Multiple scalp infarctions and bilateral orbital compression in a child with sickle cell disease

Abstract EN

Orbital compression syndromeis one of Orbital complications in sickle cell disease (SCD) in which a vaso-occlusive process affecting the marrow space of the orbital bone results in subperiosteal hematomas as a result of bone marrow infarctions.

It is one of the unusual manifestations of SCD.

Usually presents as frontal headache, fever and eyelid oedema.

Here we report an 8 year-old Sudanese girl known to have SCD since the age of one year based on HB electrophoresis, presented with bilateral orbital compression syndrome and multiple scalp infarctions, treated conservatively but at the end she undergoes operative orbital decompression when she start to developsings of optic nerve dysfunction and rapidly increasing hematomas to prevent loss of vision and to speed recovery, The ophthalmologist found that the orbital hematomas get secondary infected swab for culture and sensitivity taken and she received Gentamycin for two weeks and fortunately her vision was preserved.