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JAMA Ophthalmology Clinical Challenge
September 10, 2020

Progressive Vision Loss in a 62-Year-Old Woman

Author Affiliations
  • 1University of Miami Miller School of Medicine, Bascom Palmer Eye Institute, Department of Ophthalmology, Miami, Florida
JAMA Ophthalmol. Published online September 10, 2020. doi:10.1001/jamaophthalmol.2020.1746

Case

A 62-year-old woman with a history of chronic angle-closure glaucoma and bilateral peripheral iridotomies sought a second opinion for worsening visual acuity in the left eye for 2 months. She had been treated for glaucoma for approximately 1 year. Review of systems was negative for previous episodes of eye pain, redness, or emesis. Her medical history included hyperlipidemia, obesity, and prior cataract surgery in both eyes. On examination, best-corrected visual acuity was 20/20 OD and 20/80 OS. The patient’s pupils were equal, round, and reactive to light without a relative afferent pupillary defect. Intraocular pressure was 11 mm Hg bilaterally without medication. Slitlamp findings were notable for patent peripheral iridotomies, open angles on gonioscopy, and an asymmetrically enlarged cup-disc ratio with mild pallor of the left optic nerve. Color vision was decreased in the left eye. Visual field (VF) testing as well as ganglion cell layer (GCL) segmentation by optical coherence tomography (OCT) were obtained for both eyes (Figure 1).

Figure 1.  
Optical coherence tomography ganglion cell layer thickness map shows nasal thinning of the right eye (A) and diffuse thinning of the left eye (B). In the right eye, the relative thinning within the nasal macula compared with the temporal macula suggests injury to the papillomacular bundle. In the left eye, the diffuse thinning suggests diffuse optic nerve injury (Heidelberg Spectralis; Heidelberg). Insets show Humphrey 24-2 perimetry with minimal nonspecific defects in the right eye and a temporal scotoma in the left eye.

Optical coherence tomography ganglion cell layer thickness map shows nasal thinning of the right eye (A) and diffuse thinning of the left eye (B). In the right eye, the relative thinning within the nasal macula compared with the temporal macula suggests injury to the papillomacular bundle. In the left eye, the diffuse thinning suggests diffuse optic nerve injury (Heidelberg Spectralis; Heidelberg). Insets show Humphrey 24-2 perimetry with minimal nonspecific defects in the right eye and a temporal scotoma in the left eye.

Box Section Ref ID

What Would You Do Next?

  1. Refer to a retina specialist owing to retinal thinning

  2. Start intraocular pressure–lowering medications owing to GCL loss

  3. Order a carotid doppler

  4. Order brain magnetic resonance imaging

Discussion

Diagnosis

Nonglaucomatous optic neuropathy secondary to a pituitary adenoma

What to Do Next

D. Order brain magnetic resonance imaging

Discussion

Optic chiasm compression from a pituitary adenoma classically produces bitemporal vision loss. Most adenomas are nonfunctional or prolactinomas.1,2 Adenomas causing 3 mm or more of chiasm displacement are associated with VF defects and initially compress inferior nerve fibers leading to superior VF defects.3 Diagnosis is confirmed after tumor resection and pathologic analysis. In a 2019 study, most patients undergoing resection presented with vision loss (61.7%), followed by headache (40.0%) or endocrine dysfunction (21.8%).1

Optic neuropathies are associated with loss of retinal ganglion cells and retinal nerve fiber layer (RNFL) axons. Nasal retinal nerve fibers decussate at the optic chiasm, supplying the temporal visual hemifield. The papillomacular bundle describes the central macular retinal nerve fibers, which lie just temporal to the optic nerve; these fibers are typically not affected in glaucoma. Topographically, papillomacular bundle injury would demonstrate nasal thinning on macular GCL OCT segmentation and temporal thinning on circumpapillary RNFL OCT scans.4 The distribution of nasal retinal nerve fibers also explains the bow tie optic nerve pallor associated with compressive chiasmal lesions.4,5 Other findings may include relative afferent pupillary defect, dyschromatopsia, and bitemporal hemianopsia. However, most patients do not present with complete bitemporal hemianopsia, and, therefore, chiasmal lesions must be suspected in cases of incomplete temporal field defects or those crossing the vertical midline due to extrachiasmal extension.3 In a 2018 study, OCT GCL and RNFL analyses were able to detect chiasmal lesions significantly earlier than by clinical examination or VF testing.6 Greater preoperative RNFL/GCL thicknesses are associated with better visual recovery after tumor resection.4 Tumor recurrence is monitored at least yearly with neuroimaging and VF testing.2

