Observation of Unexplained Cases of Unilateral Upper Eyelid Retraction from Idiopathic Inflammation or an Atypical Form of Gravesʼ Orbitopathy

Abstract

Background We observed cases of unilateral upper eyelid retraction (UER) with unclear pathogenesis, where a definitive diagnosis of Gravesʼ orbitopathy (GO) could not be made and other pathologies were ruled out. The aim is to present patientsʼ findings, discuss possible underlying mechanisms, and to describe diagnostic testing and therapeutic approaches.

Patients and Methods Patients were retrospectively analyzed who presented to the Department of Ophthalmology of LMU Munich between 2016 and 2021 without a definitive diagnosis of GO or other pathological causes.

Results Thirteen patients with unexplained UER were included; all showed thickening of the levator complex in imaging. When biopsy was performed, no malignancy could be detected. Therapeutic strategies included oral steroids and surgical upper eyelid blepharotomy.

Conclusion As regards to pathogenesis, in 7/13 patients, a condition of idiopathic UER due to an idiopathic inflammatory response can be postulated rather than a variant of GO.

Zusammenfassung

Hintergrund Wir haben Fälle von unilateraler Oberlidretration (OLR) beobachtet, bei denen die definitive Diagnose einer endokrinen Orbitopathie (eO) nicht gestellt werden konnte und andere Pathologien ausgeschlossen wurden. Ziel ist es, die Befunde der Patienten vorzustellen, mögliche zugrunde liegende Mechanismen zu diskutieren und Diagnostik sowie therapeutischen Ansatz zu beschreiben.

Patienten und Methoden Patienten, die sich zwischen 2016 und 2021 in der Augenklinik der LMU München vorstellten, bei denen die definitive Diagnose einer eO und anderer Pathologien nicht gestellt werden konnte, wurden retrospektiv analysiert.

Ergebnisse Es wurden 13 Patienten mit unerklärter OLR eingeschlossen; alle zeigten eine Verdickung des Levatorkomplexes in der Bildgebung. Bei durchgeführter Biopsie konnte keine Malignität nachgewiesen werden. Therapeutische Strategien umfassten die orale Steroidtherapie und chirurgische Oberlidblepharotomie.

Schlussfolgerung Bei 7/13 Patienten handelt es sich eher um eine idiopathische OLR aufgrund einer idiopathischen entzündlichen Reaktion als um eine Variante der eO.

Conclusion Box

Already known:

• There are different reasons for upper eyelid retraction that can be classified as neurogenic, myogenic, and mechanistic.

• The most common reason for upper eyelid retraction is Gravesʼ orbitopathy.

• There are cases of unilateral upper eyelid retraction were the pathogenesis seams unexplained.

Newly described:

• We assume a form of idiopathic inflammatory upper eyelid retraction.

• A special form of unilateral GO could be in question. Further investigations are needed for more clarification.

Keywords

unilateral upper eyelid retraction - idiopathic inflammation - Gravesʼ orbitopathy

Schlüsselwörter

Unilaterale Oberlid Retraktion - endokrine Orbitopathie - idiopathische Inflammation

Publication History

Received: 11 December 2023

Accepted: 10 April 2024

Article published online:
27 May 2024

• References

• 1 Frueh BR, Musch DC, Garber FW. Lid retraction and levator aponeurosis defects in Gravesʼ eye disease. Ophthalmic Surg 1986; 17: 216-220
• 2 Grisolia ABD, Couso RC, Matayoshi S. et al. Non-surgical treatment for eyelid retraction in thyroid eye disease (TED). Br J Ophthalmol 2017; DOI: 10.1136/bjophthalmol-2017-310695.
• 3 Cruz AA, Ribeiro SF, Garcia DM. et al. Graves upper eyelid retraction. Surv Ophthalmol 2013; 58: 63-76 DOI: 10.1016/j.survophthal.2012.02.007.
• 4 Panagiotou G, Perros P. Asymmetric Gravesʼ Orbitopathy. Front Endocrinol (Lausanne) 2020; 11: 611845 DOI: 10.3389/fendo.2020.611845.
• 5 Ponto KA, Binder H, Diana T. et al. Prevalence, Phenotype, and Psychosocial Well-Being in Euthyroid/Hypothyroid Thyroid-Associated Orbitopathy. Thyroid 2015; 25: 942-948 DOI: 10.1089/thy.2015.0031.
• 6 Soroudi AE, Goldberg RA, McCann JD. Prevalence of asymmetric exophthalmos in Graves orbitopathy. Ophthalmic Plast Reconstr Surg 2004; 20: 224-225 DOI: 10.1097/01.iop.0000124675.80763.5a.
• 7 Bartley GB. The differential diagnosis and classification of eyelid retraction. Trans Am Ophthalmol Soc 1995; 93: 371-387 discussion 387-379.
• 8 Bartalena L, Kahaly GJ, Baldeschi L. et al. The 2021 European Group on Gravesʼ orbitopathy (EUGOGO) clinical practice guidelines for the medical management of Gravesʼ orbitopathy. Eur J Endocrinol 2021; 185: G43-G67 DOI: 10.1530/EJE-21-0479.
• 9 Tabasum A, Khan I, Taylor P. et al. Thyroid antibody-negative euthyroid Gravesʼ ophthalmopathy. Endocrinol Diabetes Metab Case Rep 2016; 2016: 160008 DOI: 10.1530/EDM-16-0008.
• 10 Hintschich C. Eyelid Surgery in Gravesʼ Orbitopathy. Klin Monbl Augenheilkd 2021; 238: 33-40 DOI: 10.1055/a-1325-9061.
• 11 Hintschich C, Haritoglou C. Full thickness eyelid transsection (blepharotomy) for upper eyelid lengthening in lid retraction associated with Gravesʼ disease. Br J Ophthalmol 2005; 89: 413-416 DOI: 10.1136/bjo.2004.052852.
• 12 Li D, Sun F. Observations on the Efficacy of Two Methods for the Treatment of Upper Eyelid Retraction in Thyroid-Associated Ophthalmopathy. Biomed Res Int 2021; 2021: 9514279 DOI: 10.1155/2021/9514279.
• 13 Ozturk Karabulut G, Fazil K, Saracoglu Yilmaz B. et al. An algorithm for Botulinum toxin A injection for upper eyelid retraction associated with thyroid eye disease: long-term results. Orbit 2021; 40: 381-388 DOI: 10.1080/01676830.2020.1814351.