Myonecrosis in Sickle Cell Anemia: Case Study

Abstract

BACKGROUND Myonecrosis is one of the more poorly studied, painful manifestations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of such symptoms. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and myonecrosis. CASE REPORT 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 crises. Although 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. In an actively sickling patient any invasive intervention should be avoided as it can result in ischemic necrosis of the tissues, due to interruption of capillary flow in end-arteries. CONCLUSIONS 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, off loading the extremity and early fluid resuscitation resolved the pain and swelling and prevented myonecrosis.

Figure 1.

Sagittal post contrast T1 fat saturation image. Sagittal post contrast T1 fat saturation images demonstrated focal enhancement within the plantar muscles of the forefoot, specifically within flexor digitorum and the lumbricales. In addition, there was a focal patchy abnormal increased signal within the calcaneus and distal fibula.

Figure 2.

Short axis post contrast t1 fat sat image. Short axis post contrast T1 fat saturated image showed focal enhancement within the plantar musculature, specifically the flexor digitorum and the lumbricales. The quadratus plantae muscle appears spared.

Figure 3.

Short axis T1 image. The short axis T1 image demonstrated an expansile appearance of the flexor digitorum and lumbricales.

Figure 4.

Sagittal T1. Patchy low T1 signal within the calcaneal body and the distal fibula corresponding to marrow changes from long standing sickle cell infarcts.