PLASTIC SURGERY OF SCALP AND FACIAL PLEXIFORM NEUROFIBROMAS_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

ZHAOMuxin ¹ , QIANGJinghong ² ,  LIYangqun ¹ , TANGYong ¹ , CHENWen ¹ , YANGZhe ¹ , MANing ¹ , WANGWeixin ¹ , XULisi ¹ , FENGJun ¹

1. The 2nd Department of Plastic Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Sciences, Beijing, 100144, P. R. China;
2. Department of Medical Photography, Plastic Surgery Hospital, Chinese Academy of Medical Sciences, Beijing, 100144, P. R. China;

Corresponding author：

LIYangqun, Email: liyangqun_doctor@126.com

Keywords：

Plexiform neurofibroma; Neurofibromatosis type Ⅰ; Orbitotemporal region; Endovascular embolization

DOI：

10.7507/1002-1892.20150300

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Objective To explore the plastic surgical treatment and the way to reduce hemorrhage for scalp and facial plexiform neurofibromas. Methods Between July 2004 and July 2013, 20 patients with scalp and facial plexiform neurofibromas (17 cases of neurofibromatosis type Ⅰ and 3 cases of plexiform neurofibroma) were treated, and the clinical data were retrospectively analyzed. There were 9 males and 11 females with an average age of 37 years (range, 18-56 years). The disease duration ranged from 8 to 56 years (mean, 19 years). The scalp was involved in 6 cases, the face in 7 cases, and both the scalp and face in 7 cases. The extent of involvement ranged from 4 cm×3 cm to 15 cm×13 cm. Obvious pigmentation was seen in 2 cases. Endovascular embolization was performed before surgical intervention in 4 cases. Preliminary sutures around the lesion were carried out in 18 cases having an involved range over 5 cm×5 cm. One-stage excision was performed in 17 cases, and two-stage excision in 3 cases. Wound repair and facial orthopedic treatment were performed after tumor excision. Results The intraoperative blood loss was 100-500 mL (mean, 300 mL) for patients undergoing single operation. For 3 patients undergoing two-stage excision, the blood loss of the first operation was 500, 600, and 800 mL respectively, and the blood loss of the second operation was all 50 mL. Autologous blood transfusion of 200, 400, and 400 mL was performed in 3 cases respectively. The preliminary sutures were removed at 3-7 days (mean, 5 days) after operation. All the incisions healed primarily without secondary hemorrhage and hematoma, and the flap and skin graft survived totally. Fifteen patients were followed up 1 year to 7 years (mean, 2.5 years). All patients showed significant improvement in appearance. No significant progression, expanding, and sagging were observed. Conclusion Endovascular embolization and preliminary sutures around the lesion can be used to reduce hemorrhage in resection of plexiform neurofibroma in the scalp and face. Personalized surgical plan of benign neurofibromatosis should be made to reduce the tumor mass, to improve function and appearance.

Citation： ZHAOMuxin, QIANGJinghong, LIYangqun, TANGYong, CHENWen, YANGZhe, MANing, WANGWeixin, XULisi, FENGJun. PLASTIC SURGERY OF SCALP AND FACIAL PLEXIFORM NEUROFIBROMAS. Chinese Journal of Reparative and Reconstructive Surgery, 2015, 29(11): 1401-1404. doi: 10.7507/1002-1892.20150300 Copy

1.	Lee V, Ragge NK, Collin JR. Orbitotemporal neurofibromatosis. Clinical features and surgical management. Ophthalmology, 2004, 11(2):382-388.
2.	郝永红, 宋慧峰, 许明火, 等. 巨大神经纤维瘤病诊断及整形外科治疗. 中国美容医学, 2013, 22(16):1724-1727.
3.	Korf BR. Plexiform neurofibromas. Am J Med Genet, 1999, 89(1):31-37.
4.	王炜, 林晓曦, 祁佐良, 等. 面部神经纤维瘤的整形和显微外科治疗. 上海医学, 2000, 23(7):387-389.
5.	Hivelin M, Wolkenstein P, Lepage C, et al. Facial aesthetic unit remodeling procedure for neurofibromatosis type 1 hemifacial hypertrophy:report on 33 consecutive adult patients. Plast Reconstr Surg, 2010, 125(4):1197-1207.
6.	Marchac D, Britto JA. Remodelling the upper eyelid in the management of orbitopalpebral neurofibromatosis. Br J Plast Surg, 2005, 58(7):944-956.
7.	王炜. 整形外科学. 杭州:浙江科学技术出版社, 1999:460-461.
8.	Van der Meulen JC, Moscona AR, Vandrachen M, et al. The management of orbitofacial neurofibromatosis. Ann Plast Surg, 1982, 8(3):213-220.
9.	Erb MH, Uzcatequi N, See RF, et al. Orbitotemporal neurofibromatosis:classification and treatment. Orbit, 2007, 26(4):223-228.
10.	Singhal D, Chen YC, Chen YR, et al. Soft tissue management of orbitotemporal neurofibromatosis. J Craniofac Surg, 2013, 24(1):269-272.
11.	Li J, Lin M, Shao C, et al. Blepharoplasty techniques in the management of orbito-temporal neurofibromatosis. J Plast Reconstr Aesthet Surg, 2014, 67(11):1496-1501.
12.	Bechtold D, Hove HD, Prause JU, et al. Plexiform neurofibroma of the eye region occurring in patients without neurofibromatosis type 1. Ophthal Plast Reconstr Surg, 2012, 28(6):413-415.
13.	Coban-Karatas M, Altan-Yaycioglu R, Bal N, et al. Management of facial disfigurement in orbitotemporal neurofibromatosis. Ophthal Plast Reconstr Surg, 2010, 26(2):124-126.
14.	White N, Gwanmesia I, Akhtar N, et al. Severe haemorrhage in neurofibromatoma:a lesson. Br J Plast Surg, 2004, 57(5):456-457.
15.	Tomei KL, Gupta V, Prestigiacomo CJ, et al. Spontaneous hemorrhage of a facial neurofibroma:endovascular embolization before surgical intervention. J Craniofac Surg, 2013, 24(5):e514-517.
16.	Pelage JP, Le Dref O, Mateo J, et al. Life-threatening primary postpartum hemorrhage:treatment with emergency selective arterial embolization. Radiology, 1998, 208(2):359-362.
17.	Lin YC, Chen HC. Rare complication of massive hemorrhage in neurofibromatosis with arteriovenous malformation. Ann Plast Surg, 2000, 44(2):221-224.
18.	Jones RG, Kiatisevi P, Morris DC, et al. Intravascular embolisation and surgical resection of a giant neurofibroma with intratumoural hemorrhage. Br J Radiol, 2010, 83(995):e225-229.
19.	Konno E, Kishi K. Use of the LigaSureTM vessel sealing system in neurofibroma excision to control postoperative bleeding. J Plast Reconstr Aesthet Surg, 2012, 65(6):814-817.

