Orbital imaging

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Call: within the US and Canada: 800-401-9962; outside the US and Canada: +1-314-447-8200 ORBITAL IMAGING F Allan Midyett, MD, DABR Neuroradiologist Department of Radiology Howard University Hospital Washington, D.C Suresh K Mukherji, MD, MBA, FACR Professor and Chairman Walter F Patenge Endowed Chair Department of Radiology Michigan State University East Lansing, Michigan 1600 John F Kennedy Blvd Ste 1800 Philadelphia, PA 19103-2899 ORBITAL IMAGING ISBN: 978-0-323-34037-3 Copyright © 2015 by Saunders, an imprint of Elsevier Inc No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein) Notices Knowledge and best practice in this field are constantly changing As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein Library of Congress Cataloging-in-Publication Data Midyett, F Allan, author Orbital imaging / F Allan Midyett, Suresh K Mukherji p ; cm Includes bibliographical references and index ISBN 978-0-323-34037-3 (pbk : alk paper) I Mukherji, Suresh K., author II Title [DNLM: Orbital Diseases—diagnosis Diagnostic Imaging Orbit—injuries WW 202] RE711 617.7’807572—dc23 2014038261 Content Strategist: Helene T Caprari Content Development Specialist: Amy Meros Publishing Services Manager: Catherine Jackson Senior Project Manager: Carol O’Connell Design Direction: Brian Salisbury Printed in the United States of America Last digit is the print number:  9  8  7  6  5  4  3  2  First, this book is dedicated to my children, Scott, Laura, and Brian, who probably never understood why I spent so much time on radiology Then, to my parents, who were convinced that all those years of education and training would eventually bear fruit Then, to all those patients with cancer who will probably never understand the important role radiologists and ophthalmologists play in early diagnosis and proper treatment of their very personal disease Then, to my faithful friends Cooper and Vana (the Vizslas) who unequivocally never understand why I waste “valuable” time sitting at the computer when I could be playing with them And last but not least, to my darling wife Diane, who always understands each and every day how very how much this book means to me Preface Orbital Imaging was created to serve as a handy reference and learning text It was meant to answer questions created by that “funny eye case” and to whet the reader’s appetite for more It was envisioned as a book that the reader could start in the airport and read all the way through or start in middle and read a single chapter Or better yet, read all the way through during the flight and then come back to relook at a certain chapter when the reader encounters that unusual orbital case I wanted to present pertinent orbital pathologies that included those entities we see every day, those entities we see mostly on board examinations, and some entities that are rare as hen’s teeth but some say we are “supposed to know” when we see them I wanted to give the reader a complete, although “thumbnail,” sketch in a format that hopefully could be easily remembered I wanted to package the individual components in a logical fashion so that the reader could first and foremost quickly find the section he or she is looking for But even more important I wanted the reader to be able to quickly identify the section not wanted to be reviewed at any particular time I wanted to depict those images that best demonstrated the pathologic entity with the best imaging sequences Some of these cases are truly “rare birds,” and we brought you the best images we had available But imagine the esthesioneuroblastoma, an entity that has only been reported 1000 times since it was first described in 1924 by Berger and Luc We had five cases to choose from, and we show four in this text iv As my interest in correlation between radiologic imaging and gross pathology runs long and deep, I couldn’t resist the opportunity to share a few gross pathology images with the reader, in the hope that this correlation cements the image in your psyche The orbit has a rich and long history dating back hundreds and even thousands of years The subsection “Historic Highlights” hopefully will be one that will be of interest to many when making a casual read through When the book is used as a quick reference for that eye case that has to be discussed with the clinician right now, this is one of the first sections to skip The differential section has been written with great care I have tried to find contrasting findings to help you tell the difference, if these points can be found Sure, some of these are really “Aunt Minnies” and if they are, we say so But some of these differentials are really tough, and I believe this section is potentially helpful, imparting wisdom usually found from our prized professors Well, we did get a lot of it from them But most of all, I wanted to make this textbook one that readers would enjoy reading and find that certain sections just stuck in their minds Yes, in some ways this text takes a simplistic approach You will find the statistics and the nomenclature have been rounded off To the big book purists, I apologize To those of you who seek the sleeker approach, I hope you learn something, find something to remember, and