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Chiasmal Syndrome 4 NEURO-OPHTHALMIC MANIFESTATIONS OF INTRACRANIAL TUMOURS – A CLINICAL PROFILE Dissertation Submitted for M.S.Degree(Branch III) Ophthalmology April 2014. THE TAMILNADU DR.M.G.R.MEDICAL UNIVERSITY CHENNAI CERTIFICATE This is to certify that this dissertation entitled “NEURO- OPHTHALMIC MANIFESTATIONS OF INTRACRANIAL TUMOURS-A CLINICAL PROFILE” is a bonafide work done by Dr. R.Dhivya under our guidance and supervision in the Neuro-ophthalmology department of Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Madurai during the period of her post graduate training in Ophthalmology for May 2011 – April 2014. Dr. S.Mahesh kumar Dr.Kowsalya Guide Co-Guide Head of The Department Consultant Neuro-ophthalmology Neuro-ophthalmology Aravind Eye Hospital, Aravind Eye Hospital, Madurai. Madurai Dr.M.Srinivasan Director, Aravind Eye Hospital, Madurai. DECLARATION I, Dr.R.Dhivya, hereby declare that this dissertation entitled, “NEURO- OPHTHALMIC MANIFESTATIONS OF INTRACRANIAL TUMOURS-A CLINICAL PROFILE”, is being submitted in partial fulfilment for the award of M.S. in Ophthalmology Degree by the Tamil Nadu DR. MGR Medical University in the examination to be held in April 2014. I declare that this dissertation is my original work and has not formed the basis for the award of any other degree or diploma awarded to me previously. Dr.R.Dhivya, Aravind Eye Hospital, Madurai. ACKNOWLEDGEMENT First and foremost I am thankful to Almighty for always being with me and guiding me throughout my life. I would like to express my heartfelt gratitude to my beloved parents for the dreams they have dreamt and the hardships they have been through to make me stand where I am today. I also thank my husband and my sister who had been supporting me throughout my tougher times. I take this opportunity to pay respect and homage to Dr.G.Venkatasamy, our founder. I am privileged to have on my side Dr.P.Namperumalsamy, Chairman emeritus and Director of Research, Dr.G.Natchiar, Director emeritus and chief of Department of Neuro-ophthalmology, Dr.M.Srinivasan, Director emeritus and other scholars at Aravind eye care system who had been a great source of inspiration to us. I am very grateful to Dr.R.D.Ravindran, Chairman of Aravind Eye care system for having created an environment enriched with all the facilities for learning and gaining knowledge. Words cannot express my deep sense of gratitude and heartfelt thanks to my mentor and guide Dr.S.Mahesh kumar, Professor Department of Neuro-ophthalmology who with his able guidance, optimistic attitude and constant encouragement gave me all the confidence and determination to complete my dissertation. My sincere thanks to Dr.N.Venkatesh Prajna, Director of Academics, who offered his guidance and support throughout my residency period. I take the privilege to express my gratitude and humble regards to Dr.Kowsalya, consultant in Neuro-ophthalmology for her valuable guidance, timely suggestions during my study. I am grateful to Bio-Statistician, for her guidance in statistical analysis of this study. My very sincere thanks to Mrs.Kumaragurupari, Senior Librarian for her immediate responses in providing all the articles and academic support required in the completion of this thesis. I would like to thank the para medical staffs of Neuro-ophthalmology department for their support. I would fail in my duty if I didn’t thank the countless patients who have been the learning ground for my study and my residency. CONTENTS PART - I S. No Title Page No 1. Review of literature 1 Introduction 2. Anatomy of Optic Chiasm 3 3. Chiasmal syndrome 4 4. Pituitary adenoma 7 5. Acoustic neuroma 23 6. Meningioma 28 7. Craniopharyngioma 34 8. Mid Brain and Pineal gland tumours 36 9. Malignant Brain tumours and Secondaries 38 PART - II Sl.No. Title Page No. 10. Aims & Objectives 41 11. Materials and Methods 42 12. Analysis & Results 46 13. Discussion 71 14. Limitations 75 15. Conclusion 76 16. Bibliography 17. Proforma 18. Master Chart INTRODUCTION Intracranial tumors are serious and life-threatening because of its invasive and infiltrative character in the limited space of the intracranial cavity. Primary Brain tumour is one of the major reasons for seeking neurological consultations worldwide. The clinical features and management option depends upon the site, size and type of the tumour1.The clinical features in patients with intracranial tumour may be caused by the influence on hormone secretion/depression or their mass related pressure effect on the surrounding structures or due to development of hydrocephalus1. Some Intracranial tumours present with ophthalmic abnormalities; these abnormalities could result from their effect on the visual pathway, ocular nerves and orbito-ocular tissues2. Neuro-ophthalmic signs and