Sensory exotropia versus sensory esotropia: A comparative clinical features study.

PURPOSE: This study aimed to compare the preoperative clinical features of patients with sensory esotropia (ET) and sensory exotropia (XT). METHODS: In a retrospective study, the medical records of 13,252 patients who underwent strabismus surgery were reviewed at the Farabi Eye Hospital, Iran, from 2012 to March 2022. There were 1017 patients with sensory horizontal strabismus whose, in their worse eye, had corrected distance visual acuity (CDVA) equal to or <20/160 tested with the Snellen chart. RESULTS: The mean age of patients was 29.0 ± 12.4 years [574 (56.4%) males and 443 (43.6%) females]. Sensory XT and ET were observed in 717 (70.5%) and 300 (29.5%) patients, respectively (P<.001). The mean CDVA in the strabismic and non-strabismic eyes was 1.40 ± 0.75 and 0.05 ± 0.13, respectively (P<.001). Also, the CDVA in the strabismic eyes was significantly worse in the patients with sensory XT than in the patients with sensory ET (P<.001). Sphere and spherical equivalent (SE) components were more hyperopic in both eyes of patients with sensory ET than sensory XT (P<.001). In sensory ET group, the mean horizontal deviation at far and near was significantly higher than the sensory XT group (both P<.001). The prevalence of moderate and severe amblyopia among all patients with sensory strabismus was 274 (26.9%) and 727 (71.5%), respectively (P<.001). There were 398 (39.1%) patients who needed more than one surgery. CONCLUSION: The frequency of sensory XT was about 2.5 times more than the sensory ET. Most patients with sensory ET were operated at a younger age, had better CDVA, more hyperopic spherical and SE, and higher angle of deviation compared with patients with sensory XT. The chance of reoperation in patients with sensory strabismus was about 40%.

Strabismus management in retinoblastoma survivors.

PURPOSE: To report the result of strabismus surgery in eye-salvaged retinoblastoma (Rb) patients. METHODS: A retrospective case series including 18 patients with Rb and strabismus who underwent strabismus surgery after completing tumor treatment by a single pediatric ophthalmologist. RESULTS: A total of 18 patients (10 females and 8 males) were included with a mean age of 13.3 ± 3.0 (range, 2-39) months at the time tumor presentation and 6.0 ± 1.5 (range, 4-9) years at the time of strabismus surgery. Ten (56%) patients had unilateral and 8(44%) had bilateral involvement and the most common worse eye tumor's group was D (n = 11), C (n = 4), B (n = 2) and E (n = 1). Macula was involved by the tumors in 12 (67%) patients. The tumors were managed by intravenous chemotherapy (n = 8, 47%), intra-arterial chemotherapy (n = 7, 41%) and both (n = 3, 17%). After complete treatment, the average time to strabismus surgery was 29.9 ± 20.5 (range, 12-84) months. Except for one, visual acuity was equal or less than 1.0 logMAR (≤ 20/200) in the affected eye. Seven (39%) patients had exotropia, 11(61%) had esotropia (P = 0.346) and vertical deviation was found in 8 (48%) cases. The angle of deviation was 42.0 ± 10.4 (range, 30-60) prism diopter (PD) for esotropic and 35.7 ± 7.9 (range, 25-50) PD for exotropic patients (P = 0.32) that after surgery significantly decreased to 8.5 ± 5.3 PD in esotropic cases and 5.9 ± 6.7 PD in exotropic cases (P < 0.001). The mean follow-up after surgery was 15.2 ± 2.0 (range, 10-24) months, in which, 3 (17%) patients needed a second surgery. CONCLUSION: Strabismus surgery in treated Rb is safe and results of the surgeries are acceptable and close to the general population. There was not associated with tumor recurrence or metastasis.

Uveal melanoma in the Iranian population: two decades of patient management in a tertiary eye center.