enjoy reading it! F Allan Midyett, MD Acknowledgments As I look back on a long and exciting career in radiology, I always remember first and foremost the impressive radiology showman David S Carroll, who caused me to say “Wow, I can’t believe he can read all that from the radiograph!” And then I just had to go into radiology Then I learned that Dr Carroll really was a great radiologist, but he was a fantastic showman! While Dr Carroll’s list of accomplishments was longer than this acknowledgement, it was his showmanship that caused me and others to go into radiology And then I settled down to learn under the watchful tutelage of C Allen Good No one ever accused Dr Good of being a showman, just as no one doubted he was an exceptional radiologist Dr Good set superb standards for the practice of radiology both at the Mayo Clinic and for the entire country during his more than a quarter century leading the American Board of Radiology as its president and secretary My mentors are TNTC (Too Numerous to Count) Many, like Bob Scanlon, Dave Reese, Colin Holman, Hillier L “Bud” Baker, and others, have relocated and are currently watching over us from on high And to top all this, I had the fabulous opportunity to my neuroradiology fellowship with Mauricio Castillo and Suresh Mukherji, and I caught a glimpse of academic neuroradiology like I never saw it before Their level of expertise blew my mind, and in the process I developed a love for head and neck radiology, and specifically orbital imaging Suresh Mukherji has been an incredible mentor for me in my fellowship, and has proven to be an invaluable co-author of Orbital Imaging I would like to thank the fabulous folks at Elsevier, including (but not limited to) Helene Caprari, Amy Meros, Kathryn DeFrancesco, and Carol O’Connell And while my almost half century in radiology has taught me that we can’t all be like Dave Carroll and dazzle people all the time, I will submit that these are some of my most memorable moments in radiology I hope you enjoy the book and go out and dazzle someone sometime with what you have learned from Orbital Imaging F Allan Midyett, MD v CONTENTS • PART I TRAUMA AND SURGERY MEDIAL B LOWOUT FRACTURE, BLOWOUT ORBITAL FLOOR FRACTURE, ORBITAL ExENT ERAT 10 , ORBITAL FLOOR MESH, ANTERIOR CHAMBER P ERFORATION, OCULAR LENS DISPLACEMENT, R UPTURED GLOBE, PHTHISIS BULBI, O CULAR PROSTHESIS, 14 76 19 R£TIN OBLASTOMA, 20 ORBITAL L EUKEMI A, 21 OPTIC NERVE METASTASIS, 87 22 E sTHESIONEUROBLASTOMA, 91 23 RHA8DOMYOSARCOMA, • PART IV 80 99 18 20 CONGENITAL 23 26 29 CoLOBOMA, 25 P ERSIST ENT H YPERPLASTIC PRIMARY VITREO US, 32 10 R ETINAL DETAC HMENT , • PART II 35 BENIGN TUMORS 112 26 CONGENITAL ORBITAL TERAT0.\1.A, 27 CONGENITAL A.N OPHTHALMIA, • PART V 116 121 VASCULAR 11 ORBITAL CAVERl ous HEMANG IOMA, 12 OPTIC NERVE GLIOMA, 13 OPTIC 14 POSTERIOR ORBITAL DERMOIDS, 15 ORBITAL LIPOMA, 16 ORBITAL SCHWAN OM A, 44 TERVE MENI NG IOMA, 105 24 48 54 41 28 CAROTID-CAVERNOUS FISTULA, 29 CAVERNOUS SINUS TH ROMBOSIS, 30 ORBITAL VEN OUS VA RIX, 31 VE ous L YMPHATIC M ALFORMATION, • PART VI 127 133 139 143 57 59 DEGENERATIVE • PART III 32 PosTERIOR O cuLAR STAPHYLOMA, MALIGNANT TUMORS 33 CATARACTS: B EFORE AND AFTER, 65 34 OPTIC Di sc DRUSEN, 17 O CULAR AnNEXAL L YM PHOM A, 18 OcuLARMELANOMA, vi 71 156 149 153 CONTENTS • PART VII • MUSCLE CONE INFLAMMATORY 35 GRAVES 0RBITOPATHY, 36 IDJOPATHIC ORBITAL PsEUDOTUMOR, • PART X 161 166 207 44 ORBITAL ABSCESS, 45 CYTOMEGALOVIRUS RETINITIS, 46 PoTT's PuFFv T uMoR, • PART XI 211 215 PART VIII OPTIC PATHWAY 37 OPTIC NERVE EURITIS, 175 UNCERTAIN ETIOLOGY 38 OPTIC NERVE LEUKEMIA, 178 47 PswDoTUMOR CEREBRJ, 48 WEGENER GRA1 U LOMATOSlS, • PART XII • PART IX LACRIMAL GLAND 39 LACRIMAL GLAND SARCOIDOSIS, 40 LACRIMAL GLAND L YM PHOMA, 185 188 BONY ORBIT 49 ORB ITAL PLASMACYTOMA AND MYELOMA, 41 LACRIMAL GLAND DERMOID, 42 ADENOID CYSTIC CARCINOMA 19 50 OF THE LACRIMAL GLAND, 43 223 194 SQUAMOUS CELL CARCINOMA OF THE LACRIMAL SAC, 200 233 FrnRous DvsP LASIA, ABBREVIATIONS 247 241 22 Vii PA RT I TRAUMA AND SURGERY PART OUTLINE Medial Blowout Fracture Blowout Orbital Floor Fracture Orbital Exenteration 14 Orbital Floor Mesh 18 Anterior Chamber Perforation 20 Ocular Lens Displacement 23 Ruptured Globe 26 Phthisis Bulbi 29 Ocular Prosthesis 32 10 Retinal Detachment 35 49  Orbital Plasmacytoma and Myeloma Most orbital plasmacytomas are SBPs and arise in bone with demonstrable bone destruction The rare EMP arises outside of and may not involve bone This tiny subset could be problematic when comparing with EOOM • EOOM morphology varies from a welldefined oval to infiltrating lesions • EOOM usually enhances; thus finding other foci of metastasis is habitually helpful in making the diagnosis of metastasis • Uveal metastases are bilateral in approximately 25% • Orbital metastasis are relatively uncommon and found in fewer than 1% of patients with metastatic disease • 35% to 50% of patients with symptomatic orbital metastases experience orbital symptoms before local symptoms from primary • Most patients with orbital metastasis present with diplopia or pain Most people with orbital plasmacytomas present with painless proptosis, diplopia, blurred vision, and/ or decreased visual acuity • Sclerotic metastasis may come from prostate (most common), breast (may be mixed), transitional cell carcinoma, carcinoid, medulloblastoma, neuroblastoma, mucinous adenocarcinoma, and lymphoma • Imaging patterns from orbital metastasis include intraconal diffuse, muscle only, intraconal focal, extraconal, bone mass, and others • EOOM and orbital plasmacytoma are both more frequently seen in the superior lateral extraconal quadrant No help here Orbital Fibrous Dysplasia • Fibrous dysplasia (FD) is more prevalent in children and young adults 75% of patients present at younger than 30 years of age with peak incidence from to 15 years Mean age for orbital plasmacytoma presentation is 50 to 70 years • FD comes in three basic varieties: sclerotic (35%), lytic (25%), and mixed (40%) • Sclerotic lesions predominate in the skull base with lytic lesions least common in orbital fibrous dysplasia (OFD) • Classic “ground glass” appearance in OFD Not a feature of orbital plasmacytoma • T1 Gd shows avid enhancement with OFD T1 Gd is variable with orbital plasmacytoma Often “significant contrast enhancement with central inhomogeneity.” • OFD lesions are well circumscribed but not encapsulated Orbital plasmacytomas vary from 237 somewhat circumscribed (see Figure 49-1, A and B) to obviously invasive (see Figure 49-3, B and C) • OFD may show bubbling/blistering cystic lesions Orbital plasmacytoma may progress to erosion, expansion, and destruction of bone cortex with thick peripheral ridging causing a characteristic “soap bubble” appearance • OFD commonly crosses sutures Orbital plasmacytoma can cross most anything in its path (see Figure 49-3) • OFD has no recognized gender preference Plasmacytoma male-to-female preference is 3:1 • OFD has cysts with hemorrhage Not a feature of orbital plasmacytoma • See Chapter 50: Fibrous Dysplasia 10 Hyperparathyroidism • Although hyperparathyroidism (HPT) can cause an orbital lesion that is confusing for plasmacytoma, looking at the specific radiologic features of the lesion and those available in other sites should solve the problem • “Brown tumors” of the orbit are rare but have been reported.15 • HPT may show “ground glass” appearance with mottled demineralization Not a feature of orbital plasmacytoma • HPT has subperiosteal resorption of bone, classically affecting the radial aspects of the proximal and middle phalanges of second and third fingers First described by David Pugh in 1951 Not a feature of orbital plasmacytoma.16 • HPT usually shows multiple skull defects with diffuse trabecular resorption giving granular “salt and pepper” appearance There is loss of distinction between inner and outer tables • MM may feature well-defined, “punchedout” lytic lesions in the skull (see Figure 49-2) • HPT can cause indistinct cortical margins of vascular grooves Not a feature of orbital plasmacytoma • Brown tumor of HPT is rare but can cause focal bone destruction and could be problematic in the differential diagnosis of SBP and MM, but not EMP 11 Intraosseous Meningioma • Most meningiomas are primarily extradiploic and can cause cortical thickening but they not have an intradiploic epicenter 238 PART XII  Bony Orbit • The exception is the very rare intraosseous meningioma, which could be problematic when appearing similar to an SBP • Intraosseous meningioma is a rare tumor accounting for fewer than 1% of all bone tumors • Intraosseous meningioma shows uniform avid enhancement on T1 Gd SBP shows mild contrast enhancement with central inhomogeneity varying with the amount of calcification/ossification present and correlating with that seen on CT.13 • Intraosseous meningiomas are 65% osteosclerotic, 35% osteolytic A CLOSER LOOK • The most common form of orbital plasmacytoma is a solitary soft tissue mass arising from and destroying bone.4 • Bilateral orbital plasmacytomas tend to be EMPs and not destroy bone They have a better prognosis than SBPs.11 • Two thirds of MM cases are positive for Bence Jones proteins in urine.17 Fast Facts • EMPs represent fewer than 1% of all head and neck malignancies.18 • And yet this is the area where they occur primarily! • Primary bone tumors constitute 2% or fewer of all orbital tumors.19 • 75% of EMPs occur in males • Median age for presentation of EMP is 55, 10 years younger than for MM Historic Highlights • 1847 and 1848: English physician Henry Bence Jones described and published his discovery of a protein associated with diffuse bone pain, proteinuria, and plasma dyscrasia.12,20,21 • 1873: Rustizky recognized MM as a distinct histopathologic entity.20 • Orbital involvement by MM is rare with fewer than 50 cases reported in the world literature by 1981.4,5 REFERENCES Montanˇés AU, Blanco G, Saornil MA, et al: Extramedullary plasmacytoma of the orbit, Acta Ophthalmol Scand 78:601–603, 2000 Nigel R, Seymour B, Jennifer R, et al: Solitary osseous plasmacytoma of the orbit with amyloidosis, Ophthal Plast Reconstr Surg 23:79–80, 2007 Golden N, Niryana W, Saputra H, et al: Solitary osseous plasmacytoma of the orbit with multiple myeloma: a case report, Neurol Asia 14(2):171–174, 2009 Jackson A, Kwartz J, Noble JL, et al: Multiple myeloma presenting as bilateral orbital masses: CT and MR appearances, Br J Radiol 66:181–183, 1993 Gonnering RS: Bilateral primary extramedullary orbital