Contents I

MEDICAL RADIOLOGY

Diagnostic Imaging

Editors:A. L. Baert, Leuven

M. Knauth, GöttingenK. Sartor, Heidelberg

Contents III

Goetz Benndorf

Dural Cavernous Sinus FistulasDiagnostic andEndovascular Therapy

Foreword by

K. Sartor

With 178 Figures in 755 Separate Illustrations, 540 in Color and 19 Tables

123

IV Contents

Goetz Benndorf, MD, PhDAssociate Professor, Department of RadiologyBaylor College of MedicineDirector of Interventional NeuroradiologyBen Taub General HospitalOne Baylor Plaza, MS 360Houston, TX 77030USA

Medical Radiology · Diagnostic Imaging and Radiation OncologySeries Editors: A. L. Baert · L. W. Brady · H.-P. Heilmann · M. Knauth · M. Molls · C. Nieder · K. Sartor

Continuation of Handbuch der medizinischen Radiologie Encyclopedia of Medical Radiology

ISBN 978-3-540-00818-7 e-ISBN 978-3-540-68889-1

DOI 10.0007 / 978-3-540-68889-1

Medical Radiology · Diagnostic Imaging and Radiation Oncology ISSN 0942-5373

Library of Congress Control Number: 2004116221

© 2010, Springer-Verlag Berlin Heidelberg

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Contents V

Dedicated to my parents,

Dorothea and Eberhard Benndorf

Contents VII

Foreword

Of the dural venous sinuses, the cavernous sinus is anatomically the most complex. It has an intimate topographical relationship with the internal carotid artery and the sixth cra-nial nerve (both of which pass through its meshwork) and houses the cranial nerves III, IV and V1-2 in its lateral wall. Medially it abuts the pituitary gland, while laterally it nears the temporal lobe; Meckel’s cave with the trigeminal ganglion lies immediately posterior to it. Located essentially at the center of the skull base, the cavernous sinus connects with numerous dural sinuses and veins of all three cranial fossae, as well as the orbit. Further-more, there is an abundance of dural arteries in the sellar region that are interconnected and derive from the carotid system.

Due to its specifi c vascular anatomy, the cavernous sinus is a common site of various types of arteriovenous (AV) fi stulas, which are essentially benign lesions but which may endanger both vision and cranial nerve function. These fi stulas, including their largely endovascular interventional treatment, are the topic of Götz Benndorf’s monograph. After describing the relevant anatomy of the cavernous sinus in great detail, the author con-tinues by explaining the anatomic and hemodynamic classifi cations of AV fi stulas of the cavernous sinus. This is followed by important information on etiology, pathogenesis and prevalence of the various lesions. Before embarking on the main part of his book, diagnos-tic and therapeutic radiology of AV cavernous fi stulas, Benndorf devotes an entire chapter to clinical, largely neuro-ophthalmological symptoms and signs. The radiological chap-ters, all beautifully illustrated and containing a treatise on hemodynamics, will undoubt-edly convince readers of the immense diagnostic and therapeutic experience of the author in his chosen topic. The treatment focuses on transvenous embolization rather than the transarterial approaches.

I am unaware of any publication that covers the diagnostic and interventional radiol-ogy of AV fi stulas of the cavernous sinus in such a clear, systematic and complete way as Benndorf’s book. Any interventional neuroradiologist dealing with skull base lesions needs a copy, as does any skull base surgeon. In addition, the book would not look out of place on a neurologist’s bookshelf.