BACKGROUND: To assess the characteristics and outcomes of uveal melanoma management at a tertiary center in the Middle East. METHODS: A study on 164 patients with uveal melanoma was conducted by reviewing the available medical records, ultrasound, and pathology report results. Age at diagnosis, tumor location and size, treatment mode, visual outcome, metastasis, mortality, and survival were studied. RESULTS: The mean age of patients was 52.0 ± 15.0 years, and 52.5% were male. Choroidal melanoma was the most common uveal melanoma, followed by the ciliary body and iris melanoma. The mean thickness of tumors was 8.29 ± 3.29. The majority of patients (n = 111, 67.9%) were managed by brachytherapy with ruthenium-106 plaques. Enucleation was performed primarily in 46 (28%) patients and secondarily in nine (5.5%) patients. The sexual disparity was detected as the proximity of uveal melanoma to the fovea in males. For a 61-month mean follow-up period, mortality occurred in eight of our cases, six of which were due to metastasis. The most common site for distance metastasis was the liver (5/6), followed by the lung (1/6). The five-year and eight-year overall survival (OS) rate was 0.947%± 0.019. The 5-year survival rate reached zero in metastatic patients. OS was not statistically different depending on the age, tumor diameters, the primary treatment received, or the histopathologic findings (p > 0.50 for all). CONCLUSION: In this study, individuals diagnosed with UM exhibited an OS rate of around 94% at the five-year mark, which remained consistent up to eight years. Notably, the presence of distance metastasis emerged as the sole statistically significant factor influencing overall survival.

Sensory Strabismus; A Literature Review.

Persistent unilateral or bilateral visual deprivation at any age, particularly in children, can compromise sensory fusion and result in a type of strabismus known as sensory or secondary strabismus. There are several pathologies that can induce visual impairment, such as severe anisometropia, congenital unilateral cataract, corneal opacity, retinal diseases, and optic nerve anomalies. Sensory strabismus may be horizontal or vertical or a combination of them; however, most reports indicate the development of horizontal deviation as sensory strabismus. Regardless of the direction of the sensory strabismus, early diagnosis and management of the underlying pathology are important before strabismus treatment. The primary treatment approach for patients with sensory strabismus is surgery to correct ocular misalignment and straighten the eyes. This can help to improve the patients' symptoms and diminish the negative psychosocial impacts. In this article, we review the underlying etiologies and background pathologies associated with sensory strabismus. In addition, we investigate the determinant factors of the direction of sensory strabismus and its management strategies.

Orbital primary intraosseous hemangioma in a three-month old infant: A case report.

Purpose: To report a 3-month-old boy with rapid progressive orbital intraosseous hemangioma which was treated with excisional biopsy and orbital rim reconstruction. Observation: A 3-month-old boy was referred with the aggressive growth of a mass on the right orbital region. The mass was noted to develop over 4 weeks. On presentation this firm nontender orbital mass measuring 5 × 5cm mimicked a more ominous malignancy. The spiral computed tomography scan showed a destructive mass with protrusion superiorly and inferiorly toward the orbital cavity and anterior cranial fossa. The patient underwent gross tumor resection and reconstruction of the orbital rim. Histology findings revealed an intraosseous hemangioma. There was no evidence of recurrence after 1-year follow-up. Conclusion and Importance: Due to rapid progression, the patient's age, and lesion size, this case is unique. There were additional challenges regarding complete resection, intra-cranial extension, and significant blood loss in an infant. Therefore, in the face of rapidly progressing orbital tumors in infants, despite the very low prevalence of intraosseous hemangioma, this diagnosis should be considered.

Pediatric ocular melanoma: a collaborative multicenter study and meta-analysis.

