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Abstract: A 50-year-old Caucasian Male presents with complaints of headaches and symptomatic constriction of peripheral visual field. Diagnostic MRI reveals protrusion of the cerebellar tonsils into the spinal canal indicative of Chiari Malformation Type 1. I. Case History: a. Patient Demographics: 50 year old Caucasian male; first presentation 2009 has been monitored and treated for Primary Open Angle Glaucoma. b. Chief Complaint: Presents to clinic for IOP check due to previous POAG diagnosis; currently using Latanoprost qhs OU for IOP control. Pt has noticed a decrease in peripheral vision and has difficulty with orientation and mobility during daily activities. c. Ocular/ Medical History: i. (-)injury, (-)surgery, (-)family history of ocular conditions - Diagnosed with POAG, OU in 2009 - Diabetes mellitus type II since 1999 without retinopathy, OU –Hypertension –Hypothyroidism –Headaches – diagnosed by PCP as chronic and obstructive; vague onset, frequency and location of headaches. d. Medications: – Latanoprost .005% qhs, OU –Metformin HCL 500 mg PO BID –Levothyroxine .2 mg PO BID –Amlodopine 10 mg PO QD –Vitamin B supplements (Pyridoxine HCL 25mg) II. Pertinent Findings: a. Clinical: i. VA sc OD: 20/30-2, OS: 20/30 ii. PERRL –APD iii. EOM FROM iv. Confrontation fields – Severely and equally constricted OD, OS v. SLE anterior segment: lids/lashes, conjunctiva, cornea, A/C, Iris: WNL OU vi. Angles 4/4 by VH OU vii. Tap OD: 14, OS: 14 @250pm (last drop Latanoprost last night) viii. DFE: Lens: trace NS OU, C/D ratios: OD: 0.60v/ 0.65h, deep; OS: 0.60rd, deep, Macula: scattered hard fine drusen OU, Vessels: normal 2/3 OU, Periphery: Flat and Intact 360 OU No Diabetic Retinopathy, OU b. Ancillary Testing: •HVF 24-2 2/2009 –OD: Reliable; Superior Arcuate Defect –OS: Reliable; Questionable Superior Arcuate Defect •HVF 24-2 10/12/10 –OD: Severe Constriction; GHT ONL; VFI 43% –OS: Severe Constriction; GHT ONL; VFI 31% •rNFL OCT 10/12/10 –OD: Borderline superior and inferior thinning –OS: Thinning Superiorly **show images c. Radiology Studies: a. MRI 10/2010: Chiari Type I malformation. Inferior surface of the cerebellar tonsils is located approximately 5 mm below the level of the foramen magnum. b. MRI 11/01/ 2011: Chiari Type I malformation. Inferior surface of the cerebellar tonsils is located approximately 5 mm below the level of the foramen magnum; MRI consistent with previous results. **show images III. Differential Diagnosis: a. Primary/ Leading: i. Migraines: Often associated with Chiari Malformations. MRI and other imaging studies are the main differential. Imaging of Migraine patients would show normal anatomical brain structure versus a Chiari Malformation patient. ii. Hydrocephalus: Increased Cerebrospinal Fluid; often presents with similar symptoms and can be associated with Chiari Malformation. MRI is the main differential to determine whether protrusion of the brainstem is occurring. Hallmark sign of Hydrocephalus on MRI is dilated ventricles. b. Others: i. Benign Intracranial Hypertension – Rule out disc edema on fundus exam. If disc edema is present Chiari Malformation can still be the cause. Thus imaging studies or lumbar puncture are the gold standard to rule out other underlying conditions. BIH is a diagnosis of exclusion. ii. Primary Headache Syndromes – Usually duration and onset can help rule out primary headache syndromes. Again to rule out Chiari Malformation, imaging studies are warranted. iii. Multiple Sclerosis – Inflammatory condition that attacks the myelin sheathing of nerve axons. Presents with broad array of symptoms including weakness, tingling, numbness, blurred vision, and fatigue. True differential is an MRI and neurologic exam. iv. Spinal Tumors – Mass effect may cause comparable symptoms as Chiari Malformation. MRI is used in differential diagnosis. IV. Diagnosis and Discussion a,b. Elaborate on the condition/ expound on unique features: Chiari Malformations are a group of complex brain abnormalities that affect the posterior base of the skull where the brain and spinal cord connect. Often the posterior fossa is narrow and too small to hold the cerebellum and brainstem. The cerebellum is forced downward resulting in protrusion through the foramen magnum; this herniation of the cerebellum and brainstem decreases CSF flow and can physically compress these areas of the brain. Theories suggest improper fetal development associated with: hazardous chemicals and substances, lack of vitamins and nutrients