The diagnostic key in this case is to identify that the left temporal scotoma respects the vertical meridian. Such VF defects result from lesions at or posterior to the optic chiasm, while horizontal field defects typically have intraocular causes such as retinal pathologies (choice A). For example, branch retinal artery occlusions, possibly due to emboli, would feature horizontal VF deficits and inner retinal atrophy (choice C). For cases of early or nonspecific VF defects, nasal GCL thinning is extremely suggestive of nonglaucomatous optic neuropathy rather than glaucoma; the latter typically starts with superotemporal or inferotemporal GCL thinning (choice B).4 Despite the absence of a classic bitemporal hemianopsia, optic chiasm pathology was suspected given the nasal GCL thinning observed in the right eye. Ganglion cell layer loss in the left eye was diffuse given the advanced degree of atrophy. When compressive optic neuropathy is suspected, these lesions require immediate neuroimaging to guide further management (choice D).

Patient Outcome

Brain magnetic resonance imaging revealed a 40 × 23 × 22 mm sellar mass compressing the optic chiasm from below (Figure 2). One week later, the patient underwent successful transsphenoidal pituitary tumor resection. Biopsy confirmed the diagnosis of a nonfunctioning gonadotroph pituitary adenoma. Repeat perimetric testing showed stable VF loss a few months later. Final best-corrected visual acuity was 20/20 OD and 20/50 OS.

Figure 2.  
Contrast enhancing T1-weighted coronal magnetic resonance image revealing a 40 × 23 × 22 mm sellar lesion (arrowhead) compressing the optic chiasm.

Contrast enhancing T1-weighted coronal magnetic resonance image revealing a 40 × 23 × 22 mm sellar lesion (arrowhead) compressing the optic chiasm.

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Article Information

Corresponding Author: Swarup S. Swaminathan, MD, Bascom Palmer Eye Institute, 900 NW 17th St, Miami, FL 33136 (sswaminathan@miami.edu).

Published Online: September 10, 2020. doi:10.1001/jamaophthalmol.2020.1746

Conflict of Interest Disclosures: None reported.

Funding/Support: Bascom Palmer Eye Institute receives support from a National Institutes of Health Center Core Grant (P30EY014801) and a Research to Prevent Blindness Unrestricted Grant.

Role of the Funder/Sponsor: The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

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

References
1.
Azab  MA, O’Hagan  M, Abou-Al-Shaar  H, Karsy  M, Guan  J, Couldwell  WT.  Safety and outcome of transsphenoidal pituitary adenoma resection in elderly patients.   World Neurosurg. 2019;122:e1252-e1258. doi:10.1016/j.wneu.2018.11.024PubMedGoogle ScholarCrossref
2.
Molitch  ME.  Diagnosis and treatment of pituitary adenomas: a review.   JAMA. 2017;317(5):516-524. doi:10.1001/jama.2016.19699PubMedGoogle ScholarCrossref
3.
Lee  IH, Miller  NR, Zan  E,  et al.  Visual defects in patients with pituitary adenomas: the myth of bitemporal hemianopsia.   AJR Am J Roentgenol. 2015;205(5):W512-8. doi:10.2214/AJR.15.14527PubMedGoogle Scholar
4.
Micieli  JA, Newman  NJ, Biousse  V.  The role of optical coherence tomography in the evaluation of compressive optic neuropathies.   Curr Opin Neurol. 2019;32(1):115-123. doi:10.1097/WCO.0000000000000636PubMedGoogle ScholarCrossref
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Foroozan  R.  Chiasmal syndromes.   Curr Opin Ophthalmol. 2003;14(6):325-331. doi:10.1097/00055735-200312000-00002PubMedGoogle ScholarCrossref
6.
Blanch  RJ, Micieli  JA, Oyesiku  NM, Newman  NJ, Biousse  V.  Optical coherence tomography retinal ganglion cell complex analysis for the detection of early chiasmal compression.   Pituitary. 2018;21(5):515-523. doi:10.1007/s11102-018-0906-2PubMedGoogle ScholarCrossref
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