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.     

Traumatic injuries to the porta hepatis: case report and review of the literature

Injuries to the porta hepatis present a rare but life-threatening condition. This report documents a patient surviving injuries to all three structures in the porta hepatis. A literature study sets out the guidelines for the optimal treatment of these lesions.     

Nonresectable inflammatory pseudotumor of the porta hepatis

Inflammatory pseudotumors are rare mass lesions often mistaken for malignancy. Currently, only 2 cases of pseudotumors originating from the porta hepatis have been reported in the pediatric literature, and both cases were those of patients older than 30 years. When located in the porta hepatis, pseudotumors are difficult to treat via surgical resection, and other therapeutic options must be considered. We report a pediatric case of inflammatory pseudotumor of the porta hepatis and discuss the different surgical and nonsurgical options used in treating pseudotumors.     

The surgical anatomy of the porta hepatis

The anatomy of the porta hepatis, with particular emphasis on the hilar relationships of the bile ducts to the portal vein, has been investigated in 30 fresh cadaver specimens. Meticulous dissertion delineated three major types of anatomic variations. Type A, the most common, revealed a left hepatic duct which, when it branched, sent its largest and major tributary beneath the portal vein to the lateral segment of the left lobe. Type B was characterized by the major division of the left hepatic duct running parallel to the portal vein into the hepatic sulcus. In Type C the divisions were of equal caliber. These observations should assist the surgeon in dissections of the hepatic ducts above their confluence.     

Histologic oddities at the porta hepatis in biliary atresia

Purpose

Highly unusual histologic findings at the porta hepatis in 3 infants who underwent Kasai portoenterostomy for biliary atresia are reported.

Methods

Portoenterostomy was performed using a standard operative technique. Serial transverse sections of the excised portal plate were examined by light microscopy along with sections from the distal extrahepatic biliary remnants, gallbladder, and liver biopsy.

Results

Of 61 consecutive infants who underwent Kasai portoenterostomy for biliary atresia, 3 were found to have highly unusual histologic features at the porta hepatis. All had type 3 biliary atresia. Two had hilar biliary ductules lined in part by squamous epithelium, and the third had a focus of mature hyaline cartilage surrounded by perichondrium adjacent to biliary ductules. In each case, these unusual histologic features were localized to the porta hepatis in the region of the transected portal plate.

Conclusions

The presence of hyaline cartilage at the portal plate is likely to be an expression of defective morphogenesis, thus supporting the concept of disordered embryogenesis in the etiology of biliary atresia. Squamous epithelium within biliary ductules might also reflect a similar mechanism but could alternatively be an unusual metaplastic response to inflammation at this site.     

解剖第三肝门切除巨大肝脏海绵状血管瘤

目的 为切除紧贴腔静脉的肝脏巨大海绵状血管瘤寻找一种新的安全的手术途径。方法 在患侧肝动脉结扎后,于第三肝门部位逐一分离、结扎、切断肝短静脉,使瘤体与腔静脉分离,在肝门阻断下切除瘤体。结果 全组２７例,行肝右叶及尾叶切除１３例,右半肝切除２例,右肝上段切除３例,右后叶切除２例,左三叶及尾状叶切除５例,尾状叶切除２例。绵未行全肝血流阻断。术中输血平均１１５０ｍｌ,术后随访３￣４８个月,全部情况良好。     

肝门部胆管良性狭窄再次手术的管理及并发症防治

肝门部胆管良性狭窄是胆道外科复杂而棘手的难题 ,要成功地救治肝门胆管良性狭窄再手术病人 ,完善的围手术期管理及术后并发症防治是重要的环节。现结合我院自 1 995年 1月至 2 0 0 1年 7月收治的肝门胆管良性狭窄再手术病人 2 8例的临床实践进行讨论。一、术前管理肝门胆管良性狭窄 (以下简称肝门狭窄 )因原发疾病及多次胆道手术原因、术前病人常合并梗阻性黄疸及胆管炎、胆汁性肝硬化、肾功不全等多种并发症。我院收治的 2 8例病人中有 2 0例 (71 40 % )合并梗阻性黄疸 ,胆管炎 5例 (1 7 80 % ) ,营养不良 5例(1 7 80 % ) ,贫血 1 0例 (3…     

Portoenterostomy scissors: a new instrument for surgery in the porta hepatis.

