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Ординатура / Офтальмология / Английские материалы / Surgical Atlas of Orbital Diseases_Mallajosyula_2009

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Preface

When I was a student, I often heard from my teachers saying "Proptosis is a Pandora's box". Surprisingly I continue to hear it even today! Many a time I am asked to speak on the topic titled "Proptosis is a Pandora's box", and I always change it to "Is Proptosis still a Pandora's box?" It was so, in the past, when the only imaging available was orbital venography. The information of the orbital disease process obtained with it was very meager. I salute my professor Dr. Vengala Rao, who used to perform orbitotomies in those days. It is the challenges he used to encounter, that stimulated me to take up this branch. Fortunately, advances in imaging techniques have made an immense contribution in the assessment of a case of proptosis, so that today, we know what we are dealing with. With careful clinical assessment, and knowledge in reading of CT/MRI, we can even arrive at the histopathological diagnosis of majority of cases. Hence, surprises are very few and far in between. Similarly, advances in histopathology and immunohistochemistry, anesthesia, chemo and radio therapy have made immense contributions in understanding and management of proptosis. The best example is Rhabdomyosarcoma. Today, nearly half the cases of proptosis can be managed by nonsurgical methods or with very minor surgical procedures. I thankfully acknowledge the roles of Dr Vengala Rao, my first teacher, Jack Rootman, Peter J Dolman, Brad Lemke and Mark J Lucarelli in furthering my understanding of orbital diseases.

The specialty of orbital diseases is very well advanced in North America and Europe, but not so in most other countries. Availability of ophthalmic literatures authored by orbital surgeons from the developing countries are very few. The idea of bringing this color atlas is to share two decades of my experience in orbital diseases. Though it is an atlas in principle, enough information is provided to understand the conditions and plan treatment strategies. It covers most of the common and some of the rare causes of proptosis. With color illustrations, and case presentations, I tried to make this book interesting to read and also to provide practical knowledge in clinical situations.

I thank all my contributing authors, who are internationally reputed, for their co-operation. The chapters they contributed were those in which they have a wealth of experience, viz. Jack Rootman on mesenchymal tumors, Peter J Dolman on thyroid associated orbitopathy (We rarely see such severe TAO in India ), Mark J Lucarelli on anatomy and fractures of orbit.

I hope my efforts help shatter the myth that "proptosis is a Pandora's box". If this book inspires at least some ophthalmologists to pursue this specialty with more interest and vigor and blossom into efficient orbital surgeons, the purpose of this book is served. I truly believe that "What I do, you can also do" and who knows you may do even better.

Subrahmanyam Mallajosyula

15-05-2008 Hyderabad

Acknowledgements

At the outset, I wish to acknowledge the significant roles played initially by my parents and then by my wife Rama, in my professional pursuits, which took a lot of time from my family and children Harsha and Aahlad. I greatly appreciate their cooperation.

My teacher Dr. Kotagiri Vengala Rao was the first to introduce me to orbital surgery during my postgraduation. I still relish those memories. I thank Dr. Jeffrey Nerad for introducing me to Dr. Jack Rootman. I acknowledge the role of Dr. Jack Rootman, Peter J Dolman in fine tuning my skills—both clinical and surgical. They are not only great teachers, but also wonderful human beings. My fellowship with them was made possible due to the financial assistance I received from them. I also thank the Orbis Inc. for awarding me the Ziegler's International Fellowship, which has part financed my fellowship at University of British Columbia, Vancouver. Similarly I thank the Association of Asian Indians in Ophthalmology for awarding the competitive fellowship, which financed my training with Mark J Lucarelli, Brad Lemke and Richard K Dortzbach at the University of Wisconsin, Madison. It was a great learning experience.

I thank all my contributing authors (and their supporting staff), who are all very eminent and highly reputed, for sparing their time to make this book wonderful. I thank my fellow Dr. Mohd Javed Ali, for all his assistance in proofreading. He is ever ready to help.

I wish to acknowledge the support and encouragement I received from Shri Jitendar P Vij, Chairman and Managing Director, Mr Tarun Duneja, Director (Publishing) and Mr PS Ghuman, Sr Production Manager of M/s Jaypee Brothers Medical Publishers (P) Ltd. I also thank Mr Upinder, Mr Pankaj, Mr Ram Murti and Mrs Seema Dogra of the same family (Jaypee) for their technical support.

Contents

Part One : Basic Concepts

 

1. Applied Anatomy of Orbit .................................................................................................................

