Multifocal necrotising scleritis post-MIVS in oligoarticular JIA : exploring plausible deniability.

Abstract

AIM: To report and discuss the clinical features and management strategies for paediatric surgically induced necrotizing scleritis (SINS) following microincision vitrectomy surgery (MIVS), in a patient with underlying seronegative oligoarticular juvenile idiopathic arthritis (JIA). METHODS: Case report and review of literature. RESULTS: A 10-year-old boy presented with multifocal areas of painless scleral necrosis following three-port 25-gauge pars plana vitrectomy. The area of scleral necrosis remained localized to the temporal sclerotomy sites, which were closed with 6 − 0 polyglactin sutures. There were no associated clinical signs of suppuration or discharge suggestive of an infectious etiology; the smear from the base of the scleral necrosis showed no microbial growth. Scleral melt remained progressive despite immunosuppressive therapy with intravenous methylprednisolone and cyclophosphamide. Systemically, the patient reported a recent onset of multiple joint pains without any visible joint swelling. The blood investigation returned negative for rheumatoid factor, ANA, and ANCA antibodies. Non-specific inflammatory markers (CRP) were significantly raised. The scleral necrosis stabilized following removal of the polyglactin suture. Scleral patch graft with conjunctivo-tenon flap helped to preserve the globe integrity. No recurrence or further necrosis was observed over twelve months of follow-up. CONCLUSION: This is the first report on the clinical presentation of paediatric SINS associated with JIA following MIVS. Prior surgeries, underlying autoimmunity and use of polyglactin suture could have precipitated SINS which was recalcitrant to conventional immunosuppressants. A combined medical and surgical approach was helpful to achieve disease remission.