Heidelberg Klaus Sartor

Contents IX

Preface

This volume of Medical Radiology is based to a large degree on my Ph.D. Thesis at Charité, Humboldt University (Berlin, 2002) and contains most of the original text and imaging material. During my subsequent years (2003 to 2009) at the departments of radiology, Baylor College of Medicine (BCM) and The Methodist Hospital (TMH) in Houston, this work grew substantially, and thus required more time to complete than anticipated. Nevertheless, I hope, the result represents a happy ending that was worth waiting for. The monograph stands for more than 13 years of personal experience in performing endovascular treat-ment and clinical management of patients with various types of intracranial arteriovenous shunting lesions, in particular fi stulas of dural origin involving the cavernous sinus. It is intended as a practical guide and reference for those involved in the diagnosis and treat-ment of these lesions. Completing this book would have been impossible without the moti-vation, help and support from my teachers, colleagues and friends.

Acknowledgements:I am very thankful to Christiane Kagel (University Greifswald) and Christiane Poehls (Helios Klinikum Berlin-Buch), who between 1989 and 1991, taught me the basic principles of diagnostic cerebral angiography and vascular interventional radiology. Horst Peter Molsen (Charité, Berlin), my INR fellowship director from 1991 to 1993, introduced the techniques of transvenous catheterization and embolization to me while we were treating the fi rst CSF patients together. My most sincere gratitude goes to Jacques Moret (Foun-dation Rothschild, Paris) for his generous permission to use some of his material for my thesis. During his numerous visits and lectures at the Benjamin Franklin Hospital, Free University Berlin from 1992 to 1997, Pierre Lasjaunias (†, Kremlin Bicetre, Paris) was not only a brilliant teacher, but also a great inspirer in the studies of the vascular anatomy of the cavernous sinus region. I am very much indebted to Wolfgang Lanksch (Charité, Berlin), chair of the department of neurosurgery, who was my clinical mentor and a steady supporter of my work from 1991 to 2003.

I owe many special thanks to the entire INR team at Rudolf-Virchow-Hospital, Charité, but mostly to two nurses, Angelika Wehner and Petra Schlecht, whose loyal devotion and outstanding assistance over many years played a key role in the successful performance of complex endovascular procedures. Christof Barner, immensely experienced and dedi-cated neuroanesthesiologist at Charité, became an indispensable colleague for achieving good clinical outcomes. I also wish to thank Horst Menneking (maxillo-facial surgeon, Charité) for his exceptionally skillful surgical work in those cases, where transophthal-mic SOV approaches became necessary. My colleague and friend, Andreas Bender (Charité until 1998), one of the most talented interventional neuroradiologists I had the good for-tune to meet, helped me to master the treatment challenges of several of my early patients. Stephanie Schmidt, ophthalmologist at Charité until 2003, was an outstanding clinician and a most pleasant colleague to work with.

The majority of presented case reports are of patients, for whom staff members from the departments of neurosurgery, neurology and ophthalmology at Charité provided excellent care.

X Preface

The following colleagues and friends contributed additional interesting and valuable imaging material: Jacques Moret (Foundation Rothschild, Paris), Alessandra Biondi (Pitié-Salpêtrière University Hospital, Paris); Gyula Gal (Uni-versity Hospital Odense); Michel Mawad (BCM, Houston); Jacques Dion (Emory University Hospital, Atlanta); Richard Klucznik (TMH, Houston); Maria Angeles De Miquel (Hospital Universitari de Bellvitge, Barcelona); Michael Soederman (Karolinska University Hospital, Stockholm); Winston Lim (Singapore General Hospital); Adriana Campi (Ospedale San Raffaele, University of Milan); Rob De Keizer (University Hospital, Leiden); Charbel Mounayer (Foundation Rothschild, Paris); Raimund Parsche (Ruppiner Kliniken); Bernhard Sander (MRI Praxis, Berlin) and Ullrich Schweiger (Ullsteinhausklinik, Berlin).

Special credit goes to Alessandra Biondi, Gyula Gal, Stefanie Schmidt, Diane Nino (BCM Houston), Phillip Randall (TMH Houston) and Mia Carlson (MDA, Houston) for review, suggestions and corrections of the text.