plasmacytomas, Ophthalmology 94(3):267–270, 1987 Ockrim ZK, Deutch J, Izon J: Bilateral retro-orbital plasmacytoma, Eye 14:795–797, 2000 Malik A, Narang S, Handa U, et al: Multiple myeloma presenting as bilateral orbital proptosis, Indian J Ophthalmol 57(5):393–395, 2009 Dufier JL, Trecan G, Thomas M, et al: Malignant bilateral exophthalmia as a manifestation of Kahler’s disease, Bull Soc Ophthalmol (Paris) 77:15–17, 1977 Hayes JG, Petersen M, Kakulas BA: Multiple myeloma with bilateral orbital infiltration and polyneuropathy, Med J Aust 2:276–277, 1980 10 Lim J, Yoo SW, Choi KH, et al: Extramedullary plasmacytoma of the lacrimal sac and duct invading the inferior meatus: an extremely rare occurrence, J Rhinol 19:80–82, 2012 11 Uceda-Montanes A, Blanco G, Saornil MA, et al: Extramedullary plasmacytoma of the orbit, Acta Ophthalmol Scand 78:601–603, 2000 12 Ooi GC, Chim JC-S, Au W-Y, et al: Radiologic manifestations of primary solitary extramedullary and multiple solitary plasmacytomas, AJR Am J Roentgenol 189(3):821–827, 2006 13 Selvakumar A, Noronha V, Sundaram PM: Atlas of imaging in ophthalmology, New Delhi, 2014, Jaypee Brothers, p 240 14 Chattapadhayay S, Saha A, Mukherjee A, et al: Extramedullary plasmacytoma of the orbit: a rare case, Clin Cancer Invest J 2:163–165, 2013 15 Naiman J, Green WR, d’Heurle D, et al: Brown tumor of the orbit associated with primary hyperparathyroidism, Am J Ophthalmol 90(4):565–571, 1980 16 Pugh DG: Subperiosteal resorption of bone: a roentgenologic manifestation of primary hyperparathyroidism and renal osteodystrophy, Am J Roentgenol Radium Ther 66(4):577–586, 1951 17 Hoffbrand AV, Moss P, Pettit J: Essential hematology, ed 5, New York, 2001, Wiley-Blackwell 18 Miller FR, Lavertu P, Wanamaker JR, et al: Plasmacytomas of the head and neck, Otolaryngol Head Neck Surg 19:614–618, 1998 19 Selva D, White VA, O’Connell JX, et al: Primary bone tumors of orbit, Surv Ophthalmol 49:328–342, 2004 20 Ching ASC, Khoo JB-K, Chong VF-H: CT and MR imaging of solitary extramedullary plasmacytoma of the nasal tract, AJNR Am J Neuroradiol 23:1632–1636, 2002 21 Jones HB On a new substance occurring in the urine of a patient with mollities ossium, Philos Trans R Soc 138:55–62, 1848 49  Orbital Plasmacytoma and Myeloma A B C D 239 FIGURE 49-1  ​n ​A, T1 axial image shows homogeneous fusiform abnormality along lateral aspect of left orbital wall isointense to EOM but clearly hypointense to orbital fat, displacing adjacent structures and causing exopthalmos Indistinct orbital cortex suggests bony involvement B, Axial T2 confirms findings on T1 and shows homogeneous soft tissue mass isointense to EOMs and cerebral cortex C, Axial DWI shows bright restricted diffusion corresponding to the lesion portrayed on T1 and T2 images above D, Axial ADC map shows low signal “ADC black hole” corresponding to abnormality involving left orbit demonstrated to be bright on DWI (C) 240 PART XII  Bony Orbit FIGURE 49-2  ​n ​Clear case of multiple “punched-out” lytic lesions depicted by lateral skull radiograph characteristic of MM If present, this finding can clinch the diagnosis in an otherwise complex case A B C FIGURE 49-3  ​n ​A, T1 axial show huge homogeneous, mass with “grapefruit-like” septations, destroying bone and displacing brain with intracranial mass effect and edema Clearly this tumor should have been diagnosed and treated earlier B, Axial T2 through orbits shows very complex heterogeneous abnormality filling right orbit and displacing and invading adjacent structures C, Axial T2 shows huge heterogeneous complex mass extending into anterior cranial fossa with mass effect causing considerable cerebral edema Complex heterogeneous pattern corresponds to calcific/ossific features CHAPTER 50 Fibrous Dysplasia KEY POINTS • Definition: Fibrous dysplasia (FD) is typically a benign slowly progressing tumorlike process involving bone maturation in which normal cancellous bone is replaced by immature woven bone and fibrous tissue Its predilection for the skull can cause serious orbital problems • Synonym: Lichtenstein-Jaffe disease.1 • Classic clue: Preadolescent patient presents with painless proptosis and diplopia caused by an expanding enhancing sphenoid wing mass showing intact bony cortex IMAGING • FD comes in three basic varieties: sclerotic (35%), lytic (25%), and mixed (40%) • Sclerotic lesions predominate in the cranial base with lytic lesions least common.2,3 • FD’s trademark is medullary cavity expansion preserving original bone shape while sparing cortex • A “ground glass” appearance is classic in FD However, cystic degeneration is a “red flag,” having a high association with visual impairment.2 • Although the cortex is classically preserved, severe expansion may cause cortical crevices or even “vanishing cortex” (see Figure 50-4) • Periosteal reaction suggests pathologic fracture or malignant modification.4 • Lytic lesions suggest malignant degeneration, particularly when observed to progress on imaging.2,5 Computed Tomography Features • Ground glass appearance is classic in the orbit (see Figure 50-1, D) • Expands bone, keeping original shape but sparing cortex (see Figure 50-3) • When not classic “ground glass” appearance may be quite heterogeneous (see Figure 50-3) • Ophthalmologists