03

Mark J Lucarelli, Nancy Kim

 

Orbital osteology 3; The periorbita 6; The orbital apex 6; The cavernous sinus 8; The globe 8; The extraocular muscles 9; Lids 10; The lacrimal system 13; The nerves of the orbit 14; Vascular anatomy of the orbit: Arterial supply 17; Vascular anatomy of the orbit: Venous outflow 19; Paranasal sinuses 20; Conclusion 20

2. Clinical Approach to Proptosis .........................................................................................................

23

Subrahmanyam Mallajosyula

 

Pain 23; Progression 25; Proptosis 28; Axial proptosis 29; Measurement of proptosis 35; Pulsations 37; Pupil 41; Perception of color vision 41; Prism bar-cover test (PBCT) 42; Periorbital changes 45; Lid changes 46; Conjunctival changes 48; Palpation 51; Auscultation 51; Evaluation of a case of proptosis 53

3. Imaging a Case of Proptosis: CT and MRI ....................................................................................

56

Subrahmanyam Mallajosyula, Ravi Varma

 

Evaluation of a CT scan of orbit 58; Common mistakes 59; Bony orbit 60; Eyeball 65; Enlarged extraocular muscle 69; Soft-tissue lesions 72; Lacrimal gland tumors 76; Cystic lesions of the orbit 78; Metastatic lesions 81; Contrast enhancement 83; 3-D reconstruction of the orbit 84

4. Role of Cytology in Orbital Lesions ................................................................................................

85

Geeta K Vemuganti, Anirban Bhaduri

 

Fine needle aspiration/sampling techniques 85; Intraoperative-operative diagnosis by squash and imprint cytology 85; Squash/imprint cytology 85; Case illustrations 86

5. Pathology of the Orbital Diseases ...................................................................................................

97

KS Ratnakar

 

Classification 97; Diagnosis of orbital tumors 98; Developmental lesions 98; Inflammatory lesions 100; Orbital infections 101; Cysticercosis 102; Neoplastic lesions 102; Benign tumors 103; Malignant tumors 104; Metastasis 105; Grave's disease 106; Mucocele 106

xvi Surgical Atlas of Orbital Diseases

Part Two : Disease Patterns of Proptosis

 

6. Thyroid-Associated Orbitopathy....................................................................................................

111

Peter J Dolman

 

Incidence and epidemiology 112; Risk factors and predictive variables 112; Pathogenesis 112; Course of disease 114; Clinical classification 114; The VISA classification 114; Vision/optic neuropathy 114; Inflammation/congestion 116; Strabismus/motility restriction 117; Appearance/exposure 117; General management guidelines 118

7. Orbital Infections ...............................................................................................................................

120

Shome debraj, Walinjkar Jaydeep, Mukherjee Angshuman

 

Risk factors 121; Etiological causes of orbital infections 121; Bacterial infections 121; Fungal infections 121; Parasitic infections 121; Protozoal infections 121; Diagnosis 121; Imaging studies 121; Emergency department care 122; Further inpatient care 122; Case illustrations 123

8. Orbital Inflammatory Disease ........................................................................................................

128

E Ravindra Mohan, Moupia Goswami, Vinathi Mutyala

 

Orbital amyloidosis 129; Sarcoidosis 130; Nonspecific orbital inflammatory syndrome (NSOIS) 131; Kimura’s disease 132; Wegener’s granulomatosis 132; Langerhan’s histiocytosis 133; Rosai-Dorfman disease 133; Orbital xanthogranuloma 134; Case illustrations 134

9. Orbital Lymphoma .............................................................................................................................

146

Christopher Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath

 

REAL classification 146; WHO classification of NHL 146; Modified Rye’s classification of Hodgkin’s lymphoma 147; When to suspect lymphoma 147; When to suspect idiopathic orbital inflammatory disease 148; Case illustrations 148

10. Vascular Lesions of Orbit ................................................................................................................

151

Subrahmanyam Mallajosyula, Mohd Javed Ali

 

Malformations 151; Lymphangioma 151; Orbital varices 152; Cavernous hemangioma 152; Other congenital malformations 152; Sturge-Weber syndrome 152; Wyburn-Mason syndrome 153; KlippelTrenaunay syndrome 153; Shunts 153; Carotid-Cavernous fistula 153; New growths 154; Capillary hemangioma 154; Hemangiopericytoma 155; Angiosarcoma 155; Kaposi’s sarcoma 155; Hemangioendothelioma 155; Hemangioblastoma 155; Case illustrations 157

11. Orbital Tumors of Neurological Origin........................................................................................

