Afectación muscular en la anemia drepanocítica

Myonecrosis in Sickle Cell Anemia: Case Study

“…Our patient did not have elevated CK levels, a finding which goes in favor of myonecrosis, which was also described by Tageja et al where only three patients had elevated CK/LDH levels and the remaining three did not [ 3 ]. A similar case was described by Rubio et al where the patient was a heterozygous carrier of sickle cell anemia, who presented with normal CK levels and due to non-conclusive etiologic study, an MRI was performed with a diagnosis of fasciitis [ 4 ].…”

“…Soft tissue, in particular muscle vaso-occlusive disease, is often overlooked in patients with sickle cell anemia. This is probably more common than recognized [ 4 ]. The MRI results and the clinical presentation of muscle inflammation especially after exertion, and pain on range of motion is characteristic of myositis with possible myonecrosis.…”

“…Other atypical features may include compartment syndrome from myoedema which is one of the few indications necessitating surgical intervention [ 13 ]. Another differential of this presentation could include fasciitis which has been described by Rubio et al where the patient with sickle cell trait presented with acute pain in her lower extremities and due to non-conclusive etiology, an MRI was performed which revealed perifascial hyperintensity with no significant signal changes or focal muscular hypertrophy [ 4 ]. In our case, prominent flexor digitorum brevis was noted on MRI, a phenomenon which could be related to restricted blood flow, as cited by Yasuda et al [ 15 ].…”

“…Patients should be kept warm and well hydrated, with the administration of intravenous fluids. NSAIDs is often sufficient to control pain [ 4 ]. Although muscle biopsy is the gold standard to diagnose myositis from myonecrosis, it is not advisable to perform any surgical intervention in an actively sickling patient, because it can cause ischemic necrosis due to interruption of capillary flow in end arties.…”

“…As Grove et al states: “T2 weighted sequences generally allow easy identification of intramuscular edema, showing it to be hyperintense, fat suppression T2 may increase the conspicuity of edema, is consistent with our MRI findings [ 13 ].” The authors presumptive diagnosis and treatment plan was similar to the one described by Groves et al [ 13 ]. The importance of using MRI especially in patients with sickle cell carriers and sickle cell disease with musculoskeletal pain is also well emphasized by Rubio et al [ 4 ]. If MRI scan is conclusive for myonecrosis or myositis, early physical therapy is necessary to reduce or prevent muscle atrophy in both instances.…”