• Users Online: 49
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 179-181

Posterior ethmoidal mucocele with orbital apex syndrome in a young male: A rare case report


Department of Ophthalmology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore, Tamil Nadu, India

Date of Submission04-Aug-2022
Date of Acceptance12-Oct-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
S Saudhamini
Department of Ophthalmology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore - 641 004, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1917_22

Rights and Permissions
  Abstract 


A 39-year-old male presented with painless diminution of vision in the right eye for 10 days, which was associated with drooping of the upper lid for 4 days. Vision was perception of light with 4 mm ptosis and mild proptosis and 3.5 mm pupil with grade 1 relative afferent pupillary defect (RAPD). Extraocular movements showed abduction and elevation limitation of -2, adduction and depression limitation of -1 in the right eye. Magnetic resonance imaging (MRI) showed right posterior ethmoidal mucocele causing optic nerve compression. Marsupialization of mucocele and optic nerve decompression were done. Post-surgery, vision, ptosis, and extraocular movements improved and pupil remained the same owing to the mechanical effect of the mucocele. Early identification and intervention of posterior ethmoidal mucocele is crucial in preventing visual compromise.

Keywords: Marsupialization of mucocele, orbital apex syndrome, posterior ethmoidal mucocele


How to cite this article:
Saudhamini S, Ramasamy V, Padmanaban S, Sundar D, Ravisankar V, Vignesh N. Posterior ethmoidal mucocele with orbital apex syndrome in a young male: A rare case report. Indian J Ophthalmol Case Rep 2023;3:179-81

How to cite this URL:
Saudhamini S, Ramasamy V, Padmanaban S, Sundar D, Ravisankar V, Vignesh N. Posterior ethmoidal mucocele with orbital apex syndrome in a young male: A rare case report. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:179-81. Available from: https://www.ijoreports.in/text.asp?2023/3/1/179/368193



Orbital apex syndrome (OAS) is characterized by vision loss from optic neuropathy and ophthalmoplegia due to the involvement of ocular motor nerves in the anatomical region of the orbital apex.[1]

OAS can be caused by a variety of etiologies, including inflammation, infection, vasculitis, and malignancy. An ethmoid sinus mucocele causing OAS has rarely been reported.[2],[3],[4] We report a case of OAS caused by an ethmoid sinus mucocele, which was successfully managed by functional endoscopic sinus surgery (FESS) with good visual recovery postoperatively.


  Case Report Top


A 39-year-old male presented with painless and progressive diminution of vision in the right eye (RE) for 10 days associated with drooping of upper eyelid and headache for 4 days.

On examination, visual acuity in the RE was perception of light and appreciating projection of rays in all four quadrants (PL+, PR+) and in the left eye (LE) was 20/20.

RE had 4-mm lid ptosis and axial proptosis difference of 2 mm was measured with Hertel's exophthalmometer (RE 22 mm, LE 20 mm). Pupil was 3.5 mm with grade 1 relative afferent pupillary defect (RAPD). Extraocular movements showed abduction and elevation limitation of -2, adduction and depression limitation of -1 in the right eye. Posterior segment examination was within normal limits [Figure 1].
Figure 1: Pre-operatively right eye extraocular movements showed abduction and elevation limitation of -2, adduction and depression limitation of -1

Click here to view


LE examination was unremarkable.

The patient was further referred for a detailed ear, nose, throat (ENT) examination. Diagnostic nasal endoscopy showed mucopus in the right middle meatus.

Based on the clinical examination, a diagnosis of OAS was made and magnetic resonance imaging (MRI) was advised to determine the etiology.

MRI brain and orbit showed a well-defined, large, expansile lesion of 3.5 × 3.3 × 2.5 mm with peripheral rim enhancement, involving the wall of right ethmoid air cells. The lesion was causing compression and lateral displacement of the right medial rectus and right optic nerve. It was hypointense on T1, hyperintense on T2, and showed rim enhancement on post-contrast imaging [Figure 2].
Figure 2: MRI images of right posterior ethmoidal mucocele compressing the optic nerve, hypointense on T1 (a), hyperintense on T2 (b) and rim enhancement post-contrast (c)

Click here to view


The patient was diagnosed with right posterior ethmoidal mucocele causing OAS. He was taken up for right side sinusotomy with FESS and mucocele marsupialization with right optic nerve decompression.

Postoperatively, the patient was started on intravenous steroids and antibiotics.

On the first post-op day: RE vision was counting fingers at 1 m, pupil showed grade 1 RAPD, extraocular movements showed limitation in abduction of −2, levo-elevation, and depression of −1 [Figure 3].
Figure 3: Post-operatively right eye extraocular movements showed abduction limitation of -2, levo-elevation and depression limitation of -1

Click here to view


After 1month: RE best corrected visual acuity was 20/20, colour vision, contrast sensitivity and extraocular movements were normal, pupil showed grade 1 RAPD with temporal disc pallor.