and neurosurgeons find three-dimensional computed tomography useful for surgical planning and “road mapping.” Magnetic Resonance Imaging Features • T1 is heterogeneous low to intermediate signal (isointense to brain on Figure 50-2, A and C) • T2 is heterogeneous, usually low (isointense to brain on Figure 50-2, B and D) but may have regions of higher signal (see Figure 50-4, B) • T1 gadolinium (Gd) shows avid enhancement • Cysts and hemorrhages can cause heterogeneous signals that are well demonstrated by magnetic resonance imaging and vary depending on protein content and age of hemorrhage Plain Films • Classically shows ground glass appearance (see Figure 50-1, A and B) • May be sclerotic or cystic • Lesions are well circumscribed but not encapsulated • Commonly cross sutures (see Figure 50-1, D) • Often shows bubbling/blistering cystic lesions Nuclear Radiology • Increased uptake on technetium-99m bone scans (see Figure 50-1, C) CLINICAL ISSUES Presentation • May present with proptosis, diplopia, globe displacement, decreased extraocular motility, visual impairment, or overlying soft tissue swelling.2,6 241 242 PART XII  Bony Orbit Epidemiology • FD is more prevalent in children and young adults • 75% of patients present at younger than 30 years with peak incidence from to 15 years • There is no recognized gender preference.7 • FD’s cause continues to be unknown but current theories favor developmental over traumatic and neoplastic etiologies.4 Treatment • Almost 75 years after Dandy first attempted a transcranial surgical solution, the pathoetiologic basis and treatment indications for FD’s visual impairment continue to be controversial.2,8 • Many advocate surgical decompression when the optic nerve (ON) is “markedly” compromised However, postoperative vision loss can occur following meticulous surgical technique • Conservative management is preferred unless significant functional impairment or neurologic deficits develop.4 • Cosmetic surgery usually postponed until lesions stabilize.4 • Orbital fibrous dysplasia (OFD) is rarely limited to orbit and usually requires neurosurgery • Medical management may suggest the short-term use of systemic steroids.2 Prognosis • About a quarter of lesions recur within to years following surgery.4 • Iatrogenic etiologies conceivably comprise the most common cause of vision loss in FD.2 • Radiation therapy is currently contraindicated with FD because of an associated 44% incidence of malignant transformation.9,10 PATHOLOGY • Grossly, the involved bone marrow cavity is filled with whitish abnormal appearing fibrous tissue • Microscopically, the bony matrix consists of small irregularly shaped trabecula of immature, inadequately mineralized bone • The irregular shapes shown by bony trabecula have been compared with “Chinese characters.”4 • FD is not encapsulated • Cortical bone is typically spared with FD, although cortex can sometimes be thinned and transgressed by the abnormal medullary cavity expansion • Malignant transformation occurs in 0.5%, most often after radiation therapy • May give rise to osteosarcomas, fibrosarcomas, and chondrosarcomas.4 • Cystic degeneration of tumor often a more frequent cause of symptoms than impingement on optic canal and ON.2 DIFFERENTIAL DIAGNOSIS Paget Disease • First described by Sir James Paget in 1877 • Paget disease involves inner and outer table FD widens diploic space, displacing outer table, and spares inner table • Facial bone involvement common with FD but uncommon with Paget disease.2,5 • Begins in frontal or occipital regions initially, sparing skull vertex • Well-defined lytic lesions lack sclerotic margins • Soft bone promotes basilar invagination • Paget healing phase causes “cotton-wool” appearance.11 Hyperparathyroidism • May show ground glass appearance with mottled demineralization • Indistinct cortical margins of vascular grooves • “Brown tumor” is rare but can cause focal destruction • Usually shows multiple skull defects.11 • Look for subperiosteal absorption in hand radiographs Neurofibromatosis • Bony orbital deformity in patients with neurofibromatosis usually occurs in association with plexiform neurofibroma (PNF) or optic nerve glioma (ONG).12 • Although bony changes may be confusing for FD, the associated PNF or ONG should clarify the diagnosis See Chapter 12: Optic Nerve Glioma Intraosseous Meningioma • Orbital fibrous dysplasia (OFD) is primarily intradiploic, only occasionally breaking through cortex to extend beyond 50  Fibrous Dysplasia • Most meningiomas are primarily extradiploic and can cause cortical thickening but they not have an epicenter inside bone • The exception is the very rare intraosseous meningioma, which could possibly be problematic, appearing very similar to FD • Intraosseous meningioma accounts for fewer than 1% of all bone tumors • T1 isointense to gray matter • T2 most isointense to gray matter (some hyperintense) • T1 Gd uniform avid enhancement • May cause proptosis • 65% osteosclerotic, 35% osteolytic.13 Sclerotic Metastasis • Sclerotic or blastic bony metastasis • Orbital metastasis relatively uncommon, found in fewer than 1% of patients with metastatic disease • 35% to 50% of patients with symptomatic orbital metastases experience orbital symptoms before local