|Year : 2015 | Volume
| Issue : 3 | Page : 150-152
Coexistence of optic disc drusen and idiopathic intracranial hypertension in a non-obese female
Pradeep A Venkataramana1, Preetham B Patil2, Shivabasappa V Koti1, Varna Shetty2
1 Department of Ophthalmology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India
2 Department of Radiology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India
|Date of Submission||11-Feb-2014|
|Date of Acceptance||11-Dec-2014|
|Date of Web Publication||20-Aug-2015|
Pradeep A Venkataramana
Assistant Professor, Department of Ophthalmology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad - 580 009
Source of Support: None, Conflict of Interest: None
Idiopathic intracranial hypertension (IIHT) is a headache syndrome characterized by elevated intracranial pressure with normal cerebrospinal fluid (CSF) content, normal neuro imaging with optic disc edema. Optic disc drusen (ODD) presents a diagnostic challenge as it can mimic disc edema. Although the appearance of ODD and disc edema can at times appear similar, the etiology and treatment of these two conditions differ. We report a case of young non-obese female who was referred for headache and on imaging had coexistence of ODD and IIHT.
Keywords: B scan, cerebrospinal fluid opening pressure, optic disc drusen (ODD), papilledema
|How to cite this article:|
Venkataramana PA, Patil PB, Koti SV, Shetty V. Coexistence of optic disc drusen and idiopathic intracranial hypertension in a non-obese female. J Clin Ophthalmol Res 2015;3:150-2
|How to cite this URL:|
Venkataramana PA, Patil PB, Koti SV, Shetty V. Coexistence of optic disc drusen and idiopathic intracranial hypertension in a non-obese female. J Clin Ophthalmol Res [serial online] 2015 [cited 2020 Feb 26];3:150-2. Available from: http://www.jcor.in/text.asp?2015/3/3/150/163298
Optic disc drusen (ODD) is a congenital and developmental anomaly of the optic nerve head characterized by hyaline-containing bodies. Idiopathic intracranial hypertension (IIHT) classically presents with headache and vision changes in obese women of childbearing age. Several formalized criteria for IIHT exist in the literature. Friedman and Jacobson updated modified Dandy criteria in the diagnosis of IIHT to reflect the advances of magnetic resonance imaging (MRI) and the characterization of other etiologies of IIHT. The most common clinical signs include optic disc edema, occasional abducens nerve palsy, headache, vomiting, blurred vision, diplopia, and photophobia.
| Case Report|| |
A 26-year-old non-obese female was referred with a diagnosis of bilateral papilledema. She complained of headache from past 6 months which was bilateral, non-throbbing more during evening, associated with vomiting. There was no history of weakness, numbness, parasthesia, systemic illness, chronic drug therapy, and decreased vision. General physical examination and systemic examination were unremarkable. Ocular examination revealed visual acuity of 20/20, near vision N.6, color vision testing by Ishihara plates was 15/15 and bilateral full eye movements. Pupils were equal, and reactive to light. Fundoscopy revealed bilateral hyperemic disc with blurred, elevated disc margins (Right eye having Frisen stage 1 with Left eye having Frisen stage 2 score) with obliteration of the physiological disc cupping.  Abnormal branching pattern of vessels are seen at the disc with abnormal tortuous veins around the disc [Figure 1]. Axial length in both eyes was 23.3 mm. A provisional diagnosis of papilloedema was made and neuroimaging, fundus flourescien angiography (FFA) was ordered. MRI brain showed increased fluid around bilateral optic nerves, pinched lateral and third ventricle, with partial empty sella. There was no evidence of space occupying lesion [Figure 2]a-d. Magnetic resonance venogram was normal. FFA shows leakage at the disc with staining in late phase of angiogram which was suggestive of disc edema [Figure 3]. B-scan ultrasonogram revealed evidence of hyperechoeic speck in bilateral optic nerve head region suggestive of ODD [Figure 4]. Visual fields showed mild enlarged blind spot [Figure 5].
|Figure 1: Showing blurred margins (LE >RE) with abnormal branching pattern of vessels at and around disc|
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|Figure 2: (a-d) Magnetic resonance imaging (MRI) showing increased fluid in sheaths surrounding the optic nerves on both sides, small pinched ventricles with partial empty sella|
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|Figure 3: Shows leakage at the disc with staining in late phase of angiogram|
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|Figure 4: B scan showing hyperechoeic speck in bilateral optic nerve head region suggestive of ODD|
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Imaging findings were suggestive of IIHT and cerebro spinal fluid (CSF) opening pressure with biochemical and microbial analysis was ordered. CSF opening pressure was 28 mmHg. CSF biochemical and microbiological analysis was within normal limits. Her thyroid stimulating hormone, Luteinizing hormone and follicle stimulating hormone levels were within normal limits. She was diagnosed as IIHT with coexistence of ODD bilaterally. The diagnosis was explained and the patient was started on tab acetozolamide 250 mg three times a day and was advised follow up regularly. After 2 week of starting treatment patient become symptomatically better with resolution of headache and disc edema [Figure 6]. Patient is on regular follow up for 1 year with visual fields and disc imaging which showed no progression.
