Skip to main content
SpringerLink
Log in

Etiologies of Blepharoptosis

  • Chapter
  • First Online:
Book cover Oculofacial, Orbital, and Lacrimal Surgery

  • 1335 Accesses

Abstract

Blepharoptosis is a common presenting complaint of patients presenting for medical evaluation. As etiologies vary from acute neurologic issues to mechanical age-related changes, proper diagnosis is essential. Blepharoptosis may be a sign of impending neurologic or systemic disease, further warranting diagnostic evaluations. Proper treatment relies on accurate diagnosis of the underlying cause of blepharoptosis. This chapter discusses some of the most frequently seen causes of blepharoptosis both in adults and children, in particular focusing on the more concerning, “not to be missed” etiologies such as acute third nerve palsy, Horner’s syndrome, and myasthenia gravis. Typical presentations and diagnostic evaluations are discussed. Less frequent myopathic and neurologic causes of blepharoptosis as well as congenital blepharoptosis are included as well.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Rush JA, Younge BR. Paralysis of cranial nerves III, IV, and VI. Cause and prognosis in 1,000 cases. Arch Ophthalmol. 1981;99(1):76–9.

    Article  CAS  PubMed  Google Scholar 

  2. Keane JR, Ahmadi J. Most diabetic third nerve palsies are peripheral. Neurology. 1998;51(5):1510.

    Article  CAS  PubMed  Google Scholar 

  3. Keane JR. Third nerve palsy: analysis of 1400 personally-examined inpatients. Can J Neurol Sci. 2010;37(5):662–70.

    Article  PubMed  Google Scholar 

  4. Kung NH, Van Stavern GP. Isolated ocular motor nerve palsies. Semin Neurol. 2015;35(5):539–48.

    Article  PubMed  Google Scholar 

  5. Elmalem VI, Hudgins PA, Bruce BB, Newman NJ, Biousse V. Underdiagnosis of posterior communicating artery aneurysm in noninvasive brain vascular studies. J Neuroophthalmol. 2011;31(2):103–9.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Maloney WF, Younge BR, Moyer NJ. Evaluation of the causes and accuracy of pharmacologic localization in Horner’s syndrome. Am J Ophthalmol. 1980;90(3):394–402.

    Article  CAS  PubMed  Google Scholar 

  7. Koc F, Kavuncu S, Kansu T, Acaroglu G, Firat E. The sensitivity and specificity of 0.5% apraclonidine in the diagnosis of oculosympathetic paresis. Br J Ophthalmol. 2005;89(11):1442–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Glatt HJ, Putterman AM, Fett DR. Muller’s muscle-conjunctival resection procedure in the treatment of ptosis in Horner’s syndrome. Ophthalmic Surg. 1990;21(2):93–6.

    CAS  PubMed  Google Scholar 

  9. Pang NK, Newsom RW, Oestreicher JH, Chung HT, Harvey JT. Fasanella-Servat procedure: indications, efficacy, and complications. Can J Ophthalmol. 2008;43(1):84–8.

    Article  PubMed  Google Scholar 

  10. Baylis HI, Shorr N. Anterior tarsectomy reoperation for upper eyelid blepharoptosis or contour abnormalities. Am J Ophthalmol. 1977;84(1):67–71.

    Article  CAS  PubMed  Google Scholar 

  11. Zadikoff C, Lang AE. Apraxia in movement disorders. Brain. 2005;128(Pt 7):1480–97.

    Article  PubMed  Google Scholar 

  12. Lee KC, Finley R, Miller B. Apraxia of lid opening: dose-dependent response to carbidopa-levodopa. Pharmacotherapy. 2004;24(3):401–3.

    Article  PubMed  Google Scholar 

  13. Kanazawa M, Shimohata T, Sato M, Onodera O, Tanaka K, Nishizawa M. Botulinum toxin a injections improve apraxia of eyelid opening without overt blepharospasm associated with neurodegenerative diseases. Mov Disord. 2007;22(4):597–8.

    Article  PubMed  Google Scholar 

  14. Grob D, Arsura EL, Brunner NG, Namba T. The course of myasthenia gravis and therapies affecting outcome. Ann N Y Acad Sci. 1987;505:472–99.

    Article  CAS  PubMed  Google Scholar 

  15. Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 2009;8(5):475–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Sethi KD, Rivner MH, Swift TR. Ice pack test for myasthenia gravis. Neurology. 1987;37(8):1383–5.

    Article  CAS  PubMed  Google Scholar 

  17. Fakiri MO, Tavy DL, Hama-Amin AD, Wirtz PW. Accuracy of the ice test in the diagnosis of myasthenia gravis in patients with ptosis. Muscle Nerve. 2013;48(6):902–4.