  Discussion Top


Paranasal sinus mucoceles are slow-growing benign cystic lesions containing mucoid secretions that can lead to sinus expansion, bony erosion, and extension into the orbit, cranial cavity, and nasopharynx. They represent approximately 8% of all orbital tumors, with the frontal sinus mucoceles being the most common (50%), followed by frontoethmoidal (31%), ethmoidal (16%), and sphenoidal (3%) mucoceles.[5]

Paranasal sinus mucoceles typically occur in the third to fourth decades of life, with a mild male predisposition.[6],[7] The clinical symptoms of sinus mucoceles are dependent on the region of the mass effect produced. Frontoethmoidal mucoceles tend to cause a mass effect on the ipsilateral orbit, resulting in proptosis, diplopia, and possible periorbital swelling, whereas posterior ethmoid and sphenoid mucoceles more commonly cause optic canal compression and lead to visual symptoms.[8] Surgical excision is the treatment of choice and is necessary to prevent a recurrence.

Paranasal sinus mucoceles affect the optic nerve by either mechanical compression causing ischemic injury or by direct extension of the inflammatory process to the optic nerve.[5] It has been suggested that cases with a gradual decrease in visual acuity are caused by circulatory disorders due to direct pressure of the mucocele, whereas cases that show rapid loss of visual acuity are caused by direct spread of infection or inflammation to the optic nerve.[9]

MRI is superior to computed tomography (CT) in demonstrating the relationship between the mucocele and the adjacent soft tissue, as well as differentiating mucoceles from other soft tissue neoplasms.[10] On MRI, the appearance of mucoceles varies because of alterations in the protein concentration of the obstructed mucoid secretion. High signal intensity on T2-weighted and low intensity on T1-weighted MRI typically differentiate mucoceles from other masses such as tumors. The higher protein content of mucocele mucus may increase the T1 signal intensity on MRI, with contrast enhancement localized to the peripheral cystic walls.[11]

Fleissig et al.[3] presented a 53-year-old female who underwent multiple FESS for chronic sinusitis and presented with an Onodi cell mucocele. She underwent cyst marsupialization and aspiration of a clear mucoid substance. Post-surgery, no recovery of the optic nerve function was noted, and it was suspected that ischemia of the posterior optic nerve may have occurred.

Dai et al.[4] presented a 59-year-old female who had a left ethmoid sinus mucocele compressing the optic nerve. After endoscopic sinus surgery, vision and movements returned to normal.

Our case showed right posterior ethmoidal mucocele causing right OAS and underwent FESS with marsupialization of mucocele and optic nerve decompression. After surgery, vision, ptosis, and extraocular movements improved and pupil remained the same owing to the mechanical effect of the mucocele.


  Conclusion Top


Differential diagnosis of posterior ethmoidal mucocele has to be considered in all cases of OAS, with or without history of sinusitis or status post-FESS.

Management of mucoceles extending into the orbit needs careful coordination between otolaryngologists and ophthalmologists. With timely care, symptoms can be resolved with relatively low rates of complication or recurrence.

Hence, early identification and management of posterior ethmoidal mucocele are crucial in preventing visual compromise.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Badakere A, Patil-Chhablani P. Orbital apex syndrome: A review. Eye Brain 2019;11:63-72.  Back to cited text no. 1
    
2.
Kumagai M, Hashimoto S, Suzuki H, Matsuura K, Takahashi E. Orbital apex syndrome caused by sphenoethmoid mucocele. Auris Nasus Larynx 2003;30:295-7.  Back to cited text no. 2
    
3.
Fleissig E, Spierer O, Koren I, Leibovitch I. Blinding orbital apex syndrome due to onodi cell mucocele. Case Rep Ophthalmol Med 2014;2014:453789. doi: 10.1155/2014/453789.  Back to cited text no. 3
    
4.
Dai L-B, Cheng C, Bian J, Han H-M, Shen L-F, Zhou S-H, et al. Orbital apex syndrome caused by ethmoid sinus mucocele: A case report and review of literature. Int J Clin Exp Med 2017;10:1434-8  Back to cited text no. 4
    
5.
Wang TJ, Liao SL, Jou JR, Lin LL. Clinical manifestations and management of orbital mucoceles: The role of ophthalmologists. Jpn J Ophthalmol 2005;49:239-45.  Back to cited text no. 5
    
6.
Lee TJ, Li SP, Fu CH, Huang CC, Chang PH, Chen YW, et al. Extensive paranasal sinus mucoceles: A 15-year review of 82 cases. Am J Otolaryngol 2009;30:234-8.  Back to cited text no. 6
    
7.
Conboy PJ, Jones NS. The place of endoscopic sinus surgery in the treatment of paranasal sinus mucocoeles. Clin Otolaryngol Allied Sci 2003;28:207-10.  Back to cited text no. 7
    
8.
Ting MYL, Shan M, Gantz O, Zhang-Nunes S, Wrobel B. Optic neuropathy due to an ethmoid mucocele: A case report and literature review. Case Rep Ophthalmol 2019;10:227-34.  Back to cited text no. 8
    
9.
Moriyama H, Nakajima T, Honda Y. Studies on mucocoeles of the ethmoid and sphenoid sinuses: Analysis of 47 cases. J Laryngol Otol 1992;106:23-7.  Back to cited text no. 9
    
10.
Capra GG, Carbone PN, Mullin DP. Paranasal sinus mucocele. Head Neck Pathol 2012;6:369-72.  Back to cited text no. 10
    
11.
Victores A, Foroozan R, Takashima M. Recurrent Onodi cell mucocele: Rare cause of 2 different ophthalmic complications. Otolaryngol Head Neck Surg 2012;146:338-9.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed44    
    Printed4    
    Emailed0    
    PDF Downloaded13    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]