A new design of scissors is described which has been found to be extremely useful during dissection of the porta hepatis. In particular its use is recommended for the portoenterostomy procedure of infancy (Kasai's operation).     

胰十二指肠切除术中肝门淋巴结清扫应注意的问题

迄今为止,胰腺癌病人预后仍不令人满意,其根治性切除术后5年生存率不足5%～10%。胰腺癌首要转移途径是经淋巴转移,对根治性手术标本淋巴结检查发现胰头癌淋巴结转移阳性率达56.0%～78.6%,即使肿瘤直径<2cm的小胰癌,30%～50%的病人也已发生淋巴结转移,表明大部分胰腺癌病人就诊时已属中、晚期。研究显示,根治性手术后病理检查淋巴结阴性的胰腺癌病人5年生存率为14%～57%[1～3],而淋巴结阳性的病人仅为0%～7.7%。因此,淋巴转移是影响胰腺癌病人预后的重要因素之一,极差的预后使得各国学者越来越重视此类病人的淋巴转移问题,多数学者认为广泛淋…     

不能切除的肝门区恶性肿瘤引起胆道阻塞的治疗

目的 探讨针对不同病冈导致的不能切除的肝门区恶性肿瘤引起的胆道阻塞采用各种常用治疗方法问的疗效差异.方法 回顾性分析2002年1月～2007年12月问不能切除的肝门区恶性肿瘤引起胆道阻塞的56例采用不同方式进行减黄治疗的临床资料.以术后3 d、7 d血清总胆红素、直接胆红素及碱性磷酸酶为评价指标,对减黄效果进行评价.结果 减黄效果以手术组最佳,其后依次为双侧金属支架置入术、双侧塑料支架置入术和双侧胆道内、外引流术.双侧引流明显优于单侧引流.结论 对不能切除的肝门区恶性肿瘤引起胆道阻塞的病人,如病情许可应尽量争取手术探查,减黄措施首选胆道内引流术.如行介入或内镜治疗,最好行双侧引流.     

悬吊法肝切除在第二肝门旁肝肿瘤切除术中的应用

目的:探讨肝脏悬吊法进行第二肝门旁肝肿瘤肝切除的可行性与安全性.方法:回顾性分析201 1年8月-2012年8月收治的7例第二肝门旁肝肿瘤病患者的临床资料.结果:7例术中顺利放置悬吊胶管并成功手术.行右半肝切除2例,右半肝+右肾周脂肪囊切除1例,右半肝切除+左肝内外叶2个血管瘤分别切除1例,右半肝+Ⅳ段部分切除术1例,左半肝+右肝Ⅷ段+右膈肌浆膜切除1例,右肝Ⅵ,Ⅶ段规则切除术1例.术中无断肝时误伤下腔静脉者,行右膈肌浆膜修补1例.中位手术时间375 min(295～460 min),中位出血量2 000 mL (750～8 000 mL),中位输血量1 000 mL(0～4 000 mL).术后胸腔积液2例,均经穿刺抽液恢复.无胆瘘,无腹腔感染.均痊愈出院,平均术后住院时间20 d.术后随访1～12个月,1例肝细胞癌有局部复发.结论:应用悬吊法进行第二肝门旁肝肿瘤肝切除是安全可行的.     

New clues for the developing human biliary system at the porta hepatis

The human biliary system is formed from the hepatic diverticulum, a structure which develops from the embryonic foregut in the fourth week of gestation. The cephalic portion of the hepatic diverticulum lies within the septum transversum, and gives rise to entodermal cells which become the primitive hepatocytes. The caudal part of the hepatic diverticulum is molded by mesenchyme to form the gallbladder, cystic duct, and extrahepatic bile duct. The gallbladder is initially tubular in shape, and undergoes morphological changes to become saccular during the 11th week of gestation. The extrahepatic bile duct elongates and widens as gestation progresses, and intramural mucus glands develop. There is no solid stage during the development of the extrahepatic bile duct. The extrahepatic bile duct is a well-defined tubular structure by the 6th week of gestation, whereas the intrahepatic biliary system during this period of gestation is represented by the primitive ductal plate. The ductal plate undergoes structural changes from the 11th week of gestation, beginning at the porta hepatis and progressing through gestation to the periphery of the liver. This remodeling process shapes the ductal plate from a flat sheath of biliary epithelium surrounding the portal vein branches into a network of interconnecting tubular structures. Mesenchyme plays an important role in ductal plate remodeling. The intrahepatic biliary system is in luminal continuity with the extrahepatic bile duct throughout gestation at the porta hepatis. The major bile ducts at the porta hepatis are fully formed by the 16th week of gestation. Received: September 30, 2000 / Accepted: January 10, 2001     

