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CASE REPORT
Year : 2022  |  Volume : 15  |  Issue : 3  |  Page : 412-414  

Acute soft head syndrome in sickle anemia: The first case report in Kano


1 Department of Paediatrics, Federal Medical Centre, Nguru, Yobe State, Nigeria
2 Department of Paediatrics, Cardiopulmonary Unit, Aminu Kano Teaching Hospital, Kano, Nigeria
3 Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria

Date of Submission24-Apr-2020
Date of Decision18-Jun-2020
Date of Acceptance03-Jul-2020
Date of Web Publication26-Feb-2022

Correspondence Address:
Nuhu Abubakar Garba
Department of Paediatrics, Federal Medical Centre, Nguru, Yobe State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_208_20

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  Abstract 


Sickle cell anemia is an autosomal recessive disorder resulting from insertion of the valine codon at position sixth of beta-hemoglobin gene instead of glutamate leading to the production of insoluble beta-hemoglobin chain. Although sickle anemia is mostly associated with bone pain and anemia, other manifestations also occur. Acute head syndrome is an uncommon complication resulting from skull bone infarction, cortical osteopenia, and rupture of tortuous periosteal blood vessels leading to extravasation of blood into the subgaleal space. This report therefore intends to create awareness that nontraumatic scalp swelling is an uncommon complication in sickle cell disease and no traditional practice or surgical excision should be employed in managing it.

Keywords: Acute soft head syndrome, conservative care, osteomylitis, sickle cell anemia


How to cite this article:
Garba NA, Ahmadu I, Abubakar MS, Asani MO, Aliyu I. Acute soft head syndrome in sickle anemia: The first case report in Kano. Med J DY Patil Vidyapeeth 2022;15:412-4

How to cite this URL:
Garba NA, Ahmadu I, Abubakar MS, Asani MO, Aliyu I. Acute soft head syndrome in sickle anemia: The first case report in Kano. Med J DY Patil Vidyapeeth [serial online] 2022 [cited 2022 May 21];15:412-4. Available from: https://www.mjdrdypv.org/text.asp?2022/15/3/412/338619




  Introduction Top


Sickle cell anemia (SCA) is an autosomal recessive disorder resulting from the insertion of the valine codon at position sixth of beta-hemoglobin gene instead of glutamate leading to the production of insoluble beta-hemoglobin chain.[1],[2] The mutated beta-chain becomes polymerized in the presence of precipitants such as acidosis, hypoxia, dehydration, and fever, this leading to vaso-occlusive crisis (VOC), which is a hallmark of this disease.[1],[2] Although sickle anemia is mostly associated with bone pain and anemia, other manifestations such as acute chest syndrome and acute splenic sequestration have been described, but other skeletal manifestations such as acute soft head syndrome are rare complications that have been scarcely reported.[3] Acute head syndrome which is also known as acute cephalohematoma is an uncommon complication resulting from skull bone infarction, cortical osteopenia, and rupture of tutoursed periosteal blood vessels leading to extravasation of blood into the subgaleal space.[3],[4] This report therefore intends to create awareness that nontraumatic scalp swelling is an uncommon complication in sickle cell disease and no traditional practice or surgical excision should be employed in managing it.


  Case Report Top


We report an 11-year-old known sickle anemia patient who presented with a history of headache, bone pain, jaundice, and fever. Two days at admission, he developed spontaneous, nontraumatic swelling on the left parietal area [Figure 1]. The swelling progressively increased in size; this was associated with pain. However, there was no vomiting, convulsion, or bleeding from any orifice or weakness in any part of the body. Physical examination revealed a jaundiced child, moderately pale, febrile with axillary temperature (38.2°C). Musculoskeletal examination revealed bone tenderness involving the right proximal tibia, but there was no swelling. There was fluctuant swelling on the left parietal region measured 4 cm × 3.5 cm in size, mild tenderness, and normal skin color around it. There was no crepitus around the swelling and other skull bones. The central nervous system examination was essentially normal. The pain score value was moderate using the Wong–Baker Faces Score Chart. The diagnosis of VOC with acute soft head syndrome was considered. The investigation reports showed hemoglobin of 7.0 g/dl, white blood cell of 15 × 103, and platelet (PLT) of 324 × 103 which were essentially normal. Clothing profile showed international normalized ratio of 1.2 and prothrombin time was 13 s which were within normal limits. Transcranial Doppler also revealed a normal mean middle cerebra artery velocity of 110 m/s. The skull radiography showed soft-tissue swelling with normal skull bone architecture and thus ruled out a bone fracture. The patient was given intravenous fluid, intravenous paracetamol, and codeine. He made a significant improvement and he was discharged a week later; the swelling progressively resolved by the 2nd week after discharge.
Figure 1: Left parietal swelling

