Natural history of spheno-orbital meningiomas

To investigate the natural history and the growth rate of spheno-orbital meningiomas (SOMs). Ninety patients with a diagnosis of SOM were included, and patient charts and imaging were evaluated. In a subset of 32 patients, volumetric studies were performed. The median follow-up for the entire group was 4 years (range, 1-15); the mean age was 47.8 (range, 26-93) years; 94% of the patients were female. The most common clinical signs and symptoms were proptosis (93%), visual deterioration (65%), retro-bulbar pain (23%) and diplopia (6%). In 35% of patients in this series, no visual deterioration occurred, and in 30% only mild proptosis was present. The median annual growth rate of the SOMs in the subset of 32 patients was 0.3 cm³/year (range, 0.03-1.8 cm³/year). We assessed a trend for more rapid tumour growth in younger patients and found the initial volume of the tumour (rho = 0.63) and of the soft tissue component (rho = 074) to be significantly related to the growth rate. SOMs are slow-growing tumours that cause primarily proptosis and visual deterioration. In a significant number of patients, these tumours cause minimal discomfort and symptomatology. Therefore, in the absence of risk factors, we advocate a "wait and see" policy. For patients with large SOMs or with a large soft tissue component at first visit or with fast growing SOMs (>1cm³/year), a follow-up examination every 6 months is indicate

Imaging findings in craniofacial childhood rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the commonest paediatric soft-tissue sarcoma constituting 3–5% of all malignancies in childhood. RMS has a predilection for the head and neck area and tumours in this location account for 40% of all childhood RMS cases. In this review we address the clinical and imaging presentations of craniofacial RMS, discuss the most appropriate imaging techniques, present characteristic imaging features and offer an overview of differential diagnostic considerations. Post-treatment changes will be briefly addressed

Orbital meningiomas

Vanwege hun ligging waren tumoren (zogeheten meningeomen) van de oogkas tot voor kort moeilijk te identificeren. Het openen van de schedel en het wegnemen van een stukje weefsel was vaak de enige mogelijkheid. Peerooz Saeed ontwikkelde een nieuwe diagnostische test op basis van de hoeveelheid somostatine - meningeomen nemen relatief veel van het hormoon op. Met de beeldvormende techniek scintografie kan die opname in het weefsel zichtbaar worden gemaakt. Op basis van een internationale multicenterstudie concludeert Saeed dat oogkastumoren bij volwassenen uiterst traag groeien - soms zelfs helemaal niet. Hij pleit daarom voor terughoudendheid in de behandeling

The large eyes of Wolfgang Amadeus Mozart. (On the occasion of the 250th Mozart anniversary year)

This article discusses the eyes of Wolfgang Amadeus Mozart (1756-1791) which, based on portraits, can be said to have a certain noticeable feature--some degree of exophthalmos. In fact, even contemporary observers made particular mention of Mozart's eyes being large. Although cicatricial ectropion, caused by infectious disease, cannot be excluded, mild myopia, in combination with shallow orbits or combined with a genetic predilection, would be one possible explanation for Mozart's large eyes. Graves' orbitopathy, the ophthalmic manifestation of hyperthyroidism, or hypothyroidism have both been suggested to be the cause of Mozart's large eyes. However, these diseases are unlikely causes given their topical and systemic feature

Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review

Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and limitation of abduction in the affected eye. Magnetic resonance imaging findings included an orbital mass in combination with absent nasal septum and partial destruction of the paranasal sinuses. Biopsies and histopathologic examination of the nasal cavity and the orbital mass revealed chronic inflammation. Both patients were treated with oral corticosteroids, ocular movements completely normalized but no improvement of visual acuity was noted. Intranasal cocaine abuse can cause orbital complications from chronic sinonasal inflammatory disease and these patients are at risk to develop optic neuropathy. Optic neuropathy may be caused by compression, infiltration, or ischaemi