DR. SHALIN SHAH

Dr. RUCHI GOEL

Abstract

Purpose–Langerhans cell histiocytosis (LCH) causes acute painful proptosis with osteolytic skull lesions.

Methods-7-year-male had painful sudden left eye abaxial proptosis with vertical dystopia of 2 mm, horizontal dystopia of 7 mm & left eye up and in. Pink mass at insertion of lateral rectus (LR), more prominent on adduction is noted occupying inferotemporal orbit. Retropulsion positive, limitation of left eye abduction, dilated feeder vessels +. NCCT orbit shows a single well-defined lesion along LR, with no bony erosion. An incision biopsy was done.

Results-Histopathology confirmed lymphocytes, eosinophils & histiocytes. CD 68+, S100+, CD1a+. Diagnosis was soft tissue eosinophilic granuloma – LCH variant. The primary orbital lesion regressed over 3 weeks with chemotherapy and steroids. No recurrence till 6-month follow-up.

Conclusion–Acute painful abaxial proptosis, an emergency condition needs a biopsy for accurate diagnosis & timely intervention to save vision, globe & life.

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