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CASE REPORT
Year : 2021  |  Volume : 59  |  Issue : 2  |  Page : 184-186

Orbital dermoid presenting as ptosis with monocular elevation deficiency


Department of Orbit, Oculoplasty, Reconstructive and Aesthetics Sankara Nethralaya, Medical Research Foundation, Chennai, Tamil Nadu, India

Date of Submission13-May-2020
Date of Acceptance12-Oct-2020
Date of Web Publication24-Jun-2021

Correspondence Address:
Dr. Bipasha Mukherjee
Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, 18, College Road, Chennai - 600 006., Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_110_20

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  Abstract 


Orbital dermoids are the most common benign orbital cysts accounting for 14% of all orbital masses. They are choristomas resulting from entrapment of ectoderm into the mesenchymal tissue at sites of embryologic bony fusion. Their clinical presentations vary based on the location, size, and their effects on the adnexa and orbital structures. We report a case of orbital dermoid presenting as ptosis with monocular elevation deficiency (MED). A 9-year-old boy presented with drooping of left upper eyelid since birth for which frontalis suspension surgery was done elsewhere at 5 years of age. One year later, the ptosis recurred. On examination, child had severe ptosis, inferior dystopia, and restricted elevation in the left eye. A soft nontender mass was palpated in the superior quadrant of left orbit. Imaging was suggestive of dermoid. Excision biopsy confirmed the diagnosis. At 6-week follow-up, the child regained full elevation and is awaiting ptosis correction. Superior deep dermoids may clinically mimic MED. Management includes complete excision of cyst with correction of residual ptosis as staged procedure. This case is being reported for its rare presentation and to insist the importance of performing a thorough orbital examination in all cases of congenital ptosis to avoid mismanagement.

Keywords: Dermoid, monocular elevation deficiency, ptosis


How to cite this article:
Vijay V, Mukherjee B. Orbital dermoid presenting as ptosis with monocular elevation deficiency. TNOA J Ophthalmic Sci Res 2021;59:184-6

How to cite this URL:
Vijay V, Mukherjee B. Orbital dermoid presenting as ptosis with monocular elevation deficiency. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Jul 27];59:184-6. Available from: https://www.tnoajosr.com/text.asp?2021/59/2/184/319260



Orbital dermoid cysts are benign developmental choristomas arising due to sequestration of surface ectoderm at the lines of embryonic closure. They account for 14% of all orbital tumors.[1],[2],[3],[4] Dermoid cysts are slow-growing tumors containing a cystic cavity lined by stratified squamous epithelium with epidermal appendages such as hair follicles, sebaceous glands, and teeth. The clinical presentation depends on the location of the dermoid. We report a case of superior orbital dermoid presenting as congenital ptosis with monocular elevation deficiency (MED) in a young boy.

A 9-year-old boy presented with a history of drooping of left upper eyelid since birth. There was no history of trauma, diurnal variation, or double vision. Parents reported that left eye was operated elsewhere to correct the drooping at 5 years of age. His old medical records revealed a diagnosis of left eye severe congenital ptosis with poor LPS action with MED for which he had undergone left eye frontalis suspension with silicon rod. One year later, parents noticed recurrence of the left upper eyelid droop.

On examination, the best-corrected visual acuity was 6/6, N6 in the right eye and 6/9, N6 in the left eye with − 3.50 diopters of cylinder. His left eye was displaced inferiorly by 2 mm [Figure 1]A with 2 mm of proptosis (as measured with Hertel's exophthalmometer) and restricted elevation. Squint evaluation revealed orthoposition for both distance and near with fusion noted in all gazes. He had severe ptosis with a margin reflex distance 1 of −1 mm [Figure 1]B and levator palpebrae superioris (LPS) action of 2 mm in his left eye. On palpation, a soft to firm, nontender, noncompressible, nonpulsatile cystic mass was palpated in the superomedial quadrant of the left orbit. There was no increase in left eye proptosis on Valsalva maneuver. His anterior segment, fundus examination, and intraocular pressure were normal in both the eyes. Contrast-enhanced computer tomography imaging of the orbit revealed a well-defined hypodense oval soft tissue lesion measuring 2.8 cm × 1.5 cm in the left superior orbit containing fat and a localized hyperdense area corresponding to calcification causing inferior displacement of superior rectus muscle suggestive of an orbital dermoid [Figure 2]a and [Figure 2]b. The LPS muscle was not identified separately. Excision biopsy of the dermoid was planned under general anesthesia. Excision of the intact dermoid was carried out through an upper eyelid crease incision. Intraoperatively, the silicon rod from previous frontalis suspension surgery was identified and removed. A well-encapsulated yellowish soft mass was identified in the superior quadrant. Meticulous dissection was carried out all around and the mass was excised completely [Figure 3]a. Histopathological examination of the excised mass showed a cyst wall made of stratified epithelium and lumen containing eosinophilic material, hair follicles, and foreign body granulomatous inflammation around the hair follicles confirming the diagnosis of dermoid [Figure 3]b. At 6-week follow-up, his ocular movements were full with residual ptosis of 4 mm [Figure 1A' and B']. He is awaiting ptosis correction in his left eye.
Figure 1: (A) External photograph showing fullness in left upper eyelid with severe ptosis. (B) External photograph showing mild proptosis with inferior dystopia. (A' and B') Postoperative external photograph taken at 6 weeks showing improvement in elevation and significant residual ptosis