symptoms from primary tumor • Most patients who have orbital metastasis present with diplopia or pain Most people with primary orbital tumors present with proptosis and/or visual loss.14 • Sclerotic metastasis may come from prostate (most common), breast (may be mixed), transitional cell carcinoma, carcinoid, medulloblastoma, neuroblastoma, mucinous adenocarcinoma, and lymphoma • Imaging patterns from orbital metastasis include: intraconal diffuse, muscle only, intraconal focal, extraconal, bone with soft tissue mass, and others.14 A CLOSER LOOK • Craniofacial FD is one of four FD types, which are characterized by involvement of skull and facial bones • FD is a nonfamilial bone disorder whose prevalence estimates range from “not uncommon” through “relatively uncommon,” “relatively rare,” to “rare.”2,3,9 • Looking past the subset of craniofacial FD to focus on OFD, the prevalence is best described as “rare.” • FD has a predilection for sphenoid, ethmoid, frontal, and maxillary bones.2,15 • OFD’s definitive diagnosis is based on clinical, radiologic, and histologic data • Visual impairment is the most common, most debilitating, and most feared neurologic complication of FD.2,10 243 Fast Facts • FD comprises 2.5% of all bone tumors • FD comprises 7.5% of benign bone tumors Historic Highlights • 1891: von Recklinghausen first described lesions of FD • 1938: Lichtenstein recognized the condition as a distinct entity and named it FD • 1941: Dandy published his classic monograph on orbital tumors describing the first transcranial approach for removal of OFD.2,8 • 1936 and 1937: McCune and Albright independently recognized the frequent correlation between FD and coexisting endocrinopathy.2,16,17 • The triad of polyostotic FD, cutaneous pigmentation (café-au-lait spots), and precocious puberty was subsequently dubbed McCune-Albright syndrome.2,16,17 • FD has been found in seventh century AngloSaxon skulls and in a Tennessee skull from 1480.9,18,19 REFERENCES Dähnert W: Radiology review manual, ed 3, Baltimore, 1996, Williams & Wilkins, pp 54–56 Albright F, Butler MA, Hampton AO, et al: Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precocious puberty in females, N Engl J Med 216:727–746, 1937 Bibby K, McFadzean R: Fibrous dysplasia of the orbit, Br J Ophthalmol 78:266–270, 1994 Dumont AS, Boulos PT, Jane JA, et al: Cranio-orbital fibrous dysplasia: with emphasis on visual impairment and current surgical management, Neurosurgery Focus 10:E6, 2001 Jacquemin C, Bosley TM, Svedberg H: Orbit deformities in craniofacial neurofibromatosis type 1, AJNR Am J Neuroradiol 24:1678–1682, 2003 Wells C: Polyostotic fibrous dysplasia in a 7th century Anglo-Saxon, Br J Radiol 36:925–926, 1963 McCune DJ: Osteitis fibrosa cystic: the case of a nine year old girl who also exhibits precocious puberty, multiple pigmentations of the skin and hyperthyroidism, Am J Dis Child 52:743, 1936 Tokgoz N, Oner YA, Kaymaz M, et al: Primary intraosseous meningioma: CT and MRI appearance, AJNR Am J Neuroradiol 26:2053–2056, 2005 Char DH, Miller T, Kroll S: Orbital metastases: diagnosis and course, Br J Ophthalmol 81:386–390, 1997 10 Moore AT, Buncic JR, Munro IR: Fibrous dysplasia of the orbit in childhood Clinical features and management, Ophthalmology 92:12–20, 1985 11 Larheim TA, Westesson P: Maxillofacial imaging, Berlin, 2008, Springer Verlag 12 Ricalde P, Horswell BB: Craniofacial fibrous dysplasia of the fronto-orbital region: a case series and literature review, J Oral Maxillofac Surg 59:157–168, 2001 244 PART XII  Bony Orbit 13 Michael CB, Lee AB, Patrinely JR, et al: Visual loss associated with fibrous dysplasia of the anterior skull base Case report and review of the literature, J Neurosurg 350–354, 2000 14 Gregg JB, Reed A: Monostotic fibrous dysplasia in the temporal bone: a late prehistoric occurrence, Am J Phys Anthropol 52:587–593, 1980 15 Lufkin R, Borges A, Villablanca P: Teaching atlas of head and neck imaging, New York, 2000, Thieme, pp 13–17 16 Doran SE, Gebarski SS, Hoff JT: Tumors of the skull In Youmans JR, editor: Neurological surgery, vol 4, ed 4, Philadelphia, 1996, WB Saunders, pp 2998 –3023 A C 17 Zee CS: Neuroradiology: a study guide, New York, 1996, McGraw-Hill, pp 2–3 18 Sassin JF, Rosenberg RN: Neurological complications of fibrous dysplasia of the skull, Arch Neurol 18:363– 369, 1968 19 Dandy WE: Orbital tumors: results following the transcranial operative attack, New York, 1941, Piest, pp 3–7 B D FIGURE 50-1  ​n ​A, AP radiograph shows sclerosis and distortion of bony architecture superior and medial to left orbit B, Lateral orbit shows dramatic increase in anterior skull base density C, Frontal image of technetium-99m bone scan shows increased uptake confirming activity in abnormality shown on plain films D, Axial nonenhanced computed tomography image through the anterior skull base and orbits shows a conspicuous homogeneous ground glass appearance involving the left skull base/orbital roof, crossing the suture anteriorly to involve the contralateral right frontal bone and crossing the suture posteriorly into the lesser sphenoid wing to involve left anterior clinoid 50  Fibrous Dysplasia A C 245 B D E FIGURE 50-2  ​n ​Axial images show mass protruding into medial right orbit The mass is relatively