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An 8-month-old male presents with fever and sore throat
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Occasionally, I receive feedback that my cases are too easy. This case should satisfy the “too easy” critics.
An 8-month-old male presented to his primary care physician with the complaint of 3 days of fever of up to 102°F.
At the time, he was found to have some erythema of his throat and was diagnosed with viral pharyngitis and treated symptomatically. That same evening, the mother noted the baby’s right lower extremity was a bit swollen with some erythema between the knee and ankle. He was taken to a local ED and was diagnosed with cellulitis, given an injection of an antibiotic and sent home with a prescription for an unknown oral antibiotic. The swelling worsened during the next 3 days, fever continued and the baby was taken back to the PCP, who sent the patient to the hospital for admission.
His past medical history was that of a previously healthy baby with some mild gastroesophageal reflux and an episode of respiratory syncytial virus bronchiolitis. There’s no history of injury. His immunizations are documented up to date. His family and social history are also normal.
Examination revealed a normal-appearing 8-month-old boy with a temperature of 101°F, heart rate of 180, respiratory rate of 32 and capillary refill of 3 seconds. The only positive finding was related to the right leg, which was swollen, erythematous and painful from the knee to the ankle on the lateral aspect, with no evidence of previous trauma.
Initial lab tests included elevated inflammatory markers: erythrocyte sedimentation rate (ESR) of 67 mm/hour, a C-reactive protein (CRP) level of 104 mg/L and white blood cell count of 30,000 cells/mcL. Admitting radiographs revealed the image in Figure 1.
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Figure 1: Admitting radiographs
revealed the image. |
Because of his admitting vital signs and concern for possible early sepsis, he had two blood cultures drawn, received IV fluids and had ceftriaxone (100 mg/kg/24 hours) and vancomycin (60 mg/kg per day divided every 6 hours) started. He was taken to the OR the next day and had some purulent material drained from deep soft tissue plains, and the fibula cortex drilled with no purulence seen. A Gram stain of the pus showed gram-positive cocci in clusters, but the culture was negative. For what it’s worth, his nasal methicillin-resistant Staphylococcus aureus screen was negative for MRSA but positive for MSSA.
He quickly improved during the first week of therapy and was discharged with a peripherally inserted central catheter (PICC) line on IV clindamycin (40 mg/kg per day divided every 8 hours) for presumed S. aureus sepsis, cellulitis and possible osteomyelitis with weekly follow-up. Within 1 month, his inflammatory markers had normalized, and he was clinically normal for age. After 5 weeks of IV clindamycin, his PICC accidentally came out, and the antibiotic was discontinued. An end of therapy, radiograph was ordered (Figure 2) and the patient went home. He failed to show for follow-up appointments in subsequent weeks. When the family returned to their PCP, 6 more weeks had gone by, and he was referred back to orthopedics. At that point, his clinical status and activity was still normal and the inflammatory markers were only slightly elevated in the wake of a recent upper respiratory infection. The decision was made not to go back on antibiotic therapy in lieu of close follow-up, and at 3 months after the original presentation, the radiograph revealed Figure 3. Follow-up at 5 months after the onset, his exam, ESR and CRP remained normal.
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Figure 2: After 5 weeks of IV clindamycin, his PICC accidentally came out, and the
antibiotic was discontinued. An end of therapy, radiograph was ordered. |
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Figure 3: At 3
months after the original presentation.
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What’s Your Diagnosis?
A.Gorham’s disease
B.Chronic osteomyelitis
C.Osteosarcoma
D.Bone loss secondary to acute osteomyelitis
This is an unusual and unfortunate complication of acute osteomyelitis with bone loss (answer D). The underlying cause is thought to be the inhibition of osteoblasts and increased activity of osteoclasts as a result of chemical mediators stimulated by certain bacterial infections, such as increasing receptor activator of nuclear factor-kappa-ligand (RANKL). Inflammatory mediators have also been linked to inhibiting production of osteoprotegerin (OPG), which helps regulate the action of RANKL (for those who really want to know). It is unclear why this complication occurs in a few and not the vast majority, but it may be related to certain bacteria, inducing expression of mRNA encoding for RANKL. To read more about this unusual condition, you will probably have to consult the orthopedic literature.
To correct the defect, 8 months after initial hospitalization, the patient underwent a distal fibular osteotomy and placement of an external fixator for distraction osteogenesis (Figure 4). However, this has shown to be of little benefit (Figure 5, taken 1 month later), and his condition is still being followed by pediatric orthopedics.
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Figure 4: The
patient underwent a distal fibular osteotomy and placement of an external
fixator for distraction osteogenesis. |
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Figure 5: However, this has shown to be
of little benefit (Figure 5 taken 1 month later), and his condition is still
being followed by pediatric orthopedics. |
Gorham’s disease is a rare, congenital, noninfectious loss of bone, aka disappearing bone syndrome (Gorham LW. Am J Med. 1954;17:674-682). It may look the same radiographically, but the underlying pathology has to do with the nonmalignant proliferation of vascular channels, resulting in the destruction of bone. It is often diagnosed after a pathologic fracture or as part of an evaluation of dull, aching pain. Treatment is mostly surgical, but medical and radiation-type therapies have also been used; all with mixed results.
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Figure 6: Chronic osteomyelitis can certainly result in destruction of bone, but
it is usually more localized, often walled off as an abscess. |
Chronic osteomyelitis can certainly result in destruction of bone, but it is usually more localized, often walled off as an abscess (Brodie abscess as shown in Figure 6). However, chronic osteomyelitis can also result in disappearing bone as shown in Figures 7 and 8; radiographs taken several months apart of an infant who developed chronic osteomyelitis of the proximal humerus. In either case, it usually results from inadequate medical and/or surgical therapy of acute hematogenous osteomyelitis or infection with a foreign body. The best management includes thorough surgical debridement (Figure 9) and long-term IV and oral antibiotic therapy (often up to a year).
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Figure 7: Chronic osteomyelitis can also result in
disappearing bone as shown. |
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Figure 8: Chronic osteomyelitis can also result in
disappearing bone as shown. |
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Figure 9: The best management includes thorough surgical debridement. |
Lastly, bone cancer will usually have a radiograph with a mass within the bone, often associated with a pathologic fracture (Figure 10).
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Figure 10: Bone cancer will usually have a radiograph with a mass within
the bone, often associated with a pathologic fracture. |
Columnist comment
Most of the time, strange cases turn out to be unusual manifestations of common diseases. However, once in a “Blue Moon,” you might be dealing with a common manifestation of a rare disease.
James H. Brien, DO, is Vice Chair for Education at The Children’s Hospital at Scott and White and is the Associate Professor of Pediatrics at Texas A&M University, College of Medicine, Temple, Texas. email: jhbrien@aol.com. Disclosure: Dr. Brien reports no relevant financial disclosures.
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