Ocular adnexal Langerhans cell histiocytosis clinical features and management.
OBJECTIVE
To review the clinical features, investigations, management, and outcomes of Langerhans cell histiocytosis (LCH) with ocular adnexal involvement.


METHODS
Retrospective, non-comparative, chart review of 30 patients with LCH involving the ocular adnexa treated at 6 major Australian hospitals. Clinical features, imaging findings, treatment, local and distant recurrence and outcome were evaluated.


RESULTS
Twenty-four patients (80%) were male with a mean age of presentation of 9.5 years. Females presented at an earlier age (mean age 4.1 years) with more severe involvement. Eighty percent of the cases presented with periorbital swelling. Computed tomography usually showed destructive osteolytic lesions centred on the frontal bone. Twenty-four patients (80%) had unifocal, unisystem disease. From this group, none of 13 treated with excision alone had recurrence whereas 2 of 6 treated with excision followed by chemotherapy recurred.


CONCLUSIONS
Unifocal, unisystem LCH often presents to the ophthalmologist and can usually be diagnosed on clinical and imaging grounds. Computed tomography and magnetic resonance imaging are complementary imaging studies. Biopsy is essential to confirm diagnosis. Unifocal, unisystem disease can be treated with local excision and curettage. Incomplete excision, recurrent disease or multifocal disease may require systemic chemotherapy.
Maccheron
Luke J
LJ
Eyelid, Lacrimal and Orbital Clinic, Department of Ophthalmology, Division of Surgery, Royal Brisbane and Women's Hospital and the University of Queensland Medical School, Australia.
McNab
Alan A
AA
Elder
James
J
Selva
Dinesh
D
Martin
Frank J
FJ
Clement
Colin I
CI
Sainani
Ashwin
A
Sullivan
Timothy J
TJ
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D015614:Histiocytosis / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D007223:Infant; D007801:Langerhans Cells; D008297:Male; D009916:Orbital Diseases / Q000175:diagnosis* / Q000628:therapy*