    Article  PubMed  Google Scholar 

  18. Cromar A, Lozier BK, Haven TR, Hill HR. Detection of acetylcholine receptor blocking antibodies by flow cytometry. Am J Clin Pathol. 2016;145(1):81–5.

    Article  CAS  PubMed  Google Scholar 

  19. Guptill JT, Sanders DB, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve. 2011;44(1):36–40.

    Article  PubMed  Google Scholar 

  20. Gwathmey KG, Burns TM. Myasthenia gravis. Semin Neurol. 2015;35(4):327–39.

    Article  PubMed  Google Scholar 

  21. Mao ZF, Mo XA, Qin C, Lai YR, Hackett ML. Incidence of thymoma in myasthenia gravis: a systematic review. J Clin Neurol. 2012;8(3):161–9.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Mao ZF, Yang LX, Mo XA, Qin C, Lai YR, He NY, et al. Frequency of autoimmune diseases in myasthenia gravis: a systematic review. Int J Neurosci. 2011;121(3):121–9.

    Article  PubMed  Google Scholar 

  23. Kupersmith MJ. Ocular myasthenia gravis: treatment successes and failures in patients with long-term follow-up. J Neurol. 2009;256(8):1314–20.

    Article  PubMed  Google Scholar 

  24. Suominen T, Bachinski LL, Auvinen S, Hackman P, Baggerly KA, Angelini C, et al. Population frequency of myotonic dystrophy: higher than expected frequency of myotonic dystrophy type 2 (DM2) mutation in Finland. Eur J Hum Genet. 2011;19(7):776–82.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Romeo V. Myotonic dystrophy type 1 or Steinert’s disease. Adv Exp Med Biol. 2012;724:239–57.

    Article  CAS  PubMed  Google Scholar 

  26. Day JW, Ricker K, Jacobsen JF, Rasmussen LJ, Dick KA, Kress W, et al. Myotonic dystrophy type 2: molecular, diagnostic and clinical spectrum. Neurology. 2003;60(4):657–64.

    Article  CAS  PubMed  Google Scholar 

  27. Chen RS, Huang CC, Chu NS. Coenzyme Q10 treatment in mitochondrial encephalomyopathies. Short-term double-blind, crossover study. Eur Neurol. 1997;37(4):212–8.

    Article  CAS  PubMed  Google Scholar 

  28. Rajabi MT, Tabatabaie SZ, Rajabi MB, Abrishami Y, Hosseini SS, Oestreicher J. Management of myogenic ptosis in chronic progressive external ophthalmoplegia. Iran J Neurol. 2014;13(3):185–7.

    PubMed  PubMed Central  Google Scholar 

  29. De Braekeleer M. Hereditary disorders in Saguenay-Lac-St-Jean (Quebec, Canada). Hum Hered. 1991;41(3):141–6.

    Article  PubMed  Google Scholar 

  30. Blumen SC, Nisipeanu P, Sadeh M, Asherov A, Blumen N, Wirguin Y, et al. Epidemiology and inheritance of oculopharyngeal muscular dystrophy in Israel. Neuromuscul Disord. 1997;7(Suppl 1):S38–40.

    Article  PubMed  Google Scholar 

  31. Harish P, Malerba A, Dickson G, Bachtarzi H. Progress on gene therapy, cell therapy, and pharmacological strategies toward the treatment of oculopharyngeal muscular dystrophy. Hum Gene Ther. 2015;26(5):286–92.

    Article  CAS  PubMed  Google Scholar 

  32. Hosal BM, Tekeli O, Gursel E. Eyelid malpositions after cataract surgery. Eur J Ophthalmol. 1998;8(1):12–5.

    Article  CAS  PubMed  Google Scholar 

  33. Altieri M, Truscott E, Kingston AE, Bertagno R, Altieri G. Ptosis secondary to anterior segment surgery and its repair in a two-year follow-up study. Ophthalmologica. 2005;219(3):129–35.

    Article  PubMed  Google Scholar 

  34. Loeffler M, Solomon LD, Renaud M. Postcataract extraction ptosis: effect of the bridle suture. J Cataract Refract Surg. 1990;16(4):501–4.

    Article  CAS  PubMed  Google Scholar 

  35. Kitazawa T. Hard contact lens wear and the risk of acquired blepharoptosis: a case-control study. Eplasty. 2013;19(13):e30.

    Google Scholar 

  36. Bleyen I, Hiemstra CA, Devogelaere T, van den Bosch WA, Wubbels RJ, Paridaens DA. Not only hard contact lens wear but also soft contact lens wear may be associated with blepharoptosis. Can J Ophthalmol. 2011;46(4):333–6.

    Article  PubMed  Google Scholar 

  37. Mun KS, Pailoor J, Reddy SC. Primary localised deep cutaneous amyloidosis of the eyelid. Malays J Pathol. 2005;27(2):113–5.

