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Correspondence

Myonecrosis in Sickle Cell Anemia

N Engl J Med 1999; 340:483February 11, 1999

Article

To the Editor:

Myonecrosis is a rare complication of sickle cell anemia. We report the development of myonecrosis with a sterile abscess in a 24-year-old black woman with sickle cell anemia, who was admitted to the hospital because of severe pain and numbness in the left thigh. She had had numerous admissions to the hospital for painful crises. Examination of the legs revealed mild swelling of the left thigh, without erythema or warmth. There was decreased fine sensation from midthigh to midcalf. The rest of the physical examination was unremarkable. Blood cultures were negative. Radiographs of both legs and magnetic resonance imaging of the lumbosacral spine were normal.

Over the next few days, the swelling of the left thigh increased and was followed by tense swelling of both thighs and limitation in the range of motion of the knees. There was increasing pain, and the serum creatine kinase level rose to 644 U per liter. No intramuscular injection had been administered to the affected muscle groups during the hospitalization. Magnetic resonance imaging of the thighs with gadolinium enhancement revealed a high signal intensity, with increased signals in T2-weighted images as compared with T1-weighted images.

Three weeks later, a localized, fluctuant swelling of the right thigh developed that was distinct from the underlying swelling. Computed tomographic scanning of the thigh revealed a walled-off 10-ml collection of fluid in the vastus lateralis muscle (Figure 1Figure 1Computed Tomographic Scan of the Thigh, Showing a 10-ml Collection of Fluid in the Vastus Lateralis Muscle.). Incision and drainage revealed an abscess that had a smooth interior wall and contained brownish fluid and no pus. There were rare white cells, but no organisms in the fluid, and all cultures of the fluid were negative. The patient had not received any antibiotics for 16 days. The abscess was allowed to drain and healed within about five weeks.

Myonecrosis is an overlooked, painful complication of sickle cell anemia.1,2 During painful crises, the symptoms of myonecrosis may be attributed to skeletal pathology. Myonecrosis is usually manifested by symmetric proximal muscle swelling, severe pain, warmth, increased serum creatine kinase levels, and characteristic findings on magnetic resonance imaging.1-5 Myonecrosis may result in fibrosis and contracture, making early diagnosis and early involvement of a physical therapist crucial.1,5 Another complication is the liquefaction of muscle, which can lead to the development of a sterile abscess. It is unclear whether it is necessary to drain such abscesses.

Behnam Malekgoudarzi, M.D.
Stephen Feffer, M.D.
Nassau County Medical Center, East Meadow, NY 11554

5 References
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    Feldman F, Zwass A, Staron RB, Haramati N. MRI of soft tissue abnormalities: a primary cause of sickle cell crisis. Skeletal Radiol 1993;22:501-506
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    Mani S, Duffy TP. Sickle myonecrosis revisited. Am J Med 1993;95:525-530
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    Valeriano-Marcet J, Kerr LD. Myonecrosis and myofibrosis as complications of sickle cell anemia. Ann Intern Med 1991;115:99-101
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Citing Articles (1)

Citing Articles

  1. 1

    Perla Vicari, Ruth Achkar, Kathia R.B. Oliveira, Milton L. Miszpupten, Artur R.C. Fernandes, Maria S. Figueiredo, José O. Bordin. (2004) Myonecrosis in Sickle Cell Anemia: Case Report and Review of the Literature. Southern Medical Journal 97:9, 894-896
    CrossRef