Acute soft head syndrome in a teenager with sickle cell anemia: A case report
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Date
2023
Journal Title
Journal ISSN
Volume Title
Publisher
Wiley
Abstract
ASHS is a rare complication of SCD, invariably occurring near puberty with
hitherto elusive pathogenic mechanisms. ASHS often resolves spontaneously on
conservative management, however, lack of awareness in the context of limited
access to brain imaging could pose diagnostic challenges resulting in inappropriate
management and untoward outcome. We present a case of a teenager who
presented with subtle symptoms for which the diagnosis of sickle cell anemia
(SCA) was delayed until he developed ASHS. LTM was a 16 years old boy with a
history of recurrent joints pain since the age of 6 years, with a family history of
SCA, but had initial negative sickling test. He presented with episodes of multiple
joints pain, unprovoked scalp and left orbital swelling, low-grade fever and
mild headache without any evidence for bleeding diathesis. The diagnosis of SCA
was confirmed by hemoglobin electrophoresis. Computed tomography (CT) scan
of the head revealed subgaleal heamatoma (SGH) and intraorbital haematoma
without intracranial hemorrhage (ICH). He was managed conservatively with
analgesics and hydration together with antibiotics for associated sepsis with complete
resolution of clinical symptoms within 2 weeks. This case represents a rare
scenario for a relatively mild SCA phenotype presenting with ASHS whose diagnosis
poses an enigma in the resource-limited contex. It is therefore, prudent to
recognize ASHS to avoid judicious interventions which could potentially result in
untoward clinical outcome.
Description
Full text. Available at https://doi.org/10.1002/ccr3.8174
Keywords
Citation
Magitta, N. W. F., Komanya, F. B., Alphonce, B. O., Bitesigilwe, M. D., Sindato, E. M., & Meda, J. R. (2023). Acute soft head syndrome in a teenager with sickle cell anemia: A case report. Clinical Case Reports, 11(11), e8174.