John Crompton

Perineural Spread of Cutaneous Squamous Cell Carcinoma via the Orbit: Clinical Features and Outcome in 21 Cases

OBJECTIVE To describe the clinical features, treatment, and outcome of a series of patients with perineural spread (PNS) of cutaneous squamous cell carcinoma (SCC) via the orbit. DESIGN A cohort study. PARTICIPANTS Twenty-one patients identified with PNS of cutaneous SCC via the orbit. INTERVENTION Patients were treated with various combinations of conservative or radical surgery, external beam radiation therapy, and chemotherapy. RESULTS Of 21 cases, 17 (81%) were male. Age at presentation with PNS ranged from 38 to 82 years (median, 66 years). The forehead and eyebrow were the most common site of the primary lesion. All but one had altered or decreased sensation, but only nine presented with pain. Fourteen (67%) had ophthalmoplegia at presentation, and 14 (67%) had evidence of PNS involving branches of the facial nerve. Despite combinations of radical surgery, conservative surgery, and radiation therapy, no method of treatment appeared more effective, and 14 patients died from 9 months to 5 years after presentation with PNS (median, 3 years), usually from local and intracranial disease. Two survived to 14 and 18 years, one is alive at 3 years with recurrent local and distant disease, and four are alive without evidence of disease at 2, 3, 4 and 12 months after radiation therapy. CONCLUSIONS Perineural spread of cutaneous SCC via the orbit carries a poor prognosis. Early radiation therapy may offer the best form of palliation. The role of radical surgery probably is limited once orbital involvement is apparent, as the cavernous sinus and facial nerve branches usually are involved.

Ophthalmic Manifestations of Multiple Myeloma

Purpose: To describe ophthalmic manifestations in a series of patients with multiple myeloma (MM) and review the literature. Design: Retrospective case series. Methods: Review of all cases with ophthalmic manifestations of MM seen at the Department of Ophthalmology, Royal Adelaide Hospital, between 1987 and 2002. Results: Of all patients with MM who were managed at the Royal Adelaide Hospital in the study period, only 8 were referred to the Ophthalmology Department with ophthalmic manifestations. Five males and 3 females with a mean age of 61.25 years (range 42–78) who exhibited ophthalmic manifestations were studied. Six patients (75%) had known MM at the time of their ophthalmic evaluation. Four patients (50%) had neuro-ophthalmic symptoms resulting in diplopia or visual disturbances. Three patients (37.5%) presented with orbital involvement and 1 (12.5%) with hyperviscosity retinopathy. Five patients (62.5%) died within 2 months of their ophthalmic presentation. Conclusion: Ophthalmic manifestations of MM are uncommon and diverse. They may appear at the initial presentation of the disease or occur late in the disease process. Awareness of the possible manifestations may lead to an earlier diagnosis and have a positive influence on the disease course.

Craniopharyngioma: a review of long-term visual outcome

Purpose: To assess the clinical presentation and long‐term visual outcome in a series of patients with craniopharyn‐gioma.

Traumatic chiasmal syndrome: a series of 19 patients.

Purpose: To present a clinical series of 19 patients with traumatic chiasmal syndrome.

Neuro-ophthalmology of invasive fungal sinusitis: 14 consecutive patients and a review of the literature

Invasive fungal sinusitis is a rare condition that usually occurs in immunocompromised patients and often presents as an orbital apex syndrome. It is frequently misdiagnosed on presentation and is almost always lethal without early treatment.

Neuro‐ophthalmological sequelae of horse‐related accidents

Purpose: The aim of this study was to highlight the neuro‐ophthalmological dangers associated with horse riding, and working around horses, and the importance of wearing adequate headgear to protect the rider from neuro‐ ophthalmic injuries. It raises the questions of whether the current laws regarding helmet use are satisfactory, and whether helmets currently used are of an adequate standard.