Mature cystic teratoma arising at the porta hepatis: a diagnostic dilemma

Mature cystic teratoma (MCT) is a tumor of embryological origin, which contains tissue derived from any or all germ cell lines found in an abnormal location. We present 2 cases of MCT both arising from the porta hepatis that were incorrectly thought perioperatively to be a choledochal cyst, which subsequently were demonstrated on histology to be a MCT.     

第二肝门附近复杂性肝胆管结石的外科处理

目的:探讨经第二肝门区域肝实质切开取石处理第二肝门附近复杂性肝胆管结石的临床疗效。方法:回顾性分析2008年1月—2014年1月收治的28例第二肝门附近复杂性肝胆管结石患者的临床与手术资料。结果:患者结石主要集中于II、IV、VIII段;手术方法以第二肝门区域肝实质切开取石术为主,结合胆总管切开取石术或肝部分切除术;住院期间患者死亡1例,术后并发症发生率14.8%。25例患者随访1~4年,术后结石清除率81.5%,术后再次经胆道镜取石后,最终结石清除率92.6%;结石复发率为11.1%。结论:经第二肝门区域肝实质切开取石术治疗第二肝门附近复杂性肝胆管结石,尤其是无法耐受多肝段切除的患者,具有一定的疗效。     

邻近第二、三肝门之肝海绵状血管瘤的外科治疗体会

目的探讨特殊部位肝海绵状血管瘤的外科治疗经验。方法对32例位于第二肝门附近和尾状叶内的肝海绵状血管瘤的临床资料进行回顾分析。结果本组病例均采用包膜外剥离术,血管瘤均获完全切除,手术死亡率3.1%(1/32),术中出血量50～10000ml,12例病人术中进行了输血,输血量400～4000ml不等。术后发生胆漏(1例)、膈下积液(1例)和右侧胸腔积液(3例)等并发症5例(发生率15.6%),26例获平均(3.09±0.93)年随访,未见血管瘤复发。结论熟悉肝脏解剖及熟练的手术技巧是手术成功的关键,包膜外剥离术是治疗肝海绵状血管瘤安全、有效的方法。     

CT透视引导下经皮乙酸消融治疗第二肝门部恶性肿瘤

目的　评价CT透视引导经皮穿刺乙酸注射治疗 (PAI)第二肝门区恶性肿瘤的安全性和效果。方法　搜集西南医院 2 0 0 0年 5月至 2 0 0 3年 10月在实时CT透视导向下经皮穿刺乙酸注射治疗第二肝门区恶性肿瘤 17例 ,肝细胞癌 10例、转移性腺癌 7例 ,共 2 0个病灶。病灶直径 2～ 5cm。根据病灶大小每次注入 5 0 %～ 6 0 %乙酸 2～ 6ml不等 ,乙酸内加入 1ml对比剂混匀 ,以了解乙酸在肿块内的弥散程度和范围。注射后 1周影像检查 ,根据情况重复注射 1～ 2次。结果　在实时CT透视下均能准确穿刺到靶点 ,除 12例轻中度疼痛外无其它并发症。全部病例均经B超或CT随访 ,3个月时 ,其中瘤灶缩小 14个 (70 % ) ,肿瘤大小无变化 4个 (2 0 % ) ,肿瘤增大 2个 (10 % )。 17例随访 3月 ,全部生存 ;12例随访 1年 ,9例生存 ,5例失访 ;6例随访 2年 ,3例生存 ,3例失访。结论　CTF引导下经皮乙酸消融治疗第二肝门部恶性肿瘤是安全有效的