Clinical features of sickle cell disease at altitude

Adzaku, Festus; Addae, S; Annobil, Samuel H.; Mohammed, S

doi:10.1580/0953-9859-3.3.260

Cited by 2 publications

(2 citation statements)

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“…The present case is unique because the condition is recurrent in the frontal bone, triggered by visit to a high-altitude city. Similar studies pertaining to infarctions in SCD at high-altitude areas have been conducted; however, this is the first study to conduct an exploration of orbital bone infarction in SCD at high-altitude areas [ 10 , 13 ]. However, previously OCS attacks had resolved completely with conservative measures.…”

Section: Discussionmentioning

confidence: 99%

“…It was discussed that these reported environmental factors significantly predispose sickle cell patients to acute vaso-occlusive crises. Although, a few studies have highlighted the relatively higher incidence of SCD in individuals living in high-altitude or mountainous areas [ 10 ]; the role that high altitude plays in increasing the risk of incidence of sickling cell crises has not yet been heavily explored. Earlier studies have highlighted cases of sickling cell crisis incidence in individuals flying at high-altitude areas in unpressurized aircrafts [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

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

Alghamdi

2018

BMC Ophthalmol

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BackgroundSickle cell disease is a common inherited hemoglobinopathy and is associated with high morbidity and mortality. Vaso-occlusive crises commonly occur in individuals with SCD that results in high morbidity due to end-organ ischemia and infarction. These include splenic infarction, pulmonary involvement, acute chest syndrome, and orbital compression syndrome. Ocular manifestations of SCD include anterior segment ischemia, secondary glaucoma, angoid streaks, retinopathy, and retinal artery occlusion. Commonly reported causes for the incidence of sickle cell disease are extreme temperatures, wind speed, and rainfall. This study has conducted an investigation of recurrent orbital bone sub-periosteal hematoma in a sickle cell patient that was exposed to high altitude areas.Case presentationA 12-year-old boy with SCD developed a recurrent sudden periorbital pain and swelling during a visit to high altitude area. The family reported two similar attacks previously. The patient recovered completely with timely initiated conservative treatment. The case study is about homozygous SCD with previous history of similar attack of painful periorbital swelling that resolved after conservative management. This condition was associated with proptosis, diplopia, and restriction of eye movement. Magnetic resonance imaging of the orbits showed right orbital roof subperiosteal mass adjacent to the orbital wall, which was identified as a subperiosteal haematoma, inducing proptosis. The patient was discharged after 7 days with follow up.ConclusionsInfarction of orbital bones during vaso-occlusive crises in SCD presented acutely with a rapidly progressive painful periorbital swelling. Hematomas frequently complicate the condition, along with the inflammatory swelling that may lead to the orbital compression syndrome. The condition is sight-threatening and necessitates prompt diagnosis along with appropriate management. This condition mandates prompt initiation of conservative treatment and close monitoring of the optic nerve functions to prevent permanent visual loss in young patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%