isointense to brain on T1 (A) and T2 (B) Sagittal images (C and D) show a well-circumscribed mass within marrow of orbital roof relatively isointense to brain on T1 (C) and T2 (D) Some FD subtly compromises superior cortex (black line) into anterior cranial fossa on image (D) E, Coronal T2 shows FD medial to and extending into right orbit, mildly hyperintense to brain 246 PART XII  Bony Orbit FIGURE 50-3  ​n ​Coronal NECT shows expansion of right greater wing’s orbital plate by heterogeneous increased attenuation, which enlarges bone retaining general shape and leaving cortex intact Mild asymmetric enlargement of right lesser sphenoid wing A B FIGURE 50-4  ​n ​A, Axial orbital NECT shows proptosis caused by large bony mass with ground glass appearance involving orbit and adjacent sinuses, displacing globe medially Conspicuous cystic low attenuation area anteriorly The mass extends into the contralateral ethmoid sinus B, Coronal NECT shows large complex bony lesion involving orbit, sinuses, nasal cavity, and anterior cranial fossa Cortex appears to be “vanishing” with only scant residual traces remaining Abbreviations Abbreviations listed here reference those used within this publication and not claim to be authoritative outside of this context Our choice was to use commonly used abbreviations that overlapped versus infrequently used abbreviations which might be rarely referenced While every attempt has been made to define each abbreviation when first encountered in each chapter, we are also aware that readers may start in the middle of a chapter after the abbreviation has been defined or because of a busy schedule just forgot the supplied definition This appendix is here to help provide clarity for this book ACC: adenoid cystic carcinoma ACC LG: adenoid cystic carcinoma of the lacrimal gland ADC: apparent diffusion coefficient AGCC: agenesis of corpus callosum AJCC: American Joint Committee on Cancer AJCC TNM: American Joint Committee on Cancer using TNM (tumor nodes affected and metastasis) ALL: acute lymphoblastic leukemia AML: acute myelogenous leukemia AOD: anterior orbital dermoid AP: anterior to posterior; antrochoanal polyp AVF: arteriovenous fistula AVM: arteriovenous malformation Ax: axial or transverse BBB: blood brain barrier BCC: basal cell carcinoma BMT: benign mixed tumor; bone marrow transplant BOF: blowout orbital fracture BOFF: blowout orbital floor fracture CA: congenital anophthalmia Ca11: calcification CCF: carotid-cavernous fistula CD: choroidal detachment CECT: contrast-enhanced computed tomography CHARGE syndrome: coloboma, heart anomalies, choanal atresia, retardation of growth and development, genital and ear anomalies chemosis: conjunctival swelling CISS: constructive interference in steady state CLL: chronic lymphocytic leukemia CML: chronic myeloid leukemia CMV: cytomegalovirus CMVR: cytomegalovirus retinitis CN: cranial nerve CN I: cranial nerve I: olfactory nerve CN II: cranial nerve II: optic nerve CN III: cranial nerve III: ophthalmic nerve CN IV: cranial nerve IV: trochlear nerve CN V: cranial nerve V: trigeminal nerve CN V1: cranial nerve V1: ophthalmic nerve CN V2: cranial nerve V2: maxillary CN V3: cranial nerve V3: mandibular CN VI: cranial nerve VI: abducens nerve CN VII: cranial nerve VII: facial CN VIII: cranial nerve VIII: vestibulocochlear CN IX: cranial nerve IX: glossopharyngeal CN X: cranial nerve X: vagus CN XI: cranial nerve XI: accessory CN XII: cranial nerve XII: hypoglossal CNS: central nervous system COACH syndrome: cerebellar vermis hypoplasia, oligophrenia, congenital ataxia, coloboma, hepatic fibrosis COMS: Collaborative Ocular Melanoma Study COT: congenital orbital teratoma CRAO: central retinal artery occlusion CRVO: central retinal vein occlusion CS: cavernous sinus CSF: cerebrospinal fluid CST: cavernous sinus thrombosis CT: computed tomography CTA: CT angiography CTD: CT dacryocystography CTV: CT venography CVT: cerebral venous thrombosis 3D: three dimension 3D-CRT: three-dimensional conformal radiation therapy DDx: differential diagnosis DFS: disease-free survival DNA: deoxyribonucleic acid DSA: digital subtraction angiography DWI: diffusion-weighted imaging 247 248 Abbreviations EBRT: electron beam radiation therapy ECA: external carotid artery ECD: Erdheim-Chester disease EMP: extramedullary plasmacytoma ENB: esthesioneuroblastoma EOM: extraocular muscle EOOM: extraocular orbital metastasis Fat-sat: fat saturation technique FB: foreign body FD: fibrous dysplasia FDG-PET: fludeoxyglucose positron emission tomography FDG-PET CT: fludeoxyglucose positron emission tomography with computed tomography FH: family history FLAIR: fluid-attenuated inversion recovery FOM: floor of mouth Ga67: radioisotope of gallium 67 Gd: gadolinium GI: gastrointestinal GO: graves orbitopathy GS: granulocytic sarcoma GSW: gunshot wound GU: genitourinary HA: headache HAART: highly active antiretroviral therapy HB: hemangioblastoma HCMV: human cytomegalovirus H&E: hematoxylin and eosin stain HIV/AIDS: human immunodeficiency/acquired immunodeficiency syndrome H&N: head and neck HPT: hyperparathyroidism HPV: human papillomavirus HU: Hounsfield unit I125: radioisotope of iodine 125 ICA: internal carotid artery ICH: intracranial hemorrhage IIP: increased