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Bilateral, chronic, bacterial conjunctivitis in giant fornix syndrome
  1. Patrick Commiskey,
  2. Eve Bowers,
  3. Aidan Dmitriev and
  4. Alex Mammen
  1. Department of Ophthalmology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA
  1. Correspondence to Patrick Commiskey; patrick.commiskey{at}gmail.com

Abstract

Giant fornix syndrome (GFS) results in chronic, relapsing conjunctivitis in elderly patients with enophthalmos and enlarged fornices, in which infectious material collects and perpetuates inflammation. A 98-year-old woman presented with persistent, bilateral, purulent conjunctivitis; corneal epithelial defects and progressive blepharospasm that did not respond to artificial tears, topical antibiotics and steroids and amniotic membrane grafts. Additional findings of deep-set orbits with enlarged upper fornices were diagnostic of GFS. Over the next 2 months, she responded to a combination of topical and systemic antibiotics, autologous serum eye drops, povidone-iodine forniceal rinses, and hypochlorous acid treatment of the eyelashes. GFS is an important diagnostic consideration in elderly patients with chronic conjunctivitis and deep-set orbits.

  • ophthalmology
  • anterior chamber

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Background

Giant fornix syndrome (GFS) is a rare ophthalmic condition caused by a nidus of infectious material caught in the ‘dead space’ of a large superior fornix that can result in chronic conjunctivitis, keratopathy, nasolacrimal duct obstruction,1 and blepharospasm.2 It was first described in 2004 at Moorfields Eye Hospital in a series of 12 predominantly female patients aged 77–93 who presented with primarily unilateral, chronic and relapsing conjunctivitis. The proposed underlying mechanism involves dehiscence of the levator palpebrae superioris muscle aponeurosis in elderly patients with resultant ptosis and deepening of the upper fornix. Infectious material may then accumulate in this potential space, and associated inflammation can exacerbate ptosis and cause irritative blepharospasm.2 3 GFS may be mistaken for other causes of chronic conjunctivitis, including severe dry eyes, floppy eyelid syndrome or dacryolithiasis, and may respond initially to topical therapy, making diagnosis challenging.4 This syndrome is thus an important diagnostic consideration in elderly patients with chronic relapsing conjunctivitis and deep-set orbits.

Case presentation

A 98-year-old woman with a history of chronic dry eyes presented on referral from her ophthalmologist for refractory conjunctivitis with associated persistent epithelial defects, relapsing episodes of copious ‘mucus’ discharge, and worsening blepharospasm. She denied contact lens use, mucus fishing, chronic sinusitis and autoimmune disease. Her medical history was notable for hypertension, hypothyroidism, heart failure and dementia. Her medications included amlodipine, donepezil, escitalopram, irbesartan, levothyroxine, sotalol and valsartan. Past ocular history was notable for cataract surgery in the remote past and placement of amniotic membrane grafts on the ocular surface 2 months prior to presentation. Her ocular medications were preservative-free artificial tears every hour, besifloxacin drops twice daily and erythromycin ointment at night.

Investigations

At presentation, her vision was counting fingers at one foot in the right eye (OD) and two feet in the left eye (OS). Pupils and intraocular pressure were normal. External examination was notable for bilateral deep-set eyes, deep fornices and involutional ptosis with mild eyelid erythema. The lacrimal puncta were patent with no purulent drainage. Slit lamp examination revealed 2+ diffuse conjunctival injection with papillary reaction and purulent discharge as well as paracentral 1 mm cornea epithelial defects with minimal thinning (figure 1). Tear breakup time was normal. Conjunctival smears disclosed polymorphonuclear cells and gram-positive cocci in clusters, and subsequent cultures grew methicillin-susceptible Staphylococcus aureus (S. aureus). Altogether, these findings suggested the diagnosis of GFS. A CT scan of the head from 5 years prior demonstrated normal orbital bony anatomy, well-aerated sinuses and a pocket of air deep in the upper fornix of the left eye (figure 2), which was supportive of but not necessary for clinical diagnosis.

Figure 1

Photographs of the right eye at the time of initial presentation demonstrating a deep fornix, diffuse conjunctival hyperemia and purulent discharge.

Figure 2

Axial CT scan from 5 years prior to presentation demonstrating an air bubble between the left globe and eyelid due to enophthalmos.

Treatment

Initial treatment involved sweeping of the patient’s fornices with saline-moistened cotton tip applicators followed by a course of besifloxacin drops, bacitracin ointment, autologous serum drops and oral doxycycline. After resolution of her epithelial defects at 2 weeks, her fornices were swept and rinsed with 5% povidone-iodine due to mild persistent purulent discharge. Hypochlorous acid sprays and foam were prescribed to reduce the microbial flora on her eyelids and eyelashes. She was noted to be clear of purulent discharge at three and a half weeks, and an 18-day course of topical loteprednol drops was added to address residual inflammation.