The superb clinical photographs were taken by Franz Haffner and Peter Behrend (Charité, Berlin); David Gee (Houston) produced the high-quality dry skull pic-tures. Corinna Naujok (Charité, Berlin), Charlie Thran (TMH, Houston) and Scott Weldon (BCM, Houston) helped to create the nicely colored illustrations. Dirk Emmel (Charité, Berlin) provided invaluable support for the digital storage of the image material. Richard Klucznik (TMH, Houston) was very helpful in acquiring high-resolution 3D-data for the imaging studies of the cavernous sinus.

Last, but not least, I am truly grateful to Ursula Davis (Heidelberg), Christine Schaefer (Hemsbach), Kurt Teichmann (Mauer) and Klaus Sartor (Heppenheim), whose endless patience and understanding allowed me to produce and fi nish this volume. Houston Goetz Benndorf

Contents XI

Contents

1 Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

2 Historical Considerations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

2.1 Arteriovenous Fistula and Pulsating Exophthalmos . . . . . . . . . . . . . . . . 3 2.2 Angiography. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6 2.3 Therapeutic Measures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 2.4 Embolization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

3 Anatomy of the Cavernous Sinus and Related Structures . . . . . . . . . . . . . . 15

3.1 Osseous Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 3.2 Anatomy of the Dura Mater and the Cranial Nerves . . . . . . . . . . . . . . . . 20 3.3 Vascular Anatomy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46

4 Classifi cation of Cavernous Sinus Fistulas (CSFs) and Dural Arteriovenous Fistulas (DAVFs) . . . . . . . . . . . . . . . . . . . . . . . . . . 51

4.1 Anatomic Classifi cation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51 4.2 Etiologic Classifi cation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 4.3 Hemodynamic Classifi cation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62

5 Etiology, Prevalence and Natural History of Dural Cavernous Sinus Fistulas (DCSFs) . . . . . . . . . . . . . . . . . . . . . . . . . 65

5.1 Etiology and Pathogenesis of Type A Fistulas . . . . . . . . . . . . . . . . . . . . 66 5.2 Etiology and Pathogenesis Type B–D Fistulas . . . . . . . . . . . . . . . . . . . . 67 5.3 Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79

6 Neuro-Ophthalmology in Dural Cavernous Sinus Fistulas (DCSFs) . . . . . . . . 85

6.1 Extraorbital Ocular Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86 6.2 Orbital Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 6.3 Other and Neurological Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . 91 6.4 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94

XII Contents

7 Radiological Diagnosis of DCSFs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97

7.1 Non-invasive Imaging Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . 97 7.2 Intra-arterial Digital Subtraction Angiography (DSA). . . . . . . . . . . . . . . 109

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184

8 Endovascular Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189

8.1 Techniques of Transvenous Catheterization . . . . . . . . . . . . . . . . . . . . . 189 8.2 Embolic Agents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197 8.3 Results of Transvenous Embolizations . . . . . . . . . . . . . . . . . . . . . . . . 208 8.4 Discussion of Transvenous Occlusions . . . . . . . . . . . . . . . . . . . . . . . . 228 8.5 Conclusion. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267

9 Alternative Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277

9.1 Spontaneous Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277 9.2 Manual Compression Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 9.3 Controlled Hypotension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 282 9.4 Radiotherapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284 9.5 Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289

10 Hemodynamic Aspects of DCSFs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293

10.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293 10.2 Basic Hemodynamic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . 294 10.3 Invasive Assessment of Hemodynamics . . . . . . . . . . . . . . . . . . . . . . . 295 10.4 Flow Velocity and Pressure Measurements in Brain AVMs and DAVFs. . . . . . 297 10.5 Hemodynamics and Pathophysiology in CSFs . . . . . . . . . . . . . . . . . . . 297 10.6 Flow Velocity and Pressure Measurements in DCSFs. . . . . . . . . . . . . . . . 300

