贲门失弛缓症的诊断与治疗进展

贲门失弛缓症(achalasia,AC)是一种食管运动障碍性疾病,发病机制尚未明确,可导致下食管括约肌放松和蠕动功能障碍性疾病。其发生率每年5/100 000人,患病率每年8/100 000人,表现以吞咽困难为主。诊断的金标准为高分辨食管测压(high resolution manometry,HRM),其他检查方法包括内窥镜、X线钡餐造影等,其中HRM能对AC患者进行分型并评价预后。治疗AC的方法主要有药物治疗、球囊扩张术、肉毒杆菌注射及食管支架治疗等,近年来经腹腔镜贲门肌切开术(laparoscopic heller myotomy,LHM)及经口内镜下肌切开术(peroral endoscopic myotomy,POEM)得到广泛推广。现本文对AC的诊断与治疗进展作一概述。     

贲门失弛缓症诊治的新进展

贲门失弛缓症是一种病因不明的原发性食管运动障碍性疾病,其难治性、易复发、长病程的特点严重影响患者的生活质量。近年来的诊断技术如定时食管吞钡检查、食管高分辨率测压引起了研究者的重视。临床治疗以降低食管下端括约肌压力、缓解梗阻症状为目的,治疗方法处于不断探索和更新中,此文主要就腔镜下改良Heller术及经口内镜下肌切开术进行综述。     

经口内镜下肌切开术治疗贲门失弛缓症的研究进展

贲门失弛缓症是食管神经-肌肉功能紊乱所致的疾病。治疗目标主要为降低食管下括约肌压力,解除食管梗阻。传统治疗方法包括药物治疗、内镜下肉毒素注射、内镜下球囊扩张术以及腹腔镜Heller肌切开术等,但均存在局限性。经口内镜下肌切开术(POEM)因微创、安全性高、疗效显著等特点成为研究热点。本文就POEM治疗贲门失弛缓症的研究进展作一综述。     

环形肌切开和全层肌切开治疗贲门失弛缓症长期随访研究

目的：对比内镜下全层肌切开与环形肌切开治疗贲门失弛缓症的长期临床疗效及远期并发症。方法：回顾性分析2012年6月至2014年12月于郑州大学第一附属医院消化内科行经口内镜下肌切开术治疗并定期随访的53例贲门失弛缓症患者资料,其中21例行环形肌切开,32例行全层肌切开,比较两种术式的长期临床疗效及远期并发症。 结果：环形肌切开组和全层肌切开组治疗有效率分别为90.5%（19/21）和100%（32/32）。两组术后Eckardt评分、食管下括约肌压力和4 s完整松弛压比较差异无统计学意义（P>0.05）。全层肌切开组临床相关胃食管反流发生率高于环形肌切开组（40.6％比14.3%,χ2=4.174,P=0.041）。 结论：经口内镜下环形肌切开术与全层肌切开术治疗贲门失弛缓症长期疗效相当,但全层肌切开术后临床相关胃食管反流发生率更高。     

贲门失弛缓症的研究进展

贲门失弛缓症是一种罕见的原发性食管动力障碍性疾病,以食管下段括约肌的松弛功能受损,食管蠕动波减少或消失,食管同步收缩为特征.由于本病的发病机制仍不明确,现有的治疗方式主要以缓解由食管下段括约肌引起的功能性梗阻为目的,同时保持降低食管下段括约肌压力与预防发生胃食管返流性疾病之间的平衡.本病的治疗方式包括药物治疗,介入治疗,手术治疗和中医整脊疗法.本文就贲门失弛缓症的最新治疗进展作一综述,重点讨论贲门失弛缓症现有的主要治疗方式包括气囊扩张术,Heller肌切开术及经口内镜下肌切开术(per-oral endoscopic myotomy,POEM)的进展.     

