When diagnosing patients who have sickle cell disease (SCD) with the various complex manifestations of their disease, it is considerably difficult to differentiate between an acute vaso-occlusive crisis and osteomyelitis, with multifocal osteomyelitis presenting an even more challenging situation.
A case study involving a 22-month-old female patient with SCD dealing with this issue was published in the journal Clinical Case Reports.
Osteomyelitis is frequently overlooked in individuals with SCD, as the occurrence of vaso-occlusive crisis is substantially more common and is the most often reported disease manifestation among children. To date, no laboratory test or imaging modality is available to reliably differentiate between vaso-occlusive crisis and osteomyelitis in those with SCD. It is of critical importance to establish an accurate diagnosis, as overlooking osteomyelitis can be associated with numerous dangerous implications, including bone deformities, chronic osteomyelitis, and sepsis.
Investigation into the most efficient, accurate imaging technique for multifocal osteomyelitis is warranted, with possible use of whole body magnetic resonance imaging (MRI) or positron emission tomography-computed tomography (PET-CT) scans being considered. The current case study describes a young girl with SCD who developed multifocal osteomyelitis and Salmonella bacteremia. The researchers sought to review the existing literature on the utility of various imaging studies for validation of this diagnosis.
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The patient described presented to the emergency department having experienced 3 days of left foot pain and swelling, along with the refusal to ambulate. No fever, erythema, or trauma was reported. The child was afebrile and had mild tachycardia, but did not appear to be toxic. Bilateral swelling of her feet and toes was observed, together with tenderness on palpation and greater swelling in the left foot. Both of her feet were warm and well perfused, with the sensation intact.
Laboratory assessments revealed leukocytosis, anemia, and an elevated C-reactive protein (CRP) level. No fractures, dislocations, or other abnormalities were noted on X-ray. She was admitted for pain control for an assumed vaso-occlusive pain crisis.
The young girl’s fever spiked and ceftriaxone was initiated the next day. She also received a packed red blood cell transfusion and pain medications. Her blood culture was positive for Salmonella; inflammatory markers revealed an elevated erythrocyte sedimentation rate and CRP level. At this point, the patient’s differential diagnosis was expanded to osteomyelitis vs septic joint vs bony abscess.
Based on an orthopedic consultation and aspiration of fluid from her left ankle joint, vancomycin was added to her treatment regimen. An MRI revealed osteomyelitis of her left calcaneus, outer cuneiform, and tibial diaphysis. A large plantar soft tissue abscess from the calcaneus anteriorly to her forefoot was observed as well, with an additional abscess detected lateral to the calcaneal body. She ultimately underwent irrigation and debridement of the left ankle joint and left foot plantar abscess.
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Upon worsening fever and right hand swelling on day 7 of her hospitalization, she underwent a PET-CT scan that revealed multifocal areas of hypermetabolism in her right foot and hand, which were a matter of concern regarding additional foci of osteomyelitis. Additional MRIs of her right hand revealed multifocal sites of concern for osteomyelitis and a subperiosteal abscess in the fourth metacarpal. She underwent repeat left ankle incision and drainage, with left calcaneus and lateral cuneiform drilling.
Following the 2 surgical interventions, the patient began to demonstrate clinical improvement. By day 20 of hospitalization, her CRP level decreased and was considered normal. To date, she has not experienced any osteomyelitis recurrences or other serious infections.
“There is currently no gold standard diagnostic work-up for patients with SCD,” the researchers indicated. “Missing osteomyelitis can be detrimental—causing chronic osteomyelitis and bone deformities. Each case must be evaluated individually with a focus on the history, the physical exam, the laboratory work-up, and imaging findings,” they noted. “Whereas MRIs will be accurate in these cases, PET scans can be more efficient in whole body imaging, and have greater sensitivity and specificity in diagnosing osteomyelitis,” they concluded.
Scruggs M, Pateva I. Multifocal osteomyelitis in a child with sickle cell disease and review of the literature regarding best diagnostic approach. Clin Case Rep. Published online July 2, 2023. doi:10.1002/ccr3.7288