Orbital wall infarction in child with sickle cell disease

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. Ophthalmological examination revealed no signs of pressure on the nervus opticus. The patient recovered gradually with conservative treatment.

Orbital compression syndrome in a Ugandan child with sickle cell disease: A case report

“…Ocular manifestations of SCD have been reported in the literature over the past decades. 4 These include soft head syndrome, 7 sub‐periosteal orbital hematoma, 8 orbital bone infarction, 9 and orbital compression syndrome. 10 , 11 , 12 Orbital compression syndrome (OCS) is a rare acute complication of SCD.…”

Recurrent orbital bone sub-periosteal hematoma in sickle cell disease: a case study

“…A unique feature of orbital wall infarction in SCD is the formation of hematomas, which may be orbital (subperiosteal) or intracranial (epidural) [ 8 ]. Several mechanisms have been suggested for the development of subperiosteal hemorrhage; such as extravasation of blood from necrotized vessel walls, underlying bleeding diathesis, and minor trauma [ 16 ]. Presumed cause of orbital hematoma in the present case is orbital infarction that was evident by abnormal frontal bone and bone marrow heterogeneous signals adjacent to the formation of hematomas.…”

“…Orbital wall infarction typically occurs among young patients because there is more marrow space in the orbital bone among children, unlike the adults [ 16 ]. The mean age of presentation in the reported cases was 13 years, with the youngest reported age at 2 years.…”

Hemoglobinopathies: ocular manifestations in children and adolescents

“…Although vasoocclusive crises often affect the bone marrow of vertebrae and long bones, they may involve the orbital walls in children where the marrow content is higher. [22][23][24] Presentation may be severe pain, lid edema, proptosis, ophthalmoplegia as well as diplopia. [22][23][24][25] Orbital bone infarction can lead to an orbital hematoma, which in turn may lead to orbital compartment syndrome.…”

“…[22][23][24] Presentation may be severe pain, lid edema, proptosis, ophthalmoplegia as well as diplopia. [22][23][24][25] Orbital bone infarction can lead to an orbital hematoma, which in turn may lead to orbital compartment syndrome. 26 Both osteomyelitis and orbital cellulitis may present similarly, and thus confirmation of the diagnosis of orbital bone infarction is of great importance.…”