经口内镜下肌切开术治疗贲门失弛缓症的现状和进展

贲门失弛缓症是由食管-胃交界处神经肌肉功能障碍所致的一种食管动力障碍的功能性疾病,主要特征为食管缺乏蠕动、食管下括约肌松弛受限。目前治疗方法包括肉毒素治疗、支架植入、腹腔镜Heller肌切开术和内镜下球囊扩张术,但这些治疗手段存在短期内易复发、损伤较大等不足。随着内镜技术的不断发展,新型微创术式经口内镜下肌切开术(POEM)在临床上得到广泛应用,亦可用于一些特殊患者,如Ⅲ型贲门失弛缓症、高龄、小儿、乙状结肠型贲门失弛缓症等。本文就POEM治疗贲门失弛缓症的现状和进展作一综述。     

原发性食管恶性黑色素瘤的外科诊治

目的探讨原发性食管恶性黑色素瘤的临床病理特征、诊断、鉴别诊断和外科治疗。方法回顾我院6例外科手术切除和病理学确诊原发性食管恶性黑色素瘤的相关临床资料,并分析相关文献。结果6例患者中男5例,女1例,年龄48～66岁,平均55.8岁,临床症状主要为吞咽困难和胸骨后疼痛,均经钡餐检查提示食管肿瘤性病变,食管中段4例,下段2例,术前2例误诊为食管癌,4例胃镜刷咬切片检查提示食管恶性黑色素瘤,均行食管切除和胃代食管吻合术。术后病理诊断为原发性食管恶性黑色素瘤,组织学特征与其他部位恶性黑色素瘤相似,免疫组化表达HMB45、S-100、Vimentin,不表达CgA。结论原发性食管恶性黑色素瘤临床罕见,易误诊,诊断依据临床病理特征、影像学检查,内镜刷片检查有利术前诊断,治疗原则为以外科手术切除为主的综合治疗。     

经口内镜下食管憩室肌切开术治疗食管憩室的疗效分析

探讨经口内镜下食管憩室肌切开术治疗食管憩室的中远期疗效以及术后憩室复发的危险因素。方法　对2016年5月1日—2019年8月1日在东南大学附属中大医院接受经口内镜下食管憩室肌切开术治疗的31例食管憩室病例进行回顾性研究,主要观察术后Eckardt评分、治疗成功率和复发率,并采用多因素Logistic回归模型分析术后憩室复发的独立危险因素。结果　31例均顺利完成经口内镜下食管憩室肌切开术,术后随访（30.6±11.1）个月（20~63个月）。31例术前Eckardt评分（8.2±2.4）分,术后1个月（1.4±0.7）分,术后6个月（1.4±1.1）分,术后12个月（1.3±1.1）分,术后24个月（1.3±0.9）分,与术前比较,术后各随访时间点的Eckardt评分均有明显下降（P<0.001）。术后随访1、6、12、24个月的治疗成功率分别为96.8%（30/31）、90.3%（28/31）、90.3%（28/31）和90.3%（28/31）。有3例复发,总复发率为9.7%（3/31）。Logistic回归分析结果显示,病程（P=0.038,OR=1.041,95%CI：1.002~1.080）和术前Eckardt评分（P=0.024,OR=2.299,95%CI：1.117~4.728）是影响术后憩室复发的独立危险因素。结论　经口内镜下食管憩室肌切开术治疗食管憩室的中远期疗效理想,但病程长、术前Eckardt评分高者易复发。     

经口内镜全层肌切开术与环形肌切开术治疗贲门失弛缓症临床疗效分析

目的比较经口内镜全层肌切开术与环形肌切开术治疗贲门失弛缓症(achalasia,AC)的临床疗效。方法回顾性分析2012年8月至2014年12月东华医院收治的29例行经口内镜肌切开术AC患者的临床资料。其中16例为全层肌切开组(F-POEM),13例为环形肌切开组(C-POEM),分析两种治疗方式的临床疗效及并发症发生情况。结果 29例患者均成功实施POEM术,手术成功率为100%。FPOEM组与C-POEM组手术时间为(41.23±8.02)min和(50.75±9.07)min,差异有统计学意义(P0.05),F-POEM组较C-POEM组手术时间平均缩短约10 min。F-POEM组和C-POEM组术后1月、6月及12月食管压力较术前均明显下降,差异均有统计学意义(P0.05),两组食管压力在术后1月、6月和12月各时间点相互比较,差异无统计学意义(P0.05)。以术后6个月内Eckardt评分≥4分考虑为手术失败,两组治疗有效率分别为100%(13/13)和93.75%(15/16),差异无统计学意义(P0.05)。两组皮下气肿发生率分别为46.15%(6/13)和50.00%(8/16),两组气胸发生率为15.38%(2/13)和18.75%(3/16),差异均无统计学意义(P0.05),C-POEM组术后发生反流性食管炎(3例)少于F-POEM组(5例),差异无统计学意义(P0.05)。结论经口内镜全层肌切开术与环肌切开术治疗AC患者的短期疗效相当,并发症发生率相似,但全层肌切开术可缩短手术时问,不增加术后反流的发生。     

