Pediatric uveitis complicating in cataract formation regained normal visual acuity – a challenging case

Abstract

Background: Pediatric uveitis and its complications are important causes of child blindness. Strong inflammatory response and unstable treatment compliance result in worse treatment outcome than adults and frequent recurrence. However, effective anti-inflammatory treatment can still lead to good result. Case report: We report a case of pan-uveitis in a 13-year-old patient, complicating in cataract formation who was successfully treated and regained 20/20 final visual acuity. The patient was admitted to us due to blurry vision, pain and redness on her left eye, with best-corrected visual acuity 12/20, intraocular pressure 15 mmHg, ciliary flush, anterior chamber cell and constricted pupil with poor light reflex. Her left eye was diagnosed with anterior uveitis and treated with topical prednisone acetate along with peri-orbital injection of dexamethasone. At 1-week follow-up, her left eye showed significant progress with best-corrected visual acuity being 16/20 and no inflammatory reaction detected. Due to social distancing in Covid-19 pandemic, she was unable to have follow-up examinations; her condition worsened to pan-uveitis which complicated in cataract formation and her visual acuity fell down to counting finger 0.5m. She was then treated with systemic methylprednisolone in 2 weeks and local corticosteroids in 6 months, including 3 consecutive peri-orbital injections of triamcinolone acetate with monthly interval and topical prednisone acetate; after that she underwent cataract surgery with anterior vitreous humor removed and intraocular lens implanted in the ciliary sulcus. At 1-month follow-up, best-correct visual acuity of her left eye was 20/20 and intraocular pressure was 16 mmHg, the inflammatory response of her left eye was completely in control. Conclusion: Effective anti-inflammatory treatment even after surgery (if existed) takes the decisive role in regaining patient’s vision. Cataract removal surgery on pediatric uveitis patients should combine with vitrectomy and the surgeon should be prepared to place the intraocular lens in the ciliary sulcus.

Introduction

Pediatric uveitis is currently one of the leading causes of child blindness in both developed and developing countries 1 , 2 . In children, strong inflammatory response and poor treatment compliance make uveitis difficult to treat, resulting in various complications which affect life quality of many years ahead of them 3 , 4 . Corticosteroids still takes the main role in treating pediatric uveitis, the others are either only complementary treatments or still in research 5 , 6 . High recurrence rate and various complications result in low percentage of gaining visual acuity; most patients have their visual acuity unchanged or even reduced due to complications 2 , 3 . However, effective anti-inflammatory treatment may lead to good result and preserve the patient’s baseline visual acuity.

We hereby present a challenging case of pan-uveitis in a 13-year-old child with cataract formation, who was treated with corticosteroids and then underwent cataract surgery with an intraocular lens (IOL) implanted, with best-corrected visual acuity (BCVA) at 1-month follow-up being 20/20.

Case report

A 13-year-old female patient was admitted to us due to blurry vision of her left eye (OS). Her left eye was blurry in 1 week, along with redness, mild pain and no discharge. She used unknown eye drops from a pharmacy but her eye showed no progress. Her parents had her eye checked as her condition became worse.

The best-corrected visual acuity (BCVA) of her right eye (OD) and OS were, in order, 20/20 and 12/20; the intraocular pressure (IOP) of her OD and OS were, in order, 16 and 15 mmHg. Clinical examination of her OS revealed ciliary flush, anterior chamber cell 2+, 2mm pupil with poor light reflex. Her complete blood count, VS 1h and 2h, syphilis, toxoplasma, toxocara, CMV and other immune tests showed no abnormality. Her OS was then diagnosed with aseptic anterior uveitis and treated with topical prednisone acetate 1% q3h, topical atropine 1% q12h and 1 peri-orbital injection of 0.5 ml dexamethasone 4 mg/ml. One week later, her OS had BCVA 16/20, IOP 15mmHg and no anterior chamber cell detected. She was continued treating with topical prednisone acetate 1% 4 times/day only and appointed to have her eye checked after 2 more weeks. However, due to social distancing in Covid-19 pandemic, she was unable to continue follow-up check.