PURPOSE: To investigate clinical manifestations and prognoses in pediatric patients (≤12 years old) with ocular melanoma. METHODS: This was a retrospective, multicenter cohort study with individual participant data (IPD) meta-analysis pooling available published cases, and unpublished cases from an international collaboration of seven ocular oncology centers. RESULTS: There were 133 eyes of 133 pediatric patients with choroidal or ciliary body (n = 66 [50%]), iris (n = 33 [25%]), conjunctival (n = 26 [19%]), and eyelid (n = 8 [6%]) melanoma. Overall, the mean patient age at presentation was 7 years (median, 8; range, 1-12 years), with 63 males (49%). The mean age by tumor site was 6.50 ± 3.90, 7.44 ± 3.57, 9.12 ± 2.61, and 5.63 ± 2.38 years, for choroid/ciliary body, iris, conjunctiva, and eyelid melanoma, respectively (P = 0.001). Association with ocular melanocytosis was seen in 15%, 11%, 4%, and 0%, respectively (P = 0.01). Frequency of ocular melanoma family history did not vary by tumor site (7%, 17%, 9% and 12%, resp. [P = 0.26]). After mean follow-up of 74, 85, 50, and 105 months (P = 0.65), metastasis was seen in 12%, 9%, 19%, and 13% of choroid/ciliary body, iris, conjunctiva, and eyelid melanoma, respectively. Death was reported in 5%, 3%, 8%, and 0%, respectively, with survival analysis indicating higher mortality in choroidal/ciliary body and conjunctival melanoma patients. CONCLUSIONS: Ocular melanoma in the pediatric population is rare, with unique clinical features and outcomes. Iris melanoma accounts for about one-third of pediatric uveal melanoma cases.

Massive Uveal Relapse of Retinoblastoma Presumed to Be Choroidal Tumorous Involvement: Case Series.

We report the choroidal and ciliary body invasion by retinoblastoma (RB) in a salvaged eye after complete and successful primary treatment. Case 1: A 25-month-old boy was referred due to group B RB lesions based on the International Classification of RB (ICRB; groups A-E) in the right eye (OD). His left eye (OS) was enucleated because of advanced group E RB. After 47 months of uneventful follow-up (F/U), a new lesion recurred and was treated with transpupillary thermotherapy. Four months later, a fast-growing pigmented subretinal mass was detected that was treated by brachytherapy with the apical dose of 80 Gy. Three weeks later, the lesion regressed completely, and no recurrence happened after 6 years of F/U. Case 2: A 4-month-old girl with a deletion in chromosome 13 was referred for bilateral RB. OD was enucleated because of unresponsive RB and anterior segment involvement. In OS, group B lesions had multiple recurrences after systemic chemotherapy. After a while, a single mass appeared in the nasal periphery which was controlled well with brachytherapy. Four months later, AC involvement was controlled with IAC, intravitreal, and intracameral chemotherapy, but posterior synechia and cataract appeared later. One year after the last treatment, UBM showed a ring-shaped ciliary body mass. Her parents refused enucleation again, and she received intravenous chemotherapy. Two years later, magnetic resonance imaging showed orbital and optic canal involvement with a deformed globe. In conclusion, RB recurrence can appear as local choroidal and ciliary body involvement even after a time of complete remission. The role of B-scan and UBM in early diagnosis and successful treatment is valuable.

No-split, no-tenotomy transposition of only the superior rectus muscle combined with medial rectus recession in patients with complete abducens nerve palsy.

PURPOSE: To evaluate the outcomes of no-split, no-tenotomy transposition of only the superior rectus muscle combined with medial rectus recession in patients with complete abducens nerve palsy. METHODS: In this procedure, the temporal margin of the superior rectus muscle, 10 mm posterior to the insertion, was secured with a nonabsorbable suture and sutured to the sclera at a distance of 12 mm from the limbus in the superotemporal quadrant, halfway between the superior rectus and lateral rectus. Success was defined as distance and near alignment of ≤8Δ and no diplopia in primary position 6 months after surgery. RESULTS: A total of 8 patients with abducens nerve palsy underwent the procedure. At the last follow-up, the mean postoperative change in primary position deviation was 42.6Δ ± 8.1Δ (range, 34Δ-57Δ) for distance and 42.1Δ ± 7.5Δ (35Δ -57Δ) for near, a significant reduction (P = 0.012). Abduction deficit also improved significantly (P = 0.010). Postoperatively, no vertical deviation or torsional diplopia was induced. At the 6 months' follow-up, compared with the first postoperative visit, an esodrift at near developed in 1 patient. Of the 8 cases, 6 fulfilled the criteria for success. CONCLUSIONS: In our small study cohort, no-split, no-tenotomy superior rectus transposition and medial rectus recession improved esotropia and abduction limitation without inducing significant vertical deviations or torsional diplopia.

