Acute Soft Head Syndrome (Subgaleal Haematoma) with Periorbital Oedema as a Rare Presentation in Sickle Cell Disease
  • Rehab Yusuf AL-Ansari
    Adult Hematology Unit, Internal Medicine Department, KFMMC, Dhahran, Kingdom of Saudi Arabia
  • Maan Al Harbi
    Internal Medicine Department, KFMMC, Dhahran, Kingdom of Saudi Arabia
  • Nawaf Al-Jubair
    Neuroradiology unit, Radiology Department, KFMMC, Dhahra, Kingdom of Saudi Arabia
  • Leena Abdalla
    Adult Hematology Unit, Internal Medicine Department, KFMMC, Dhahran, Kingdom of Saudi Arabia

Keywords

Sickle cell, subgaleal hematoma, periorbital edema, soft head syndrome, orbital compression syndrome

Abstract

Background: Sickle cell disease is a genetic condition frequently found in Africa and the Arabian Peninsula. Uncommon complications include subgaleal haematoma (soft head syndrome) and periorbital oedema.
Case presentation: A 17-year-old male patient presented with body aches and progressive right parieto-temporal and frontal head swelling. Physical examination revealed puffiness of the right eye that progressed rapidly to reddish periorbital oedema sparing the extraocular muscle and pupil response to light. CT and MRI of the brain suggested multiple subgaleal haematomas (soft head syndrome) and right periorbital oedema.
Conclusion:Subgaleal haematoma (soft head syndrome) and periorbital oedema are uncommon complications of sickle cell disease. Management is conservative rather than surgical.

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References

  • Adekile A, Makani J. Sickle cell disease in Africa and the Arabian Peninsula: current management and challenges. In: Costa F, Conran N, editors. Sickle Cell Anemia. Springer; 2016, p. 339–370.
  • Ganesh A, William R, Mitra S, Yanamadala S, Hussein SS, Al-Kindi M, et al. Orbital involvement in sickle cell disease: a report of five cases and review literature. Eye 2001;15:774–780.
  • Foula M, Hassan A, AlQurashi A, Alsaihati A, Sharroufna M. Spontaneous subgaleal hematoma in a patient with sickle cell disease: a case report and literature review. Clin Case Rep 2019;7:2220–2224.
  • Alqurashi M, Raslan O, Gmati G. Spontaneous subgaleal hematoma in a sickle cell disease patient: a case report. J Med Cases 2020;11(2):46–48.
  • Jastaniaha W. Epidemiology of sickle cell disease in Saudi Arabia. Ann Saudi Med 2011;31(3):289–293.
  • Garty I, Koren A, Katzumi E. Uncommon sites of bone infarction in a sickle cell anemia patient. Eur J Nucl Med 1983;8(8):367–368.
  • Koizumi K, Suzuki S, Utsuki S, Nakahara K, Niki J, Mabuchi I, et al. A case of non-traumatic subgaleal hematoma effectively treated with endovascular surgery. Interv Neuroradiol 2010;16(3):317–321.
  • Arends S, Coebergh JA, Kerkhoffs JL, van Gils A, Koppen H. Severe unilateral headache caused by skull bone infarction with epidural haematoma in a patient with sickle cell disease. Cephalalgia 2011;31(12):1325–1328.
  • Dahdaleh NS, Lindley TE, Kirby PA, Oya H, Howard 3rd MA. A "neurosurgical crisis" of sickle cell disease. J Neurosurg Pediatr 2009;4:532–535.
  • Emerson GG, Lutty GA. Effects of sickle cell disease on the eye: clinical features and treatment. Hematol Oncol Clin North Am 2005;19(5):957–973.
  • Sokol JA, Baron E, Lantos G, Kazim M. Orbital compression syndrome in sickle cell disease. Ophthalmic Plast Reconstr Surg 2008;24:181–184.

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    Published: 2020-07-30
    Issue: 2020: Vol 7 No 10 (view)

    How to cite:
    1.
    AL-Ansari RY, Al Harbi M, Al-Jubair N, Abdalla L. Acute Soft Head Syndrome (Subgaleal Haematoma) with Periorbital Oedema as a Rare Presentation in Sickle Cell Disease. EJCRIM 2020;7 doi:10.12890/2020_001766.

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