贲门失弛缓症的治疗

贲门失弛缓症是一种病因尚未明确、累及食管平滑肌和下食管括约肌（LES）的动力障碍性疾病。以吞咽时食管体部蠕动消失、LES松弛障碍为特征。本病的治疗方式包括药物、注射肉毒杆菌毒素、气囊扩张术和肌切开术,旨在降低LES压力,促进食管排空,多数患者可获得较理想的结局。本文就贲门失弛缓症的治疗现状作一综述。     

Update on therapeutic interventions for the management of achalasia

Achalasia is a primary esophageal motility disorder. It is the absence of peristalsis in the esophageal body and inability of the lower esophageal sphincter to relax, which characterizes this rare condition. Its features typically include dysphagia, regurgitation, chest pain, and weight loss. The ultimate goal in treating achalasia is to relieve the patient's symptoms, improve esophageal emptying, and prevent further dilatation of the esophagus. Current treatment modalities targeted at achalasia include pharmacological therapy, endoscopic therapy, and surgery. This review focuses on the current therapeutic options and explores the role of peroral endoscopic myotomy in the management armamentarium.     

Treatment of idiopathic achalasia with per-oral esophageal myotomy

Achalasia is a rare esophageal motility disorder that necessitates the disruption of the lower esophageal sphincter. Patients with achalasia should be evaluated in a systematic, multidisciplinary fashion. Workup should include upper endoscopy, esophagography, and high-resolution manometry. The gold standard for surgical treatment is laparoscopic Heller myotomy with partial fundoplication. Per-oral esophageal myotomy is a novel endoscopic technique that has gained considerable traction over the past decade. The procedure includes the creation of a submucosal tunnel and a selective circular myotomy of the lower esophageal sphincter. Common intra-operative hazards include bleeding within the submucosal tunnel and capnoperitoneum. Significant complications are rare. Patients experience excellent dysphagia relief that is on par with laparoscopic Heller myotomy at moderate-term follow up. Post-operative gastroesophageal reflux disease occurs in greater than one-third of patients, and the vast majority of cases are readily controlled with an anti-secretory medication. Although data is sparse, there is a growing body of literature that supports the long-term durability of per-oral esophageal myotomy.     

Management of achalasia: surgery or pneumatic dilation

Achalasia is an esophageal motility disorder of unknown cause, characterised by aperistalsis of the esophageal body and impaired lower esophageal sphincter relaxation. Patients present at all ages, primarily with dysphagia for solids/liquids and bland regurgitation. The diagnosis is suggested by barium esophagram or endoscopy and confirmed by esophageal manometry. Achalasia cannot be cured. Instead, our goal is to relieve symptoms, improve esophageal emptying and prevent the development of megaesophagus. The most successful therapies are pneumatic dilation and surgical myotomy. The advantages of pneumatic dilation include an outpatient procedure, minimal pain, return to work the next day, mild if any GERD, and can be performed in any age group and even during pregnancy. Pneumatic dilation does not hinder future myotomy, and all cost analyses find it less expensive than Heller myotomy. Laparoscopic myotomy with a partial fundoplication has the advantage of being a single procedure, dysphagia relief is longer at the cost of more troubling heartburn, and a myotomy may be more effective treatment in adolescents and younger adults, especially men. Over a two year horizon, the clinical success of pneumatic dilation and laparoscopic myotomy are comparable in a recent large European randomised trial. The prognosis for achalasia patients to return to near-normal swallowing and good quality of life are excellent, but few are "cured" with a single treatment and intermittent "touch up" procedures may be required.     

