体表神经纤维瘤的手术治疗探讨

目的：探讨体表神经纤维瘤的切除及创面修复的方法。方法：24例患者均行手术,其中切除后直接缝合13例,皮瓣转移6例,皮片移植4例,1例行肿瘤切除术同时行截肢术。术中采用肿胀麻醉技术,控制性降压技术等治疗方法。结果：20例患者Ⅰ期愈合,4例部分伤口裂开,经换药而愈。有两例巨大神经纤维瘤术中予以输血,其余病人未予输血。结论：对体表神经纤维瘤行切除手术,术中采用合适技术,可显著减少术中出血,降低手术风险,创面修复应根据瘤体大小及部位选择不同的修复方法。     

巨大神经纤维瘤的整形治疗

神经纤维瘤多发生于体表，虽然属良性肿瘤，但是如不及时手术治疗，可生长巨大，使患者外形丑陋，功能受到严重影响。1998年及2004年我们分别收治2例巨大神经纤维瘤，经整形手术治疗，效果满意，报告如下。     

巨大神经纤维瘤的围手术期护理

神经纤维瘤病（neurofibromatosis,NF）是由于神经嵴细胞分化异常而导致的多系统损害的常染色体显性遗传病,常累及神经、肌肉、骨骼、内脏、皮肤等系统[1-2]。临床上主要以皮肤牛奶咖啡色斑、皮下多发性神经纤维瘤为主要特征,肿物较大时因重力作用而下垂可呈＂囊袋＂状。皮肤外表现可有橡皮病、骨骼畸形、中枢神经系统的肿瘤及相应压迫症状[3]。目前尚无有效方法能够预防或逆转NF的特征性病变,主要是对症治疗[4]。     

巨大神经纤维瘤的手术切除及瘤体皮肤回植

目的:总结巨大神经纤维瘤的手术切除与切取瘤体皮肤修复创面的经验。方法:对1999年8月～2004年4月收治的7例巨大神经纤维瘤进行回顾性分析。臀部2例,背部2例,腰部1例,上肢1例,下肢1例。面积在36cm×25cm～55cm×38cm,瘤体切除后,反取瘤体皮肤使之成为中厚层皮片,回植创面。结果:术中出血少,回植皮片成活良好,回访6个月～4年以上,皮片生长稳定,外观满意,未见瘤体复发。结论:巨大神经纤维瘤可完整或大部分手术切除,切取的瘤体皮肤回植创面,可取得良好的外观。     

颈椎管内神经鞘瘤和神经纤维瘤的手术治疗

目的：探讨颈段椎管内神经鞘瘤和神经纤维瘤的手术治疗效果。方法：回顾性分析经手术治疗的63例颈段椎管内神经鞘瘤和神经纤维瘤患者的临床资料及治疗效果。结果：神经鞘瘤52例，神经纤维瘤11例；全切除57例，大部分切除6例；痊愈54例，好转7例，恶化1例，死亡1例。结论：术中避免损伤脊髓、保护好椎动脉及处理好椎旁静脉丛是提高全切率、改善预后的关键。     

皮肤巨大神经纤维瘤的治疗及围手术期护理

神经纤维瘤（neurofibroma,NF）是一种常见的良性肿瘤,常累及神经、肌肉、骨骼、器官和皮肤,生长速度较慢,但在青春期和妊娠期可出现阶段性生长加速[1]。皮肤神经纤维瘤可为单发或多发,主要分布于躯干、四肢和面部,在儿童期即可出现,青春期后进展明显[2]。皮肤神经纤维瘤如果生长迅速或过大,妨碍患者活动、影响美观,伴有疼痛或有明显压迫症状时,应行手术治疗。我科于2000～2010年先后收治6例皮肤巨大神经纤维瘤病患者,手术难度大,经过精心治疗和护理后痊愈出院,现将护理体会报道如下。     

头面部巨大神经纤维瘤的整形治疗

神经纤维瘤多发于头面颈部[1 ] ,虽属良性 ,如果瘤体较大 ,致使面部五官畸形及功能障碍 ,常需手术治疗改善。1994年及 1998年 ,我们收治头面部巨大神经纤维瘤 2例 ,经整形手术治疗 ,效果满意。1　临床资料例 1　男 ,13岁。右头面部肿瘤逐渐增大 12年 ,于 1994年 2月入院。体检 :全身散在大小不等的色素斑和皮肤结节 ,右侧头面部包括右颞、额、上下睑、耳、鼻及唇颌部因不规则肿物隆起 ,范围约 40ｃｍ× 2 0ｃｍ× 15ｃｍ ,局部皮肤色泽灰黑 ,粗糙、质地柔软 ,边界不清 ,无明显条索感 ,无压痛 ,肿物呈袋状下垂 ,牵拉五官肥厚变形 ,功能障碍。…     

Neurofibroma of the porta hepatis

Neurofibromas are generally well-circumscribed tumors of the nervous system, often found in association with neurofibromatosis 1 (NF1). They uncommonly present as a single sporadic form in the retroperitoneum and in the pancreas. We present a case of a 40-year-old man who presented with a 4- to 5-year history of right upper quadrant pain with associated nausea and vomiting. Medical imaging showed a 3.8 by 3.4-cm mass encasing the hepatic artery and the porta hepatis. Surgical resection was performed, with removal of the gallbladder. The mass was histologically diagnosed as a neurofibroma. At 1 year following his surgery, the patient is doing well.     