1. Lee V, Ragge NK, Collin JR. Orbitotemporal neurofibromatosis. Clinical features and surgical management. Ophthalmology, 2004, 11(2):382-388.
2. 郝永红, 宋慧峰, 许明火, 等. 巨大神经纤维瘤病诊断及整形外科治疗. 中国美容医学, 2013, 22(16):1724-1727.
3. Korf BR. Plexiform neurofibromas. Am J Med Genet, 1999, 89(1):31-37.
4. 王炜, 林晓曦, 祁佐良, 等. 面部神经纤维瘤的整形和显微外科治疗. 上海医学, 2000, 23(7):387-389.
5. Hivelin M, Wolkenstein P, Lepage C, et al. Facial aesthetic unit remodeling procedure for neurofibromatosis type 1 hemifacial hypertrophy:report on 33 consecutive adult patients. Plast Reconstr Surg, 2010, 125(4):1197-1207.
6. Marchac D, Britto JA. Remodelling the upper eyelid in the management of orbitopalpebral neurofibromatosis. Br J Plast Surg, 2005, 58(7):944-956.
7. 王炜. 整形外科学. 杭州:浙江科学技术出版社, 1999:460-461.
8. Van der Meulen JC, Moscona AR, Vandrachen M, et al. The management of orbitofacial neurofibromatosis. Ann Plast Surg, 1982, 8(3):213-220.
9. Erb MH, Uzcatequi N, See RF, et al. Orbitotemporal neurofibromatosis:classification and treatment. Orbit, 2007, 26(4):223-228.
10. Singhal D, Chen YC, Chen YR, et al. Soft tissue management of orbitotemporal neurofibromatosis. J Craniofac Surg, 2013, 24(1):269-272.
11. Li J, Lin M, Shao C, et al. Blepharoplasty techniques in the management of orbito-temporal neurofibromatosis. J Plast Reconstr Aesthet Surg, 2014, 67(11):1496-1501.
12. Bechtold D, Hove HD, Prause JU, et al. Plexiform neurofibroma of the eye region occurring in patients without neurofibromatosis type 1. Ophthal Plast Reconstr Surg, 2012, 28(6):413-415.
13. Coban-Karatas M, Altan-Yaycioglu R, Bal N, et al. Management of facial disfigurement in orbitotemporal neurofibromatosis. Ophthal Plast Reconstr Surg, 2010, 26(2):124-126.
14. White N, Gwanmesia I, Akhtar N, et al. Severe haemorrhage in neurofibromatoma:a lesson. Br J Plast Surg, 2004, 57(5):456-457.
15. Tomei KL, Gupta V, Prestigiacomo CJ, et al. Spontaneous hemorrhage of a facial neurofibroma:endovascular embolization before surgical intervention. J Craniofac Surg, 2013, 24(5):e514-517.
16. Pelage JP, Le Dref O, Mateo J, et al. Life-threatening primary postpartum hemorrhage:treatment with emergency selective arterial embolization. Radiology, 1998, 208(2):359-362.
17. Lin YC, Chen HC. Rare complication of massive hemorrhage in neurofibromatosis with arteriovenous malformation. Ann Plast Surg, 2000, 44(2):221-224.
18. Jones RG, Kiatisevi P, Morris DC, et al. Intravascular embolisation and surgical resection of a giant neurofibroma with intratumoural hemorrhage. Br J Radiol, 2010, 83(995):e225-229.
19. Konno E, Kishi K. Use of the LigaSureTM vessel sealing system in neurofibroma excision to control postoperative bleeding. J Plast Reconstr Aesthet Surg, 2012, 65(6):814-817.

Chinese Journal of Reparative and Reconstructive Surgery

PLASTIC SURGERY OF SCALP AND FACIAL PLEXIFORM NEUROFIBROMAS

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