Dominant dystrophic epidermolysis bullosa (DDEB) is a rare inherited skin disorder characterized by extreme fragility of the skin and mucosa that poses significant challenges for anesthetic management. Herein, we report a case of a 65-year-old woman with DDEB who underwent a 13-hour surgery for mandibular cancer under general anesthesia. To prevent skin and mucosal injuries, meticulous perioperative strategies were implemented, including the use of barrier films and pressure-relieving pads and avoiding adhesive devices. High-flow nasal cannula oxygenation enabled awake fiberoptic intubation without the need for mask ventilation, minimizing the risk of blister formation and airway trauma. Despite the prolonged duration, no pressure ulcers or new skin injuries developed, other than minor epidermal peeling of the lips. This case demonstrates that comprehensive preoperative planning and intraoperative protection can ensure safe anesthesia in patients with DDEB undergoing long surgical procedures.

Ten patients, aged 3 to 43 years, with the tetralogy of Fallot underwent in situ pulmonary valve replacement (PVR) 13 times. The implanted valves were a St. Jude Medical prosthesis (3 times) and a bioprosthetic valve (10 times). In 5 patients PVR was performed at the time of radical repair and in the remaining 5 patients PVR was performed after radical repair. Three patients underwent re-PVR at 6 to 13 years after the first PVR. There was one operative death in re-PVR 14 years after the first PVR and one patient died from congestive heart failure 4 years after PVR. In the patients with the tetralogy of Fallot, the rate of PVR in those who had undergone open Brock's operation were significantly higher than that of the patients without open Brock's operation (p<0.05). Actuarial survival rates at 5 years and 10 years were 88.9% and 88.9%, respectively. Rates of freedom from reoperation at 5 years and 10 years were 88.9% and 59.3%, respectively. Although the early operative results are satisfactory, re-PVR is mandatory in the future. Thus the indications of PVR should be considered carefully.