|Figure 6: Post treatment with Acetozolamide showing decrease in the disc swelling and margins appearing clear|
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| Discussion|| |
ODD are formed by abnormal axonal metabolism in optic nerve leading to intracellular mitochondrial calcification forming calcified micro bodies and as calcium continues to deposit on these microbodies and so ODD increase in size.  ODD is found in approximately 0.34% of the population, bilateral in 75% of cases and have no sex predilection.  Impairment of visual acuity is rare in ODD, but insignificant visual field defects may occur in up to half of cases. The funduscopic appearance of ODD depends on their location within the optic nerve head. The disc vasculature shows emerging vessels which are more numerous with anomalous branching pattern.  Children and young adults are vulnerable to misdiagnosis, such as papilledema.
Friedman and Jacobson in 2003 updated modified Dandy criteria in the diagnosis of IIHT which additionally includes no evidence of hydrocephalus, mass, structural, or vascular lesion on MRI or contrast-enhanced computed tomogram (CT) for typical patients, and MRI and MR venography for all others and no other cause of intracranial hypertension identified.  Ophthalmologic signs of IIHT consist of diminished visual acuity, visual field loss, 6th nerve palsy, and papilledema. Absence of papilledema has been reported in many patients with IIHT, but its absence is suggestive of an alternative etiology for headache and vision loss. 
The primary goals of management in IIHT are restoration of visual acuity and resolution of papilledema. Conservative measures like weight loss is recommended in patients without vision loss as the predominant presentation. Carbonic anhydrase inhibitors like acetozolamide and topiramate with systemic corticosteroids are used to decrease intracranial pressure. Therapeutic lumbar puncture is another treatment method to decrease elevated CSF pressure immediately but effects are short-lived.
Aggressive treatment measures like Lumbo peritoneal shunt surgery and optic nerve sheath fenestration are reserved for those who continue to experience vision loss despite conservative management and in patient who initially present with rapid vision loss. Venous sinus stent placement is used recently to treat IIHT on the basis of several imaging studies showing stenosis of the cerebral venous sinuses and the presumption that venous outflow obstruction constitutes the underlying etiology of most cases of IIHT.  Frequent follow-up, including visual field testing at intervals of 1 month, 3 months, and then every 3-6 months, is advisable. 
Other causes of papilledema include tumors of the brain, malignant hypertension, bilateral compressive thyroid ophthalmopathy, bilateral simultaneous anterior ischemic optic neuropathy, bilateral central retinal vein occlusion, and carotid cavernous fistula. After extensive literature search and to our best knowledge only nine cases are reported in literature and our case is the 10 th case. ,,,,
This case emphasizes the importance of detailed clinical history taking and enquires must be made regarding neurological symptoms prior to accepting the diagnosis of pseudopapilledema. The ophthalmologist, neurologist, and the reporting radiologist must therefore consider ODD when dealing with atypical cases of disc swelling to prevent delay in appropriate management.
| References|| |
Frisen L. Swelling of the optic nerve head: A staging scheme. J Neurol Neurosurg Psychiatry 1982;45:13-8.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47:515-32.
Tso MO. Pathology and pathogenesis of drusen of the optic nervehead. Ophthalmology 1981;88:1066-80.
Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492-5.
Wall M. Idiopathic intracranial hypertension. Neurol Clin 2010;28:593-617.
Passi N, Degnan AJ, Levy LM. MR imaging of papilledema and visual pathways: Effects of increased intracranial pressure and pathophysiologic mechanisms. AJNR Am J Neuroradiol 2013;34:919-24.
Krasnitz I, Beiran I, Mever E, Miller B. Coexistence of optic nerve head drusen and pseudotumour cerebri: A clinical dilemma. Eur J Ophthalmol 1997;7:383-6.
Rossiter JD, Lockwood AJ, Evans AR. Coexistence of optic disc drusen and idiopathic intracranial hypertension in a child. Eye (Lond) 2005;19:234-5.
Fong CY, Williams C, Pople IK, Jardine PE. Optic disc drusen masquerading as papilloedema. Arch Dis Child 2010;95:629.
Granger RH, Bonnelame T, Daubenton J, Dreyer M, McCartney P. Optic nerve head drusen and idiopathic intracranial hypertension in a 14-year-old girl. J Pediatr Ophthalmol Strabismus 2009; 46:238-40.
Komur M, Sari A, Okuyaz C. Simultaneous papilledema and optic disc drusen in a child. Pediatr Neurol 2012;46:187-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]