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Lalita Turaga

Lalita Turaga

Advocate Illinois Masonic Hospital/Stroger Hospital of Cook County, USA

Title: Myonecrosis in sickle cell anemia: Case study

Biography

Biography: Lalita Turaga

Abstract

Statement of the problem: Soft tissue, muscle vaso-occlusive disease, is often over-looked and poorly understood complication in patients with sickle cell anemia. We present a case study of a patient in sickle cell crisis with an episode of acute pain and swelling to the intrinsic muscles of the foot as a prominent feature of the crisis.
 
Theoretical Orientation: Myonecrosis is among the more infrequent clinical presentations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of symptoms relating to sickle cell anemia and is limited to anecdotal case reports and case series. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and potential vaso-occlusion. Besides the rarity of the condition, this may be secondary to the fact that sickle cell myonecrosis is a poorly recognized complication amongst diagnosing physicians, and signs and symptoms may not
always be corroborated by positive muscle injury biomarkers.
 
Findings: Muscle biopsy is considered the gold standard for the diagnosis of myositis or myonecrosis, a low intensity signal on T1 and high intensity signal on T2 at the affected muscle belly can be as conclusive as imaging studies.
 
Conclusion & Significance: Early recognition is critical in sickle cell disease management, allowing for prompt and aggressive fluid resuscitation, which remains a cornerstone in the management of most sickle cell vaso-occlusive crises. In this instance, offloading the extremity and early fluid resuscitation proved critical in resolving the pain and swelling while averting progression
to more permanent form of muscle damage and necrosis.