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305

11 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309

Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313

Glossary XIII

Glossary

ACC Anterior condylar confl uensACT Angiographic computed tomography (contrast enhanced DynaCT; based on C-arm mounted Flat panel technology)ACV Anterior condylar vein AFR Artery of the foramen rotundum AMA Accessory meningeal artery APA Ascending pharyngeal arteryAVM Arteriovenous malformation

BP Basilar plexus BSC Boston Scientifi c Corporation

CCF(s) Carotid cavernous fi stula (direct fi stula)CN(s) Cranial nerve(s)CS(s) Cavernous sinus(es) CSF(s) Cavernous sinus fi stula(s) (AV shunt involving the cavernous sinus in general)CTA Computed tomographic angiography (based on conventional CT technology)CVD Cortical venous drainage

DAVF(s) Dural arteriovenous fi stulaDAVS Dural arteriovenous shuntDCCF Dural carotid cavernous fi stulaDCSF(s) Dural cavernous sinus fi stula(s) (indirect fi stula)DSA Digital subtraction angiographyDynaCT Siemens term for cross sectional (CT-like) imaging using rotating C-arms

ECA External carotid artery

F French (Charrière; 1 french = 1/3 mm)FLP Foramen lacerum plexusFrV Frontal veinFV Facial vein

i.a. Intraarteriali.v. IntravenousICA Internal carotid arteryICAVP Internal carotid artery venous plexusICS Intercavernous sinus IJV Internal jugular vein ILT Inferolateral trunkIMA Internal maxillary artery IOF Inferior orbital fi ssure (infraorbital) IOV Inferior ophthalmic vein IPCV Inferior petroclival vein IPS Inferior petrosal sinus IPV Intrapetrosal vein

XIV Glossary

JB Jugular bulb

LCS Laterocavernous sinusLCV Lateral condylar vein LVD Leptomeningeal venous drainage

MHT Meningohypophyseal trunkMIP Maximum Intensity projections MMA Middle meningeal arteryMPR Multiplanar reconstructionsMRA Magnetic resonance angiographyMTA Marginal tentorial artery (medial tentorial artery)MTV Middle Temporal vein

NBCA N-butyl-cyanoacrylate

OA Ophthalmic artery

PCP Posterior clinoid processPCV Posterior condylar veinPP Pterygoid plexus PPF Pterygopalatine fossaPVP Prevertebral plexus

RAFL Recurrent artery of the foramen lacerum

SMCV Superfi cial middle cerebral veinSOF Superior orbital fi ssure (supraorbital)SOV Superior ophthalmic vein SPPS Sphenoparietal sinus SPS Superior petrosal sinus SS Sigmoid sinus SSD Surface shaded DisplaySTA Superfi cial temporal artery

TAE Transarterial embolizationTVO Transvenous occlusion

UV Uncal vein

VA Vertebral arteryViA Vidian arteryVRT Volume rendering technique

Alternative Treatment Options 1

Introduction 1

ophthalmic vein (SOV), causing signs and symp-toms very similar, albeit milder, to those observed in patients with direct high-fl ow carotid cavernous fi stulas (CCFs).

Signifi cant improvements in angiographic im-aging technology over the last 15 years, such as the introduction of three-dimensional digital subtracted angiography (3D-DSA), have resulted in better understanding of the specifi c arterial and venous anatomy, opening the doors for novel treatment options. In combination with the ad-vances made in endovascular tools and devices, transvenous occlusion using various transfemoral or percutaneous access routes has become increas-ingly popular.

Numerous case reports and small case series have been published, creating a wealth of infor-mation in the medical literature. However, the data scattered through journals of various clinical disciplines namely neuroradiology, neurosurgery, neurology and ophthalmology.

Regarding therapeutic options for patients with CSFs, Hamby (1966) stated: “The best possibility theoretically would be to induce thrombosis that would close the sinus completely. This appears to be hardly possibly, by currently known techniques, in the face of the tremendous arterial inf low of blood”. This concept was reiterated by Mullan (1974), and 40 years later, transvenous occlusion (TVO) techniques play a dominant role in the management of patients with DCSFs. Because TVO of DCSFs can often be performed success-fully today with high efficacy and low morbidity, it has widely replaced microneurosurgery. On the other hand some controversy about its proper in-dication, associated complication rates and the use of therapeutic alternatives persists.