    PubMed  Google Scholar 

  38. Yang CC, Vagefi MR, Davis D, Mamalis N, Anderson RL, McCann J. Gouty tophus of the upper eyelid. Ophthal Plast Reconstr Surg. 2008;24(5):404–6.

    Article  PubMed  Google Scholar 

  39. Scuderi G, Mutolo MG, Lambiase A, Recupero SM. Surgical management of concomitant ptosis and cicatricial entropion in a patient with ocular cicatricial pemphigoid. Int J Ophthalmol Clin Res. 2015;2(3):32.

    Google Scholar 

  40. Koursh DM, Modjtahedi SP, Selva D, Leibovitch I. The blepharochalasis syndrome. Surv Ophthalmol. 2009;54(2):235–44.

    Article  PubMed  Google Scholar 

  41. Langley KE, Patrinely JR, Anderson RL, Thiese SM. Unilateral blepharochalasis. Ophthalmic Surg. 1987;18(8):594–8.

    CAS  PubMed  Google Scholar 

  42. Sarnat HB, Flores-Sarnat L, Kherani F. Palpebral myopathology of congenital ptosis. Presented at the 57th annual meeting of the German Society for Neuropathology and Neuroanatomy. Erlangen, Germany; 2012.

    Google Scholar 

  43. Crawford JS. Repair of ptosis using frontalis muscle and fascia lata: a 20-year review. Ophthalmic Surg. 1977;8(4):31–40.

    CAS  PubMed  Google Scholar 

  44. Hamed LM. Associated neurologic and ophthalmologic findings in congenital oculomotor nerve palsy. Ophthalmology. 1991;98(5):708–14.

    Article  CAS  PubMed  Google Scholar 

  45. Langmann A, Lindner S. Congenital third nerve palsy in septo-optic dysplasia. Br J Ophthalmol. 2004;88(7):969.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Smith SJ, Diehl N, Leavitt JA, Mohney BG. Incidence of pediatric Horner syndrome and the risk of neuroblastoma: a population-based study. Arch Ophthalmol. 2010;128(3):324–9.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Yamada K, Hunter DG, Andrews C, Engle EC. A novel KIF21A mutation in a patient with congenital fibrosis of the extraocular muscles and Marcus Gunn jaw-winking phenomenon. Arch Ophthalmol. 2005;123(9):1254–9.

    Article  CAS  PubMed  Google Scholar 

  48. Kirkham TH. Familial Marcus Gunn phenomenon. Br J Ophthalmol. 1969;53(4):282–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Demirci H, Frueh BR, Nelson CC. Marcus Gunn jaw-winking synkinesis: clinical features and management. Ophthalmology. 2010;117(7):1447–52.

    Article  PubMed  Google Scholar 

  50. Bowyer JD, Sullivan TJ. Management of Marcus Gunn jaw winking synkinesis. Ophthal Plast Reconstr Surg. 2004;20(2):92–8.

    Article  PubMed  Google Scholar 

  51. Ibrahim HA. Use of the levator muscle as a frontalis sling. Ophthal Plast Reconstr Surg. 2007;23(5):376–80.

    Article  PubMed  Google Scholar 

  52. Fukushima Y, Wakui K, Nishida T, Ueoka Y. Blepharophimosis sequence and de novo balanced autosomal translocation [46,XY,t(3;4)(q23;p15.2)]: possible assignment of the trait to 3q23. Am J Med Genet. 1991;40(4):485–7.

    Article  CAS  PubMed  Google Scholar 

  53. De Baere E, Dixon MJ, Small KW, Jabs EW, Leroy BP, Devriendt K, et al. Spectrum of FOXL2 gene mutations in blepharophimosis-ptosis-epicanthus inversus (BPES) families demonstrates a genotype – phenotype correlation. Hum Mol Genet. 2001;10(15):1591–600.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Departments of Ophthalmology and Neurology, Rush University Medical Center, Chicago, IL, USA

    Alexander Grigalunas & Aimee J. Szewka

Authors
  1. Alexander Grigalunas
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Aimee J. Szewka
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Aimee J. Szewka .

Editor information

Editors and Affiliations

  1. Eyelid and Facial Plastic and Reconstructive Surgery and MediSpa, Diseases and Surgery of the Orbit & Lacrimal System Neuro-ophthalmology, Glenview/Chicago, IL, USA

    Adam J. Cohen

  2. Oculoplastics, Orbital, and Cosmetic Facial Surgery, University of Wisconsin, Madison Department of Ophthalmology and Visual Sciences, Madison, WI, USA

    Cat Nguyen Burkat

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Grigalunas, A., Szewka, A.J. (2019). Etiologies of Blepharoptosis. In: Cohen, A., Burkat, C. (eds) Oculofacial, Orbital, and Lacrimal Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-14092-2_2

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-14092-2_2

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-14090-8

  • Online ISBN: 978-3-030-14092-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation