Acute soft head syndrome in a teenager with sickle cell anemia: A case report
Key Clinical Message Sickle cell disease (SCD) rarely presents with acute soft head syndrome (ASHS) often posing a diagnostic dilemma. Recovery is typically spontaneous, however, in the context of lack of awareness and limited brain imaging it could potentially lead to poor outcome. Abstract ASHS is...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-11-01
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| Series: | Clinical Case Reports |
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| Online Access: | https://doi.org/10.1002/ccr3.8174 |
| _version_ | 1797449730551185408 |
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| author | Ng'weina F. Magitta Francisca B. Komanya Baraka O. Alphonce Mbelwa D. Bitesigilwe Emmanuel M. Sindato John R. Meda |
| author_facet | Ng'weina F. Magitta Francisca B. Komanya Baraka O. Alphonce Mbelwa D. Bitesigilwe Emmanuel M. Sindato John R. Meda |
| author_sort | Ng'weina F. Magitta |
| collection | DOAJ |
| description | Key Clinical Message Sickle cell disease (SCD) rarely presents with acute soft head syndrome (ASHS) often posing a diagnostic dilemma. Recovery is typically spontaneous, however, in the context of lack of awareness and limited brain imaging it could potentially lead to poor outcome. 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. |
| first_indexed | 2024-03-09T14:30:17Z |
| format | Article |
| id | doaj.art-f54fbf20aa4a46249bc71f4ecc77d9bd |
| institution | Directory Open Access Journal |
| issn | 2050-0904 |
| language | English |
| last_indexed | 2024-03-09T14:30:17Z |
| publishDate | 2023-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical Case Reports |
| spelling | doaj.art-f54fbf20aa4a46249bc71f4ecc77d9bd2023-11-28T04:14:45ZengWileyClinical Case Reports2050-09042023-11-011111n/an/a10.1002/ccr3.8174Acute soft head syndrome in a teenager with sickle cell anemia: A case reportNg'weina F. Magitta0Francisca B. Komanya1Baraka O. Alphonce2Mbelwa D. Bitesigilwe3Emmanuel M. Sindato4John R. Meda5Department of Internal Medicine, School of Medicine & Dentistry University of Dodoma Dodoma TanzaniaDepartment of Internal Medicine Benjamin Mkapa Hospital Dodoma TanzaniaDepartment of Internal Medicine Benjamin Mkapa Hospital Dodoma TanzaniaDepartment of Radiology & Imaging Benjamin Mkapa Hospital Dodoma TanzaniaDepartment of Internal Medicine, School of Medicine & Dentistry University of Dodoma Dodoma TanzaniaDepartment of Internal Medicine, School of Medicine & Dentistry University of Dodoma Dodoma TanzaniaKey Clinical Message Sickle cell disease (SCD) rarely presents with acute soft head syndrome (ASHS) often posing a diagnostic dilemma. Recovery is typically spontaneous, however, in the context of lack of awareness and limited brain imaging it could potentially lead to poor outcome. 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.https://doi.org/10.1002/ccr3.8174acute soft head syndromebone infarctionextramedullary hematopoiesissickle cell anemiasubgaleal haematoma |
| spellingShingle | Ng'weina F. Magitta Francisca B. Komanya Baraka O. Alphonce Mbelwa D. Bitesigilwe Emmanuel M. Sindato John R. Meda Acute soft head syndrome in a teenager with sickle cell anemia: A case report Clinical Case Reports acute soft head syndrome bone infarction extramedullary hematopoiesis sickle cell anemia subgaleal haematoma |
| title | Acute soft head syndrome in a teenager with sickle cell anemia: A case report |
| title_full | Acute soft head syndrome in a teenager with sickle cell anemia: A case report |
| title_fullStr | Acute soft head syndrome in a teenager with sickle cell anemia: A case report |
| title_full_unstemmed | Acute soft head syndrome in a teenager with sickle cell anemia: A case report |
| title_short | Acute soft head syndrome in a teenager with sickle cell anemia: A case report |
| title_sort | acute soft head syndrome in a teenager with sickle cell anemia a case report |
| topic | acute soft head syndrome bone infarction extramedullary hematopoiesis sickle cell anemia subgaleal haematoma |
| url | https://doi.org/10.1002/ccr3.8174 |
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