Visual function assessment, ocular examination, and intervention in children with developmental delay: A systematic approach. Part 1.

Children with special needs form a unique subset with regards to visual function and examination techniques needed to assess them. With more awareness among the general public, neurologists, and pediatricians, these children are referred for assessment to the ophthalmologist or optometrist and sometimes even to the rehabilitation professional at an early age. This clinical practice guideline and review gives a systematic approach for examining the visual functions of a child with special needs. It outlines the procedures to be followed with equipment needed in clinical practice. Functional vision assessment guidelines are also included. This is the first part in a two-part series, with the first part presenting clinical examination guidelines and the second presenting intervention and vision enhancement techniques.

Meningioma causing visual impairment: outcomes and toxicity after intensity modulated radiation therapy.

PURPOSE: To evaluate ophthalmologic outcomes and toxicity of intensity modulated radiation therapy (IMRT) in patients with meningiomas causing visual deficits. METHODS AND MATERIALS: A prospective observational study with formal ophthalmologic and clinical assessment of 30 consecutive cases of meningioma affecting vision treated with IMRT from 2007 to 2011. Prescriptions were 50.4 Gy to mean target dose in 28 daily fractions. The median follow-up time was 28 months. Twenty-six meningiomas affected the anterior visual pathway (including 3 optic nerve sheath meningiomas); 4 were posterior to the chiasm. RESULTS: Vision improved objectively in 12 patients (40%). Improvements were in visual field (5/16 patients), color vision (4/9 patients), acuity (1/15 patients), extraocular movements (3/11 patients), ptosis (1/5 patients), and proptosis (2/6 patients). No predictors of clinical response were found. Two patients had minor reductions in tumor dimensions on magnetic resonance imaging, 1 patient had radiological progression, and the other patients were stable. One patient experienced grade 2 keratitis, 1 patient had a minor visual field loss, and 5 patients had grade 1 dry eye. CONCLUSION: IMRT is an effective method for treating meningiomas causing ophthalmologic deficits, and toxicity is minimal. Thorough ophthalmologic assessment is important because clinical responses often occur in the absence of radiological change.

Gamma knife radiosurgery for choroidal hemangioma.

PURPOSE: Patients with choroidal hemangioma (CH), a benign ocular hamartoma, frequently presents with visual disturbance as a result of exudative retinal detachment (RD), which originates in subretinal fluid accumulation. We report our experience using the Leksell Gamma Knife in the management of symptomatic CH. METHODS AND MATERIALS: Seven patients with symptomatic CH (circumscribed form in 3 patients and diffuse form in 4) were treated with the Leksell Gamma Knife at our institution during a 7-year period. All patients presented with exudative RD involving the macula that resulted in severe visual deterioration. The prescription dose to the target margin was 10 Gy in all cases. The mean tumor volume receiving the prescription dose was 536 mm3 (range, 151-1,057). The clinical data were analyzed in a retrospective fashion after a mean follow-up of 34.4 months (range, 9-76). RESULTS: The resolution of exudative RD was achieved within 6 months, and the visual acuity of the affected eye had improved at the latest follow-up examination (p=.018) in all patients. No recurrence of exudative RD occurred. Thinning of the CHs was observed in most patients; however, symptomatic radiation toxicity had not developed in any of the patients. CONCLUSION: Symptomatic CHs can be safely and effectively managed with Gamma Knife radiosurgery using a marginal dose of 10 Gy.

Visual outcome and tumor control after conformal radiotherapy for patients with optic nerve sheath meningioma.

PURPOSE: Optic nerve sheath meningioma (ONSM) is a rare tumor that almost uniformly leads to visual dysfunction and even blindness without intervention. Because surgical extirpation carries a high risk of postoperative blindness, vision-sparing treatment strategies are desirable. METHODS AND MATERIALS: We retrospectively reviewed the outcomes of 25 patients (25 optic nerves) with ONSM, treated at a single institution with conformal fractionated radiotherapy by either stereotactic photon or proton radiation. Primary endpoints were local control and visual acuity. RESULTS: The patients presented with symptoms of visual loss (21) or orbital pain (3) or were incidentally diagnosed by imaging (3). The mean age was 44 years, and 64% were female patients. The indication for treatment was the development or progression of symptoms. Of the patients, 13 were treated with photons, 9 were treated with protons, and 3 received a combination of photons and protons. The median dose delivered was 50.4 gray equivalents (range, 45-59.4 gray equivalents). Median follow-up after radiotherapy was 30 months (range, 3-168 months), with 3 patients lost to follow-up. At most recent follow-up, 21 of 22 patients (95%) had improved (14) or stable (7) visual acuity. One patient had worsened visual acuity after initial postirradiation improvement. Of the 22 patients, 20 (95%) had no radiographic progression. Three patients had evidence of asymptomatic, limited retinopathy on ophthalmologic examination, and one had recurrent ONSM 11 years after treatment. CONCLUSIONS: Highly conformal, fractionated radiation therapy for symptomatic primary ONSM provides tumor control and improvement in visual function in most cases, with minimal treatment-induced morbidity. Longer follow-up is needed to assess the durability of tumor control and treatment-related late effects.