The patient continued follow-up check after the social distancing had been lifted, approximately 4.5 months after her first check. She told that because of treatment discontinuation, her OS became worse. At the time of examination, her OS had BCVA counting finger (CF) 0.5m and IOP 12 mmHg. Clinical examination of OS revealed ciliary flush, anterior chamber depth abnormality and cell 4+, posterior synechia, white cataract formation, dark pupillary reflex and unapproachable fundus ( Figure 1 ). B-scan ultrasonography of OS showed patchy vitreous opacification, dense chorioretinal echo ( Figure 2 ). Her OS was diagnosed with panuveitis complicated in cataract, and treated with oral methylprednisolone 1mg/kg q24h, topical prenisone acetate 1% q3h, topical atropine 1% q12h and one periorbital injection of 0.5ml triamcinolone 40 mg/ml. After 2 weeks, her OS had BCVA unimproved, IOP 15 mmHg, ciliary flush resolved, anterior chamber only 1+, posterior synechia and cataract remained; oral corticosteroids and topical atropine were stopped and she was appointed to have a recheck after 2 weeks. On the next follow-up, her OS still had BCVA unchanged, normal IOP, anterior chamber cell only nil; the liberal region of pupil still had light reflex, and the orange color of fundus could still be seen through unopacified regions of the lens. She was continued to treat with topical prednisone acetate 1% q6h and 2 more injections of peri-orbital 0.5ml triamcinolone 40 mg/ml with monthly interval. At 1 month after the last injection, her OS still had unchanged BCVA, normal IOP and no anterior chamber cell. Triamcinolone injection was stopped and she continued topical prednisone acetate 1% q8h in 3 months, with monthly check of IOP (all normal). After 3 months, she underwent cataract surgery and had intraocular lens (IOL) implanted. One day before the operation, she had one more periorbital injection of 0.5ml triamcinolone 40 mg/ml.

Figure 1 . The patient’s left eye (white cataract and posterior synechia can be observed)

Figure 2 . B-scan ultrasound image of left eye (patchy opacification of nearly whole vitreous cavity can be observed)

In the operation, the lens was found to become white and phacolytic, resulting in abnormal anterior chamber depth; anterior capsule became attached to posterior capsule. The anterior surface of the lens was cover with a thick, firm fibrotic membrane with neovascularization. After making the incison and filling the anterior chamber with viscoelastic, the fibrotic membrane cannot be removed with either 26-gauge needle or capsulorhexis pince. Hence, the mass of the fibrotic membrane, anterior capsule and posterior capsule was removed by Vannas scissors with a 6-milimeter round cut and then taken out through the incision. After that, the remaining cortex and anterior vitreous humor were removed by a 23-gauge vitrectomy cutter. The IOL was placed in the ciliary sulcus, with the haptic being placed at 3 o’clock and 9 o’clock position. Viscoelastic was sucked out (no sucking beneath the IOL), and the incision was closed after injecting intracameral antibiotic. Post-operation medication included oral ofloxacin 0.2g b.i.d, paracetamol 0.5g t.i.d in 5 days and topical levofloxacin 0.5% q3h, topical prednisone acetate 1% q3h.

At 1 day post-operation, her OS had uncorrected visual acuity (UCVA) 4/20 and IOP 42 mmHg, conjunctival injection, corneal edema, centrated IOL. Her IOP was corrected with oral acetazolamide 0.25g b.i.d, kaleorid 0.6g q24h in 5 days and topical Combigan (timolol 0.5% + brimonidine 0.2%) q12h. At 1week post-operation, her OS had UCVA 6/20, BCVA 20/20, IOP 15 mmHg, normal cornea and anterior chamber, pupil 3mm with normal light reflex, a small piece of the fibrotic membrane remained, and centrated IOL; the patient was treated with topical levofloxacin 0.5% q3h, topical prednisone acetate 1% q3h and topical Combigan q12h. At 1-month post-operation (the patient had stopped using Combigan for 2 days), her OS had BCVA 20/20, IOP 16 mmHg, normal cornea and anterior chamber and centrated IOL; the iris slightly attached onto the fibrotic membrane ( Figure 3 ). Her fundus image showed normal fundus ( Figure 4 ). She was treated with topical fluorometholone 0.1% q6h and appointed to have recheck after 3 months.

Figure 3 . The OS at 1-month follow-up (stable IOL, the iris attached slightly onto the fibrotic membrane)

Figure 4 . Fundus image of OS at 1-month follow-up (The image was a bit blurry because the pupil was small and partially covered by the fibrotic membrane, however a clear vitreous humor and normal fundus can still be observed)

Discussion

Diagnosis

There was no difficulty in making a definitive diagnosis in this patient as she had almost all typical signs and symptoms of uveitis 2 , 7 . Laboratory tests – including syphilis, toxoplasma, toxocara – showed no abnormality, allowing us to exclude infectious causes and use corticosteroids in treatment 7 , 8 , 9 . On later stage, besides the signs of anterior uveitis and cataract formation, B-scan image of diffuse and patchy vitreous opacification helped confirm the diagnosis of panuveitis 2 , 7 .

