Novel cause of bilateral proptosis in a child: orbital extramedullary haematopoietic tissue due to iron deficiency anaemia

Abstract

SummaryChildhood proptosis is an emergency with prevalence of 1.2% in hospital admissions. It has a wide spectrum of aetiologies ranging from infective and inflammatory to neoplastic. Iron deficiency anaemia (IDA)-induced extramedullary haematopoesis (EMH) is known to cause hepatosplenomegaly but proptosis is very rare. We describe a girl in middle childhood with subacute bilateral proptosis because of orbital EMH, secondary to chronic nutritional IDA. There was no hepatosplenomegaly. Haemogram depicted severe microcytic hypochromic anaemia with wide red cell distribution width. Red blood cell indices were suggestive of IDA, but serum ferritin was elevated. Bone marrow examination showed absent iron and MRI brain was suggestive of EMH tissue in the orbits. Red blood cell transfusion was given before attempting a biopsy after which the proptosis dramatically reduced and disappeared. IDA is a common entity that rarely can cause EMH at sites other than liver and spleen.

Keywords: Ophthalmology; Paediatrics.