in maternal diet, infection, prescription or illegal drug and alcohol consumption, and hereditary tendency. There are four types of Chiari Malformations based on their associated anatomical abnormalities. Chiari Malformation Type I (CM1) is the most common and least severe. CM1 is typically congenital and presents in adulthood. CM1 is medically defined as the displacement of the Cerebellar tonsils 5mm or more below the foramen magnum as seen on imaging studies. Some common symptoms are: •Chronic Headaches * most common symptom –Cough, coital and exertion headaches –Chronic Migraine; three times more prevalent in CM than in general population •Palatial Tinnitus and other auditory symptoms •Neck pain – often radiating down the spine •Dizziness/ Vertigo – increased with neck extension •Vague pains throughout body •Impaired balance and coordination •Nausea/ vomiting •Poor memory, cognition and concentration •Polyuria •Irritable bowel syndrome •Difficulty Swallowing •Changes in voice Ocular Symptoms are: •Photophobia •Visual Blurring •Peripheral field loss/ blind spots •Downbeat Nystagmus •Diplopia •Mydriasis •Papilledema V. Treatment: a. Treatment and response to treatment: i. Surgical Intervention is the only treatment to correct a Chiari Malformation. Some symptoms of this condition such as pain and headaches can be medically treated with analgesics. ii. New surgical studies have shown excellent success in the improvement of quality of life of patients after the procedure. iii. Goals of Surgery are to decompress the cervicomedullary junction and restore CSF flow in the region of the foramen magnum. b. Refer to Research: i. Retrospective study of 66 patients (mean age 15 years) – 32 patients presented with CM 1 alone and 34 patients in combination with syringomyelia. ii. Retrospective review of 25 patients with Chiari Malformation and syringiomyelia with or without scoliosis were reviewed over the past 20 years. iii. Cerebrospinal Fluid flow imaging by phase- contrast MR technique is being used to monitor CSF flow dynamics in several disorders including Chiari Malformation and hydrocephalus. c. Bibliography: 1.Tisell M, Wallskog J, Linde M. Long-term outcome after surgery for Chiari I malformation. Acta Neurol Scand 2009: 120: 295-299. 2.Verma R, Praharaj HN. Unusual association of Arnold- Chiari malformation and vitamin B12 deficiency. Department of Neurology: 2012 July 09. 3.Chiari Malformation. http://emedicine.medscape.com. Updated March 2, 2012. 4.deSouza, Ruth-Mary, Zsolt Zador, David Frim. Chiari malformation type I: related conditions. Neurological Research 2011: Vol. 33: 278- 284. 5.Cinalli G, Renier D, Sebag G. Chiari ‘malformation’ in Crouzon syndrome. Arch Pediatr 1996; 3: 433-439. 6.Banik R, Lin D, Miller NR. Prevalence of Chiari I malformation and cerebellar ectopia in patients with psuedotumor cerebri. J Neurol Sci 2006; 247: 71-75 7.Royo- Salvador MB. Syringomyelia, scoliosis and idiopathic Arnold-Chiari malformations: a common etiology. Rev Neurol 1996; 24: 1241-50. 8.Kaplan Y, Oksuz E. Chronic migraine associated with the Chiari type I malformation. Clin Neurol Neurosurg 2008; 110: 818 – 22. 9.Pettorini B, Gao A, Rodrigues D. Acute deterioration of a Chiari I malformation: an uncommon neurosurgical emergency. Childs Nerv Syst 2011; 27: 857 – 860. 10.Alzate JC, Kothbauer KF, Jallo GI. Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurg Focus. 2001 Jul 15; 11. 11.Chiari Malformation. http://www.wichiaricenter.org. The Wisconsin Chiari Center. c 2012. 12.Chiari Malformation Fact Sheet. http://www.ninds.nih.gov. National Institute of Neurologic Disorders and Stroke. Last updated 2/1/12. 13.Durai R, Fernandes C. Arnold- Chiari Malformation. Images in Medicine. British Journal of Hospital Medicine, March 2010, Vol 71, No 3. 14.Chiari Malformations. http://www.webmd.com 15.Chiari Malformation. Columbia Neurosurgery. http://www.columbianeurosurgery.com. 16.Chiari Malformation. The Chiari Institute. http://www.chiariinstitute.com. VI. Conclusion: a. Clinical Pearls, take away points: i. When symptoms of Chiari Malformations seriously impair the patient’s quality of life, it is best to consider surgery. ii. The goal of surgery for CM is to create more intracranial space and improve CSF circulation. iii. Physician should inquire regarding developmental history of the child: Sitting up? Crawling? Walking? Talking? Also measure the circumference of the head. iv. No two cases of Chiari Malformation are alike. v. MRI is the main diagnostic procedure for determining this condition.