Clinical analysis of 11 cases of orbital Langerhans cell histiocytosis

Objective: To study the clinical and imaging features, the diagnosis and management of orbital Langerhans cell histiocytosis.

Methods: The symptoms, signs, imaging features, treatment and follow-up results of 11 cases of orbital Langerhans cell histiocytosis were reviewed and analyzed, including 7 cases of eosinophilic granuloma and 4 cases of Hand-Schueller-Christian disease.

Results: Eosinophilic granuloma occurred primarily in children and teenagers and appeared as orbital lesions only. Hand-Schueller-Christian disease occurred in children under 5 years old, with multiple lesions of histiocytosis. Typical cases showed the Hand-Schueller-Christian triad: exophthalmos, diabetes insipidus and bony skull defects. Patients usually complained of exophthalmos and orbital masses. B scan and color Doppler showed soft tissue masses in the orbit. CT showed soft tissue masses and bony skull defects. MRI showed orbital tumor and intracranial involvement.

Conclusions: Characteristic imaging features are helpful for the diagnosis of orbital Langerhans cell histiocytosis. The treatment includes surgical excision, local radiotherapy or chemotherapy. The prognosis is related to the types of lesion, eosinophilic granuloma shows the best prognosis and infantile Hand-Schueller-Christian disease shows the worst prognosis.