Neurofibromatosis type 1 (NF1), a common genetic disease, is of particular interest to the plastic surgeons, as it affects skin with various clinical manifestations. Diffuse neurofibroma and massive plexiform neurofibroma, uncommon but distinctive forms, may produce gross disfigurement such as elephantiasis neurofibromatosis. Although the majority of patients require surgical procedure for cosmetic and functional reasons, the associated vascular malformations consist of excessive vascular fragility make most surgeons hesitant to address them. In fact, surgical resection of neurofibroma may cause life-threatening hemorrhage and require massive transfusion. The authors tried a safe surgical procedure, "tie up technique", for treatment of massive diffuse neurofibroma. The details of the technique is as follows: (1) grasp up tumor to be excised, (2) pierce through the base of tumor with a needle and suture, (3) tie up the base of tumor tightly 2 - 3 cm in width and repeat it all over the base. After that, blood supply to the tumor is completely cut off and tumor resection can be completed without bleeding. All of ligation sutures are removed after wound closure. This simplified and faster technique is strongly recommended to excise pendulous cutaneous diffuse neurofibroma and plexiform neurofibroma without major nerve involvement expected to occur massive hemorrhage.
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