Addition of intravitreal carboplatin with melphalan for management of vitreous seeding in retinoblastoma.

PURPOSE: To evaluate the efficacy and toxicity of intravitreal carboplatin plus melphalan for the treatment of vitreous seeds in eyes with retinoblastoma (RB). METHODS: This retrospective series at a tertiary referral center included 22 consecutive RB patients who had received intravitreal carboplatin (16 µg per 0.05 ml) combined with melphalan (30 µg in 0.03 ml) [IVi (Ca-Me)] for treatment of vitreous seeds. Tumor control and drug toxicities were recorded. RESULTS: There were 22 eyes of 22 patients, divided into primary group (n = 13) without history of previous intravitreal chemotherapy (IViC) and refractory group (n = 9) with history of previous IViC using melphalan and/or topotecan. The demographics and clinical findings of the primary and refractory groups did not differ significantly. The 6-month follow-up revealed complete vitreous seed control (77% vs. 89%, p = 0.47). Vitreous seed recurrence was detected in 1 eye of each group at 6 months. During the next 18-month follow-up period, no recurrence of seed was observed. The response to IVi (Ca-Me) was not significantly influenced by previous IViC (p = 0.70), primary systemic or intra-arterial chemotherapy (p = 0.45), or the type of regression (p = 0.35). The most common tumor treatment complications were retinal detachment (RD) (n = 2), early hypotony (n = 2) and late hypotony (n = 4, unrelated), cataract (n = 2), and severe pigment dispersion (n = 1). Enucleation was performed in 8 eyes, for total RD (n = 1), phthisis bulbi (n = 5), and extensive solid tumor recurrence (n = 2). There was no case of orbital invasion, systemic metastasis, or death. CONCLUSION: Based on this interventional case series for primary and refractory vitreous RB seeds, carboplatin plus melphalan therapy may be effective with few toxic side effects.

Superior Rectus Transposition Surgery: Safety, Efficacy, and Place in Therapy.

Abduction limitation in esotropic Duane retraction syndrome (DRS), esotropic Mobius syndrome, and sixth nerve palsy is one of the difficult-to-manage problems in strabismus surgery. The procedure of superior rectus transposition (SRT) was introduced by Johnston et al. In this procedure, the superior rectus (SR) muscle is disinserted and sutured adjacent to the insertion of lateral rectus (LR) muscle. The purpose of this review is to explore literature about efficacy and safety of SRT and its usage in strabismus surgery.

Clinical characteristics and surgical approach in Duane retraction syndrome: a study of 691 patients.

PURPOSE: To evaluate the clinical findings of different types of Duane retraction syndrome (DRS). STUDY DESIGN: Retrospective. METHODS: This study was performed on 691 patients with DRS who underwent surgery. Clinical examinations included laterality, abnormal head posture (AHP), corrected distance visual acuity (CDVA), refractive error, amblyopia, deviation, overshoots, and type of surgery. RESULTS: The mean age of patients with DRS was 16.7 ± 12.5 (range 1.0-73) years. The patients included 396 (57.3%) women and 295 (42.7%) men (P < 0.001). DRS type I, was observed in 429 (62.1%), II in 168 (24.3%), III in 88 (12.7%) and IV in 6 (0.9%) patients. Unilateral DRS was observed OS in 628 (90.9%) [471 (%78.9) and OD in 157 (21.1%) eyes (P < 0.001)]. O ther clinical findings were AHP (n = 522, 78.1%), overshoot (n = 236, 34.2%) and amblyopia (n = 118, 17.1%). The prevalence of overshoot in types I, II, and III was 17.5% (75/429), 60.7% (102/168) and 64.8% (57/88), respectively (P < 0.001). The prevalence of amblyopia was significantly lower in patients with AHP (80/522, 15.3%) compared to patients with normal head posture (38/169, 22.5%) (P = 0.023). The mean angle of deviation in the primary position (PP) at distance was 21.7 ± 11.5 △ for esotropic group and 17.8 ± 12.4 △ for exotropic group. Sixty-two (9.0%) patients required second surgery for resolving residual misalignment (1.1 surgeries for each patient). CONCLUSIONS: About two-thirds of DRS patients had AHP, one-third had overshoots, and one-sixth had amblyopia. The results show that different types of DRS are associated with different epidemiological and clinical characteristics.

