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Multiple Fractures in an Infant With Hepatoblastoma and Beckwith-Wiedemann Syndrome

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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.
Title: Multiple Fractures in an Infant With Hepatoblastoma and Beckwith-Wiedemann Syndrome
Description:
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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