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Progressively Worsening Unilateral Ptosis in a Woman in Her 60s

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.

On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.

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Unilateral ptosis secondary to mantle cell lymphoma of the left lacrimal gland

D. Obtain orbital imaging with intravenous contrast

The etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.

Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.

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Article Information

Corresponding Author: Shannon S. Joseph, MD, MSc, Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, 1000 Wall St, Ann Arbor, MI 48105 (sjshan@med.umich.edu).

Published Online: March 24, 2022. doi:10.1001/jamaophthalmol.2021.5716

Conflict of Interest Disclosures: None reported.

Meeting Presentation: Contents of this article were presented in part at the Women in Ophthalmology 2021 Summer Symposium; August 27, 2021; in Amelia Island, Florida.

Additional Contributions: We thank the patient for granting permission to publish this information.

Kim  JS , Liss  J .  Masses of the lacrimal gland: evaluation and treatment.   J Neurol Surg B Skull Base. 2021;82(1):100-106. doi:10.1055/s-0040-1722700PubMedGoogle ScholarCrossref
Rose  GE , Wright  JE .  Pleomorphic adenoma of the lacrimal gland.   Br J Ophthalmol. 1992;76(7):395-400. doi:10.1136/bjo.76.7.395PubMedGoogle ScholarCrossref
Gündüz  AK , Yeşiltaş  YS , Shields  CL .  Overview of benign and malignant lacrimal gland tumors.   Curr Opin Ophthalmol. 2018;29(5):458-468. doi:10.1097/ICU.0000000000000515PubMedGoogle ScholarCrossref
Kim  JS , Al-Lozi  A , Leyngold  IM .  Malignant orbital tumors: current approach to diagnosis and management.   Curr Ophthalmol Rep. 2021;9:1-9. doi:10.1007/s40135-020-00262-wGoogle ScholarCrossref
Aronow  ME .  Ocular adnexal lymphoma: evidence-based treatment approach.   Int Ophthalmol Clin. 2015;55(1):97-109. doi:10.1097/IIO.0000000000000049PubMedGoogle ScholarCrossref
Knudsen  MKH , Rasmussen  PK , Coupland  SE ,  et al.  Clinicopathological features of ocular adnexal mantle-cell lymphoma in an international multicenter cohort.   JAMA Ophthalmol. 2017;135(12):1367-1374. doi:10.1001/jamaophthalmol.2017.4810PubMedGoogle ScholarCrossref
Dreyling  M , Campo  E , Hermine  O ,  et al; ESMO Guidelines Committee.  Newly diagnosed and relapsed mantle cell lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.   Ann Oncol. 2017;28(4)(suppl_4):iv62-iv71. doi:10.1093/annonc/mdx223PubMedGoogle ScholarCrossref
Jenkins  C , Rose  GE , Bunce  C ,  et al.  Clinical features associated with survival of patients with lymphoma of the ocular adnexa.   Eye (Lond). 2003;17(7):809-820. doi:10.1038/sj.eye.6700379PubMedGoogle ScholarCrossref
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Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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