Outcome and follow-up

At 8 weeks, there was significantly less conjunctival injection and purulent discharge with marked improvement in ocular discomfort and blepharospasm (figure 3). She was back to her baseline level of vision in both eyes (OD 20/50 and OS counting fingers two feet), with her vision limited by bilateral bullous keratopathy that was worse in the left eye and corneal scarring from her persistent epithelial defects. She was maintained on a regimen of saline rinses of her fornices twice daily, autologous serum drops, sodium chloride ointment at night and oral doxycycline every other day without recurrence of conjunctivitis for a follow-up period of 3 months. She was subsequently noted to have a mild recurrence of purulent discharge from her left upper eyelid punctum and fornices and was diagnosed with a complete left-sided nasolacrimal duct obstruction. Due to her advanced age, she was managed conservatively with antibiotic drops.

Figure 3

Photographs of both eyes at 8 weeks after initial presentation demonstrating resolution of discharge and improvement of conjunctival injection. Deep fornices and eyelid laxity are still apparent.

Discussion

This patient presented with classic findings of GFS including relapsing episodes of copious mucopurulent discharge in the setting of enlarged fornices and enophthalmos with radiologic evidence of an air bubble under the upper eyelid.3 5 Beyond the previously described underlying mechanism of age-related dehiscence of the levator muscle aponeurosis, it has also been proposed that the development of an enlarged fornix in GFS may occur due to age-related generalised atrophy of orbital tissue as one of the enophthalmos syndromes.3 6 The other enophthalmos syndromes include silent sinus syndrome7 8 and silent brain syndrome,9 which conversely develop secondary to increased orbital volume from bony remodelling and overlap clinically with GFS since malposition of the eyelids relative to the globe in these entities can lead to ocular surface disease.6 Uncommon features of this case included bilateral involvement and progressive blepharospasm. Blepharospasm may be caused by contraction of the orbicularis oculi muscle secondary to irritation from infectious material in the fornix, which has been shown to resolve with injection of botulinum toxin.2

Treatment of GFS focuses on removal of infectious material from the fornix, administration of topical and systemic antibiotics, optimisation of the ocular surface, and in some cases, restoration of the forniceal space with eyelid fillers or fornix reconstruction.4 Complications such as nasolacrimal duct obstruction and corneal neovascularisation are treated accordingly. Doxycycline and minocycline are commonly used systemic adjuncts to topical therapy given that S. aureus is the most common bacterium implicated in GFS.3 10 However, conjunctival cultures may be helpful since Pseudomonas aeruginosa has also been isolated in GFS.11

Povidone-iodine 5% eyedrops are a safe and inexpensive antiseptic that have also been shown to effectively treat GFS.12 The drops can be self-administered if the patient is able to tolerate the associated burning and stinging, which helps to mitigate frequent outpatient visits for irrigation and fornix sweeping. They are also less toxic and equally effective as 10% povidone-iodine solution, which has been used to irrigate the fornix in treatment of GFS after failed topical antibiotics and steroids.13 14 Additionally, there are reports of subtarsal or subconjunctival injection of antibiotics and corticosteroids for patients with GFS who have failed aggressive topical and systemic treatments.15

Chronic, relapsing GFS resistant to medical and mechanical therapy can be corrected surgically with 4–5 mm of fornix reconstruction under local anaesthesia.16 17 This process minimises superior fornix dead space and the potential for developing a nidus of infection. In a case series of six surgical patients, five reported complete resolution of symptoms and all reported symptomatic improvement by 18 months.18 Alternatively, hyaluronic acid gel filler has also been used for functional treatment of GFS. Like surgical correction, this minimally invasive injection shortens the superior fornix to eliminate dead space.19

In conclusion, this unusual presentation of bilateral GFS with associated blepharospasm improved with topical and systemic antibiotics, 5% povidone-iodine rinses, and hypochlorous acid eyelid treatments. To he best of our knowledge, there is only one other report of blepharospasm in GFS, which responded to botulinum toxin injection rather than medical therapy.

Learning points

  • Giant fornix syndrome (GFS) causes chronic, relapsing, purulent conjunctivitis that affects elderly patients.

  • Enophthalmos is a key feature of this syndrome that leads to development of an enlarged fornix where an infectious nidus can form and perpetuate inflammation.

  • Treatment of GFS involves manual forniceal debridement, antibiotics, steroids and surgical reconstruction of the fornix in some cases.

  • The nasolacrimal duct system should always be checked for obstruction, which may precede or follow GFS.

  • Blepharospasm in GFS may improve with medical treatment alone.

Ethics statements

Patient consent for publication

References

Footnotes

  • Twitter @actualpatrick

  • Contributors Concept and design: PC and AM. Data collection: PC and EB. Analysis and interpretation; writing the article; critical revision of the article and final approval of the article: PC, EB, AD and AM.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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