Skull Bone Osteomyelitis in A Sickler With Acute Soft Head Syndrome Managed Surgically; A Rare Case Report

Abstract

Mohammed Awad Elzain MD, IFAANS, Modathir Mohammed Osman Fadul, Mohamed Hashim Siddiq, Mohammed Hajo Ibrahim, Mohammed Altair Ali, Omer Elshaikh Omer and Zaghloul Mahmoud Moussa

Introduction: Acute soft head syndrome is an extremely rare complication of sickle cell disease that mostly affects children and young persons. It occurs due to focal necrosis of skull bone secondary to occlusion of blood supply to that region by micro-thrombi. Upon reviewing the literature, only 3 cases were reported and the three of them were managed conservatively. In this paper we would like to present a rare case with acute soft head syndrome as the 1st case with acute soft head syndrome that is been managed surgically.

Case Description: A 17 years old young male, known case of sickle anemia presented with spontaneous forehead swelling for about 10 days duration and pus discharge from the forehead swelling. CT-brain reported Left frontal scalp soft tissue mass with infiltration and destructive changes to the underlying frontal bone. There was no intracranial extension. CT with contrast showed septations with enhancement of the walls of the subgaleal collection and enhancement of the underlying dura. The abscess was evacuated, the bone was looking infected and the periosteum also was unhealthy looking, therefore both were excised. The inner table and outer table were involved at different levels, the abnormal bone was nibbled away until normal edges were encountered. Histopathology revealed XANTHOGRANULOMATOUS INFLAMMATION WITH FIBROPLASTIC REACTION AND CONGESTION COMPATIBLE WITH ORGANIZING ABSCESS. The patient was discharged in a good shape, with intact neurological function, GCS 15/15 afebrile and healthy-looking surgical wound. He was scheduled for elective cranioplasty with custom-made bone flap later on on elective basis.

Conclusion: This case represents a unique presentation of these conditions with osteomyelitis other than the usual presentation with necrosis. This draws our attention to the fact that not all of these cases can be managed conservatively. Brain imaging with contrast is an important diagnostic tool that can pick up the cases that might need surgical intervention. The early surgical intervention fasten patient’s recovery and prevents the spread of infection if any. More studies need to be carried out to establish international guidelines for managing this rare condition.

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