Management of primary choroidal lymphoma presenting as extensive serous retinal detachment and salmon patch of bulbar conjunctiva: a case report.

BACKGROUND: We describe the outcome of ultra-low-dose radiotherapy plus intravitreal methotrexate and rituximab injections for a patient with primary choroidal lymphoma who presented with nodular conjunctival salmon patches and extensive serous retinal detachment. CASE PRESENTATION: A 34-year-old Iranian man presented with a nodular patch of bulbar conjunctiva in the right eye, and 1+ vitritis. A nearly complete shallow serous retinal detachment, retinal folds, and multifocal yellow choroidal infiltrates were seen during a fundus examination of the right eye. Enhanced depth imaging optical coherence tomography revealed macular retinal folds and an uneven, undulating, "seasick" appearance of the choroidal surface with choriocapillaris compression, intraretinal and subretinal fluid, and clusters of optically dense material at the outer retinal level. An incisional biopsy of the conjunctival lesion confirmed the diagnosis of primary choroidal lymphoma with epibulbar involvement. The patient was treated with ultra-low-dose "boom-boom" radiation (4 Gy delivered in two fractions over two consecutive days) as well as intravitreal methotrexate and rituximab injections. After a year, the lesions had completely disappeared, with no adverse effects or recurrence. CONCLUSION: Ultra-low-dose (boom-boom) radiotherapy combined with intravitreal chemotherapy and/or immunotherapy may be an effective treatment for primary choroidal lymphoma with anterior epibulbar extension and diffuse subretinal fluid with favorable response and minimal side effects.

Retinal Optical Coherence Tomography Angiography Findings following Retinoblastoma Treatment by Chemotherapy.

Purpose: To investigate the macular microvascular changes after different kinds of chemotherapy in patients with extramacular retinoblastoma (RB). Methods: In this study, 28 eyes of 19 patients with bilateral RB treated with intravenous systemic chemotherapy (IVSC group) and 12 eyes of 12 patients with unilateral RB treated with intra-arterial chemotherapy (IAC group) were compared with 6 normal fellow eyes of 6 patients with unilateral RB treated with IVSC (IVSC fellow eye group), and 7 normal fellow eyes of 7 unilateral RB patients treated with IAC (IAC fellow eye group), as well as 12 age-matched normal eyes. Enhanced depth imaging optical coherence tomography measurements of central macular and subfoveal choroidal thickness (CMT and SFCT) as well as optical coherence tomography angiography measurements such as retinal superficial capillary density (SCD), deep capillary density (DCD), and choriocapillaris density were documented. Results: Images of 2 eyes in the IVSC group and 8 eyes in the IAC group were excluded from the final image analysis due to severe retinal atrophy. Overall, 26 eyes with bilateral RB treated with IVSC and 4 eyes of 4 patients with unilateral RB treated with IAC were compared with the mentioned control groups. Best-corrected visual acuity was 1.03 logMAR in the IAC patients compared to 0.46 logMAR in the IVSC group at the time of imaging. While the CMT and SFCT were lower in the IAC group in comparison with the IAC fellow eye and normal groups (P < 0.05 for all), no remarkable difference was observed between the IVSC group and the control groups based on the mentioned parameters. Although the SCD showed no significant difference between the IVSC and control groups, this parameter was significantly lower in the eyes receiving IAC relative to the corresponding fellow eye group (P = 0.042) and normal control eyes (P = 0.047). The mean DCD was considerably lower in both the treatment groups compared to the control groups (P < 0.05 for all). Conclusion: Our study showed a substantial decrease in SCD, DCD, CMT, and choroidal thickness in the IAC group, which may explain the lower visual outcome in this group.

