Faculty Collaboration Database

Eosinophilic granuloma of the orbit: a paradox of aggressive destruction responsive to minimal intervention. Trans Am Ophthalmol Soc 2003;101:93-103; discussion 103-5

Date

02/20/2004

Pubmed ID

14971567

Pubmed Central ID

PMC1358978

Scopus ID

2-s2.0-1142286533 (requires institutional sign-in at Scopus site)   46 Citations

Abstract

PURPOSE: To describe the findings and outcomes in eosinophilic granuloma (unifocal Langerhans cell histiocytosis [LCH]) of the orbit, and to explain the paradox of aggressive bone destruction responsive to minimal intervention.

METHODS: Retrospective, consecutive, interventional case series of patients treated from 1985 through 2001. Minimum inclusion criteria were demonstration of CD1a positivity or Birbeck granules, treatment by a single surgeon, systemic evaluation by a pediatric oncologist, and follow-up of 12 months. A pathogenetic construct was assembled from general LCH concepts and the specific orbital findings.

RESULTS: Seven patients met study criteria. All were male, 2 to 16 years of age. All had eyelid or forehead swelling and osteolytic defects, with symptoms of 2 to 6 weeks' duration. All underwent incisional biopsy, with frozen-section examination suggestive of LCH in 6 of 7 cases. The 2 earliest patients received low-dose irradiation after simple biopsy. The 5 most recent patients had subtotal curettage at the time of biopsy; 4 of 5 received simultaneous intralesional corticosteroid injection. In all cases, systemic evaluation showed no other focus of LCH, reossification was timely, and no local recurrence or additional focus was noted in follow-up of 1 to 17 years.

CONCLUSIONS: Transient immune dysfunction may provoke the cytokine-mediated proliferation of pathologic Langerhans cells within the hematopoietic marrow of the anterolateral frontal bone. These cells cause osteolysis through elaboration of interleukin-1 and prostaglandin E2. Corticosteroids can inhibit the mediators. We recommend incisional biopsy, frozen-section provisional diagnosis, subtotal curettage, intralesional corticosteroid instillation, postoperative systemic evaluation, and long-term follow-up.

Author List

Harris GJ, Woo KI

Author

Gerald J. Harris MD Professor in the Ophthalmology and Visual Sciences department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Child
Child, Preschool
Curettage
Eosinophilic Granuloma
Follow-Up Studies
Glucocorticoids
Humans
Injections, Intralesional
Male
Methylprednisolone
Orbit
Orbital Diseases
Radiotherapy
Retrospective Studies
Tomography, X-Ray Computed
Triamcinolone