associated with extraocular extension and orbital tumor formation.

Orbital Malignant Peripheral Nerve Sheath Tumor With Heterologous Elements in a 15-Year-Old Girl

A 15-year-old girl presented with painful proptosis in the right eye. Imaging studies demonstrated an inferiorly located orbital mass. Histopathological examination of the exenteration specimen was consistent with malignant peripheral nerve sheath tumor. This case is a rare example of orbital malignant peripheral nerve sheath tumor presumably arising from the infraorbital nerve with heterologous histologic elements.

Malignant transformation of retinocytoma treated with intra-arterial chemotherapy

patients who experienced IOP elevation and cataract progression within 3–6 months also required cataract surgery because of decreased visual acuity secondary to cataract progression, although IOP was stabilized in both by glaucoma treatment. Another patient experienced cataract progression within 10 weeks after implantation, but without IOP elevation. The findings in our patient differ from those in previous reports, in that our patient experienced delayed IOP elevation without cataract progression. As our patient did not report visual discomfort related to the intracapsular location of the dexamethasone implant, she did not present for follow-up examinations for several months. Therefore, the time at which IOP became elevated was unclear. Macular edema persisted after injection of the dexamethasone implant, as reported previously. As the components of the lens (65% water and 35% protein) and vitreous (99% water) differ, the sustained release of dexamethasone through hydrolysis may have been inhibited, resulting in no change in the size of the implant in the lens and persistent macular edema. This suggests that, if a dexamethasone implant is inadvertently injected into the lens, leaving the implant in place has no beneficial effects on macular edema but has risks of complications such as IOP elevation, thus indicating the need for prompt removal. In summary, the inadvertent injection of a dexamethasone implant into the crystalline lens may lead to delayed elevation of IOP without cataract progression. Because of the possibility of delayed elevation of IOP, cataract surgery, including extraction of the implant, should be considered even if there are no immediate complications.