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the United Kingdom), and, in accordance with our philosophy, each centre is represented by specialists in internal medicine and ophthalmology. A further requirement of EUGOGO membership is that each participating centre must have combined thyroid-eye clinics in which the patient can be seen simultaneously by physicians from both disciplines. It is gratifying to note that the number of combined thyroid-eye clinics has been increasing slowly but steadily throughout Europe over the last decade, thereby enhancing the quality of patient care.

      EUGOGO meets twice a year, and we have had many lengthy and at times heated discussions on how to assess the eye changes in a most objective manner and what the best treatment should be. It has taken us several years to reach an acceptable degree of agreement among ourselves, again illustrating the many pitfalls in the management of Graves’ orbitopathy as well as the importance of using a multidisciplinary approach. Only then could we embark on prospective clinical trials (the size of the group allows completion of clinical studies in a much shorter time than possible for each individual centre), on teaching courses how to investigate and treat the patient with Graves’ orbitopathy (so far 3 courses have been held – Thessaloniki 2005, Pisa 2006, and Mainz 2007), and on papers in scientific journals describing the outcome of our studies and recommendations on disease management.

      The present book reflects our current thinking on Graves’ orbitopathy: all authors are EUGOGO members. The internal consistency between chapters is the result of our ongoing discussions during the annual EUGOGO meetings. The outline of the book is unusual by its choosing the question-and-answer format. The purpose is to enable the book to be also used as a quick reference source for the practising physician confronted with an ordinary or extraordinary management question: most likely the answer can be found in one of the 194 questions asked in this book. We do not apologize for some overlap between chapters (especially on smoking and thyroid treatment), because these issues are relevant to many aspects of Graves’ orbitopathy and it facilitates fast retrieval of the information you are looking for. Topics such as unilateral eye disease, childhood Graves’ orbitopathy, and disease-specific quality-of-life assessment which are not easily found elsewhere receive the attention they deserve. The editors welcome feedback from the readership: please send your comments to us, also on how future editions could be improved. You may also consult the EUGOGO website (www.eugogo.eu).

      We would like to thank the patients with Graves’ orbitopathy who cooperated in our endeavours to better understand their disease. We also thank S. Karger AG, Medical and Scientific Publishers, for their efforts to edit, produce, and publish the book within a very short time. Last but not least, we are grateful to the authors for their excellent contributions. It again proves how much can be accomplished by a group of dedicated people.

      Wilmar M. Wiersinga, President EUGOGO, Editor

      George J. Kahaly, Treasurer EUGOGO, Co-Editor

      Amsterdam and Mainz, June 2007

Diagnosis and Pathogenesis

      Wiersinga WM, Kahaly GJ (eds): Graves’ Orbitopathy: A Multidisciplinary Approach – Questions and Answers.

      Basel, Karger, 2017, pp 1–25 (DOI: 10.1159/000475944)

      ___________________

Clinical Manifestations

      A. Jane Dickinson^a · Christoph Hintschich^b

      ^aNewcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK; ^bUniversity Eye Hospital, Ludwig-Maximilian University Munich, Munich, Germany

What Are the Common Signs and Symptoms of Early Graves’ Orbitopathy?

Most patients initially notice a change in appearance. This includes redness in the eyes or lids, and swelling or feeling of fullness in one or both upper eyelids, and/or bags under the eyes [1]. The single most common presenting sign is eyelid swelling, followed by eyelid lagging behind eyeball movement on downgaze (von Graefe sign) [2].

During early Graves’ orbitopathy (GO), 40% of patients also develop symptoms that relate to ocular surface irritation comprising a gritty sensation, light sensitivity (photophobia), and excess tearing [3–5].

What Are Other Signs and Symptoms of Graves’ Orbitopathy?

With ongoing disease, the most frequent sign is upper eyelid retraction, which affects 90–98% of patients at some stage [3, 6] and frequently varies with attentive gaze (Kocher sign) [7]. Indeed, if upper eyelid retraction is absent, then it is appropriate to question the diagnosis [7]. The contour of the retracted upper eyelid often shows lateral flare (Fig. 1) [8], an appearance that is almost pathognomonic for GO.

Exophthalmos (also known as proptosis) is also very frequent and correlates significantly with lower lid retraction [9]; these patients are more likely to show incomplete eyelid closure (lagophthalmos). Many such patients, especially those with a wide palpebral fissure, will show punctate inferior corneal staining with fluorescein [9, 10].

Double vision is rare at presentation but fairly common later, when it is initially noticed either on waking, when tired, or on extremes of gaze [4, 5, 11]. Hence, many patients presenting to tertiary centres show restriction of ocular excursions in one or more directions of gaze. Eye movements may be accompanied by aching; however, if the orbit is extremely congested, then the patient may also develop orbital ache unrelated to gaze [12].

Fig. 1. Assessment of the palpebral aperture. The midpoint of the pupil is chosen regardless of lateral flare. In this example, upper eyelid retraction and lower eyelid retraction both measure +1 mm with the limbus as a reference point. Note that the normal adult eyelid position would measure –2 mm.

Only about 5% of patients report visual symptoms such as alteration in colour perception or blurring of vision, which may be either patchy or generalized [4, 5, 11]. These visual symptoms are potentially significant

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