Advances in Pediatric Transplantation

Certain fractures in children are highly specific to child abuse. Metabolic bone disease frequently develops in adults and children with cholestatic liver disease (CLD), resulting in weakened bones and a predisposition to pathologic fractures. Fractures in patients with rickets and osteopenia may mimic a bone response to an inflicted injury, which raises concerns about child abuse in children. To explore the outcomes of children with CLD who developed pathologic fractures in the setting of metabolic bone disease, Mercedes Martinez, MD, Medical Director for the Intestinal Transplant Program at the Center for Liver Disease and Abdominal Organ Transplantation at NewYork-Presbyterian, and other researchers at Columbia University including Dana Goldner, MD, Dulce Barrios, MD, Jillian McGuire, LCSW, Susan Brodlie, MS, RD, Jocelyn Brown, MD, and Steven Lobritto, MD, conducted a retrospective chart review of 397 pediatric patients with a diagnosis of CLD who were followed in the NewYork-Presbyterian/Columbia University Irving Medical Center outpatient hepatology clinic between 2010 and 2014 for the incidence of fractures.

Several studies have identified impairment of bone mass and mineral development in children with CLD, particularly those with biliary atresia and Alagille syndrome. In addition, the risk for fractures is higher in pediatric patients following LT than in the general pediatric population. In this study, the Columbia researchers identified 15 patients with CLD who developed pathologic fractures in the setting of metabolic bone disease.

• For 10 (67%) of the 15 patients, bone fractures occurred post-liver transplant (LT), with 7 (70%) of those 10 cases occurring within 1 year of LT. These 7 cases had all been exposed to high-dose systemic steroids within the 6 months before fracture occurrence, which is known to cause increased bone resorption, decreased bone formation, and calcium malabsorption.
• Five out of the 10 families were reported to Child Protection Services agencies for concern of child abuse.
• In all 5 cases, the CPS agencies ultimately determined that there was no evidence of abuse.

The findings point to the need for increased clinical awareness of fracture risk, even after the resolution of CLD, particularly during the peritransplant period when this risk may be greatest. In addition, the presence of osteoporosis or rickets on radiological examination may suggest other possible etiologies for fracture aside from nonaccidental trauma.