162

Christopher M Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath

 

Optic nerve glioma 162; Optic nerve meningioma 163; Orbital schwannoma (neurilemmoma) and neurofibroma 165; Case illustrations 166

12. Mesenchymal Tumors .......................................................................................................................

170

E Weis, J Rootman

 

Mesenchymal soft tissue tumors 170; Rhabdomyosarcoma 170; Rhabdomyoma 172; Leiomyoma 172; Leiomyosarcomas 172; Adipose tumors 172; Liposarcoma 174; Fibrous tissue tumors 174; Histiocytic tumors 175; Fibrous histiocytoma 175; Malignant tumors of uncertain type 175; Rhabdoid tumor 175

Contents

xvii

13. Bone Tumors of Orbit .......................................................................................................................

180

Venkatesh C Prabhakaran, Dinesh Selva

 

Clinical presentation 180; Osteoma 180; Fibrous dysplasia 181; Ossifying fibroma 182; Osteoblastoma 183; Chondroma 183; Cholesterol granuloma 184; Aneurysmal bone cyst 184; Giant cell lesions 184; Osteogenic sarcoma 184; Chondrosarcoma 186; Mesenchymal chondrosarcoma 186; Ewing’s sarcoma 186; Langerhan’s cell histiocytosis (LCH) 186; Intraosseous hemangioma 186

14. Tumors of Lacrimal Gland ...............................................................................................................

190

Raman Mittal

 

Classification 190; Epithelial cyst (Dacryops) 190; Case illustrations 191; Pleomorphic adenoma 191; Adenoid cystic carcinoma 194

15. Cystic lesions of Orbit ......................................................................................................................

199

Golam Haider, Subrahmanyam Mallajosyula, Mohd Javed Ali

 

Classification 199; Dermoid and epidermoid cysts 200; Teratomas 201; Cephalocele 201; Microphthalmos with cyst 202; Mucocele 202; Cysts of the optic nerve sheath 203; Hematic cyst 203; Simple cyst 204; Retention cyst 204; Lacrimal ductal cyst 204; Implantation cyst 205; Dacryocele 205

16. Parasitic Cysts of Orbit .....................................................................................................................

207

Subrahmanyam Mallajosyula, Mohd Ather, Modini Pandarpurkar

 

Cysticercosis 207; Case illustrations 208; Hydatid cyst of orbit 217

 

17. Orbital Fractures ................................................................................................................................

220

Alon Kahana, Mark J Lucarelli, Cat N Burkat, Richard K Dortzbach

 

Introduction 220; Anatomy 220; Imaging 226; Implant materials 227; General operative considerations

 

229; Pediatric patients 231; Timing of surgery 231; Decision: repair or not repair 232; Floor fractures 233;

 

Medial wall fractures 236; Lateral wall and zygomatico maxillary fractures 238; Late and secondary

 

fracture repair 238

 

18. Secondary and Metastatic Orbital Tumors .................................................................................

244

Kasturi Bhattacharjee, Harsha Bhattacharjee, Ganesh Kuri, Shyamanga Borooah

 

Orbital extension of intraocular tumors 244; Orbital extension of retinoblastoma 244; Orbital extension of

 

medulloepithelioma 246; Orbital extension of uveal melanoma 247; Orbital extension of lacrimal sac

 

tumors 250; Orbital extension of eyelid tumors 252; Basal cell carcinoma (BCC) 252; Sebaceous carcinoma

 

of the eyelid 253; Squamous cell carcinoma of the eyelid 255; Malignant melanoma of eyelid 256; Orbital

 

extension of intracranial tumors 257; Orbital extension of conjunctival tumors 258; Squamous cell

 

carcinoma of the conjunctiva 258; Malignant Melanoma of the conjunctiva 259; Orbital extension of

 

tumors of the nasal cavity and paranasal sinus 260; Orbital extension of nasopharyngeal tumor 262;

 

Metastatic orbital tumors 263

 

xviii Surgical Atlas of Orbital Diseases

Part Three : Management Strategies: Surgical

 

19. Decision Making ................................................................................................................................

271

Subrahmanyam Mallajosyula

 

Intraconal lesion 273; Reese-Berke’s incision 273; Steps of Reese-Berke approach 274; Steps of superior lidcrease incision 275; Apical conal lesions 279; Lesions of superior peripheral space 279; Thyroid associated orbitopathy 285

20. Orbitotomies ........................................................................................................................................

288

Ramesh Murthy, Anirban Bhaduri, Vikas Menon, Santosh G Honavar

 

General principles 288; Approaches 289; Anterior orbitotomy 289; Swinging lower eyelid flap 289; Lateral orbitotomy 290; Stallard-Wright lateral orbitotomy 290; Transfrontal orbitotomy 291; Complications 291; Postoperative management 291; Case illustrations 291

21. Multidisciplinary Approach to Proptosis ....................................................................................

