Management of Neurofibromatosis Type 1 of Mid and Lower Face

位於中下臉部的第一型多發性神經纖維瘤之治療報告

林雍球(Yung-Chiu Lin) ； 歐令奮(Ling-Feng Ou) ； 顏榮信(Jung-Hsing Yen) ； 陳伊呈(I-Chen Chen) ； 唐友文(Yu-Wen Tang)

《中華民國整形外科醫學會雜誌》 10卷4期 (2001/12) Pp. 254-263

https://doi.org/10.7096/JPSA.200112.0254

摘要

第一型多發性神經纖維瘤(Type I Neurofibromatosis)是一種常見的體顯性遺傳疾病，發生率約每三千個新生兒中有一位。在身體檢查時，大多可在身上找到六個以上的咖啡牛奶斑點(Café au lait spots)，及數個神經纖維瘤(Neurofibroma)。
這類病人常因腫瘤過度增生造成醜形，而尋求治療。回顧已往文獻，有關切除面部病灶之報告不多；本篇主旨為就兩位病患接受顏面部腫瘤切除及重建手術治療的經驗檢討。
手術目標首先在保留原有剩餘的神經功能，避免因手術而造成功能喪失；其次對於病灶之切除範圍方面，以回復正常外觀為主，不要求做完全切除手術，因為這類腫瘤的界限往往不明顯。
手術技巧方面：
(1)施予低血壓全身麻醉、以及縫合結紮，以降低手術中出血。
(2)採多次分區切除治療，以縮短手術時間、減少出血量。
(3)以拉皮手術之方式，將腫瘤從皮瓣下切除、並將皮瓣拉撐，可使臉形較平整。
(4)保留神經肌肉組織以減少功能損失；並適時重建五官的變形。

並列摘要

Neurofibromatosis type I (von-Recklinghausen's disease, NF1) is a common autosomal dominant hereditary disease. Patients are characterized by multiple café-au lait spots and neurofibromas. Plexiform neurofibroma, especially those in the head and neck, frequently elicits major cosmetic and functional impairment. We present two cases of plexiform neurofibroma of face who underwent serial tumor ablation and reconstructive surgeries. Because the infiltrating nature of plexiform neurofibroma makes complete excision difficult, our surgical strategy was to preserve the remaining neuromuscular function and remove as much of the tumor as possible to restore the normal appearance.
When using surgical techniques, we recommend the following:
(1) Surgery is performed under hypotensive anesthesia and liberal suture ligation to decrease intraoperative bleeding.
(2) Performing serial excisions shortens the operation time and decrease blood loss. The plan of serial excisions is mainly based on ”aesthetic unit”concept.
(3) Applying technique of rhytidectomy smoothes the facial contours.
(4) Preserving the neuromuscular tissue improves the function; and restores normal anatomic position and profile of the involved mouth, nose and ear.

並列關鍵字

neurofibromatosis type 1 ； Von-Recklinghausen's disease ； plexiform neurofibroma