Port Use in Duchenne Muscular Dystrophy


Clinical Management

Poster Number: 20


Kristy Riddle, RN, BSN, CCRC, The University of Texas Southwestern Medical Center, Kaitlin Batley, MD, UTSW/Children's Health Dallas, Susan Iannaccone, MD, UTSW

Introduction: With FDA approval of exon-skipping medications, use of central venous access ports in the Duchenne Muscular Dystrophy (DMD) population is increasing. Currently approved medications are administered intravenously once per week. Patients with DMD often have veinous access issues due to frequent administrations, and comorbid autism and developmental delay may complicate this. Therefore, port placement is common. Unlike many patients who utilize ports for shorter course of treatment, DMD patients may require lifelong use of ports. To better understand long-term use of ports in this population, we analyzed the frequency and types of port complications in our patients.

Objective: To determine the frequency and nature of complications of central venous access ports in the DMD population and then create a standard of care plan for management of ports in DMD patients.

Methods: This was a retrospective chart review of DMD patients receiving exon-skipping therapy through the Neuromuscular Clinic at Children’s Health. We received expedited review by the UTSW IRB.

Results: From 2017 to 2022, 25 boys with DMD had ports placed, with ages ranging from 3-19 years old. Seven underwent revisions. Complications included 2 infections, one pneumothorax, one hematoma, and numerous TPA injections and sheathing procedures for clotted ports. One patient experienced migration of the tip of the catheter out of the Superior Vena Cava. Over half of the patients have experienced a port complication, with timing ranging from one day to several years after placement. Most complications occurred within 6 months of placement.

Discussion: Patients with DMD are at increased risk for infection due to immunosuppression from chronic steroid use. They may also experience catheter displacement related to growth, as ports remain in place for several years and are often placed early in childhood. In addition, complications such as fibrin sheaths are common due to their prolonged use.