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Correct differential diagnosis in cases of blurred optic disc margins is a challenging task for ophthalmologists. The reliable differentiation of pseudopapilloedema and true papilloedema has significant implications for proper patient management. Conditions that give rise to pseudopapilloedema include small crowded discs, tilted discs and optic nerve head drusen. Conditions that cause bilateral true swelling of the optic nerve head with initially good visual acuity include those that are secondary to raised intracranial pressure (optic disc edema, ODE). The majority of cases, however, present with unilateral optic nerve head swelling and normal intracranial pressure. They have systemic signs or symptoms which either precede ocular manifestation or have ophthalmoscopic signs other than elevation of the optic disc pointing to its diagnosis. Ancillary testing has been utilized to aid in identification of true ODE or swelling, including ultrasonography, fluorescein angiography, cranial and orbital MRI with venography, and lumbar puncture. Optical coherence tomography is also evolving as a modality for differentiation of buried optic disc drusen from ODE. This presentation will discuss each modality, with examples, advantages, and disadvantages for each., Competing Interests: Interessenkonflikt: Die Autoren geben an, dass kein Interessenkonflikt besteht., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: A broad differential diagnosis has to be considered in a patient with swollen discs. Myeloproliferative disorders such as leukemia and lymphoma can in rare cases cause infiltrative optic neuropathy., History and Signs: Two patients initially presented with slowly progressive severe visual loss. History was unremarkable except for previously noted slightly elevated white blood cell count for which - according to their general physicians - no treatment or work-up was required. At presentation, bilateral disc swelling was present. Magnetic resonance imaging showed enhancement of the entire optic nerves sparing the chiasm. No other intracranial lesion was found. Cerebrospinal fluid contained no malignant cells., Therapy and Outcome: After bone marrow aspiration the diagnosis of non-Hodgkin's lymphoma and granulocytic leukemia, respectively, was made. Treatment resulted in visual recovery., Conclusion: Work-up in a patient with swollen discs should always include white blood cell count. If the result is abnormal further exploration should be pursued. Elevated white blood cell count may be the only hint of optic nerve infiltration caused by a myeloproliferative disorder and its treatment can result in remarkable recovery.

Background: Ocular involvement in panarteritis nodosa (PAN) has been reported to occur in 10 to 20% of patients. In 3 patients with acute visual disturbance we point out unusual findings., Patients: Case 1. A 40-year-old man initially presented with papilledema together with partial optic atrophy in both eyes, later polyneuropathy, gangrene of the toes and myalgic pains developed. Caliber changes in the small arteries in the liver were seen angiographically and recognized as signs of PAN. Under treatment with cyclophosphamide und prednisone no relapse occurred during a follow-up of 2 years. Case 2. In a 67-year-old man who suffered from arterial hypertension and coronary heart disease, central retinal artery occlusion occurred, at first in the left and then later in the right eye. The clinically suspected diagnosis of PAN (arterial hypertension, myalgia, polyneuropathy) was confirmed by a muscle biopsy. During a follow-up of 4 years--including treatment with prednisone and cyclophosphamide--no relapse occurred. Case 3. A 16-year-old adolescent with throbbing headaches and a thickened right temporal artery reported visual disturbances. These were due to an inflammation of choroidal vessels of the right eye appearing as an initial sign of PAN. Histology revealed a necrotising arteritis of the temporal artery. He presented with signs of Raynaud's disease, cachexia and arterial hypertension. Multiple vasculitic changes were detected by aorto-arteriography. Five months after the visual deterioration an anterior spinal artery syndrome with quadriplegia developed. After a follow-up of 2 years and treatment with prednisone und cyclophosphamide, he still had paralysis of both legs. The visual acuity was 1.0 in each eye., Conclusion: PAN should be considered in differential diagnosis in patients with acute inflammatory signs of the optic nerve head, the choroid and/or the retina together with general signs of the disease. If the disease is suspected, a muscle biopsy is indicated.

Background: The circumscript cutaneous sclerosis is an inflammato-edematous erythema of the skin, usually leading to a plaque-like sclerosis and cutaneous atrophy. The etiology of this disease remains unknown, but some authors presume a systemic infection with Borrelia burgdorferi as the underlying pathological condition., Patient: We present a case of a 66-year-old male patient who suffered from episcleritis of his left eye. Funduscopy showed an unilateral papillary edema. Visual acuity was 1.0. The patient had multiple erythemata of the skin of the body-trunk and the arms. On histological examination the lesions were compatible with circumscript sclerosis. Neurological examination was normal. Brain imaging (MRI), CSF examination and serology showed no pathological findings, although the patient presented with raised antibodies against Borrelia burgdorferi (IgG positive, IGM negative). Intravenous antibiotic treatment and topical steroids for three weeks resulted in a complete recovery of the scleritis, but had no effect on the papillary edema., Conclusion: Inflammatory pathological findings of the eye can be associated with circumscript sclerosis, a disease, that is normally limited to the skin. The pathological mechanism remains unclear. The presumption of an underlying borreliosis could not be confirmed in this case.

Background: In glaucomatous optic disc morphology follow-up, changes due to loss of nerve fibres should be distinguished from changes without loss of nerve fibres., Patient and Method: The intraocular pressure-dependent neuroretinal rim change in a 25-year-old white woman's optic nerve head, suffering from unilateral congenital glaucoma, had been documented papillometrically and by means of Laser Scanning Tomography. For papillometry, the optic disc outlines obtained from 15-degree stereo slides were evaluated planimetrically. To compute the volume of the optic disc excavation, 32 axially staggered optical sections were registered with a laser scanning retina tomograph (Heidelberg Engineering, Heidelberg) and computed three-dimensionally., Results: At an intraocular pressure level of 9 mm Hg a diminution of the planimetrical cup to disc ratio of about 20% and a reduction of the tomographically obtained excavation volume of about 55% was seen in comparison to 44 mm Hg one day before.--After 6-month follow-up, these parameters returned to their initial readings., Conclusion: This phenomenon was attributed to an acute post-barotraumatic tissue edema.--To our mind, this single observation illustrates an acute change in optic disc morphology documented and quantified by laser scanning tomography.

Fluorescein angiography has been underrated as an exploratory tool in the diagnosis of blurred optic disks. The ten cases presented here had blurred disks of diverse etiology; fluorescein angiography helped in the differential diagnosis and therapeutic approach. Irrigation of the disk, type, magnitude and location of dye leakage, as well as objectivation of peripapillary and retinal vascular characteristics all help to explain the pathogenic mechanisms leading to different types of optic disk edema. Although it is known tha papilledema due to increased intracranial pressure can not be differentiated from locally caused blurring of disks, there remain a number of mechanical, ischemic and inflammatory conditions that can be diagnosed by means of fluorescein angiography. Thus, optic neuritis, ischemic optic neuropathy, papillovasculitis and juxtapapillary chorioretinitis are some of the diseases in which chorioretinitis are some of the diseases in which angiography may help in differential diagnosis and lead to adequate therapeutic approaches.

We examined 160 patients with pseudopapilledema caused by deep and hidden drusen. The advantages of ophthalmochromoscopy versus fluorescein angiography in diagnosing deep drusen are demonstrated. The role of fluorescein examination in differentiating pseudopapilledema from genuine papilledema is pointed out. After many years of observation of patients suffering from drusen and of their relatives, and by studying the histologic data, the dynamics of the process were revealed. A classification of drusen according to depth of location is suggested. Hidden, deep and amputated drusen are distinguished. The conclusion is that deep and hidden drusen are the most frequent cause of pseudopapilledema.