Ganglioglioma of the lateral ventricle presenting with blepharospasm : case report and review of the literature

Gangliogliomas of the lateral ventricle are unusual tumours of the central nervous system. We report a 25-year-old female who presented with a 4-week history of generalized headaches associated with left-sided blepharospasm. Clinical examination revealed bilateral papilloedema but no focal neurological deficits. Computed tomography and magnetic resonance imaging demonstrated a large lesion within the left lateral ventricle. Gross total surgical resection of the lesion was performed through a para-sagittal, trans-callosal approach. Histopathological analysis revealed the diagnosis of ganglioglioma. The post-operative course was uneventful, with complete resolution of both the headaches and blepharospasm. The presentation with an ipsilateral blepharospasm has never previously been reported in the setting of a lateral ventricular tumour.

New onset diplopia: 14 years after retinal detachment surgery with a hydrogel scleral buckle.

In 1979, the hydrogel explant (Miragel, Waltham, MA, USA) was introduced as a scleral buckling material in the surgical management of retinal detachment.1 It was widely used in the 1980s and early 1990s as it was initially believed to be well tolerated, less prone to infection, and easy to manipulate.2 However, long term complications related to swelling and fragmentation of the explant have been reported over recent years,3–6 resulting in discontinuation of its use in 1995. A 36 year old healthy man presented on 2003 with symptoms of mild right ocular discomfort. Past ocular history included a right retinal detachment repair 14 years previously, using a 907 (3×5 mm) Miragel scleral buckle (Miragel, Medical Instruments Research Associates, Waltham, MA, USA), sutured to the inferior sclera. On examination, visual acuity was …

Orbital invasion by esthesioneuroblastoma.

Purpose: Esthesioneuroblastoma is a rare malignancy arising from the olfactory mucosa in the nasal fossa, which can invade the orbit producing ophthalmic symptoms and signs. The purpose of this study was to present a case series and review the literature to assess the characteristics of orbital involvement by esthesioneuroblastoma. Methods: Retrospective case series of all patients treated for biopsy-proven esthesioneuroblastoma at the Royal Adelaide Hospital between 1992 and 2004. Results: Nine patients (5 male, 4 female) with a mean age of 50 years (range, 20 to 66 years) were reviewed. One case was classified as Kadish stage A, 1 as Kadish stage B, and 7 as Kadish stage C. Mean time from onset of symptoms to diagnosis was 17 months (range, 2 to 24 months). Radiologic orbital invasion was present in 4 cases and was associated with ophthalmic symptoms or signs in 3 of these cases. These included proptosis (2 cases), periorbital pain (1 case), decreased visual acuity (1 case), extraocular muscle restriction (1 case), and chemosis (1 case). One patient with symptoms secondary to orbital invasion was initially referred to and assessed by an ophthalmologist. All patients who had or went on to have development of orbital invasion had advanced disease at diagnosis. Treatment was surgical (9/9), with the addition of radiotherapy (6/9) and chemotherapy (2/9). Mean follow-up was 3.6 years (range, 0.5 to 8.5 years). Six of 9 patients had tumor recurrence. Two had metastasis. Five of 9 patients remained alive. Conclusions: Orbital invasion by esthesioneuroblastoma is not uncommon. It is important to be aware of this malignancy because a significant proportion of patients will present with ophthalmic signs and symptoms.

Neuro-ophthalmic manifestations of intracranial cavernous hemangiomas.

PURPOSE: To describe the neuro-ophthalmic manifestations of patients with intracranial cavernous hemangiomas (cavernomas). Methods A retrospective review of all patients with intracranial cavernomas with neuro-ophthalmic manifestations who were treated at the Royal Adelaide Hospital in Australia between 1994 and 2004. Results There were nine patients (three men and six women), with a mean age of 39 years (range 22–61). There was one cerebellar lesion, two thalamic, four pontine, one midbrain/pontine, and one midbrain. Ophthalmic presentations included internuclear ophthalmoplegia (one patient), third cranial nerve (CN) palsy (one patient), fourth CN palsy (one patient), and sixth CN palsy (six patients). Three patients underwent extraocular muscle surgery, and six were treated medically or observed. In five patients the diplopia resolved, in three it was only mild, and in one patient no significant change was noted during the follow-up period. Conclusions Diplopia is the main neuro-ophthalmic manifestation of intracranial cavernomas. Sixth CN palsy is the most common cause. Neurosurgical or conservative treatment leads to improvement in most cases, and later use of spectacles or extraocular muscle surgery may lead to further improvement.