Landmark detection for fusion of fundus and MRI toward a patient-specific multimodal eye model.

Ophthalmologists typically acquire different image modalities to diagnose eye pathologies. They comprise, e.g.

ranibizumab in the management of advanced Coats disease Stages 3B and 4: long-term outcomes.

Background: Laser photocoagulation and cryotherapy to completely destroy telangiectatic vessels and ischemic retina in Coats disease is barely applicable in advanced cases with total retinal detachment, and globe survival is notoriously poor in Stages 3B and 4. Anti-vascular endothelial growth factor intravitreal injections may offer new prospects for these patients.

Methods: This study is a retrospective review of all consecutive patients with Coats disease treated with neoadjuvant or adjuvant intravitreal ranibizumab plus conventional and amblyopia treatment as appropriate.

Intravitreal chemotherapy for vitreous seeding in retinoblastoma: Recent advances and perspectives.

For decades intravitreal chemotherapy (IViC) remained virtually banished from the therapeutic armamentarium against retinoblastoma, except as a heroic attempt of salvage before enucleation in only eyes with refractory vitreous seeding. Very recently, we have initiated a reappraisal of this route of administration by (1) profiling eligibility criteria, (2) describing a safety-enhanced injection procedure, (3) adjusting the tumoricidal dose of melphalan, and (4) reporting an unprecedented efficacy in terms of tumor control of vitreous seeding. Since then, intravitreal chemotherapy is being progressively implemented worldwide with great success, but still awaits formal validation by the ongoing prospective phase II clinical trial.

Anterior uveitis and cataract after rubella vaccination: a case report of a 12-month-old girl.

Many reports associating uveitis after vaccination have been reported, including 2 cases after measles, mumps, and rubella (MMR) vaccine. We report the case of a 12-month-old girl who developed a unilateral anterior uveitis with rubeosis and cataract 3 months after an MMR vaccination at 9 months of age. Aqueous humor analysis showed the presence of more rubella-specific immunoglobulin G in the affected eye than in the unaffected one.

Intravitreal chemotherapy for vitreous disease in retinoblastoma revisited: from prohibition to conditional indications.

Background: Tumour control of vitreous seeds remains challenging owing to their resistance to radiation and systemic chemotherapy.

Objective: To describe the short-term efficacy of intravitreal melphalan for vitreous disease in retinoblastoma using a new injection technique and dose.

Methods: This study is a retrospective non-comparative review of 23 consecutive heavily pretreated patients (23 eyes) with active vitreous seeding and eligible for intravitreous chemotherapy (IViC).

Profiling safety of intravitreal injections for retinoblastoma using an anti-reflux procedure and sterilisation of the needle track.

Background: The preservation of globe integrity has always been a major concern during the treatment of retinoblastoma for fear of extraocular or metastatic spread. Intravitreal chemotherapy has been attempted as a desperate salvage therapy only for eyes with refractory retinoblastoma. Published data on the safety and efficacy of this route are, however, limited.

Phenotypic variability of retinocytomas: preregression and postregression growth patterns.

Aim: To describe the incidence of retinocytomas, their variability at presentation and their growth patterns both before and after regression.

Methods: Medical notes of the 525 patients of the Jules-Gonin Eye Hospital Retinoblastoma Clinic between 1964 and 2008 were reviewed and the charts of 36 patients with retinocytomas and/or phthisis bulbi were selected.

Results: The proportion of patients with retinocytomas and/or phthisis bulbi was 3.

Risk assessment of recurrence in sporadic retinoblastoma using a molecular-based algorithm.

Purpose: Most RB1 mutations are unique and distributed throughout the RB1 gene. Their detection can be time-consuming and the yield especially low in cases of conservatively-treated sporadic unilateral retinoblastoma (Rb) patients. In order to identify patients with true risk of developing Rb, and to reduce the number of unnecessary examinations under anesthesia in all other cases, we developed a universal sensitive, efficient and cost-effective strategy based on intragenic haplotype analysis.

Ultrasound biomicroscopy evaluation of anterior extension in retinoblastoma: a clinicopathological study.

Background: Extension of retinoblastoma cells into the posterior chamber is a criterion for group E according to the international classification of intraocular retinoblastoma. Currently, the anterior extension of retinoblastoma is based on the presence of tumour cells in the anterior chamber assessed by biomicroscopy.

Aim: To determine the value of ultrasound biomicroscopy (UBM) in the assessment of posterior chamber involvement in advanced retinoblastoma.

Chiasmatic infiltration secondary to late malignant transformation of retinoma.

Purpose: To report the lethal course of malignant transformation of retinoma in an adult.

Methods: Case report. A 40-year-old patient presented with retinoma in his right eye and retinoblastoma in his left eye.

Occurrence of sectoral choroidal occlusive vasculopathy and retinal arteriolar embolization after superselective ophthalmic artery chemotherapy for advanced intraocular retinoblastoma.

Background: Superselective ophthalmic artery chemotherapy (SOAC) has recently been proposed as an alternative to intravenous chemoreduction for advanced intraocular retinoblastoma. Preliminary results appear promising in terms of tumor control and eye conservation, but little is known regarding ocular toxicity and visual prognosis. In this study, we report on the vascular adverse effects observed in our initial cohort of 13 patients.

