Clinical features and recurrence patterns of pediatric idiopathic orbital inflammatory disease

Idiopathic orbital inflammatory disease (IOID) is a non-specific orbital inflammatory condition that is uncommon in children. Due to limited data and the unique characteristics of the developing immune system in children, this study examined the clinical features, imaging subtypes, treatment outcomes, and recurrence patterns.

Pediatric patients (≤ 18 years) diagnosed with IOID at Beijing Tongren Hospital, Capital University between January 2020 and January 2025 were included in this retrospective review. Clinical data, imaging characteristics, treatment regimens, and recurrence patterns were collected. Cases were further classified into six anatomical subtypes based on orbital imaging.

A total of 10 patients (12 eyes) were included, with a mean age of 9.9 ± 3.2 years. Imaging subtypes included diffuse (5, 50%), posterior scleritis (3, 30%), dacryoadenitis (1, 10%), and orbital apex involvement (1, 10%). Clinical symptoms were conjunctival injection (9, 90%), optic disc edema (8, 80%), decreased visual acuity (8, 80%), eyelid swelling (7, 70%), periorbital pain (6, 60%), ocular pain (4, 40%), and ptosis (4, 40%). Orbital MRI revealed posterior scleral and/or Tenon's capsule thickening with enhancement (8, 80%), optic nerve or sheath enhancement (5, 50%), lacrimal gland enlargement (4, 40%), and cavernous sinus thickening or dural enhancement (2, 20%). All patients received systemic corticosteroids, with a mean follow-up duration of 17.9 ± 12.5 months. Nine patients experienced recurrence, with seven of them related to steroid dependence in diffuse-type cases. Final visual acuity was ≥ 0.3logMAR in all relapsed cases.

Pediatric IOID frequently presents with diffuse or deep orbital involvement on imaging. While corticosteroid therapy is generally effective, these subtypes may be more prone to steroid-dependent relapse during tapering. The association between steroid tapering and relapse remains to be fully substantiated, and further studies are needed to explore this potential relationship. Imaging-based classification may help predict recurrence risk and guide individualized long-term management strategies for pediatric IOID.