Paediatric Orbital Juvenile Xanthogranuloma: A Case Series and Review of the Literature

Ming-Han H Lee; Ebony J Smith; Thomas G Hardy; Nicole Graf; Krishna Tumuluri

doi:10.1097/IOP.0000000000002696

Paediatric Orbital Juvenile Xanthogranuloma: A Case Series and Review of the Literature

Ophthalmic Plast Reconstr Surg. 2024 Apr 29. doi: 10.1097/IOP.0000000000002696. Online ahead of print.

Authors

Ming-Han H Lee^{1

2

3}, Ebony J Smith^{1

4}, Thomas G Hardy^{5

6

7}, Nicole Graf^{2

8

9}, Krishna Tumuluri^{1

2

3}

Affiliations

¹ Department of Ophthalmology, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead.
² School of Medicine, The University of Sydney.
³ Save Sight Institute, Sydney, New South Wales.
⁴ Department of Ophthalmology, Sunshine Coast University Hospital, Queensland.
⁵ Orbital, Plastic and Lacrimal Service (OPAL), Royal Victorian Eye and Ear Hospital, Melbourne.
⁶ Department of Ophthalmology, Royal Children's Hospital.
⁷ Department of Surgery, Royal Melbourne Hospital, University of Melbourne, Parkville, Victoria.
⁸ Department of Histology, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead.
⁹ School of Medicine, Western Sydney University, Campbelltown, Sydney, New South Wales, Australia.

PMID: 38687290
DOI: 10.1097/IOP.0000000000002696

Abstract

Purpose: Juvenile xanthogranuloma (JXG) is a subtype of histiocytosis characterised histologically by foamy non-Langerhan cells with Touton giant cells. It typically manifests as a single self-limiting cutaneous nodule in the paediatric population. Orbital JXG is extremely rare, and its clinical course and management are not well understood or defined. Herein we present 3 cases of orbital JXG and provide a detailed literature review.

Methods: Review of 3 cases with orbital JXG and literature review of all published cases.

Results: Three presented cases demonstrate the heterogeneous clinical course of orbital JXG. Although centred around the use of steroids, there is neither robust evidence nor consensus on its management. The wider JXG literature is currently concentrated around the classification of JXG with respect to histiocytosis, especially the exclusion of extracutaneous JXG as separate diseases. This separation is based on clinical, histopathological, and molecular findings. It is unclear where orbital JXG best fits in this emerging classification of JXG.

Conclusion: Our review of the cases and literature on orbital JXG show that it may manifest with variable clinical course and its molecular pathogenic mechanism may be different to that of the cutaneous JXG.