The purpose of this monograph was to collect and discuss much of the radiological and imaging information available. It aims to summarize and

In reference to cavernous sinus fi stulas (CSFs) caus-ing pulsating exophthalmos, Walter Dandy (1937)wrote: “The study of carotid-cavernous aneurysm – the clinical ensemble – the variation and capricious results of treatment – have been told and retold, and most admirably. Medical literature can scarcely claim more accurate and thorough studies than upon this subject.”

More than 70 years later, a similar statement can be made relating to a subgroup of CSFs, the arterio-venous shunts between small dural branches aris-ing from the external and internal carotid arteries and the cavernous sinus, also called dural cavern-ous sinus fi stulas (DCSFs). Indeed much has been written about these fi stulas, which were recognized relatively late as a separate entity among CSFs, and which can be clinically perplexing and sometimes quite diffi cult to diagnose or to treat. The cure of patients, on the other hand, is one of the most re-warding in the spectrum of modern neuroendovas-cular treatments.

The initial angiographic descriptions by Castaigne et al. (1966), Newton and Hoyt (1970) and Djindjan et al. (1968) focused mainly on their peculiar arterial supply, which later became the basis for a widely used anatomic classifi cation (Barrow et al. 1985).

The cavernous sinus itself represents a rather complex venous reservoir, embedded in the base of the skull and traversed by the cavernous carotid artery and four cranial nerves. It functions as a con-fl uens, receiving multiple cerebral and intra cranial afferent veins (tributaries) and drains into various efferent veins or dural sinuses.

Despite numerous studies, etiology, pathophysi-ology and clinical course of these fi stulas are to date only partially understood.

Because the arteriovenous shunts develop with-in the dural walls of the cavernous sinus (CS), their fl ow is usually directed towards the superior

2 1 Introduction

facilitate access to currently existing knowledge on these complex, incompletely understood, and some-times challenging lesions.

Views and opinions stated below refl ect personal experience in clinical and endovascular manage-ment of patients with DCSFs, demonstrating the evolution of minimal invasive techniques, particu-larly the increasing use of transvenous approaches to the CS.

Insights into all aspects of these interesting cere-brovascular lesions, including their anatomy, etiol-ogy, classifi cation, clinical presentation, imaging techniques and hemodynamics, are provided. Vari-ous current treatment options and their role in pa-tient management are described, such as conserva-tive management, manual compression, controlled hypotension, radiosurgery, surgery, but foremost endovascular therapy.

Percutaneous catheterization techniques are cov-ered in greater detail with great emphasis on trans-venous access routes and the progress that has been made since Halbach et al. (1989) published the fi rst relevant series.

This volume is intended as a reference and a guide for neuroradiologists, neurosurgeons, neurologists and ophthalmologists, who see patients with DCSFs in their practice.

References

Dandy W (1937) Carotid-cavernous aneurysms (pulsating exophthalmos). Zentralbl Neurochir 2:77–206

Castaigne P, Laplane D, Djindjian R, Bories J, Augustin P (1966) Spontaneous arteriovenous communication be-tween the external carotid and the cavernous sinus. Rev Neurol (Paris) 114:5–14

Newton TH, Hoyt WF (1970) Dural arteriovenous shunts in the region of the cavernous sinus. Neuroradiology 1:71–81

Djindjian R, Cophignon J, Comoy J, Rey J, Houdart R (1968) Neuro-radiologic polymorphism of carotido-cavernous fi stulas. Neurochirurgie 14:881–890

Djindjian R, Manelfe C, Picard L (1973) External carotid-cav-ernous sinus, arteriovenous fi stulae: angiographic study of 6 cases and review of the literature. Neurochirurgie 19:91–110