Primary solitary eosinophilic granuloma of the brain and postoperative radiotherapy: a clinical case.

A case of solitary eosinophilic granuloma (EG) of the brain in a 53-year-old woman is presented. The tumor developed in the anterior cranial fossa causing destruction of the skull basis and the retrobulbar space, accompanied by vision impairment. The tumor was partially excised and the patient was irradiated postoperatively with 40 Gy 6 MV x-rays, followed by 10 cycles of chemotherapy. Follow-up computed tomography (CT) showed no response at 30 Gy and no progression during chemotherapy. Attention is drawn to cases of EG indolent in growth but resistant to radiation and chemotherapy.

Preliminary visual outcomes after three-dimensional conformal radiation therapy for optic nerve sheath meningioma.

PURPOSE: We assessed visual outcomes, local control, and toxicity associated with three-dimensional conformal radiation therapy (3D-CRT) for primary optic nerve sheath meningiomas (ONSM). METHODS: Twenty-three patients diagnosed with ONSM were evaluated at the University of Michigan between 1986 and 2001. Fourteen patients were treated with 3D-CRT. Detailed pre- and postradiation treatment ophthalmologic examinations and MRIs were performed on all patients. Clinically significant visual acuity change was defined as a >or=three line change on the Snellen chart. Mean deviation change of >or=three decibels was defined as a clinically significant visual field change. Radiographic progression was defined as any increase in size on MRI. Acute and late toxicity was scored according to RTOG criteria. RESULTS: Median follow-up was 51.3 months. Five patients had a clinically significant improvement in visual acuity. Seven had stable acuity, and only 2 worsened. Nine patients had clinically significant visual field improvement. One patient developed early radiation retinopathy, 1 experienced orbital pain, 1 developed dry eye, and 2 developed iritis. No patient has required additional treatment, and none have demonstrated radiographic progression. CONCLUSION: 3D-CRT is effective in controlling tumor growth while improving or preserving vision in most patients with optic nerve sheath meningiomas.

Plasma cell granuloma--an enigmatic lesion: description of an extensive intracranial case and review of the literature.

We present an unusual case of intracranial plasma cell granuloma in a 70-year-old man with a 6-month history of progressive visual disturbance. The lesion extensively involved the cranial base, extended into the frontal region, and reached the floor of the third ventricle in the suprasellar area. Microscopic examination of multiple diagnostic transsphenoidal biopsies showed an inflammatory proliferation with a predominance of cells that were immunohistochemically determined to be polyclonal plasma cells. Ultrastructural analysis confirmed the presence of numerous mature plasma cells in a mixed inflammatory proliferation. In situ hybridization for Epstein-Barr viral RNA revealed no evidence of viral expression. The patient was treated with steroid therapy and radiotherapy, without any appreciable reduction of the lesion's size. He is alive with persistent severe visual disturbance 14 months after the diagnosis. We discuss the etiopathogenetic, diagnostic, and therapeutic issues related to this entity, and review the literature.

Pituitary tumours presenting in the elderly: management and outcome.

OBJECTIVE: In elderly patients there are few data on the efficacy and safety of pituitary surgery and radiotherapy (DXT). The aim of the present study was to assess the mode of presentation, treatment and outcome of patients > 64 years with a pituitary tumour presenting to a regional neuroendocrine service. DESIGN: A retrospective case note review of all patients with a pituitary tumour, from 1986 to 1993, was performed with DXT information from computerized records. PATIENTS: Forty-four patients were identified: median age 70 (65-83) years; 25 males; followed for a mean of 34 (range 0-84) months. RESULTS: The commonest presenting symptom was with visual disturbance (24 patients) with a further 9 with asymptomatic visual field defects. Five patients had acromegaly and two prolactinoma. Thirty-two patients underwent transsphenoidal surgery (TSS) (with post-operative DXT in 14 cases) and 6 craniotomy. Two patients had DXT as the primary procedure and four were observed. Visual fields improved in 21 of 29 patients. Perioperative complications occurred significantly more often after craniotomy (5/6) than after TSS (6/32) (P < 0.01). Eight patients died during the follow-up period (three deaths tumour related; one death followed DXT). CONCLUSIONS: Pituitary adenoma is a remediable cause of visual disturbance in the elderly. Most tumours were non-functioning. Age alone is not a contraindication to active treatment particularly with transsphenoidal surgery and radiotherapy.