Progressive optic neuropathy in idiopathic intracranial hypertension after optic nerve sheath fenestration. 2009

Byron N Wilkes, and R Michael Siatkowski
Dean McGee Eye Institute, Department of Ophthalmology, University of Oklahoma College of Medicine, Oklahoma City, Oklahoma, USA.

A 16-year-old woman complaining of headache and declining vision in both eyes had papilledema, normal brain imaging, and a lumbar puncture showing a moderately high opening pressure (35 cm H2O) and normal cerebrospinal fluid constituents. For a diagnosis of idiopathic intracranial hypertension (IIH), she was treated with acetazolamide and methylprednisolone, but vision worsened, so she underwent bilateral optic sheath fenestration (ONSF). Within the 1st postoperative week, vision had improved and papilledema was less prominent. However, by the 14th postoperative day, vision had worsened and headache persisted. Lumbar puncture showed a very high opening pressure (65 cm H2O), so she underwent ventriculoperitoneal shunting. Although there was a slight initial improvement in vision, it eventually declined further. This case emphasizes that ONSF may yield initial improvement in vision and reduction in papilledema yet not prevent eventual visual loss in IIH. Whether the visual loss in this patient resulted from persistently elevated intracranial pressure after ONSF or was prefigured before ONSF occurred is unresolved. It is a reminder that patients with IIH must be monitored carefully after ONSF. If there is a suggestion of further visual loss, shunting should be considered if intracranial pressure is high.

UI MeSH Term Description Entries
D008775 Methylprednisolone A PREDNISOLONE derivative with similar anti-inflammatory action. 6-Methylprednisolone,Medrol,Metipred,Urbason,6 Methylprednisolone
D009900 Optic Nerve The 2nd cranial nerve which conveys visual information from the RETINA to the brain. The nerve carries the axons of the RETINAL GANGLION CELLS which sort at the OPTIC CHIASM and continue via the OPTIC TRACTS to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the SUPERIOR COLLICULI and the SUPRACHIASMATIC NUCLEI. Though known as the second cranial nerve, it is considered part of the CENTRAL NERVOUS SYSTEM. Cranial Nerve II,Second Cranial Nerve,Nervus Opticus,Cranial Nerve, Second,Cranial Nerves, Second,Nerve, Optic,Nerve, Second Cranial,Nerves, Optic,Nerves, Second Cranial,Optic Nerves,Second Cranial Nerves
D009901 Optic Nerve Diseases Conditions which produce injury or dysfunction of the second cranial or optic nerve, which is generally considered a component of the central nervous system. Damage to optic nerve fibers may occur at or near their origin in the retina, at the optic disk, or in the nerve, optic chiasm, optic tract, or lateral geniculate nuclei. Clinical manifestations may include decreased visual acuity and contrast sensitivity, impaired color vision, and an afferent pupillary defect. Cranial Nerve II Diseases,Foster-Kennedy Syndrome,Optic Disc Disorders,Optic Disk Disorders,Optic Neuropathy,Second Cranial Nerve Diseases,Cranial Nerve II Disorder,Neural-Optical Lesion,Disc Disorder, Optic,Disk Disorder, Optic,Disorder, Optic Disc,Foster Kennedy Syndrome,Lesion, Neural-Optical,Neural Optical Lesion,Neural-Optical Lesions,Neuropathy, Optic,Optic Disc Disorder,Optic Disk Disorder,Optic Nerve Disease,Optic Neuropathies,Syndrome, Foster-Kennedy
D011559 Pseudotumor Cerebri A condition marked by raised intracranial pressure and characterized clinically by HEADACHES; NAUSEA; PAPILLEDEMA, peripheral constriction of the visual fields, transient visual obscurations, and pulsatile TINNITUS. OBESITY is frequently associated with this condition, which primarily affects women between 20 and 44 years of age. Chronic PAPILLEDEMA may lead to optic nerve injury (see OPTIC NERVE DISEASES) and visual loss (see BLINDNESS). Benign Intracranial Hypertension,Idiopathic Intracranial Hypertension,Intracranial Hypertension, Benign,Intracranial Hypertension, Idiopathic,Hypertension, Benign Intracranial,Hypertension, Idiopathic Intracranial
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000086 Acetazolamide One of the CARBONIC ANHYDRASE INHIBITORS that is sometimes effective against absence seizures. It is sometimes useful also as an adjunct in the treatment of tonic-clonic, myoclonic, and atonic seizures, particularly in women whose seizures occur or are exacerbated at specific times in the menstrual cycle. However, its usefulness is transient often because of rapid development of tolerance. Its antiepileptic effect may be due to its inhibitory effect on brain carbonic anhydrase, which leads to an increased transneuronal chloride gradient, increased chloride current, and increased inhibition. (From Smith and Reynard, Textbook of Pharmacology, 1991, p337) Acetadiazol,Acetazolam,Acetazolamide Sodium, (Sterile),Acetazolamide, Monosodium Salt,Ak-Zol,Apo-Acetazolamide,Diacarb,Diamox,Diuramide,Défiltran,Edemox,Glauconox,Glaupax,Huma-Zolamide,Ak Zol,AkZol,Apo Acetazolamide,ApoAcetazolamide,Huma Zolamide,HumaZolamide
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D013508 Ophthalmologic Surgical Procedures Surgery performed on the eye or any of its parts. Ophthalmologic Surgical Procedure,Ophthalmological Surgical Procedures,Procedure, Ophthalmologic Surgical,Procedures, Ophthalmologic Surgical,Surgical Procedure, Ophthalmologic,Surgical Procedures, Ophthalmologic,Ophthalmological Surgical Procedure,Procedure, Ophthalmological Surgical,Procedures, Ophthalmological Surgical,Surgical Procedure, Ophthalmological,Surgical Procedures, Ophthalmological
D014792 Visual Acuity Clarity or sharpness of OCULAR VISION or the ability of the eye to see fine details. Visual acuity depends on the functions of RETINA, neuronal transmission, and the interpretative ability of the brain. Normal visual acuity is expressed as 20/20 indicating that one can see at 20 feet what should normally be seen at that distance. Visual acuity can also be influenced by brightness, color, and contrast. Acuities, Visual,Acuity, Visual,Visual Acuities

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