April 2014
Volume 55, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2014
Diplopia as a Presenting Symptom of Adenoid Cystic Carcinoma of the Head and Neck
Author Affiliations & Notes
  • Omar Ozgur
    Ophthalmology, New York Eye & Ear Infirmary, New York, NY
  • Kateki Vinod
    Ophthalmology, New York Eye & Ear Infirmary, New York, NY
  • Peter Chang
    Ophthalmology, New York Eye & Ear Infirmary, New York, NY
  • Payal Patel
    Ophthalmology, New York Eye & Ear Infirmary, New York, NY
  • Lisabeth Hall
    Ophthalmology, New York Eye & Ear Infirmary, New York, NY
  • Elizabeth Maher
    Ophthalmology, New York Eye & Ear Infirmary, New York, NY
  • Footnotes
    Commercial Relationships Omar Ozgur, None; Kateki Vinod, None; Peter Chang, None; Payal Patel, None; Lisabeth Hall, None; Elizabeth Maher, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science April 2014, Vol.55, 4095. doi:
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      Omar Ozgur, Kateki Vinod, Peter Chang, Payal Patel, Lisabeth Hall, Elizabeth Maher; Diplopia as a Presenting Symptom of Adenoid Cystic Carcinoma of the Head and Neck. Invest. Ophthalmol. Vis. Sci. 2014;55(13):4095.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract
 
Purpose
 

To report 2 cases of adenoid cystic carcinoma (ACC) of the head and neck manifesting as multiple cranial nerve (CN) palsies causing diplopia as the presenting symptom, and to propose pathophysiologic mechanisms for this atypical presentation.

 
Methods
 

Two patients with ACC presenting with binocular diplopia were identified. Patient 1 (P1) was a 49-year-old man with vertical diplopia for 2 months, and right (R) forehead numbness for 2 weeks. Vision was 20/20 OU, and abduction and depression deficit OD were consistent with R CN IV palsy, with hypoesthesia in the R CN V1 distribution. Patient 2 (P2) was a 49-year-old woman with horizontal diplopia for 1 day. Vision was 20/30 OD and 20/25 OS. R facial hypoesthesia, abduction deficit OD, and R facial weakness were consistent with CN V, VI, and VII palsies. She soon developed R CN IV palsy, followed by R CN III palsy.

 
Results
 

P1’s MRI showed R superolateral orbital inflammation with normal lacrimal gland (LG) without localizable mass. After a failed trial of oral steroids for presumed orbital pseudotumor, repeat MRI showed probable extension into the cavernous sinus (CS). R orbital biopsy showed orbital ACC from ectopic LG tissue. P2’s MRI showed an enhancing mass in the R CS. Endonasal biopsy demonstrated ACC. Fine needle aspiration biopsy confirmed the R submandibular gland as the primary source. Both patients were referred to oncology.

 
Conclusions
 

ACC is a rare malignant epithelial tumor that, in the head and neck, most commonly arises in the salivary glands, and less often, the LG. We describe 2 cases of ACC presenting atypically with diplopia. In P1, invasion of the superior orbital fissure likely accounts for CN IV involvement, resulting in vertical diplopia. In P2, perineural spread by ACC into the CS affecting CN VI resulted in horizontal diplopia. Review of the literature confirms that diplopia as a presenting symptom of ACC is very rare. To our knowledge, only 8 reports exist of ACC presenting with diplopia, due either to mass effect, perineural invasion, or CS involvement.

 
 
Figure 1a. P1 with left head tilt. 1b. Incisional biopsy, H&E stain, demonstrating tubular (yellow arrow) and cribriform growth pattern (red arrow). 1c. T1-weighted MRI orbits showing R superolateral orbital inflammation with normal LG.
 
Figure 1a. P1 with left head tilt. 1b. Incisional biopsy, H&E stain, demonstrating tubular (yellow arrow) and cribriform growth pattern (red arrow). 1c. T1-weighted MRI orbits showing R superolateral orbital inflammation with normal LG.
 
 
Figure 2a. P2 with abduction deficit OD. 2b. MRI brain and orbits demonstrating enhancement of CN III, IV, V1, and VI as they leave the CS (red oval).
 
Figure 2a. P2 with abduction deficit OD. 2b. MRI brain and orbits demonstrating enhancement of CN III, IV, V1, and VI as they leave the CS (red oval).
 
Keywords: 724 strabismus: etiology • 624 oncology • 522 eye movements  
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