Conjunctival Resection for Peripheral Ulcerative Keratitis
Alhussain B, AlRasheed R, Almudhaiyan T and Alsultan S
Published on: 2021-07-24
Purpose: To report a case of a progresive peripheral ulcerative keratitis (PUK) managed successfully with conjunctival resection.
Observations: A 61-year-old female not known to have any rheumatological disease. Presented with a crescent-shaped ulceration and peripheral corneal thinning. Patient was admitted for medical management with topical fortified antibiotics along with systemic steroids and methotrexate with no response. Conjunctival resection was performed for signs of progression. Three days after conjunctival resection complete epithelialization occurred with no recurrence during her follow up visits.
Conclusions and Importance: Surgical management of PUK is used in cases of impending perforation to preserve globe integrity. We encourage the use of this local treatment method whenever medical management fails to heal ulceration with worsening of the condition.
KeywordsPeripheral Ulcerative Keratitis, Conjunctival Resection, Inflammation
Peripheral ulcerative keratitis (PUK) refers to a crescent-shaped ulcerative inflammation of the juxtalimbal corneal stroma associated with corneal epithelial defect. Patients usually present to the clinic with a history of pain and redness in the involved eye. Other symptoms might include tearing, photophobia, and decreased vision . The peripheral cornea has distinct morphologic and immunologic characteristics in which they predispose it to inflammatory reactions . Limbal and conjunctival vessels are prone to the deposition of immune complexes . In PUK, resection of the perilimbal conjunctiva associated removes those immune complexes, and decreases production of collagenases and proteinases, which consequently promotes resolution of inflammation, and decreases production of collagenases and proteinases, which consequently promotes resolution of inflammation . Conjunctival resection is controversial, because it is thought that PUK may recur once the conjunctiva heals back to the limbus .
A 61 year-old female known case of hypertension and hypothyroidism. Patient was not known to have any rheumatological disease. Presented to the emergency room with a 3 weeks history of right eye pain, redness, tearing and decrease of vision. Best corrected visual acuity (BCVA) on presentation was 20/50 in the right eye and light perception in the left eye. Slit lamp examination of the right eye showed normal lid architecture. A crescent-shaped non infiltrative marginal ulceration with 90% peripheral corneal thinning between 3 and 7 o’clock (Figure 1).
Figure 1: Sli tlamp photo showing the juxtalimbal ulceration and thinning with superficial peripheral vascularization.
No filaments were seen, no scleral involvement and no leak. The anterior chamber was deep with occasional cells. Patient was pseudophakic with a clear 1 piece intraocular lens in the bag. Her cataract surgery was done 1 year prior to presentation. The remainder of the eye exam was unremarkable. Examination of the left eye showed sensory esotropia since childhood with old corneal scar and mature cataract. The patient was admitted for medical management, and corneal scrapings were collected from the right eye ulcer avoiding areas of extreme thinning. Gram stain was negative, and later on culture and sensitivity for bacteria and fungi were also negative. Medical regimen included fortified antibiotics Cefazolin 50 mg/ml and Ceftazidime 50 mg/ml every 1 hour alternating and cyclopentolate 1% every 8 hours. Also, frequent lubrication with preservative free eye drops every 1 hour and a bandage contact lens was placed over the cornea of the right eye. Investigation included: CBC, RF, ANA, c-ANCA, p-ANCA, ESR, CRP, PPD, and a chest X-RAY. ESR and CRP showed high abnormal values. ANCA also showed a positive Result. On the second day of admission, Prednisolone acetate 1% eye drops was started on a gradual dosing with BID then increased to QID, fortified antibiotics Cefazolin and Ceftazidime were stopped and replaced by Moxifloxacin QID. After the negative results of PPD and chest x-ray, systemic oral Prednisolone was started with 40 mg daily along with antacids and Doxycycline 100 mg BID. Rheumatology still did not reach a final diagnosis and they started oral Methotrexate 12.5 mg weekly for a positive result of ANCA. Despite systemic and topical management for more than one week, the patient showed neglectable improvement in terms of vision, pain, and clinical picture (Figure 2).
Figure 2: Slit lamp photo showing the same lesion after medical management.
The patient also developed another similar lesion superonasally. Then the decision was made to intervene surgically with conjunctival resection without AMT placement (Figure 3).
Figure 3: Photo a Intra operative photo showing 4 mm wide strip of perilimbal conjunctiva adjacent to the areas of stromal ulceration was excised between 2 and 6 oclock.
