Percutaneous interventional radiology procedures in patients with epidermolysis bullosa: modifications and challenges.

OBJECTIVE

The purpose of this study is to describe the role of interventional radiology in palliation and maintenance of nutritional support in children with epidermolysis bullosa, with a focus on safety and required procedural modifications.

MATERIALS AND METHODS

This was a retrospective study of all patients diagnosed with epidermolysis bullosa who underwent interventional radiology procedures between January 1991 and December 2008 at a pediatric tertiary care institution. The type of epidermolysis bullosa, the patient's age, the indication and type of interventional radiology procedure, modifications used, and complications were recorded.

RESULTS

Fifteen patients (9 boys and 6 girls) with a diagnosis of epidermolysis bullosa underwent a total of 87 procedures during 82 different patient interventional radiology visits. Twenty-seven esophageal dilatations for symptoms of dysphagia, 11 percutaneous gastrostomy tube placements resulting from failure to thrive, 30 maintenance procedures, 15 central venous access device insertions, and three other procedures (nasojejunal tube insertion, liver biopsy, and inferior vena cava filter insertion) were performed. Modifications were related to anesthetic management, access, tissue handling, and dressings. Complications were categorized according to Society of Interventional Radiology grades: Minor A (n = 6), Minor B (n = 4), Major C (n = 7), Major D (n = 2), Major E (n = 0), and Major F (n = 0).

CONCLUSION

Our experience suggests that interventional radiology procedures can be done successfully and safely when utmost care is given to skin and mucosal protection. Wound healing is adequate and should not deter intervention.