Clinical experience of surgically treating giant neurofibromatosis-1

The surgical treatment for giant neurofibromatosis-1 (NF-1) requires comprehensive measures. Presently, there is no systematic description of surgical treatment. Because of its high level of risk, we want to share our clinical experience. From 2011 to 2014, patients (n = 8, 5 female and 3 male patients, aging from 31 to 45 years-old) were included in the study. The tumours were located on the trunk (n = 5) or face (n = 3). In addition to routine examination, blood storage was also prepared. Preoperative consultation from related departments was critical at first. Related artery embolisation was also carried out. In the operation, we checked thromboelastography, based on which reasonable blood component transfusion was implemented. Autologous blood transfusion was also ready. An instrument of copper needle or ring ligation was used to reduce haemorrhage before the surgery. Protruding or drooping portions of the tumours were excised. A pressurised bandage was applied when the surgery was completed. After the surgery, besides the routine monitoring of vital signs, re-haemorrhage should be detected in time. Then, we should decide whether blood transfusion or surgery was required again. Expanders were implanted in one female patient with facial injuries before removing the tumour. Then, expanded flaps were applied to repair the secondary wound. According to the above clinical route, after an average of 1-year follow-up, no patients died, and other unforeseen events did not occur. Wounds healed well in all patients. The tumor was excised as much as possible. No facial nerve paralysis occurred in the facial sites. Expanded flaps necrosis WAS not encountered. It is essential to design the educational clinical route for treating NF-1 when a giant protruding tumour is advised to be excised, which can minimise the risk of surgery and assure us of the maximum range of resection.