Case report

A 60 year old moderately obese man with well controlled rheumatoid arthritis (RA) presented to the eye clinic with recurrent red and gritty eyes. A diagnosis of dry eye syndrome with blepharitis was made. He was also found to have a mucocoele of the left lacrimal sac. Lid hygiene and ocular lubricants yielded an initial encouraging response and he was discharged.

He re-presented 5 years later with similar symptoms and reduced visual acuity (VA) of 6/36 in the left eye. A diagnosis of dry eye syndrome with secondary corneal epithelial changes was made. Topical lubricants failed to relieve the condition satisfactorily and he was therefore scheduled for punctal occlusion. However, upon admission for this 8 weeks later, he was found to have an asymptomatic perforation of the left cornea, with a VA of 6/60. The perforation was treated with glue and a bandage contact lens, topical antibiotic, steroids, and lubricants. Systemic immunosuppression was considered in view of the history of RA, but was withheld as a heavy growth of staphylococcus had been cultured from the cornea. The right eye demonstrated signs of dryness but was otherwise healthy with VA of 6/9.

Bilateral punctal occlusion was undertaken as planned, and in addition a left dacrocystorhinostomy (DCR) to eliminate the mucocoele as a potential reservoir of infection. The eye, however, continued to slowly deteriorate, with persisting mucopurulent discharge, despite the DCR. Eventually uncontrolled endophthalmitis developed requiring evisceration.

He re-presented 8 months later with reduced VA of 6/60 in the right eye secondary to a corneal melt (Fig 1A). A chronic mucopurulent discharge had also developed in the right eye, but no lacrimal sac mucocoele was identifiable. On this occasion, however, it was noted on examination that while everting the eyelids, all four lids exhibited excessive laxity (Fig 1B). This, together with a florid papillary tarsal conjunctival reaction and the chronic mucus discharge, led to a diagnosis of RA associated dry eye syndrome exacerbated by FES.

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Figure 1

Right eye before eyelid shortening showing (A) the corneal melt and (B) marked laxity of the upper and lower lids with a chronic mucus discharge.

All four eyelids were immediately subjected to considerable shortening by pentagonal excision; the corneal melt was treated with a bandage contact lens, with topical antibiotic, steroids and lubricants. The response to surgery was dramatic with complete resolution of discharge and gradual spontaneous repair of the corneal melt (Fig 2). The VA eventually recovered to 6/9.

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Figure 2

Postoperative photograph of right eye demonstrating the marked improvement.

Comment

FES occurs most frequently in middle aged obese males,¹ although it has been described in young, slim males, females, and one child.² Typically, the upper tarsus is rubbery and the upper eyelid everts easily with gentle upward pressure. A florid papillary conjunctivitis and chronic mucus discharge are common. Severe corneal involvement is rare, with only four reports in the literature of ulceration in association with FES and only two cases of perforation.^2–4

Although the exact pathophysiology of FES is uncertain, a sequence of events that may lead to its development and to the secondary corneal changes has been proposed.^{1, 4} Unknown predisposing factors, possibly congenital, create a floppy upper tarsus. Whereas examination of post-excisional specimens has revealed normal tarsal collagen, elastin fibres are nearly absent.⁵ It is unclear whether this finding is causative or secondary. During sleep, a local pressure induced ischaemia may develop in the tarsus that, when relieved, results in a reperfusion injury which could injure tarsal elastin. In addition, there is a high incidence of obstructive sleep apnoea in FES patients and nocturnal dips in Pao₂ could further contribute to the local ischaemia and subsequent elastin damage.²

Corneal involvement may occur through one or more mechanisms. Spontaneous nocturnal lid eversion resulting from pressure of the pillow on the lax upper lid may lead to repeated trauma of the corneal epithelium. Lash ptosis may contribute to this direct trauma. The cornea, however, may be damaged from a more subtle but important mechanism. Affected lid specimens demonstrate a marked polymorphonuclear infiltrate, which may be the sequelae of the reperfusion injury described above; this tarsal infiltrate and the associated papillary response may have direct toxic effects on corneal epithelium and stroma.⁴ It is perhaps intuitive that the corneal complications found in FES may be more severe when, as in our case, co-existing pathologies are present. Blepharitis and RA associated dry eye may both independently cause significant corneal pathology.

This case serves as a reminder that multiple pathologies may contribute to the clinical picture. If FES is not to be missed, ocular examination must include lid eversion and inspection of the tarsus.