Case Report of International Journal of Case Reports
Acute soft head syndrome and a mini review of bone and neurologic complications in patients with sickle cell disease
Ehab Hanafy1, Saad Al Amri2, AbdulKareem Alenazi2, Amal Al Balawi2, Nadia Abdullah3, Nazim Faisal1, Gihan Mahmoud1
1Prince Sultan Oncology Center, King Salman Armed Forces Hospital, Tabuk, Kingdom of Saudi Arabia.
2 Pediatric Department, King Salman Armed Forces Hospital, Tabuk, Kingdom of Saudi Arabia.
3 Faculty of Medicine, Tabuk University, Tabuk, Kingdom of Saudi Arabia.
Introduction: Acute soft head syndrome is a rare complication in patients with sickle cell disease (SCD) that is characterized by progressive skull pain and swelling, it is usually related to skull bone infarction and full recovery is achieved with conservative management. This case represents a very rare association of sickle cell disease and acute soft head syndrome that necessitates to be differentiated from other bone and neurologic presentations occurring in this entity of patients.
Case: We report a rare case of an 11 years old Saudi male patient with sickle cell disease who developed acute soft head syndrome that presented with severe headache. An underlying osteomyelitis of skull and lower limb bones couldn’t be excluded and the patient had full recovery after conservative treatment.
Conclusion: Acute soft bone syndrome seldomly occurs in SCD, however, its presentations mimics those of bone and neurologic complications so differentiation is essential in order to set up a clear plan of management.
Keywords: Sickle cell disease, acute soft head syndrome, stroke, headache, osteomyelitis
How to cite this article:
Ehab Hanafy, Saad Al Amri, AbdulKareem Alenazi, Amal Al Balawi, Nadia Abdullah, Nazim Faisal, Gihan Mahmoud. Acute soft head syndrome and a mini review of bone and neurologic complications in patients with sickle cell disease. International Journal of Case Reports, 2019 4:80. DOI: 10.28933/ijcr-2019-06-2606
1. Hassan A. Al-Jafar, Raed Alroughani, Thamer A.Abdullah, and Fatma Al-Qallaf. Neurological Complications in Sickle Cell Disease. International Journal of Clinical and Experimental Neurology, vol. 4, no. 1 (2016): 9-18. doi: 10.12691/ijcen-4-1-2.
2. Arends S, Coebergh JA, Kerkhoffs JL, et al (2011) Severe unilateral headache caused by skull bone infarction with epidural haematoma in a patient with sickle cell disease. Cephalalgia31:1325–28.
3. Hoffbrand, A. V, Higgs, Douglas R, Keeling, David, Mehta, Atul B. (2016) Postgraduate Haematology. 7th edition. Blackwell Publishing Ltd., Oxford, UK.
4. Ekaterini Solomou, Pantelis Kraniotis, Alexandra Kourakli, and Theodore Petsas. Extent of Silent Cerebral Infarcts in Adult Sickle-Cell Disease Patients on Magnetic Resonance Imaging: Is There a Correlation with the Clinical Severity of Disease? Hematol Rep. 2013 Jan 25; 5(1): 8-12.
5. Berkelhammer LD, Williamson AL, Sanford SD, Dirksen CL, Sharp WG, Margulies AS, et al. Neurocognitive sequelae of pediatric sickle cell disease: a review of the literature. Child Neuropsychol 2007;13:120-131.
6. Jastaniah W. Epidemiology of sickle cell disease in Saudi Arabia. Ann Saudi Med. 2011;31(3):289–293. doi:10.4103/0256-4947.81540.
7. Akodu SO, Njokanma OF, Diaku-Akinwumi IN, Ubuane PO, Adediji UO. Acute soft head syndrome in children with sickle cell anaemia in lagos, Nigeria. Indian J Hematol Blood Transfus. 2014;30(Suppl 1):67–69. doi:10.1007/s12288-013-0251-6.
8. Al-Dabbous IA, Al-Jama AH (1996) Acute soft head syndrome in sickle cell disease (A case report). Saudi Med J17:804–07.
9. Fortin-Boudreault R, Story E, Simpson E, Johnston D, Chretien C (2016) Spontaneous Subgaleal Hematoma: An Unusual Complication of Sickle Cell Disease. J Clin Case Rep 6: 681. doi:10.4172/2165-7920.1000681.
10. Kalala Okito JP, Van Damme O, Calliauw L. (2004) Are spontaneous epidural haematoma in sickle cell disease a rare complication? A report of two new cases. Acta Neurochir (Wien)146:407 10.
11. Dahdaleh NS, Lindley TE, Kirby PA, et al. (2009) A. J Neurosurg Pediatr4:532–35
12. Saito N, Nadgir RN, Flower EN, Sakai O (2010) Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics 30: 1021-1034.
13. da Silva Junior GB, Daher Ede F, da Rocha FA. Osteoarticular involvement in sickle cell disease. Rev Bras Hematol Hemoter. 2012;34(2):156–164. doi:10.5581/1516-8484.20120036
14. Redwood AM, Williams EM, Desal P, et al: Climate and painful crisis of sickle-cell disease in Jamaica. Br Med J 1:66, 1976.
15. Fontalis A, Hughes K, Nguyen MP, et al. The challenge of differentiating vaso-occlusive crises from osteomyelitis in children with sickle cell disease and bone pain: A 15-year retrospective review. J Child Orthop. 2019;13(1):33–39. doi:10.1302/1863-2548.12.180094
16. Berger E, Saunders N, Wang L, Friedman JN (2009) Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis.Arch Pediatr Adolesc Med 163: 251-255.
17. Miller ST, Sleeper LA, Pegelow CH, et al: Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 342:83, 2000.
18. Diggs LW: Bone and joint lesions in sickle-cell disease. Clin Orthop Relat Res 52:119, 1967.
19. Milner PF, Kraus AP, Sebes JI, et al: Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med 325:1476, 1991.
20. Strouse, John J., Sophie Lanzkron, and Victor Urrutia. The epidemiology, evaluation and treatment of stroke in adults with sickle cell disease. Expert review of hematology 4.6 (2011): 597-606.
21. Webb, Jennifer, and Janet L. Kwiatkowski. Stroke in patients with sickle cell disease. Expert review of hematology (2013).
22. Switzer, Jeffrey A., et al. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. The Lancet Neurology 5.6 (2006): 501-512.
This work and its PDF file(s) are licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.