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A 9-year-old female with complicated comorbidities presents with swelling of leg, knee
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Fifteen years ago, a 9-year-old female with Riley-Day syndrome — hereditary sensory and autonomic neuropathy type III — was being followed by orthopedics with a 1-week history of progressive swelling of her left leg about the knee and distal thigh.
It was initially thought to be due to an injury because she was afebrile and was known to have self-destructive behavior in the past, including fractures. However, on the day of admission a week later, she was found to have a temperature of 101°F. Examination at that time was positive for increased swelling and erythema about the knee, and an arthrocentesis was performed revealing purulent fluid with gram-positive cocci, and she was admitted and treated with vancomycin. The culture grew methicillin-sensitive Staphylococcus aureus and treatment was changed to nafcillin. Radiographs of the femur on admission are shown in Figures 1 and 2 (pre-dates routine MRI).
James H. Brien
Due to her PMHx of congenital insensitivity to pain with anhidrosis, she has a long history of thermal instability, including recurrent high fevers without infections, as well as multiple infections complicating frequent injuries, including fractures. She was also found to have some mild to moderate developmental delay with several psychiatric diagnoses.
Radiographs of the femur on admission are shown in Figures 1 and 2.
Source: Brien JH
As 2 weeks of treatment of her septic knee approached, her inflammatory markers were noted to be rising, along with swelling of the left thigh at the knee and hip. The current radiograph of the left leg is shown in Figure 3.
The current radiograph of the left leg is shown in Figure 3.
What’s Your Diagnosis?
A. Healing fractures
B. Chronic recurrent multifocal osteomyelitis
C. Bone tumor
D. Advanced osteomyelitis
This is a very complicated child, with a rare case of congenital insensitivity to pain that was presumably hereditary sensory and autonomic neuropathy (HSAN) type III, or Riley-Day syndrome. However, she had a fairly straight-forward case of severe bacterial osteomyelitis. The patient had a history of inability to sweat (anhidrosis), which would more correctly be HSAN-IV. There are at least five types of HSAN with variations of similar features. In this patient, in addition to the insensitivity to pain, she had temperature instability due to the anhidrosis, along with developmental delay and some self-destructive behavior, placing her at increased risk of infections becoming advanced before diagnosis. In fact, malignant hyperthermia and sepsis are the leading causes of death in these children, usually before adulthood.
The patient had extensive surgery to debride the infection, utilizing antimicrobial-impregnated methyl methacrylate beads (Figures 4 and 5).
While all can see that Figure 3 was grossly abnormal, when the radiographs in Figures 2 and 3 were retrospectively reviewed by radiology, it was apparent that there were some lytic lesions in the distal femur that, although subtle, were probably associated with the osteomyelitis diagnosed later. The patient had extensive surgery to debride the infection, utilizing antimicrobial-impregnated methyl methacrylate beads (Figures 4 and 5); returning at least once to the operating room for more irrigation and debridement. Methicillin-sensitive S. aureus and Klebsiella pneumoniae were recovered from the deep tissues. She was treated with culture-directed IV antimicrobial therapy for 6 weeks and, eventually, went home on oral cephalexin, and was then lost to follow-up after moving from the area.
Antibiotic-impregnated beads have been used in complicated bone infections since the 1970s to fill in the dead spaces with poor circulation. There are scientific animal studies showing good elution characteristics into bone and granulation tissue, but very little prospective human data on efficacy and safety. Almost all published studies are small retrospective reviews. However, many of us believe that the animal data and human experience would seem to point toward benefit for severe, complicated bone infections.
A healing fracture shows callus formation (Figure 6). A tumor may be accompanied by a pathologic fracture, as well as a mass on plain radiograph (Figure 7).
A healing fracture shows callus formation (Figure 6) and not periosteal elevation along the entire length of the femur, as well as the bone destruction shown in this case.
A tumor may be accompanied by a pathologic fracture, as well as a mass on plain radiograph (Figure 7), and would not involve the entire length of the bone.
Recurrent multifocal osteomyelitis is a benign condition of unknown etiology that results in multiple bone lesions with a history of recurrence. Since its description in the early 1970s, it has been found to be associated with synovitis, acne, palmoplantar pustulosis, hyperostosis and osteitis (SAPHO syndrome). Propionibacterium acnes has been isolated from the bone in a few cases, prompting some to recommend treating with antimicrobials, but has not been consistent. The mainstay of treatment has been anti-inflammatory drugs and close follow-up.
Columnist Comments
During this time of family gatherings, the Brien family wishes you all a Happy Thanksgiving and hope you have a healthy winter. It’s not too late for your influenza immunization. It is good for you, your family and your patients. STOP READING THIS AND GET IT NOW!
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James H. Brien, DO, is vice chair for education in the department of pediatrics at McLane Children’s Hospital at Scott & White/Texas A & M College of Medicine in Temple, Texas. He is also a member of the Infectious Diseases in Children Editorial Board.
Disclosure: Brien reports no relevant financial disclosures.