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Multiple Fractures in an Infant With Hepatoblastoma and Beckwith-Wiedemann Syndrome
Toni Eimicke1, Jonathan Swartz2
1Pediatric Endocrinology, The Barbara Bush Children's Hospital at Maine Medical Center, Portland, ME 04102, USA.
Insights
Children with hepatoblastoma experience more fractures. A premature infant with Beckwith-Wiedemann syndrome and hepatoblastoma showed fracture healing after zoledronic acid (ZA) treatment, suggesting a potential therapeutic role.
Area of Science:
- Pediatric Oncology
- Pediatric Endocrinology
- Pediatric Orthopedics
Background:
- Hepatoblastoma in children is associated with an increased incidence of fractures.
- Limited data exist on the specific causes and management of fractures in this population.
Observation:
- A premature 4-month-old infant with Beckwith-Wiedemann syndrome and hepatoblastoma presented with multiple severe fractures.
- While prematurity and metabolic bone disease were considered, they did not fully explain the fracture severity.
Findings:
- The patient received chemotherapy and surgical resection for hepatoblastoma.
- Following treatment with zoledronic acid (ZA), skeletal surveys showed fracture healing and no new fractures.
- Long-term follow-up indicated continued absence of new fractures and appropriate motor development.
Implications:
- This case highlights the potential for zoledronic acid (ZA) in managing pathological fractures in pediatric cancer patients.
- Understanding compounded fracture risks (prematurity, chronic illness, hepatoblastoma) is crucial for prevention and treatment strategies.
- Further research is needed to elucidate fracture pathophysiology and guide preventative measures in hepatoblastoma patients.
Abstract:
Children with hepatoblastoma have an increased incidence of fractures, but data are limited. Previous reports document an average of 4 fractures per child with hepatoblastoma. We present a severe case of a premature 4-month-old with multiple fractures in the setting of Beckwith-Wiedemann syndrome and hepatoblastoma. Although prematurity is a known risk for metabolic bone disease, it did not entirely explain the severity. Our patient underwent chemotherapy and surgical resection of his hepatoblastoma. Once deemed stable, he received a dose of zoledronic acid (ZA). One month post treatment with ZA, a skeletal survey revealed healing of the rib and femoral fractures and no new fractures. Five months post ZA, the skeletal survey revealed no new fractures and motor development was appropriate. An extensive search revealed scant literature on the rate or cause of pathologic fractures in patients with newly diagnosed hepatoblastoma. A better understanding of fracture risk in this population may guide prevention strategies, screening, and treatment. In our case, prematurity and substantial chronic illness may have compounded the known fracture risk associated with hepatoblastoma and may provide insight into the pathophysiology and prevention of fractures in this setting.
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