The procedure was conducted in the main ophthalmology operating theater, under a sterile technique using topical anasthesia with Oxybuprocaine 0.4%. A 4 mm wide strip of perilimbal conjunctiva adjacent to the areas of stromal ulceration was excised between 2 and 7 oclock, with cauterization of perilimbal blood vessels. Antibiotic ointment was applied and the eye was patched. Patient was prescribed fluorometholone 0.1% BID, and lubricating eye drops every 4 hours. Three days later, her symptoms of pain and redness had decreased significantly. Corneal reepithelialization was complete with significant improvement in stromal thickness with BCVA being 6/24 (Figure 3b).
Figure 3b: Slit lamp photo using cobalt-blue filter with fluorescein dye application, showing complete epithelialization of the lesion; three days post-op.
During one week, one month, and four months follow ups complete epithelialization was maintained (Figure 4). The dose of oral prednisolone was slowly tapered. Patient was discharged with regular follow ups with ophthalmology and rheumatology.
Figure 4: (left to right) Slit lamp photo showing complete epithelialization at 1 week, 1 month and 4 months respectively.
PUK is an inflammatory corneal condition estimated to affect 8-10 persons per million per year . Local and systemic diseases are associated with PUK and they can be classified into systemic and ocular infectious and non-infectious causes. Systemic causes include, and not limited to, rheumatoid arthritis, relapsing polychondritis, polyarteritis nodosa and granulomatosis with polyangitis. The ophthalmologist must be familiar with the list of systemic associations to tailor the systemic treatment in collaboration with a rheumatologist . Rheumatoid arthritis is considered the most common cause of non-infectious PUK accounting for 34% . Laboratory investigations are based on the findings of the patient and they include complete and differential blood cell counts, platelet count, erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), antinuclear antibody (ANA), antineutrophil cytoplasmic antibodies (Anti-CCP), chest X-ray examination, and liver enzymes more investigations can be done based on its results (6). PUK is associated with significant ocular and systemic morbidity . In a retrospective review of 47 patients with PUK, 32% had impending corneal perforations, 43% of patients had best corrected visual acuity of 20/400 or worse at their presentation . Peripheral cornea has a distinctive feature in which it is predisposed to inflammatory reactions that contribute in the etiology of PUK.. The limbus and the peripheral cornea derive part of their nutrient supply from the capillary arcades extending 0.5 mm into the cornea . This close proximity of blood and lymphatic vessels resulted in more langerhans cells, C1 complement, IgM in peripheral cornea compared to central cornea . Activation of complement pathways and recruitment of inflammatory cells are major steps in peripheral corneal stromal destruction aided by collagenases and proteases secreted by neutrophils and macrophages .
The mainstay management PUK is reducing inflammation by local and systemic treatment. Local treatment aims to reduce inflammation, promote epithelialization, and to minimize stromal loss . Stepwise approach in treatment of those patients is appropriate. Local treatment of PUK consists of lubrication, 1% Medroxyprogestrone drops, prophylactic antibiotics and a bandage contact lens. Systemic management includes oral steroids and steroid sparing agents [3, 10]. Topical steroids may potentiate further thinning and perforation due to its effect in inhibiting collagen formation and for that reason it should be used with caution [2,4,5,7]. Glucocorticoids are started at a dose of 1 mg/kg/day (maximum of 60 mg daily), followed by a tapering schedule based on clinical response . In our case topical with systemic management including oral steroids and Methotrexate did not stop the progression of the disease. Surgical management of PUK is used in cases of impending perforation to preserve globe integrity.
Several surgical options exist depending on the size of perforation. These include the use of a tissue adhesive with a bandage contact lens, a lamellar graft, and tectonic corneal grafting . Tissue adhesives, like cyanoacrylate have been shown to prevent further stromal thinning by eliminating acute inflammatory cells from the affected site and preventing influx of white blood cells from the tear film. It has been used successfully in impending perforations [2,10,11]. One of the modalities of local treatment is conjunctival resection adjacent to PUK with or without amniotic membrane transplant. Conjunctival resection removes the proteolytic enzymes that lead to corneal ulceration and stromal loss by interrupting the local immunologic events [2,4,12,13]. The procedure is done in a minor surgery room under local anesthesia, and the risks undergoing conjunctival resection are minimal. The use of perilimbal conjunctival resection for the treatment of peripheral corneal ulceration for different causes has been reported previously [12-15]. The use of this procedure is usually done when medical treatment fails to heal the ulcer. It also has been reported that complete healing of rheumatoid corneal ulcerations occurred within a few days after the procedure which suggests a cause and effect relationship. It can shorten the hospital stay and prevent perforation . This method was reported to be effective long years ago yet it is not widely used. In our case complete epithelialization with no recurrence was observed through the continuous follow ups. We encourage the use of this local treatment method whenever medical management fails to heal ulceration with worsening of the condition. A stepwise approach must always be considered with careful monitoring of the patient's condition. Close follow ups are needed and collaboration with rheumatology is a must to reach the best management approach.
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