DR. MEVIS PHONGLO

Dr.Jyoti Bhuyan

Abstract

Langerhans cell histiocytosis(LCH), a rare disease is characterised by clonal proliferation of immature Langerhans cells believed to occur in 1:2000,000 children.

A 6 year old male child presented with protrusion of right eyeball and increased frequency of micturition. The child has delayed developmental milestones with inability to speak. On ocular examination, the child follows light, mild conjunctival congestion and proptosis in the right eye was found.Dilated veins over temporal area and kyphosis seen. Recurrent middle ear infection and reduced hearing were associated features. MRI reveals bilateral proptosis(R>L), diffuse cerebral atrophy, hypoplastic anterior lobe of the pituitary gland with multiple osteolytic lesions seen in the cranium,cranio-vertebral junction, mastoid bones and spine suggestive of LCH. The child was treated with high dose prednisolone in consultation with a paediatrician. Improvement in proptosis was seen after 1 week.The child is now under follow-up.