Update on the endoscopic treatments for achalasia

Achalasia is the most common primary motility disorder of the esophagus and presents as dysphagia to solids and liquids. It is characterized by impaired deglutitive relaxation of the lower esophageal sphincter. Highresolution manometry allows for definitive diagnosis and classification of achalasia, with type Ⅱ being the most responsive to therapy. Since no cure for achalasia exists, early diagnosis and treatment of the disease is critical to prevent end-stage disease. The central tenant of diagnosis is to first rule out mechanical obstruction due to stricture or malignancy, which is often accomplished by endoscopic and fluoroscopic examination. Therapeutic options include pneumatic dilation(PD), surgical myotomy, and endoscopic injection of botulinum toxin injection. Heller myotomy and PD are more efficacious than pharmacologic therapies and should be considered first-line treatment options. Per oral endoscopic myotomy(POEM) is a minimally-invasive endoscopic therapy that might be as effective as surgical myotomy when performed by a trained and experienced endoscopist, although long-term data are lacking. Overall, therapy should be individualized to each patient's clinical situation and based upon his or her risk tolerance, operative candidacy, and life expectancy. In instances of therapeutic failure or symptom recurrence re-treatment is possible and can include PD or POEM of the wall opposite the site of prior myotomy. Patients undergoing therapy for achalasia require counseling, as the goal of therapy is to improve swallowing and prevent late manifestations of the disease rather than to restore normal swallowing, which is unfortunately impossible.     

经口内镜下肌切开术对贲门失弛缓症患者食管动力的影响

目的探讨经口内镜下肌切开术(peroral endoscopic myotomy,POEM)初次治疗对贲门失弛缓症(achalasia,AC)患者术后食管动力的影响。方法纳入2012年1月至2016年6月期间于首都医科大学附属北京友谊医院就诊并行POEM治疗的AC患者,按研究设计完成各项检查、POEM治疗及随访观察,比较各型AC患者的POEM治疗成功率以及POEM治疗前后食管动力的改变。结果POEM术后6个月随访时,Ⅰ型AC患者的症状缓解率为100.0%(13/13),Ⅱ型为95.5%(42/44),Ⅲ型为90.1%(10/11)。与术前比较,术后1~6个月内下食管括约肌静息压[10.5(6.9,15.8)mmHg比24.6(18.3,35.1)mmHg,1 mmHg=0.133 kPa]、4 s整合松弛压[6.0(3.7,8.8)mmHg比21.8(15.3,28.0)mmHg]、上食管括约肌静息压[43.4(33.7,57.3)mmHg比45.3(33.2,71.1)mmHg]、上食管括约肌残余压[1.5(0.0,4.6)mmHg比3.9(1.1,6.9)mmHg]均明显改善(P均<0.05)。术后6个月,食管腔扩张的最宽直径较术前明显减小[(3.0±0.7)cm比(3.9±1.1)cm,P<0.001],总Eckardt评分较术前明显降低[1(0,2)分比6(5,8)分,P<0.001]。POEM术后,Ⅰ型AC患者食管体部均未出现蠕动恢复,Ⅱ型AC患者中有4例(9.1%,4/44)较术前恢复弱蠕动或期前收缩,Ⅲ型AC患者中10例(90.9%,10/11)较术前出现正常蠕动波、期前收缩或弱蠕动的比例增加。结论POEM术后不仅食管胃交界部流出道梗阻得到改善,而且食管体部动力也一定程度上发生改变,其中部分患者体部动力有一定恢复;但是这种变化在3个AC亚型表现不同,Ⅲ型最明显,其次是Ⅱ型,Ⅰ型则无明显改变。     

