Papilledema

Papilledema can be an issue with NF2. Papilledema is the swelling of the optic nerve as it enters the back of the eye due to raised intracranial pressure.

Increased intracranial pressure can lead to hearing vascular noises, whooshing sounds, or pulsatile tinnitus.

Dire consequences of sustained elevated intracranial pressures if not recognized in time will cause visual loss or blindness. If you are a person with NF2, make sure to get your eyes checked regularly.

Papilledema

Arch Ophthalmol, Dilip A. Thomas, MD; Jonathan D. Trobe, MD; Wayne T. Cornblath, MD1

Symptoms of Papilledema:

Symptoms related to papilledema when caused by increased pressure include headache and nausea with vomiting and a machinery-like sound. Twenty-five percent of people with advanced severe papilledema also will develop some visual symptoms. Typically, the visual changes are recurring brief episodes lasting less than 30 seconds in which the vision turns gray or blacks, sometimes described as if a veil has fallen over the eyes. The symptoms usually affect both eyes at once. Visual blackouts often are triggered by a change in position, such as by standing up very suddenly, or they may be triggered by coughing or straining in the chest or abdomen. Occasionally, people with papilledema can have an experience of flashing lights, often seen in an arc shape. Other visual changes occur over time, including a smaller field of vision with a larger blind spot and, ultimately, blindness, if successful treatment is not given.2

Papilledema is usually bilateral.

Under certain special circumstances, papilledema can be unilateral. Intracranial lesions that exert pressure on one optic nerve often leads to unilateral optic atrophy. If these lesions are large enough, they may cause increased intracranial tension and papilledema in the opposite eye.

A space occupying lesions such as tumor or decrease of absorption of cerebrospinal fluid, change in the dynamics of cerebrospinal flow through the ventricles (for example by obstruction of the 4th ventricle), or rarely by increase of flow of cerebrospinal fluid.3

Need to emphasizes that low spinal cord tumors can cause elevated intracranial pressure without causing markedly elevated cerebrospinal fluid protein or cells, or any myelopathic manifestations, perhaps by obstructing sacral cerebrospinal.

The association of papilledema and spinal tumor is an unusual but well-known phenomenon.4

Obstructive Hydrocephalus occurs when CSF flow is obstructed within the ventricular system or in its outlets to the arachnoid space, resulting in impairment of the CSF from the ventricular to the subarachnoid space. The most common form of noncommunicating hydrocephalus is obstructive and is caused by intraventricular or extraventricular mass-occupying lesions that disrupt the ventricular anatomy.

References:

  1. Dilip A. Thomas, et al. Visual Loss Secondary to Increased Intracranial Pressure in Neurofibromatosis Type 2, Arch Ophthalmol. 1999;117(12):1650-1653.
  2. InteliHealth, reviewed by Harvard Medical School, Optic Nerve Swelling (Papilledema).
  3. Madjid Samii, et al. Neurofibromatosis Neurosurgical Treatment and Follow-up. European Neurological Review, 2007;(2):14-16.
  4. Costello F, Kardon RH, et al. Papilledema as the presenting manifestation of spinal schwannoma.J Neuroophthalmol. 2002 Sep;22(3):199-203.

Further Readings:

  • Definition of PapilledemaMedicineNet.com – Papilledema: Swelling of the head of the optic nerve, a sign of increased intracranial pressure. The optic nerve head, also called the optic disk or papilla, is the area where the optic nerve (the nerve that carries messages from the eye to the brain) enters the eyeball. …
  • Edmond J. FitzGibbon, M.D., PapilledemaeyeWiki – Papilledema refers to swelling of the optic disc from increased intracranial pressure (ICP)]. It must be distinguished from optic disc swelling from other causes which is simply termed “optic disc edema”. Papilledema must also be distinguished from pseudo-papilledema such as optic disc drusen. Since the root cause of papilledema is increased intracranial pressure (ICP) this is an alarming sign which may presage such entities as brain tumor, CNS inflammation, or idiopathic intracranial hypertension (IIH). …
  • James Garrity, MD, PapilledemaMerck Manual – Papilledema is swelling of the optic disk due to increased intracranial pressure. All other causes of optic disk swelling, such as that caused by malignant hypertension or thrombosis of the central retinal vein, do not involve increased intracranial pressure and therefore are not causes of papilledema. There are no early symptoms, although vision may be disturbed for a few seconds. Papilledema requires an immediate search for the cause. Diagnosis is by ophthalmoscopy with further tests, usually brain imaging, to determine cause. Treatment is directed at the underlying condition. …
  • Leslie Pham, MD and Michael Wall, MD, Papilledema GradesThe University of Iowa – various images of Papilledema progression.
  • Dr. Kanthamani, Papilledema :Neurosurgery, On the Web – Papilledema is a, non-inflammatory passive swelling of the optic disc, produced by raised intra-cranial tension (ICT). It occurs as a result of interrupted axoplasmic flow in the optic nerve due to transmitted elevated cerebrospinal fluid pressure in the subarachnoid space around it. This in turn causes venous congestion. …
  • Papilledema Pictures, Definition, Causes, Symptoms and TreatmentPrimeHealthChannel – Doctors and medical researchers define Papilledema as a medical condition that affects the eyes of a person. The condition is characterized by a swelling or inflammation of the optic nerves at the rear part of the eye. This happens due to an increasing pressure inside the brain. The optic nerves are responsible for carrying messages from the eyes to the brain. …
  • PapilledemaNational Cancer Institute (NIH-NCI) – Swelling around the optic disk, the area where the optic nerve (the nerve that carries messages from the eye to the brain) enters the eyeball. Papilledema occurs when increased brain pressure caused by tumors or other problems results in swelling of the optic nerve.
  • Understanding PapilledemaIntracranial Hypertension Research Foundation – Papilledema, or swelling of the optic nerves, is often considered a hallmark sign of intracranial hypertension. But what does your doctor see when he looks into your eyes? Read on to find out what papilledema is, why it develops, what a venous pulsation is and whether papilledema is necessary for an IH diagnosis.