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Figure 2: Contrast enhanced computed tomography of the orbit coronal section (a) and axial section (b) showing well-defined oval hypodense soft tissue lesion in the left superior extraconal space and a localized hyperdense area corresponding to calcification causing inferior displacement of superior rectus muscle

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Figure 3: (a) Intraoperative photograph showing the dermoid being excised completely. (b) Histopathology picture showing hair follicle (asterisk) and giant cells indicating granulomatous foreign body inflammation around the hair follicles

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Orbital dermoid cysts are slow-growing lesions and may be located superficially in the periorbital region or deep within the orbit. These lesions may also cause bony remodeling of the adjacent orbital walls and very rarely bony erosion with extension into the temporal fossa or intracranial space.[5] The clinical presentation of dermoids depends on their location, size, and associated features. Superficial dermoids usually present as a well-circumscribed, freely mobile, soft to firm, subcutaneous mass whereas the deeper lesions present with orbital signs such as proptosis and/or diplopia based on their location within the orbit.[3] In our patient, the location of the dermoid was in the superior extraconal space causing inferior dystopia and ptosis. Other common presentation of dermoid is a sudden onset inflammatory reaction following trivial trauma, which causes the cyst wall to rupture and subsequent release of its contents leading to an inflammatory response. As these are slowly progressing lesions, deeper lesions within the orbit may often be missed in the initial stages. Our patient too presented with severe ptosis and elevation restriction initially and hence was misdiagnosed as congenital ptosis with MED elsewhere. Such long-standing deep dermoid cysts, especially when located in the superior quadrant, can induce pressure effect over the levator and superior rectus muscles causing the stretching of the muscle fibers leading to ptosis, motility restriction, and hypotropia. However, the possibility of coexistence of congenital ptosis along with an orbital dermoid cyst cannot be ruled out in this case. Our patient is similar to cases previously reported, where the dermoid presented with ptosis and elevation restriction.[6],[7],[8] Review of literature showed case reports of dermoid presenting atypically as Type II Duane's retraction syndrome,[9] meningocele,[10] and ectopic cilia with draining sinus fistulas.[11],[12] Imaging helps in clinching the diagnosis. Complete surgical excision of the dermoid forms the mainstay of treatment as an incomplete excision may lead to recurrence.[13]

To conclude, orbital dermoid cysts can rarely have atypical presentations. Hence, the treating ophthalmologist should carry out a thorough orbital examination in all cases of unilateral congenital ptosis including palpation of the upper eyelid and look out for subtle orbital signs such as dystopia or proptosis due to any underlying mass lesion. Management of superior orbital dermoid presenting as ptosis with MED includes complete excision of the dermoid, followed by squint correction in case of any residual vertical deviation, and finally ptosis correction, as a staged procedure.

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.
Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111:997-1008.  Back to cited text no. 1
    
2.
Bonavolontà G, Strianese D, Grassi P, Comune C, Tranfa F, Uccello G, et al. An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthalmic Plast Reconstr Surg 2013;29:79-86.  Back to cited text no. 2
    
3.
Rootman J. Diseases of the Orbit: A Multidisciplinary Approach. 2nd ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2003. p. 53-69.  Back to cited text no. 3
    
4.
Pryor SG, Lewis JE, Weaver AL, Orvidas LJ. Pediatric dermoid cysts of the head and neck. Otolaryngol Head Neck Surg 2005;132:938-42.  Back to cited text no. 4
    
5.
Borodic GE. Cystic Lesions of the Orbit. In: Albert DM, Miller JW, editors. Albert and Jacobiec's Principles and Practice of Ophthalmology. 3rd ed. Oxford, UK: Elsevier; 2007. p. 2903-6.  Back to cited text no. 5
    
6.
Srikanth R, Meenakshi S, Chaterjee R, Mukherjee B. Orbital dermoid mimicking a monocular elevation deficiency. Oman J Ophthalmol 2012;5:118-20.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Correa Pérez ME, Sánchez-Tocino H, Mateos GB. Dermoid cyst in childhood, diagnosed as ptosis. Arch Soc Esp Oftalmol 2010;85:215-7.  Back to cited text no. 7
    
8.
Oberoi P, Ramasubramanian S, Mukherjee B. Orbital dermoid presenting as a case of ptosis with monocular elevation deficiency. Can J Ophthalmol 2016;51:e29-31.  Back to cited text no. 8
    
9.
Gatzonis S, Charakidas A, Papathanassiou M, Paikos P. Multiple orbital dermoid cysts located within the lateral rectus muscle, clinically mimicking Duane syndrome type II. J Pediatric Ophthalmol Strabismus 2002;39:324.  Back to cited text no. 9
    
10.
Wani BN, Jajoo SN, Bhole AM. Transilluminant naso-orbital dermoid cyst masquerading as meningocele. J Craniofac Surg 2010;21:930-2.  Back to cited text no. 10
    
11.
Mukherjee B, Banerjee M, Agarkar S. Orbital dermoid cyst presenting as chronic osteomyelitis. J AAPOS 2018;22:480-1.e1.  Back to cited text no. 11
    
12.
Wang Y, Su F, Li Y, Xiao L. Orbital dermoid cyst with sinus tract mimicking ectopic cilia. J AAPOS 2010;14:532-3.  Back to cited text no. 12
    
13.
Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. 3rd ed. Philadelphia: W B Saunders Company; 2000. p. 2906.  Back to cited text no. 13
    


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