intracranial pressure IOI: idiopathic orbital inflammation IOP: idiopathic orbital pseudotumor; increased orbital pressure; intraocular pressure IP: inverted papilloma ISSVA: International Society for the Study of Vascular Anomalies IV: intravenous LCH: Langerhans cell histiocytosis LG: lacrimal gland LGD: lacrimal gland dermoid LGL: lacrimal gland lymphoma LGS: lacrimal gland sarcoidosis LM: lymphatic malformation LOCS III: Lens Opacities Classification System III LRS: lens replacement surgery MALT: mucosa-associated lymphoid tissue MBOF: medial blowout fracture MCA: middle cerebral artery MEC: mucoepidermoid carcinoma M:F: male-to-female ratio M:F 1:1: male-to-female ratio is to MGS: morning glory syndrome MIBG: metaiodobenzylguanidine MM: multiple myeloma MMF: mycophenolate MOG: malignant optic glioma MR: magnetic resonance MRA: magnetic resonance angiogram MRD: magnetic resonance dacryocystography MRI: magnetic resonance imaging MRSA: methicillin resistant staphylococcus aureus MRV: magnetic resonance venogram MS: multiple sclerosis NB: note well NECT: nonenhanced computed tomography NF: neurofibroma, neurofibromatosis NF- I: neurofibromatosis type I NF1: neurofibromatosis type I NF-II: neurofibromatosis type II NF2: neurofibromatosis type II NHL: non-Hodgkin lymphoma NSGCT: nonseminomatous germ cell tumor OA: orbital abscess OAL: ocular adnexal lymphoma OCH: orbital cavernous hemangioma; ocular cavernous hemangioma OD: optic disc; orbital dermoid; right eye, from Latin, oculus dextra ODD: optic disc drusen OE: orbital exenteration OFD: orbital fibrous dysplasia OGM: olfactory groove meningioma OL: orbital lipoma; orbital lymphoma OLD: ocular lens displacement OM: ocular melanoma ON: optic nerve ONG: optic nerve glioma ONHD: optic nerve head drusen ONL: optic nerve leukemia ONM: optic nerve meningioma ON Met: optic nerve metastasis ONN: optic nerve neuritis ONS: optic nerve sarcoidosis; optic nerve sheath ONSM: optic nerve sheath meningioma OP: ocular prosthesis   Abbreviations OPG: optic pathway glioma OS: orbital schwannomas; left eye, from Latin, oculus sinister OT: ocular toxocariasis OU: both eyes; from Latin, oculi uterque OVV: orbital venous varix PALG: pleomorphic adenoma lacrimal gland PB: phthisis bulbi PD: proton density PE: physical examination PES: partially empty sella PET: positron emission tomography PHPV: persistent hyperplastic primary vitreous PNET: primitive neuroectodermal tumor PNF: plexiform neurofibroma POD: posterior orbital dermoid POS: posterior ocular staphyloma post-Gd: post-gadolinium PPT: Pott’s puffy tumor PRN: as needed PTC: pseudotumor cerebri Pts: patients RB: retinoblastoma RBI: retinoblastoma tumor suppressor gene RD: retinal detachment RG: ruptured globe RLF: retrolental fibroplasia RMS: rhabdomyosarcoma R/O: rule out ROP: retinopathy of prematurity RöRx: radiation therapy SAH: subarachnoid hemorrhage SBP: solitary bone plasmacytoma SCC: squamous cell carcinoma SCC LG: squamous cell carcinoma of lacrimal gland 249 SCCA: squamous cell carcinoma SCRT: stereotactic conformal radiotherapy SDH: subdural hemorrhage SI: signal intensity SOV: superior ophthalmic vein SQ: subcutaneous STD: sexually transmitted disease STIR: short tau inversion recovery T1: MRI relaxation parameter T1 Gd: gadolinium enhanced T1 imaging T1WI: T1 weighted images T2: MRI relaxation parameter T2WI: T2 weighted images TAO: thyroid associated orbitopathy TCA: traumatic cerebral aneurysm TCC: transitional cell carcinoma TED: thyroid eye disease TEs: time to echoes, echo delay time THS: Tolosa-Hunt syndrome TNM: TNM classification of malignant tumors (uses tumor size, nodes effected, and metastatic site) TS: tuberous sclerosis TSH-R: thyroid-stimulating hormone-r (an antigen) URI: upper respiratory infection US: ultrasound VHL: von Hippel-Lindau syndrome VLM: venous lymphatic malformation WG: Wegener granulomatosis WHO: World Health Organization WI: weighted image Smarter search Faster answers Smarter, Faster Search for Better Patient Care Unlike a conventional search engine, ClinicalKey is specifically designed to serve doctors by providing three core components: Comprehensive Content The most current, evidence-based answers available for every medical and surgical specialty Trusted Answers Content supplied by Elsevier, the world’s leading provider of health and science information Unrivaled Speed to Answer Faster, more relevant clinical answers, so you can spend less time searching and more time caring for patients Start searching with ClinicalKey today! Visit ClinicalKey.com for more information and subscription options ... enophthalmos,restrictivestrabismus,or infraorbitalnumbness • Blowout orbital fractures(BOFs)may involvethe orbital floor,medial orbital wall,orrarelythe orbital roof,while sparingthe orbital rim • Therearetwogenerallyaccepted... have learned from Orbital Imaging F Allan Midyett, MD v CONTENTS • PART I TRAUMA AND SURGERY MEDIAL B LOWOUT FRACTURE, BLOWOUT ORBITAL FLOOR FRACTURE, ORBITAL ExENT ERAT 10 , ORBITAL FLOOR MESH,... CONGENITAL ORBITAL TERAT0.1.A, 27 CONGENITAL A.N OPHTHALMIA, • PART V 116 121 VASCULAR 11 ORBITAL CAVERl ous HEMANG IOMA, 12 OPTIC NERVE GLIOMA, 13 OPTIC 14 POSTERIOR ORBITAL DERMOIDS, 15 ORBITAL
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