Evaluation of radiation maculopathy after treatment of choroidal melanoma with ruthenium-106 using optical coherence tomography angiography.

BACKGROUND: To assess the impact of brachytherapy on macular microvasculature utilizing optical coherence tomography angiography (OCTA) in treated choroidal melanoma. METHODS: In this retrospective observational case series, we reviewed the recorded data of the patients with unilateral extramacular choroidal melanoma treated with ruthenium - 106 (106Ru) plaque radiotherapy with a follow-up period of more than 6 months. Automatically measured OCTA retinal parameters were analysed after image processing. RESULTS: Thirty-one eyes of 31 patients with the mean age of 51.1 years were recruited. Six eyes had no radiation maculopathy (RM). From 25 eyes with RM, nine eyes (36%) revealed a burnout macular microvasculature with imperceptible vascular details. Twenty-one non-irradiated fellow eyes from the enrolled patients were considered as the control group. Foveal and optic disc radiation dose had the highest value to predict the burnout pattern (ROC, AUC: 0.763, 0.727). Superficial and deep foveal avascular zone (FAZ) were larger in irradiated eyes in comparison to non-irradiated fellow eyes (1629 µm2 vs. 428 µm2, P = 0.005; 1837 µm2 vs 268 µm2, P = 0.021; respectively). Foveal and parafoveal vascular area density (VAD) and vascular skeleton density (VSD) in both superficial and deep capillary plexus (SCP and DCP) were decreased in all irradiated eyes in comparison with non-irradiated fellow eyes (P < 0.001). Compared with non-irradiated fellow eyes, irradiated eyes without RM had significantly lower VAD and VSD at foveal and parafoveal DCP (all P < 0.02). However, these differences at SCP were not statistically significant. CONCLUSION: The OCTA is a valuable tool for evaluating RM. Initial subclinical microvascular insult after 106Ru brachytherapy is more likely to occur in DCP. The deep FAZ area was identified as a more critical biomarker of BCVA than superficial FAZ in these patients.

Intra-Arterial Chemotherapy: New Hope for Adult Retinoblastoma Treatment-A Case Report and Brief Review.

BACKGROUND: Adult retinoblastoma (RB) is a rare intraocular tumor, leading to delayed diagnosis and, eventually, enucleation of the involved eyes. Therefore, this diagnosis should be considered if attributable signs and symptoms occurred. Here, the authors report a case of an adult group D RB, treated successfully with intra-arterial chemotherapy (IAC) as primary treatment followed by cryotherapy. The authors briefly review the literature on the prognosis and management of adult RB. Case Presentation. A 22-year-old man has noticed floaters in his right eye for 2 months. Right eye examination revealed diffuse white spherical calcified vitreous seeds in all quadrants and a large white endophytic mass in the superonasal quadrant with engorged feeding vessels. Based on clinical data, the group D RB tumor was classified and the IAC was started with 2 medications, melphalan (5 mg) and topotecan (1 mg), for 3 cycles. Trans-scleral triple freeze-thaw cryotherapy was used as an adjunctive treatment at the latest hospital visit. Thirteen months after the last treatment, the RB tumor showed type 4 regression (flat chorioretinal scar) and no evidence of recurrence was identified. CONCLUSION: It seems that IAC, as the first line of treatment, either alone or in conjunction with adjuvant therapies may allow us to salvage the globe of adult patients involved with RB.