经口内镜下肌切开术治疗贲门失弛缓症的初探

目的探讨经口内镜下肌切开术（POEM）治疗贲门失弛缓症（AC）的疗效和可行性。方法研究2010年8月至2010年12月确诊为AC并接受POEM治疗的8例患者的临床资料。患者年龄16～62岁,平均43岁,病程2-20年,平均8．4年。POEM的主要步骤包括：食管黏膜层切开;分离黏膜下层,建立黏膜下“隧道”;胃镜直视下切开环形肌;金属夹关闭黏膜层切口。结果8例患者均成功接受POEM术,手术时间45-115min,平均68．5min,黏膜下隧道长度8～13cm,平均9．5cm,环形肌切开长度7～11cm,平均8．5cm,无1例出现与POEM相关的严重并发症。术后随访1～4个月,平均2．5个月,7例吞咽困难明显得到解除;1例术后15d出现进食困难及呕吐,胃镜检查发现黏膜下窦道形成,行内镜下窦道切开。结论作为一种新的微创治疗方法,POEM治疗AC短期疗效肯定,可以迅速解除AC患者吞咽困难,但其长期疗效及远期并发症仍有待随访观察。     

A case of long myotomy and fundoplication for diffuse esophageal spasm

We report a case of diffuse esophageal spasm (DES) successfully treated by esophageal long myotomy and Dor’s fundoplication. The patient was a 52-year-old man with a history of hepatitis C and severe chronic heart failure due to hypertensive cardiomyopathy. He had also undergone hemodialysis for chronic renal failure for 10 years. He had complained of dysphagia for 10 years. Diffuse esophageal spasm was diagnosed by fluoroscopy and esophageal manometry. We performed esophageal long myotomy through the opened hiatus and Dor’s fundoplication. The upper extent of the myotomy was confirmed by intraoperative endoscopic ultrasonography used to detect muscle thickening and low compliance of the esophageal wall. The procedure and postoperative recovery were uneventful, and the patient’s symptoms were relieved. This approach appears to be a potentially useful means of treating severe symptoms of DES resistant to conservative therapy, with ultrasound endoscopy being a helpful means of confirming the extent of esophageal myotomy.     

The surgical management of motility disorders

Surgical treatment is either the therapy of choice or a facultative procedure in various types of esophageal motility disorders. In achalasia, cardiomyotomy, frequently combined with fundoplasty, achieves good or excellent results in >80% of cases, and is, therefore, advised in cases when pneumostatic dilatation fails. Diverticulectomy and myotomy of the upper or lower esophageal sphincter are proven procedures to treat cervical and epiphrenic diverticula, leading to good/excellent results or at least an improvement in more than 95%. If, exceptionally, parabronchial diverticula require therapy, they should be excised transthoracically. Cervical myotomy is indicated in cases of cervical achalasia, when sufficient pharyngeal propulsion is preserved. In systemic diseases like scleroderma reflux induced complications may require surgical intervention in medically intractable cases. In these rather few cases, subtotal gastrectomy with a Roux-en-Y anastomosis is advised. In patients suffering from diffuse esophageal spasm or symptomatic nutcracker esophagus, extended esophageal myotomy can relieve symptoms. If a clear diagnosis is provided, about 75% of patients will have an improvement of symptoms.     

Data analyses and perspectives on laparoscopic surgery for esophageal achalasia

In general,the treatment methods for esophageal achalasia are largely classified into four groups,including drug therapy using nitrite or a calcium channel blocker,botulinum toxin injection,endoscopic therapy such as endoscopic balloon dilation,and surgery. Various studies have suggested that the most effective treatment of esophageal achalasia is surgical therapy. The basic concept of this surgical therapy has not changed since Heller proposed esophageal myotomy for the purpose of resolution of lower esophageal obstruction for the first time in 1913,but the most common approach has changed from openchest surgery to laparoscopic surgery. Currently,the laparoscopic surgery has been the procedure of choice for the treatment of esophageal achalasia. During the process of the transition from open-chest surgery to laparotomy,to thoracoscopic surgery,and to laparoscopic surgery,the necessity of combining antireflux surgery has been recognized. There is some debate as to which type of antireflux surgery should be selected. The Toupet fundoplication may be the most effective in prevention of postoperative antireflux,but many medical institutions have selected the Dor fundoplication which covers the mucosal surface exposed by myotomy. Recently,a new endoscopic approach,peroral endoscopic myotomy(POEM),has received attention. Future studies should examine the long-term outcomes and whether POEM becomes the gold standard for the treatment of esophageal achalasia.