We presented an SJS patient with two conjunctival cysts extending into the orbit without previous ocular surgery or trauma. SJS can be secondary to drugs, infection, malignancies, or an idiopathic reaction. Chronic inflammation of the ocular surface in SJS leads to goblet cell destruction, scarring of the tarsal conjunctiva, eyelid margin keratinization, eyelid malpositioning, entropion, and trichiasis. These changes in addition to the secondary dry eye due to lacrimal gland dysfunction can cause corneal micro trauma, corneal epithelial defect, corneal ulcer, perforation, and limbal stem cell deficiency and conjunctivalization 7.
Orbital cysts can be classified by their manifestation, pathogenesis, or histopathology. They are primary or secondary to trauma, surgery, or inflammation 8. Conjunctival epithelial cysts are simple cysts lined by conjunctival epithelium containing goblet cells. Primary conjunctival cysts are usually located in the supranasal side of the orbit without proptosis or globe displacement. However, larger cysts can limit eye movement, displace the globe, and induce refractive error 8, 9. In contrast, the location of secondary conjunctival cysts following trauma or surgery depends on the type of surgery or site of trauma.
Conjunctival cysts are relatively rare ocular manifestations in the chronic phase of SJS/TEN. However, the conjunctival cysts may seriously affect patients with SJS. Besides the cosmetic issues, the larger cysts may exacerbate the dry eye, cause Dellen formation, or prevent implantation of mini scleral lenses like MSD. MSD lenses are among the best treatment options for patients with SJS due to significant visual improvement and reducing the microtrauma caused by eyelid margin irregularity and keratinization 5.
To the best of our knowledge, to date, five studies reported conjunctival or orbital cysts in patients with SJS (Table1)10, 11. The epithelial cysts may originate from the conjunctiva or the lacrimal glands. It is assumed that chronic inflammation in SJS may cause adhesions between the palpebral and bulbar conjunctiva with subsequent entrapment of epithelial cells and cyst formation. Moreover, cyst enlargement may be the result of mucus secretion by conjunctival epithelium. The irregularities and cysts prevent tears from reaching the conjunctival surface 6.
Table 1
Summary of studies reporting conjunctival and orbital cysts in patients with SJS
study
|
age
|
sex
|
laterality
|
Manifestation
|
Time of SJS
|
Cause of SJS
|
treatment
|
f/u duration
|
outcome
|
Singh et al.20085
|
11
|
m
|
OU
|
conjunctival cyst at the medial of inferior fornix
|
3 years before
|
-
|
observation
|
-
|
-
|
Memarzadeh et al. 200612
|
17
|
m
|
OU
|
inferior anterior orbit and lower eyelids cysts
|
1 y before
|
tetracylcine
|
marsupialization
|
1 year
|
No recurrence
|
Desai et al.19926
|
10
|
f
|
OD
|
Superior anterior orbit. Conjunctival origin
|
5 years
|
phenobarbital,erythromycin,acetaminophen,aspirin
|
Cyst excision
|
-
|
-
|
Goodglick, T A et al.199210
|
|
f
|
OU
|
palpebral conjunctival cysts
|
|
allopurinol
|
|
|
|
Gerald et al. 198311
|
|
|
|
lacrimal gland cyst
|
|
|
marsupialization of the wall
|
|
|
The present study
|
13
|
m
|
OD
|
Conjunctival cyst, adjacent to the medial rectus muscle
|
3 years
|
Indomethacin, cephalexin
|
Cyst excision
|
6 months
|
No recurrence
|
SJS: Stevens-Johnson Syndrome; OU: Oculus Uterque; OD: Oculus Dexter; OS: Oculus Sinistra |
Surgical management of conjunctival cysts consists of two approaches, including complete intact excision versus marsupialization, which means unroofing the cyst. While complete cyst resection using meticulous resection remains the standard technique, preserving the intact walls is not always possible. Memarzadeh et al 12 advocated that marsupialization in large conjunctival cysts located in the inferior fornix could preserve the epithelial lining to be used in fornix reconstruction. However, marsupialization may not be a good choice in deeper orbital/ conjunctival cysts. In our patient, despite confirmed adhesion of the cyst to the eyelids and medial rectus sheet, complete cyst resection was possible for both adjacent cysts, and the inferior fornix was reconstructed with AMT. Because of the inflammatory process, in patients with SJS, the conjunctival cysts may have a more confirmed attachment to the adjacent tissue and extraocular muscles. So meticulous dissection is needed to complete the excision of such conjunctival/orbital cysts. However, in superficial epithelial conjunctival cysts with inadvertent cyst rupture or a high probability of rupture, marsupialization can be alternatively used.
In conclusion, we presented the surgical management of an SJS patient with two large conjunctival cysts located in the nasal and inferonasal orbital cavities. Due to lid deformity, ptosis, and movement limitation, the patient underwent surgical cyst resection and inferior fornix reconstruction. Cyst excision benefited our patient in terms of mini scleral lens fitting, resolution of lagophthalmos, and DED improvement.