A New Rapidly Growing Dome-Shaped Choroidal Lesion in an Eye with Treated Retinoblastoma.

BACKGROUND: To describe an extensive untreatable choroidal metastasis by retinoblastoma in the treated patient which was clinically indistinguishable from regular tumor recurrence. METHODS: A 24-month-old girl without a family history of retinoblastoma (RB) was discovered to have group C RB in her right eye and group D in her left eye. The patient received 12 cycles of intravenous chemotherapy, intra-arterial chemotherapy (IAC), and intravitreal chemotherapy for the left eye and focal adjuvant therapy (laser thermotherapy and cryotherapy) for both eyes. Six months after the last treatment, fundus examination showed a regressed tumor in both eyes. Ten months after the last treatment, except for in addition to tumor recurrence, rising intraocular pressure was noticed in the left eye. While doing IAC for the left eye, a very rapid growing yellowish dome-shaped mass was found which had doubled in size in two weeks. Enucleation was considered for her. RESULTS: Pathology evaluation of the enucleated eye revealed a very massive dome-shaped choroidal metastasis invasion with poorly differentiated RB tumor. Prophylactic systemic chemotherapy was performed for the patient. CONCLUSION: Choroidal metastasis in RB patients is often diagnosed based on pathology reports, but it may rarely be seen in clinical examinations especially if the pattern of tumor recurrence and growth is abnormal.

Uveal Melanoma Metastasis to the Contralateral Eye Structures: A Retrospective Comparative Analysis of 13 Consecutive Patients.

PURPOSE: To report uveal melanoma (UM) metastasis to the contralateral ocular and periocular structures. DESIGN: Retrospective study. PARTICIPANTS: Thirteen patients with UM metastasis to the contralateral ocular and periocular structures were included. METHODS: Clinical records were reviewed retrospectively. MAIN OUTCOME MEASURES: The development and time to onset of contralateral ocular and periocular metastasis, systemic metastasis, and death. RESULTS: Of the 13 000 treated UM patients, 13 patients were diagnosed with UM metastasis to the contralateral ocular and periocular structures. Mean patient age at primary UM diagnosis was 60 years (median, 60 years; range, 37-87 years). The primary uveal melanoma was in the choroid (n = 11) or ciliary body (n = 2) and was treated with brachytherapy (n = 11), proton beam radiotherapy (n = 1), or enucleation (n = 1). Systemic metastasis developed in 11 patients (85%) at a mean of 66 months (median, 34 months; range, 12-216 months) after diagnosis of the primary UM. All 11 patients (100%) showed liver metastasis and 8 patients (62%) also showed extrahepatic metastasis. The sites of metastasis to the contralateral ocular or periocular structures included the choroid in 4 patients (31%), the orbit in 7 patients (54%), and the eyelid in 2 patients (15%). One patient with eyelid metastasis demonstrated concurrent conjunctival nodule. Mean time to diagnosis of contralateral ocular or periocular metastasis was 94 months (median, 48 months; range, 9-375 months). Contralateral choroidal metastasis was multifocal in 3 of 4 patients (75%). Of 7 patients with orbital metastasis, 5 showed extraocular muscle involvement with restricted ocular motility. Treatment for contralateral choroidal metastasis included brachytherapy (n = 2), transpupillary thermotherapy (n = 1), and observation (n = 1). Treatment for contralateral periocular (orbit or eyelid) metastasis was excision (n = 5), external beam radiotherapy (n = 2), and observation (n = 2). Of 13 patients, death was documented in 11 patients at a mean of 17 months (median, 9 months; range, 3-54 months) as a result of systemic UM metastasis (n = 10) or unrelated cause (n = 1). CONCLUSIONS: Metastasis resulting from UM to the contralateral ocular and periocular structures is rare and generally occurs in patients with disseminated metastasis. Orbital tissue is the most common site of involvement, and these patients have short life expectancy.

GROWTH OF PRESUMED CHOROIDAL NEVUS INTO MELANOMA OVER 4 YEARS IN BAP1 TUMOR PREDISPOSITION SYNDROME.