299

Subrahmanyam Mallajosyula, B Ranganadha Reddy, M Chandrasekhar Reddy

 

Surgical anatomy 299; ENT approach to proptosis 300; Various etiological factors of proptosis in ENT 300; Sinus diseases causing proptosis 301; Purulent infections 301; Extensive nasal polyposis 301; Mucormycosis 301; Allergic fungal sinusitis 301; Frontoethmoidal mucocele 302; Tumors of paranasal sinuses causing proptosis 302; Fibrous dysplasia 302; Hemangiopericytoma 302; Juvenile nasopharyngeal angiofibroma 303; Squamous cell carcinoma 303; Rhabdomyosarcoma 303; Non-Hodgkin’s lymphoma 303; Esthesio-neuroblastoma 304; Caldwell-Luc operation 304; Lateral rhinotomy/medial maxillectomy 305; Total maxillectomy 306; Patterson’s operation 305; FESS 309; Neurosurgical approaches of proptosis 309; Transcranial approach 311; Extracranial approach 309; Case illustrations 313

22. Orbital Exenteration ..........................................................................................................................

318

Ramesh Murthy, Anirban Bhaduri, Sima Das, Santosh G Honavar

 

Indications 318; Patient preparation 318; Surgical procedure 318; Types 319; Management of the exenterated socket 320; Prosthesis 320; Complications of exenteration 320; Case illustrations 321

23. Orbital Prosthesis...............................................................................................................................

327

Kuldeep Raizada

 

Types of prosthesis 327; Complete prosthesis 327; Factors that affect the fit of an orbital prosthesis 328; Preparation of the patient 328; Impression of the orbital defect 328; Casting 329; Sculpting 329; Moulding 330; Using the desired material 331; Fabrications of ocular prosthesis 331; Assemble of prosthesis 331; Care of your prosthesis 331; Storing the prosthesis 332; Preventing mishaps 333

Part Four : Management Strategies: Nonsurgical

 

24. Medical Management of Proptosis ................................................................................................

337

Subrahmanyam Mallajosyula, Mohd Javed Ali

 

Nonspecific inflammations of the orbit (NSOIS) 337; Specific inflammations of the orbit 338; Orbital cellulitis 338; Rhino-orbital mucormycosis 338; Chronic granulomatous infections 338; Parasitic infestations 338; TolosaHunt syndrome 339; Capillary hemangioma 339; Acute intraorbital hemorrhage and emphysema 340; lymphoprolifarative and other neoplastic lesions 340; Case illustrations 340

Contents xix

25.Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy .. 344

Vijay Anand P Reddy, Nitin More, Ramesh Murthy, Anirban Bhaduri, Santosh G Honavar

Introduction 344; Ionizing radiation 344; Radiation therapy delivery methods 344; External beam radiation (teletherapy) 344; Internal radiation therapy (brachy therapy) 345; Plaque radiotherapy 346; Principles of anti-neoplastic therapy 347; Capillary hemangioma 348; Basal cell and squamous cell carcinoma 348; Tumors of lacrimal gland 348; Malignant conjunctival tumors 349; Intraocular lymphoma 349; Retinoblastoma 349; Choroidal melanomas 349; Chemoreduction regimen 350; Oculor metastasis 352; Rhabdomyosarcoma 352; Orbital lymphoma 352; Grave’s ophthalmopathy 353; Optic nerve glioma 353; Optic nerve meningioma 353; Sequelae of radiation therapy 353

26. Carotid-Cavernous Fistulae: Role of Interventional Radiologist ..........................................

356

D Ravi Varma, D Radhika Varma

 

Pathophysiology 357; Clinical features 357; When to suspect CCF 359; Radiological investigations 359; Management of CCF 360; Direct CCF 360; Indirect CCF 363; Prognosis 363

27. Ocular and Systemic Associations of Proptosis .........................................................................

366

Subrahmanyam Mallajosyula, Mohd Javed Ali

 

Capillary hemangiomas 366; Neurofibromatosis 366; Craniofacial dysostosis 367; Encephalocele 367; Wegener’s granulomatosis 367; Wyburn-Mason syndrome 367; Hurler’s syndrome 368; Nonspecific orbital inflammation syndrome 368; Sclerosing inflammation of the orbit 368; Osteoma 368; Orbital hamartoma (tuberous sclerosis) 368; Hemangioblastom 368

Index .....................................................................................................................................................

369