Neurofibroma of the bile duct: a rare cause of obstructive jaundice

Neurofibromas of the common bile duct are extremely rare. The lack of specific clinical or radiological features makes preoperative diagnosis in the absence of histology difficult. We report the case of a female patient who presented with obstructive jaundice and evidence of a common bile duct stricture on imaging. She underwent an exploratory laparotomy, and intraoperative frozen section confirmed clear margins and a benign lesion. Excision of the extrahepatic bile duct and A Roux-en-Y hepaticojejunostomy was performed. We discuss the clinical features and management of neurofibromas of the bile duct in light of the literature.     

Neurofibroma of the leg presenting as chronic lymphedema

Summary A case of long standing Elephantiasis neuromatosa simulating chronic lymphedema is presented. The look of the patient for cosmetic repair and the macroscopical appearance during the operation led to the right diagnosis.     

Neurofibroma of the common bile duct: a rare cause of obstructive jaundice

Neurofibroma of the common bile duct is extremely rare. Fewer than 20 cases have been reported in the literature. We report a case of neurofibroma in a 47-year-old female presented with obstructive jaundice. Preoperative imaging failed to diagnose the case. The patient was treated with en-bloc excision of the extrahepatic biliary tree and Roux-en-Y hepaticojejunostomy.     

Mediastinal Neurofibroma Originating from the Left Intrathoracic Phrenic Nerve: Report of a Case

We report a case of mediastinal neurofibroma originating from the left phrenic nerve in a 42-year-old woman who was referred to us after a routine chest X-ray showed a smooth, round abnormal shadow in the left middle lung field adjacent to the heart. We resected a 25 × 20 × 20-mm tumor by video-assisted thoracic surgery. Histopathological examination confirmed that the lesion was a mediastinal neurofibroma originating from the left phrenic nerve without von Recklinghausens disease. Neurogenic mediastinal tumors originating from the phrenic nerve are very rare, and to the best of our knowledge, no other case of a mediastinal neurofibroma originating from the phrenic nerve in a patient without von Recklinghausens disease has ever been reported.     

Retroperitoneal Transdiaphragmatic Robotic-Assisted Laparoscopic Resection of a Left Thoracolumbar Neurofibroma

Objective:

Robotic technology has been used in a variety of surgical procedures for its 3D magnification and precision. Minimally invasive techniques have already become common in neurosurgery; however, robotic-assisted procedures in neurosurgery are still a relatively new frontier. This report describes the first use of robotic technology to resect a left thoracolumbar neurofibroma.

Case Report:

A 19-year-old male with a family history of neurofibromatosis was diagnosed with a suspected 3-cm x 4-cm neurofibroma in the T12-L1 left paraspinal area. His only complaint was back pain requiring narcotic analgesics. He had no other findings on physical examination or laboratory/radiologic workup.

Methods:

After consulting urologic robotic surgeons, it was agreed to use the da Vinci robot (Intuitive Surgical, Sunnyvale, CA) for the resection of this mass. Following retroperitoneal laparoscopic access, the urologic surgeons opened the diaphragm and began the initial mobilization of the mass laparoscopically. The robot was docked, and the neurosurgeon operated the robot at the console to resect the mass from its nerve origin. There were no complications, and the mass, a confirmed neurofibroma, was completely removed. The patient was discharged on postoperative day 2; his back pain resolved, requiring no analgesia by the end of the first postoperative week.

Conclusion:

This case provides early evidence that robotic assistance can be successfully used for the resection of a paraspinal neurofibroma.     

Video-Assisted Thoracoscopic Surgery for Localized Neurofibroma of the Esophagus: Case Report and Review of the Literature

Esophageal submucosal tumors are less common than other gastrointestinal tract tumors. Leiomyoma is the most common benign esophageal SMT, accounting for more than 70% of these tumors. We report on a case of a 56-year-old woman with a 3-cm diameter midthoracic esophageal submucosal tumor. Magnetic resonance imaging suggested leiomyoma or neurofibroma. Video-assisted thoracoscopic surgery was performed to enucleate the tumor from the esophageal wall by splitting the muscle layers. The postoperative course was uneventful, and the patient was discharged on postoperative day 8. Immunohistochemical staining confirmed the diagnosis of esophageal neurofibroma. Gastrointestinal tract involvement of neurofibromatous lesions is rare and occurs most frequently as a systemic manifestation of von Recklinghausen disease. Cases of localized esophageal neurofibroma with prior or subsequent evidence of generalized neurofibromatosis have rarely been documented. This is a rare case of isolated esophageal neurofibroma without classic systemic manifestations of generalized neurofibromatosis, and it is the first reported case treated by video-assisted thoracoscopic surgery.