Igg4-related dacryoadenitis with fibrous mass in a 19-month-old child: Case report and literature review

Abstract

A 19-month-old boy presented with eyelid swelling, proptosis, and upgaze limitation in the OD. The radiological study showed a homogeneous mass enclosing the lacrimal gland. Near-total tumor excision revealed IgG4-related orbital disease. Eosinophilia, serum IgG4, and IgE elevations were detected without extraorbital involvement. The patient received oral prednisolone for 4 months postoperatively and remained relapse-free for 27 months. Among the 17 well-documented pediatric cases of IgG4-related orbital disease in the literature (including this case), 59% were female, and the median age was 10 years; 2 patients were under 2 years old. The disease was unilateral in 82% of the patients and caused a soft tissue mass in 88%, involving the lacrimal gland in 53%. Nine patients received immunosuppression only, 4 surgery and immunosuppression, and 2 only surgical excision. Treatment results were reported in 13 patients, and all were favorable. IgG4-related dacryoadenitis with a fibrous mass may occur in very young children, responding well to surgical excision and steroids. Although pediatric IgG4-related orbital disease is not well-characterized yet, it may manifest differently from its adult variant.