Newton TH, Hoyt WF (1968) Spontaneous arteriovenous fi stula between dural branches of the internal maxil-lary artery and the posterior cavernous sinus. Radiology 91:1147–1150

Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT (1985) Classifi cation and treatment of spon-taneous carotid-cavernous sinus fi stulas. J Neurosurg 62:248–256

Hamby W (1966) Carotid-cavernous fi stula. Springfi eldMullan S (1974) Experiences with surgical thrombosis of in-

tracranial berry aneurysms and carotid cavernous fi stu-las. J Neurosurg 41:657–670

Halbach VV, Higashida RT, Hieshima GB, Hardin CW, Pri-bram H (1989) Transvenous embolization of dural fi stulas involving the cavernous sinus. AJNR Am J Neuroradiol 10:377-383

2.1 Arteriovenous Fistula and Pulsating Exophthalmos 3

Historical Considerations 2

C O N T E N T S

2.1 Arteriovenous Fistula and Pulsating Exophthalmos 3

2.2 Angiography 6

2.3 Therapeutic Measures 9

2.4 Embolization 10

References 11

cose and it will have a pulsatile jarring motion on account of the stream from the artery. It will make a hissing noise, which will be found to correspond with the pulse for the same reason. The blood of the tumor will be altogether or almost entirely fl uid because of its constant motion”.

Cleghorn (1769) suggested the name “aneuris-mal varix” for the direct communication. Without postmortem evidence for his conclusions, Benjamin Travers in 1809 described fi rst pulsating exophthal-mos and designated it as “Aneurysma per anstomo-sin” or “cirsoid aneurysms of the orbit” (Fig. 2.1) (Travers 1811).

Three years later on April 7, 1813, Dalrymple (1815) operated a second, similar case of pulsating exophthalmos and followed Travers’ explanation for its etiology, as did the majority of subsequent writers until 1823.

In these years the French anatomist Breschet (1829) studied in detail the vascular anatomy of the brain and skull, as well as the head and neck area and provided color plates in most outstanding qual-ity (Fig. 2.2).

Guthrie (1827) recorded during the same pe-riod the fi rst necropsy of a patient with pulsating exophthalmos and found instead of a “cirsoid aneu-rysm” an aneurysm of the ophthalmic artery: “On the death of the patient an aneurism of the ophthal-mic artery was discovered on each side, of about the size of a large nut”. Thus, he was led to believe er-roneously that it was the usual lesion in pulsating exophthalmos and advocated this as etiology of all reported cases of pulsating exophthalmos. This was supported by Busk’s (1839) autopsy fi ndings of an-other case.

However, in France, four years prior to the Busk report, it was Baron (1835) who is given credit for being the fi rst to discover a direct communication between the internal carotid and the cavernous sinus. Even though his report was so brief that it was

2.1 Arteriovenous Fistula and Pulsating Exophthalmos

William Hunter in 1757, is credited with recognizing an arteriovenous aneurysm as direct communica-tion between the artery and vein, while previous observers having interpreted the lesion as a simple aneurysm (Hunter 1762). He studied two patients in whose arms the vessels had been injured by phle-botomy, an operation extensively practiced at that time not only by physicians but also charlatans and barbers. His fi rst accurate appraisal of an arterio-venous communication, described not only the bruit and the palpable thrill at the site of communication but also the marked dilatation and tortuosity of the artery at the site of the fi stula:

“In a former paper upon aneurysm, I took notice of a species of that complaint, which, so far as I know, had not been mentioned by any other author; where there is an anstomosis or immediate connection be-tween the artery and the vein at the part where the patient let blood in consequence of the artery being wounded through the vein; so that blood passes im-mediately from the trunk of the artery into the trunk of the vein and so back into the heart. It will differ in its symptoms from the spurious aneurysm princi-pally thus. The vein will be dilated or become vari-