Preliminary experience in treatment of papillary and macular retinoblastoma: evaluation of local control and local complications after treatment with linear accelerator-based stereotactic radiotherapy with micromultileaf collimator as second-line or salvage treatment after chemotherapy.

Purpose: To determine the local control and complication rates for children with papillary and/or macular retinoblastoma progressing after chemotherapy and undergoing stereotactic radiotherapy (SRT) with a micromultileaf collimator.

Methods And Materials: Between 2004 and 2008, 11 children (15 eyes) with macular and/or papillary retinoblastoma were treated with SRT. The mean age was 19 months (range, 2-111).

(106)Ruthenium brachytherapy for ciliary recurrence with supraciliary effusion in retinoblastoma.

Purpose: A 2-year-old boy with syndromic bilateral retinoblastoma resulting from a (del(13)(q12.3q14.3)) developed a recurrent tumor measuring 2.

Modern cataract surgery for radiation-induced cataracts in retinoblastoma.

Background: Surgery of radiation-induced cataracts in children with retinoblastoma (RB) is a challenge as early intervention is weighted against the need to delay surgery until complete tumour control is obtained. This study analyses the safety and functional results of such surgery.

Methods: In a retrospective, non-comparative, consecutive case series, we reviewed medical records of RB patients ≤ 14 y of age who underwent either external beam radiotherapy or plaque treatment and were operated for radiation-induced cataract between 1985 and 2008.

Germline mutations in retinoma patients: relevance to low-penetrance and low-expressivity molecular basis.

Purpose: To study phenotype-genotype correlation in patients who have retinoma, which is a benign tumor resembling the post irradiation regression pattern of retinoblastoma (RB).

Methods: We selected patients who had retinoma and positive family history for RB and patients who had retinoma in one eye and either retinoma or RB in the other eye. The study included 22 patients with available DNA: 18 from 11 families and four sporadic cases.

New developments in external beam radiotherapy for retinoblastoma: from lens to normal tissue-sparing techniques.

Historically, retinoblastoma was treated with external beam radiotherapy (EBR) and for many years this was the accepted standard of care. With greater knowledge of radiation-induced morbidity and mortality, the trend over the past decade has shifted towards primary chemotherapy for most globe conservative treatments. Such a radical change in treatment modalities has restrained EBR to second-line and salvage indications with little consensus regarding dose, timing and techniques.

(106)Ruthenium brachytherapy for retinoblastoma.

Purpose: To evaluate the efficacy of (106)Ru plaque brachytherapy for the treatment of retinoblastoma.

Methods And Materials: We reviewed a retrospective, noncomparative case series of 39 children with retinoblastoma treated with (106)Ru plaques at the Jules-Gonin Eye Hospital between October 1992 and July 2006, with 12 months of follow-up.

Results: A total of 63 tumors were treated with (106)Ru brachytherapy in 41 eyes.

Differential diagnosis of leukocoria and strabismus, first presenting signs of retinoblastoma.

Leukocoria in infants is always a danger signal as retinoblastoma, a malignant retinal tumor, is responsible for half of the cases in this age group. More common signs should also be considered suspicious until proved otherwise, such as strabismus, the second most frequent sign of retinoblastoma. Less frequent manifestations are inflammatory conditions resistant to treatment, hypopyon, orbital cellulitis, hyphema or heterochromia.

Retinopathy in Danon disease.

Objective: To evaluate visual function in 2 boys and their maternal aunt affected with Danon disease due to a mutation in the X-linked lysosome-associated membrane protein-2 (LAMP2) gene.

Methods: Linkage analysis using microsatellite markers from the X chromosome was done in family members from the paternal side. Visual acuity testing, fundus analysis, fluorescence angiography, and full-field electroretinography were performed in all 3 patients.

Trilateral retinoblastoma with suprasellar tumor and associated pineal cyst.

Trilateral retinoblastoma (TRb) is a well-known syndrome associating hereditary retinoblastoma (Rb) with an intracranial neuroblastic tumor arising usually in the pineal region, rarely at the suprasellar or parasellar site. It develops in most cases after diagnosis of Rb. The outcome is usually fatal because of secondary spinal dissemination.

Shorter time to diagnosis and improved stage at presentation in Swiss patients with retinoblastoma treated from 1963 to 2004.

Objectives: Retinoblastoma is the most frequent intraocular malignancy in children. Early diagnosis is essential for globe salvage and patient survival. The aim of our study was to determine how time to diagnosis of retinoblastoma has evolved over a 40-year period in Switzerland.

Benign pineal cysts in children with bilateral retinoblastoma: a new variant of trilateral retinoblastoma?

Purpose: Patients with hereditary retinoblastoma (Rb) develop in 4%-8% a malignant midline tumor called trilateral Rb (TRb). We report in this study on benign pineal cysts observed in patients investigated for TRb.

Patients And Methods: Between September 1990 and December 2001, 172 patients were screened for TRb.

Metastatic melanoma in the eye and orbit.

Objective: Presentation of a large series of patients with metastatic melanoma involving the eye and orbit.

Design: Retrospective clinical study.

Participants: Thirteen cases of metastatic melanoma involving intraocular tissues, 6 cases of metastatic melanoma in the orbit, and 1 case of metastatic melanoma involving both the eye and the orbit, treated in Lausanne between 1986 and 2002, were identified from the computer files of the ocular oncology and orbitopalpebral surgery units of Jules Gonin Hospital.