Objectives In this retrospective study, we aimed to determine the incidence and distribution of fractures in patients with Alagille syndrome, one of the leading inherited causes of pediatric cholestatic liver disease. Methods Surveys regarding growth, nutrition, and organ involvement were distributed to patient families in the Alagille Syndrome Alliance or The Children’s Hospital of Philadelphia research database. Patients with a history of fracture were identified by their response to one question, and details characterizing each patient’s medical, growth, and fracture history were obtained through chart review and telephone contact. Results Twelve of 42 patients (28%) reported a total of 27 fractures. Patients experienced fractures at a mean age of 5 years, which contrasts with healthy children, in whom fracture incidence peaks in adolescence. Fractures occurred primarily in the lower extremity long bones (70%) and with little or no trauma (84%). Estimated incidence rate calculations yielded 399.6 total fractures/10,000 person years (95% CI = 206.5, 698.0) and 127.6 femur fractures/10,000 person-years (95% CI = 42.4, 297.7). There were no differences in gender, age distribution or organ system involvement between the fracture and no-fracture groups. Conclusions Children with Alagille syndrome may be at risk for pathologic fractures, which manifest at an early age and in a unique distribution favoring the lower extremity long bones. While this preliminary study is limited by small sample size and potential ascertainment bias, the data suggest that larger studies are warranted to further characterize fracture risk and to explore factors contributing to bone fragility in these children.

No abstract

No abstract

Objectives: We describe a novel adjunctive treatment for aortobronchial fistulas.Methods: A foam sclerosing compound consisting of sodium tetradecyl sulfate (STS), lipiodol, and air has been successfully used in the treatment of bleeding gastric varices. We describe a 65-year-old cirrhotic patient with a saccular aortic arch aneurysm who required emergency treatment for a symptomatic aortobronchial fistula. The patient originally presented with a contained rupture of a thoracic aortic aneurysm and underwent urgent placement of a thoracic aortic stent graft. Postoperatively, he developed significant hemoptysis due to an aortobronchial fistula. A chest computed tomography demonstrated a proximal type I endoleak with enlargement of the aneurysmal sac. With the patient deemed not a candidate for open surgical repair, we chose to reinforce the proximal endograft seal zone with a Palmaz stent and ablation of the aneurysm sac with STS/lipiodol foam. The patient recovered from the procedure well and, thereafter, was no longer symptomatic.Results: Thrombosis of the thoracic aneurysm sac and ablation of the aortobronchial fistula led to cessation of bleeding symptoms.Conclusions: In selected cases, sterile STS/lipiodol foam ablation is a potential adjunct to TEVAR for treating symptomatic aortobronchial fistulas.

No abstract

stent types are similar. CSs are associated with an increased risk of postoperative acute limb ischemia and subsequent open revascularization.