PURPOSE: To report a case of presumed choroidal nevus that eventually grew into melanoma in a patient with family history of choroidal melanoma and germline BAP1 mutation. METHODS: Case report. RESULTS: A 55-year-old healthy white woman with a family history of uveal melanoma in her father, paternal aunt, and paternal cousin was referred for evaluation of an asymptomatic small pigmented choroidal lesion in her right eye, measuring 2 mm × 2 mm in basal diameter and 1 mm in thickness. There were no clinical risk factors. The patient was advised routine monitoring but returned 4 years later with intermittent photopsia. The choroidal mass demonstrated growth and suggestive of transformation into melanoma, measuring 9 mm × 6 mm in basal diameter and 2.5 mm in thickness with overlying orange lipofuscin pigment and no associated subretinal fluid. Fine-needle aspiration biopsy disclosed Chromosome 3 mosaic monosomy and Chromosomes 6 and 8 disomy. Iodine 125 plaque radiotherapy was provided. Based on growth to melanoma and strong family history of uveal melanoma, BAP1 germline mutation testing was performed, and the results were positive. CONCLUSION: This case demonstrates growth of a presumed choroidal nevus into melanoma in the setting of underlying germline BAP1 mutation. We suggest that small pigmented choroidal lesions be monitored closely in patients with germline BAP1 mutation or with family history of uveal melanoma, even in the absence of known local risk factors predictive of tumor growth.

INTRAVITREAL MELPHALAN FOR TREATMENT OF VITREOUS SEEDING FROM CHOROIDAL MELANOMA.

PURPOSE: To report treatment of vitreous seeding of choroidal melanoma with monthly injections of intravitreal melphalan. METHODS: Case report. RESULTS: A 70-year-old white woman noted floaters in her left eye, and further examination revealed visual acuity of 20/30 in both eyes. Funduscopically, there was a mushroom-shaped choroidal melanoma in her left eye, measuring 9 mm in basal dimension and 4.8 mm in thickness. Notably, there was apical retinal invasion of melanoma with mild vitreous hemorrhage, without vitreous seeding. The tumor was treated with iodine-125 plaque radiotherapy using an apex dose of 70 Gy over 99 hours, designed to include the retinal invasion. The melanoma demonstrated complete regression into a nearly flat scar of 1 mm and remained stable over 4 years. Five years after radiotherapy, there were diffuse vitreous pigmented seeds of presumed melanoma origin, emanating from the site of retinal necrosis. This progressively worsened over the following 18 months, suspicious for viable melanoma cells, as visual acuity concurrently declined to 20/100. Treatment with intravitreal melphalan (10 µg/0.05 mL) was delivered on a monthly basis for 12 cycles, resulting in vitreous seeds regression, and preservation of the eye. Final visual acuity was 20/200. There were no treatment-related complications. CONCLUSION: Intravitreal melphalan can be considered in cases of vitreous seeding from uveal melanoma.

Tamoxifen Induced Pachychoroid Pigment Epitheliopathy with Reversible Changes After Drug Discontinuation.

We report a case of breast cancer with clinically significant retinal toxicity induced by 7 years' Tamoxifen consumption in which patient's visual acuity and paraclinical findings remarkably improved after drug discontinuation. A 49-year-old woman with a history of breast cancer and Tamoxifen consumption was referred to our clinic for evaluation and treatment of gradual and progressive decrease visual acuity of both eyes (more prominent in right eye). Funduscopy showed bilateral macular pigmentary changes, with diffused tiny yellow crystals and reduced macular tessellation. On spectral-domain optical coherence tomography (SD-OCT), there was retinal pigment epithelium (RPE) abnormality and ellipsoid zone discontinuity accompanied by retinal thinning and choroidal thickness that was more prominent in the right eye. One year after discontinuation of the drug, visual acuity was significantly improved and SD-OCT revealed some surprising recoveries in the photoreceptor layers especially in her right eye.