symptoms of intracranial tumours are visual loss, headache,vomiting, giddiness, double vision, nerve palsies, pupillary abnormalities, ptosis, proptosis,visual field defects and optic nerve head changes like optic atrophy, papilloedema etc. Visual signs and symptoms form major presentation in certain types of brain tumours and may be of great help in early diagnosis when initial signs are unspecific. The most common Intracranial tumours that presents with ophthalmic features are Pituitary adenoma, Meningioma, Acoustic schwannoma, Craniopharyngioma,Glioma, metastatic tumours1 etc. Patients with intracranial tumours can present with various symptoms like 1. Defective vision 2. Defective visual field/colour vision 3. Headache 4. Vomiting 5. Giddiness/Fainting 6. Seizures 7. Tinnitus 8. Hard of hearing 9. Transient obscuration of vision 10. Double vision etc…, The major cause for visual field defect is due to compression of optic chiasm known as chiasmal syndrome1.Pituitary adenomas are the most common cause for chiasmal syndrome. ANATOMY OF OPTIC CHIASM The optic chiasm is at the junction of two optic nerves. The nasal fibers of each optic nerve decussate (cross) across the chiasm to the opposite side while the temporal fibers are uncrossed to form the optic tract of the same side. This allows the right half of the visual field to end up on the left side of the brain and the left half of the visual field to locate to the left side of the Brain. At the optic chiasm, 53% of the fibres from the nasal retina cross the midline to join the uncrossed temporal fibers. These nasal fibers carry information from the temporal visual field. Similarly, the temporal fibres carry information from the nasal field. Optic chiasm is clinically very important because many important structures are located around it. The Pituitary gland is situated below the optic chiasm in the sella turcica. The sella turcica is covered in front by tuberculum sellae and behind by the dorsum sellae. The supraclinoid branches of the internal carotid artery flank the chiasm. The cavernous sinuses are located lateral and inferior to the chiasm. The frontal lobe of the Brain lies above the chiasm and behind the optic chiasm lies the third ventricles. CHIASMAL SYNDROME Compression of fibres of optic chiasm leads to chiasmal syndrome. Chiasmal syndrome is divided into anterior, middle and posterior. Anterior chiasmal syndrome involves the junction of the optic nerve and optic chiasm. Middle chiasmal syndrome involves the decussating fibers in the body of the optic chiasm while posterior chiasmal syndrome involves the distal fibers. The classic anterior chiasmal lesion produces central scotoma, and contralateral superotemporal field defect. This is known as Junctional scotoma6. Compression of Middle fibres of optic chiasm involve the uncrossed temporal fibres result in a nasal or binasal hemianopia. Lesions in the body of the chiasm most commonly damage the crossing nasal fibers. This leads to a bitemporal hemianopia. Posterior chiasmal syndrome affects the macular fibres which cross more posteriorly. This leads to paracentral bitemporal field defect. Posterior chiasmal compression may also involve the optic tract and cause a contralateral homonymous hemianopia. Longstanding damage to optic nerve fibres can lead to Optic disc pallor. If the lateral uncrossed fibers are spared then, the pallor appears in a bow-tie configuration6. In this type of lesion the fibres affected are retinal ganglion cells nasal to the macula in the papillomacular bundle. Compressive lesions often cause headache and may compress the third ventricle leading to hydrocephalus. The major cause of chiasmal syndrome is compressive lesions like pituitatry adenomas, craniopharyngiomas and meningiomas 5. Non functional pituitary adenomas can present with visual field defects which may be the earliest manifestation.Field defects are rare in functional pituitary tumours6. Other causes of chiasmal syndrome are Ø Gliomas, Ø Multiple sclerosis Ø Trauma Ø Metabolic Ø Toxic Ø Infectious (very rare) Gliomas of optic chiasmis commonly associated with Neurofibromatosis type1. A : Lesion in right optic nerve – right complete blindness B : Lesion in Optic chiasm – Bitemporal Hemianopia C:Lesion in optic tract – Incongruous Homonymous hemianopia D:Lesion of optic radiation in parietal lobe – Superior quadrantanopia E:Lesion in visual cortex – Congruous homonymous hemianopia sparing the macula. Lesion involving the tip of the visual cortex causes congruous homonymous macular defct. Pupillary reflexes are normal and optic atrophy do not occur following visual cortex lesion. PITUITARY TUMOURS CLINICAL ANATOMY: The pituitary gland is known as the master endocrine gland which is of a size of pea situated in a bony
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