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
Wiersinga WM, Kahaly GJ (eds): Graves’ Orbitopathy: A Multidisciplinary Approach – Questions and Answers.
Basel, Karger, 2017, pp 1–25 (DOI: 10.1159/000475944)
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A. Jane Dickinsona · Christoph Hintschichb
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 markers of dysthyroid optic neuropathy (DON), and as they may not be volunteered, they should be specifically elicited from all patients with progressive or otherwise symptomatic disease as detecting subtle evidence of DON is crucial. If DON is significantly asymmetrical (30%), then an afferent pupil defect will also be apparent [13].
Sight-threatening corneal ulceration is even less common than DON, but potentially devastating. It presents as an area of corneal staining, sometimes with thinning or abscess and very occasionally perforation. Corneal ulceration can only develop when normal corneal protection is lost. This occurs in those patients who not only have lagophthalmos (see above), but whose cornea remains visible when the eyelids are closed. In 90% of normal individuals the eyeball rotates upwards on eyelid closure to protect the cornea. If the inferior rectus muscle is tight as is common in GO, then this normal reflex (the Bell’s phenomenon) is lost, leaving the cornea in a more vulnerable position (Fig. 2). It is not known whether patients with extreme eyelid retraction are at greater risk of ulceration, but it is clear that sight-threatening ulceration can develop in patients without severe eyelid retraction.
Other unusual signs and symptoms of GO include superior limbic keratoconjunctivitis, inflammation of the caruncle and/or plica (see the section “How Are These Signs Assessed?” below) and episodes of globe subluxation (where