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  Discussion Top


Acute soft head syndrome is a rare complication of skull manifestation of SCA.[3],[4] The exact mechanism of acute soft head syndrome is not completely understood, but the proposed pathogenesis includes extramedullary hemopoiesis involvement of the flat bones of the skull, bone infarction, osteopenia resulting in cortical bone thinning, and disruption of periosteal structure with extravasation of blood into the subgaleal space.[3],[4],[5],[6],[7] Common symptoms in acute soft head syndrome are headache and swelling; similar findings were also reported by Akodu et al.[4] In our index case, other differential diagnoses of scalp swellings which included trauma, blood dyscrasia, and osteomyelitis were considered; the skull radiography essentially rules out skull fracture. However, brain computer tomography that could give better information was not done due to financial constraints. Transcranial Doppler was normal and this ruled out the possibility of cerebral ischemia, and central nervous system examination was essentially normal. Although it may be difficult in distinguishing severe VOC with osteomylitis in SCA patients,[5] the possibility of osteomyelitis was ruled out in the absence of fever, severe parietal bone tenderness, hyperemia, and no warmness around the mass that may suggest severe acute inflammation commonly associated with acute osteomyelitis. Aspiration of the swelling to further characterize it was discouraged due to clinical improvement and the entertained diagnosis. The body temperature was also normal after 48 h of admission, which was before the onset of the swelling, which could have been persistently high in patients with osteomyelitis depending on the microbiology profile. The clotting profile, liver function test, and PLT count were within normal limits and these had ruled out bleeding diastasis as a possible cause of the swelling. The patient was subsequent discharged following improvement in pain and the child was actively ambulating. The patient was seen after 48 h and again a week later with monitoring of the scalp swelling. The swelling spontaneously regressed at 2nd week of follow-up, and spontaneous regression is the natural history of acute soft head syndrome, as reported in previous communications.[4],[5]


  Conclusion Top


Acute soft head syndrome is a rare complication in SCA. Headache, scalp swelling, and bone pain are the symptoms that may be common to the acute soft head syndrome. The management is supportive of use of intravenous fluid, analgesia, and occasionally use of antibiotic if an infection is suspected, but spontaneous regression is the norm.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Micheal RD, Melissa JF, Elililoff PV. Heamoglobinopathies. In: Robert KS, Bonita FS, editors. Nelson Textbook of Paediatrics. 20th ed. Philadelphia: Elsevier; 2015. p. 1663-1665  Back to cited text no. 1
    
2.
Federal Ministry of Health. National Guideline for the Control and Management of Sickle Cell Aneamia; 2014. Available from: http://www.health.gov.ng. [Last accessed on 2020 Apr 02].  Back to cited text no. 2
    
3.
Emily RM, Jeffery LM. Sickle cell aneamia. Drug 2012;72:895-906.  Back to cited text no. 3
    
4.
Akodu SO, Njokanma OF, Diaku-Akinwumi IN, Ubuane PO, Adediji UO. Acute soft head syndrome in children with sickle cell aneamia in Lagos, Nigeria. Indian J Heamatol Blood Transfus 2014;30:67-9.  Back to cited text no. 4
    
5.
Fortin-Boudreault R, Story E, Simpson E, Johnston D, Chretien C. Spontaneous subgaleal hematoma: An unusual complication of sickle cell disease. J Clin Case Rep 2016;681. Available from: https://www.hilarispublisher.com/open-access/spontaneouss-subgaleal-hematoma-an-unusual-complication-of-sickle-celldisease-2165-7920-1000681.pdf. [Last accessed on 2020 Apr 02].  Back to cited text no. 5
    
6.
Ibrahim AA, Ali HA. Soft head syndrome in sickle cell disease. Saudi Med J 1996;17:804-7.  Back to cited text no. 6
    
7.
Mahmud A, Ahmed FE, Mahmud S. Sickle cell aneamia: Image from head to toe. Egypt J Radiol Nucl Med 2013;44:547-61.  Back to cited text no. 7
    


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