Medical treatment

Treating uveitis in children is a challenge to even experienced ophthalmologists. Strong inflammatory response, patient’s poor compliance and difficulties in follow-up reduce treatment results considerably 2 , 8 , 9 . The delay in admission to hospital results in severe condition and low baseline visual acuity, and then also contributes to poorer prognosis than the adults. In another study of us, only 23.4% of eyes gained visual acuity after treatment, 69.2% of eyes had at least one complication; 51.4% of eyes had baseline visual acuity less than 6/20 2 . Low percentage of gaining visual acuity was also concluded in other studies 1 , 4 .

Corticosteroids is still first-line treatment in aseptic uveitis 8 , 9 ; the others either take the role of complementary treatment or are still in research 5 , 6 . Local corticosteroids is the first choice in treating monocular anterior uveitis. Options of local corticosteroids include topical drop, periorbital/subtenon injection and intravitreal injection; among those, intravitreal injection is the most effective. However, due to risk of ocular hypertension and endopthalmitis, intravitreal injection of corticosteroids is only indicated to patients with panuvetis, or refractory to other choices 8 , 9 . Our patient was initially treated with topical and periorbital injection of corticosteroids and showed progression before being unable to have follow-up checks in social distancing.

When the patient developed panuveitis, in order to support local corticosteroids, we combined systemic corticosteroids in a short amount of time; after 2 weeks, when the inflammatory response reduced considerably, she was stopped systemic corticosteroids. Periorbital injection of triamcinolone was maintained in 3 months (until no inflammatory response present) and topical prednisone acetate was maintained in 6 months with taper to prevent ocular hypertension.

The patient was given 1 more periorbital injection of triamcinolone 1 day before the surgery to prevent the post-operating exacerbation of uveitis. Some authors chose intravitreal injection of triamcinolone right after the surgery as an alternative way 10 .

Surgical treatment

The decision to perform cataract surgery on this patient is also a big challenge due to strong post-operating inflammatory response, as well as the prognosis of improving visual acuity is not certain 11 , 12 . On clinical examination, the patient’s pupil still had light reflex in regions without posterior synechia; as well as the orange color of the fundus could still be observed through clear regions of the lens. On B-scan ultrasound images, despite the opacification of vitreous humor, the choroid and retina was intact, no retinal traction or detachment was observed. Therefore, we decided to perform cataract surgery and implant an IOL. A 3-piece IOL with thin haptic was chosen in case we need to implant the IOL into the ciliary sulcus.

In the operation, the mass of anterior capsule, posterior capsule and the fibrotic membrane was dissected with Vannas scissors to prevent uncontrolled damaged of anterior capsule and late posterior capsule opacification. A 23-gauge vitrectomy cutter was used to remove the remaining cortex and anterior vitreous humor to prevent vitreous spilling in the anterior chamber. Without posterior capsule, the IOL was implanted in the ciliary sulcus. We thought that the unremovable piece of the fibrotic membrane (at 6-7 o’clock) might attach to the iris after the surgery; therefore, we placed the haptic at 3 and 9 o’clock position to prevent the IOL to be distorted.

After the surgery, the patient’s OS had 20/20 BCVA with no abnormal astigmatism, centrated IOL, which demonstrated that IOL distortion did not happen although the iris did attach to the fibrotic membrane itself ( Figure 3 ). The inflammatory response was completely controlled with quiet anterior chamber and clear vitreous humor. The IOP of the OS increased transiently, was fully controlled with glaucoma medications and returned to normal at 1-month follow-up.

Conclusion

Effective anti-inflammatory treatment with corticosteroids, even after surgery (if existed), takes the decisive role in treating uveitis in children. Cataract removal surgery on pediatric uveitis patients should be combined with vitrectomy. The surgeon should be fully prepared to implant the IOL in the ciliary sulcus.

INFORMED CONSENT

The informed consent, which allow the pathological information and images of the patient being published, has been read and signed by the patient’s blood-related parents.

ABBREVIATIONS

BCVA: best-corrected visual acuity

IOL: intraocular lens

IOP: intraocular pressure

OD (occulus dexter): right eye

OS (occulus sinister): left eye

UCVA: uncorrected visual acuity

CONFLICTS OF INTEREST

The author(s) declare no conflict of interest in this study

AUTHORS’ CONTRIBUTION

THB was involved in the medical care of the patient, designed and wrote the manuscript.

ACKNOWLEDGEMENTS

The author(s) would like to thank our patient and her family, who kindly agreed with using her case for the purpose of science and education worldwide.