Orbital wall infarction in child with sickle cell disease

Acta Clinica Belgica
C JanssensM Wojciechowski

Abstract

We present the case of a 17-year-old boy, known with homozygous sickle cell disease, who was admitted because of generalised pain. He developed bilateral periorbital oedema and proptosis, without pain or visual disturbances. In addition to hyperhydration, oxygen and analgesia IV antibiotics were started, to cover a possible osteomyelitis. Patients with sickle cell disease are at risk for vaso-occlusive crises, when the abnormally shaped red blood cells aggregate and block the capillaries. Such a crisis typically presents at a location with high bone marrow activity, as the vertebrae and long bones. At an early age, the bone marrow is still active at other sites, for example the orbital wall, and thus infarction can also occur there. Thus, in young persons with sickle cell disease, it is important to consider orbital wall infarction in the differential diagnosis, since the approach is different from osteomyelitis. If the disease is complicated by an orbital compression syndrome, corticosteroids or surgical intervention may be necessary to preserve the vision. In our patient, an MRI of the orbitae demonstrated periorbital oedema with bone anomalies in the orbital and frontal bones, confirming orbital wall infarction. Ophthalmolog...Continue Reading

References

May 10, 2005·British Journal of Haematology·Antonio Almeida, Irene Roberts
Jun 4, 2008·Ophthalmic Plastic and Reconstructive Surgery·Jason A SokolMichael Kazim
Jul 16, 2010·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Naoko SaitoOsamu Sakai
Apr 5, 2014·Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus·Nilufer IlhanEsra Ayhan Tuzcu

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