Table 1.
Demographics and clinical characteristics of patients with port use complications.
| Case | Disorder | Age (years) | Sex (M/F) | Time with TIVAD | Complication | Removal of TIVAD? (Y/N/reinserted) | Resolution |
|---|---|---|---|---|---|---|---|
| 1 | Alpha mannosidosis | 18 | M | 3 years | Skin thinning and discoloration; small bruise over TIVAD site | N | Change from lidocaine/prilocaine cream to ethyl chloride spray |
| 2 | MPS II | 19 | M | 2 years, 8 months | Skin atrophy; bruise around TIVAD site; small scab over port | Reinserted | Change from lidocaine/prilocaine cream to ethyl chloride spray |
| 3 | MPS VI | 20 | M | 8 months | Skin burn from overuse of tetracaine cream | Reinserted | Change from tetracaine to lidocaine/prilocaine cream |
| 4 | MPS I | N/A | N/A | 2 years | Infective endocarditis | Y | Valve replacement |
| 5 | MPS II | 28 | M | 8 years | Thrombus | Y | Warfarin treatment; after port removal, used peripheral infusions |
| 6 | MPS II | 29 | M | 3 years | Port failure; subsequent difficulties placing pacemaker | Y | Successful placement of pacemaker after third complex procedure |
| 7 | MPS I | 26 | F | 2 years; 6 months; 5 years; ongoing | Multiple port failures (fourth port in use); collateral vessel interference with angiography | N | Continue with fourth TIVAD; however, distorted vasculature noted |
| 8 | MPS VI | 27 at time of death | F | 1 year; 6 years | Fatal, generalized infection; port was considered infection entry route | – | – |
MPS, mucopolysaccharidosis; N/A, not available; TIVAD, totally implantable vascular access device.